13managing The Critically Ill Child-Freemedicalbooks2014

Managing the Critically Ill Child
A Guide for Anaesthetists and Emergency

Physicians
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Cambridge Books Online Cambridge University Press, 2013
Managing the Critically

Ill Child
A Guide for Anaesthetists and Emergency
Physicians
Edited by
Richard Skone
Specialist Registrar, Paediatric Intensive Care Medicine and Anaesthetics, Birmingham Childrens Hospital, Birmingham, UK
Fiona Reynolds
Paediatric Intensivist, Paediatric Intensive Care Unit, Birmingham Childrens Hospital, Birmingham, UK
Steven Cray
Consultant Paediatric Anaesthetist, Birmingham Childrens Hospital, Birmingham, UK
Oliver Bagshaw
Consultant in Anaesthesia and Intensive Care, Birmingham Childrens Hospital, Birmingham, UK
Kathleen Berry
Consultant in Paediatric Emergency Medicine, Birmingham Childrens Hospital, Birmingham, UK
CAMBRIDGE UNIVERSITY PRESS
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Cambridge University Press 2013
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Library of Congress Cataloguing in Publication data
Managing the critically ill child : a guide for anaesthetists and
emergency physicians / edited by Richard Skone ... [et al.].
p. cm.
Includes index.
ISBN 978-1-107-65232-3 (Paperback)
1. Pediatric emergencies. 2. Critically ill childrenMedical care.
I. Skone, Richard
RJ370.M34 2013
618.920 0025dc23
2012028035
ISBN 978-1-107-65232-3 Paperback
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Every effort has been made in preparing this book to provide accurate
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Contents
List of contributors
vii
Section 1 The District General

Hospital Setting
10 The child with seizures

Katie Z. Wright
Setting up a department for

managing a sick child 1
Adrian P. Jennings and Julian Berlet
11 The child with diabetic

ketoacidosis 104
Ian Jenkins
Team approach and organization

Richard Skone
Equipment 14
Fiona Reynolds
Stabilization of the critically

ill child: the initial approach
Richard Skone
21
The child with sepsis 33

Matthew D. Christopherson and
Paul Baines
The child with cardiac disease 48

John Smith, with illustrations by
Mazyar Kanani
The child with shortness of

breath 64
Brian Shields and Benjamin Stanhope
The child with asthma 76
Josephine Langton and Stuart
Hartshorn
The child with decreased
consciousness and coma 87
Mark D. Lyttle
122
14 The child with anaphylaxis

Nick Sargant
12 Inherited metabolic
conditions 112
Saikat Santra
13 Paediatric toxicology
Marius Holmes
Section 2 Clinical
Conditions
95
130
15 The child with stridor 137

Ed Carver and Tim Day-Thompson
16 The difficult paediatric
airway 146
Oliver Masters and Alistair
Cranston
17 The surgical abdomen
Suren Arul
18 Paediatric trauma
Karl Thies
159
169
19 The child with raised intracranial

pressure 180
Phil Hyde
20 The child with burns 192
Sapna Verma and Manu Sundaram
21 Blood product administration
in children 201
Oliver Bagshaw
v
vi
Contents
22 The sick neonate presenting

with shock 211
Fiona Reynolds
Section 4 The Childrens

Hospital Setting
Section 3 What You Could Be

Expected to Do in a District
General Hospital
228
25 Anaesthetizing for a surgical

emergency 235
Andy Tatman and Richard
Pierson
26 Managing children on an adult
critical care unit 245
Helga Becker
27 Pain management in
children 250
Ursula Dickson
28 Children with complex needs and
disability 258
Kate Skone and Ian
Wacogne
29 When a child dies
Fiona Reynolds
32 Fluid therapy 288

Adrian Plunkett
33 Pharmacology in children
Rhian Isaac
23 Ongoing management of the

successful arrest 219
Barney Scholefield
24 Referring-team-led transfers
Andrew J. Baldock and
Gareth D. Jones
31 Ventilation 277
J. Nick Pratap
34 Neonates 309
Richard Skone
Section 5 Golden Rules

35 Quick reference for
emergencies 321
Kasyap Jamalapuram
36 Drug infusions 323
Kasyap Jamalapuram
37 Top tips for practical
procedures 325
Steven Cray
38 UK vaccination schedule
Richard Skone
329
39 Common syndromes in the

critically ill child 330
Oliver Bagshaw
266
30 Child protection 270

Kate Skone and Geoff
Debelle
300
Index
340
Suren Arul
Consultant Paediatric Surgeon,
Birmingham Childrens Hospital,
Birmingham, UK
Matthew D. Christopherson
Specialist Registrar in Paediatric Intensive
Care, Alder Hey Childrens NHS
Foundation Trust, Liverpool, UK
Oliver Bagshaw
Consultant in Anaesthesia and Intensive
Care, Birmingham Childrens Hospital,
Birmingham, UK
Alistair Cranston
Consultant Anaesthetist,
Birmingham, UK
Paul Baines
Consultant in Paediatric Intensive Care, Alder
Hey Childrens NHS Foundation Trust;
Wellcome Trust Biomedical Ethics Research
Fellow, Department of Professional Ethics,
Keele University, Staffordshire, UK
Steven Cray
Consultant Paediatric Anaesthetist,
Birmingham, UK
Andrew J. Baldock
Specialist Registrar in Anaesthesia,
Southampton University Hospital,
Southampton, UK
Helga Becker
Consultant Anaesthetist, Dudley Group
NHS Foundation Trust, West Midlands, UK
Tim Day-Thompson
Birmingham School of Anaesthesia,
Birmingham, UK
Geoff Debelle
Consultant Paediatrician,
Birmingham, UK
Julian Berlet
Consultant Anaesthetist, Worcester Royal
Hospital, Worcester, UK
Ursula Dickson
Consultant in Paediatric Anaesthesia,
Birmingham, UK
Kathleen Berry
Consultant in Paediatric Emergency
Medicine, Birmingham Childrens
Hospital, Birmingham, UK
Stuart Hartshorn
Ed Carver
Consultant Paediatric Anaesthetist,
Birmingham, UK
Marius Holmes
Specialist Registrar in Emergency
Medicine, West Midlands Deanery, UK
vii
viii
Phil Hyde
Consultant Paediatric Intensivist, Paediatric
Intensive Care Unit, Southampton General
Hospital, Southampton, UK
Rhian Isaac
Paediatric Pharmacist, Birmingham
Childrens Hospital, Birmingham, UK
Kasyap Jamalapuram
Specialist Registrar, Paediatric Emergency
Medicine, West Midlands Deanery, UK
Ian Jenkins
Consultant in Paediatric Anaesthesia and
Intensive Care, Royal Hospital for
Children, Bristol, UK
Richard Pierson
Specialist Registrar in Anaesthetics,
West Midlands Deanery, UK
Adrian Plunkett
Consultant Paediatric Intensivist,
Birmingham, UK
J. Nick Pratap
Assistant Professor in Anaesthesia and
Paediatrics, Cincinnati Childrens Hospital and
University of Cincinnati, Cincinnati, OH, USA
Fiona Reynolds
Paediatric Intensivist, Paediatric Intensive
Care Unit, Birmingham Childrens
Adrian P. Jennings
Specialist Registrar in Paediatric
Anaesthesia, Birmingham Childrens
Saikat Santra
Consultant in Paediatric Inherited
Metabolic Disorders, Birmingham
Gareth D. Jones
Consultant in Anaesthesia and Paediatric
Intensive Care, University Hospital
Southampton NHS Trust, Southampton, UK
Nick Sargant
Medicine, Bristol Royal Hospital for
Mazyar Kanani
Fellow in Congenital Cardiothoracic
Surgery, Great Ormond Street Hospital,
London, UK
Barney Scholefield
Clinical Research Fellow, Paediatric
Intensive Care Unit, Birmingham
Josephine Langton
Emergency Medicine, Birmingham
Brian Shields
Specialist Registrar in General Paediatrics,
University Hospitals Coventry and
Warwickshire NHS Trust, Coventry, UK
Mark D. Lyttle
Medicine, Bristol Royal Hospital for
Kate Skone
Neurodisability, Birmingham Childrens
Oliver Masters
Birmingham, UK
Richard Skone
Specialist Registrar, Paediatric Intensive Care
Medicine and Anaesthetics, Birmingham
John Smith
Consultant Paediatric Cardiothoracic
Anaesthetist, Paediatric Intensive Care
Unit, Freeman Hospital, Newcastle
upon Tyne, UK
Benjamin Stanhope
Clinical Lead, Emergency Department,
Birmingham, UK
Manu Sundaram
Emergency Medicine, Birmingham
Andy Tatman
Consultant Anaesthetist, Birmingham
Karl Thies
Consultant in Paediatric Anaesthesia,
Birmingham, UK
Sapna Verma
Ian Wacogne
Consultant Paediatrician, Birmingham
Katie Z. Wright
Specialist Registrar in Paediatric and
Adult Emergency Medicine,
Birmingham, UK
ix
Section 1
Chapter
The District General Hospital Setting
Setting up a department for

managing a sick child
Adrian P. Jennings and Julian Berlet
Introduction
In 2009 nearly 5000 UK children were admitted to 28 paediatric intensive care units
(PICUs) from outlying hospitals, accounting for 64% of their unplanned workload.
A designated retrieval team performed the transfer to PICU in 80% of cases with a median
time of arrival at the patients bedside of 2 hours.
Life-saving interventions required during the first few hours of stabilization remain the
responsibility of the referring district general hospital (DGH) and cannot be deferred until
the arrival of the retrieval team. Figure 1.1 demonstrates the frequency with which referring
teams perform interventions. Any hospital that potentially manages sick children should
have a series of systems in place that anticipate and ease the process of managing a critically
ill child.
Endotracheal intubation
Mechanical ventilation
Central venous access
First inotropic agent
Osmolar therapy
0%
20%
40%
60%
80%
Percentage of interventions
100%
Referring hospital
Retrieval team
Figure 1.1. Proportion of interventions performed by referring hospitals and intensive care retrieval teams
during stabilization of critically ill children. (Reproduced from S Lampariello, M Clement, AP Aralihond, et al.
Stabilization of critically ill children at the district general hospital prior to intensive care retrieval: a snapshot of
current practice. Arch Dis Child 2010;95:681685, with permission from BMJ Publishing Group Ltd.)
Managing the Critically Ill Child, ed. Richard Skone et al. Published by Cambridge University Press.
Cambridge University Press 2013.
1
http://dx.doi.org/10.1017/CBO9781139107815.001
Section 1: The District General Hospital Setting
Changing practice in district general hospitals (DGH)

Decreasing experience
Many UK hospitals have withdrawn surgical services for children younger than 2 years of
age. This leaves many non-specialist anaesthetists with a duty of care to manage sick
children out of normal working hours. Nevertheless, public expectation of standards of
care has increased and DGH emergency departments (EDs) have maintained open access
for all children.
The Tanner report

In 2006 the Department of Health published a report entitled The acutely or critically sick
or injured child in the district general hospital a team response. The working group
established that concerns about deskilling in the management of critically ill children were
shared by many specialties, including paediatrics and ED medicine.
The report recommended that services for the critically ill child should be planned
within a network, with the local tertiary hospital acting as a source of advice and support
while stabilization is performed in the local DGHs. It suggested six generic skills which
can be expected of all personnel involved in the care of critically sick or injured children in
the DGH:
to recognize the critically sick or injured child
to initiate appropriate immediate treatment
to work as part of a team
to maintain and enhance skills
to be aware of issues around safeguarding children
to communicate effectively with children and carers.
There was also emphasis on the importance of considering the whole patient pathway.
Pre-hospital
The report commented that paramedic crews should transport sick children to the most
appropriate paediatric facility, and make a clinical judgement to drive-by a local DGH if
the hospital does not have a team capable of stabilizing the critically ill child.
Resuscitation/stabilization
The report encouraged a team approach with shared responsibility. It emphasized that
following the initial stages of resuscitation of a critically ill collapsed child, stabilization
and further management should not be left solely to the anaesthetist.
Transfer
The sickest children require transfer to the local tertiary paediatric centre and this transfer
should be performed by a designated paediatric retrieval team. However, in certain cases, it
may be necessary for the DGH team to facilitate transfer (see Chapter 24).
Chapter 1: Setting up a department for managing a sick child
Organizing a DGH to manage a critically ill child

Preparation
Relative to adult practice, the resuscitation and stabilization of critically ill children is a rare
event. This increases the need for clear planning in order to ensure a smooth pathway for
each patient. As a minimum each hospital should aim to have in place:
clear communication pathways within and between departments
easily available hospital protocols and guidelines for the management of common
paediatric emergencies
appropriate range of drugs and equipment
adequate, ongoing training of staff in paediatric emergencies.
Location
Critically ill children usually present to the ED; however, they may also deteriorate in
theatre, wards or other clinical areas. It is not practical to equip all clinical areas to
stabilize a sick child. Local guidelines should be in place stating where resuscitation and
stabilization should occur until the childs condition improves or the retrieval team
arrives.
Designated areas should have appropriate neonatal and paediatric resuscitation equipment (see Table 1.1).
Children should ideally not be managed directly alongside adults and the need to
support the family should be remembered. Parents and, where possible, children should
be consulted when designing areas of care.
Equipment
Studies show that DGHs continue to perform the vast majority of key stabilization
procedures on critically ill children (Figure 1.1). Therefore it is essential that paediatric
and neonatal emergency equipment be readily available, well maintained and organized in a
systematic fashion. Staff should undergo regular updates on its use, especially if used
infrequently. A robust system should be in place to ensure that every area has rapid access
to this equipment in the event of a paediatric emergency. Formal checks of drugs and
equipment should be performed daily.
When equipment is procured in the DGH, it is useful to ensure suitability for use with
both adult and paediatric patients as this will allow adult practitioners to use equipment
with which they are familiar, and minimize cost.
The emergency equipment should be arranged in a structured manner so that staff using
it infrequently can find the necessary items quickly and easily. This can be achieved in
different ways:
system-based
airway equipment of all sizes in one drawer, circulation in another
weight-based
one tray containing all the equipment for a child of a given weight.
Team composition
Stabilization of a critically ill child requires a team of competent individuals. As a minimum
this should include:
a paediatrician or ED consultant
an anaesthetist or intensivist
a paediatric trained nurse.
Clinicians from other services such as surgery or radiology may also need to be involved.
All staff should be trained in the specific needs of children and their families, including
child protection and consent.
It may sometimes be necessary for staff within the DGH to undertake transfer to the
regional PICU if the condition is time-critical (see Chapter 24) or if the retrieval team
is unavailable. Suitably trained staff to facilitate the transfer must be available at all
times.
Support services
Services such as haematology, chemical pathology, radiology and blood transfusion should
meet the requirements of children. Pharmacy staff with specialist paediatric knowledge
should be available to ensure safe and effective drug management.
Hospitals with no acute paediatric units

Where a DGH with no on-site inpatient paediatric facilities offers children unrestricted
access via the ED, the challenges of being able to manage the critically ill child are even
greater. Drive-by policies agreed with the ambulance service and close links to other
hospitals with paediatric facilities are crucial.
Table 1.1. Minimum set of equipment for paediatric emergency trolley. Further equipment should be kept
easily accessible if needed, e.g. ventilators.
System
Suggested equipment needed
Airway and breathing
Oxygen mask with reservoir bag

Self-inflating ventilation bag
Oropharyngeal airway (size 00 to 4)
Nasopharyngeal airway
Face mask (all sizes)
Endotracheal tubes
Uncuffed (from size 2.5 up)
Cuffed (from size 3.5 up)
Laryngoscope
Spare batteries
Range of blades (straight and curved)
Bougie (size 5 and 10 Fr)
End-tidal CO2 monitor
Laryngeal mask airway (all sizes)
Stethoscope
Yankeuer sucker
Magills forceps
Nebulizer mask
Tracheostomy tubes
Circulation
Intravenous cannulas (24G upwards)

Intraosseous needles
Wide range of syringes
Selection of needles
Arterial blood gas syringe/capillary blood gas tubes
IV administration/blood giving set
Dressings
Three-way taps and bungs
Defibrillator with size appropriate pads
Drugs
Locally agreed emergency drugs should be kept on the trolley,

e.g. adrenaline
Other
Blood glucose monitor

Lubricating gel
Scissors
Nasogastric tubes
Tape
Blood sampling bottles
Monitoring equipment, including ECG electrodes etc.
Stopwatch
Resuscitation chart
Algorithms, protocols and guidelines
Teamwork and training

A team approach is essential in order to utilize the skill-mix of different practitioners.
Local policies should define the members of the paediatric resuscitation team, the team
leader (for example, emergency medicine consultant or paediatrician) and the roles
expected to be performed by each member of the team.
Front-line medical staff should be trained in paediatric resuscitation and receive annual
updates. The departments should aim to support staff as they improve their knowledge and
skills through:
practising skills such as intraosseous needle insertion on purpose-built equipment
completion of an accredited paediatric life-support course
repeated attendance at these courses for the purpose of recertification/revalidation
secondment to a local paediatric centre
locally organized training (involving all departments)
regular audit
interdepartmental morbidity/mortality meetings
tutorials and e-learning modules.
Practice scenarios are a good opportunity to test equipment and review or develop local
guidelines. Audit and morbidity/mortality meetings are an essential opportunity for the
team to reflect on how actual cases are managed and identify areas of improvement.
Table 1.2. Factors to be taken into consideration when planning paediatric emergency management.
Factors
Actions needed in preparing for a sick child
Staff
Regular scenario training

Audit
Multidisciplinary morbidity and mortality meetings
Adequate clinical governance
Paediatric life-support training
Trained in using/setting up appropriate equipment
Equipment
Age-appropriate
Laid out systematically
Familiar to practitioners, i.e. same as adult if appropriate
Appropriate drug availability
Immediate availability of life-saving equipment
Central store of more specialized equipment
Environment
Prompts, e.g. wall chart with age-appropriate GCS

Clear guidelines and protocols
Child-friendly
Support for family
Procedural
Age-specific early warning scores to identify sick inpatients

Rapid escalation policy
Paediatric trained emergency response team
Good communication with support services
Clear lines of communication with tertiary centre
Policies for transferring patient staff availability, training, etc.
Relationship with the tertiary centre

A good relationship between a DGH and the local tertiary paediatric centre is essential if
care of the acutely ill or injured child is to be well organized and effective. Links and
coordination need to exist on several different levels.
Networks
The creation of paediatric service networks has helped improve communication over the
last decade. In many regions, paediatricians, surgeons and paediatric anaesthetists have
formed groups that create regional guidelines, conduct peer reviews of services and organize
study days. There is no substitute for face-to-face meetings between clinicians as a way of
developing closer links between local and tertiary centres. Outreach education and feedback
sessions in local units help to foster the feeling of partnerships between DGHs and regional
PICUs.
Retrieval services
In the UK, the development of regional retrieval teams has greatly improved links with
DGHs. They provide facilities such as:
single point of access to PICU
rapid response
advice throughout the process of managing a child
web-based drug calculators
feedback sessions (two-way)
teaching days
attendance at morbidity and mortality meetings.
Much of the responsibility for achieving a good relationship with the tertiary centre still lies
with staff in the DGH. Liaising with specialist clinicians regarding equipment purchases,
agreeing joint protocols and discussing individual patients is an effective way of forging
links. Visiting the tertiary centre to observe or participate in a clinical attachment often
helps to create strong links.
Summary
The burden of stabilizing critically ill children falls to all members of the ED, anaesthetic
and paediatric team within a DGH. It is important that plans are put in place to help make
the situation as straightforward and safe as possible.
Golden rules

Preparation is key to managing a sick child

Have appropriate equipment available and easy to access
Develop clear guidelines that are easy to access
In-hospital, scenario-based training can improve teamwork and highlight potential
problems
Further reading
Guidance on the provision of Paediatric
Anaesthesia Services. Royal College of
Anaesthetists, 2010. www.rcoa.ac.uk/docs/
GPAS-Paeds.pdf.
Lunn JN. Implications of the national
confidential enquiry into perioperative
deaths for paediatric anaesthesia. Paediatric
Anaesthesia 1992;2:6972.
Standards for Children and Young People in
Emergency Care Settings 2012: Developed by
the Intercollegiate Committee for Standards
for Children and Young People in

Emergency Care Settings. www.rcpch.ac.uk/
emergencycare.
The acutely or critically sick or injured child in
the District General Hospital: A team
response. Department of Health, 2006.
http://www.dh.gov.uk/Consultations/
ClosedConsultations/
ClosedConsultationsArticle/fs/en?
CONTENT_ID=4124412&chk=lIVmJg.
Tomlinson A. Anaesthetists and care of the
critically ill child. Anaesthesia
2003;58:309311.
Section 1
Chapter
Team approach and organization

Richard Skone
Introduction
A critically ill/injured child, depending on where they present, will be looked after by many
of the following professionals:
paediatrician
ED physician
ED nurse
paediatric nurse
anaesthetist
intensivist
surgeon
operating department practitioner (ODP).
Within this team there are often two specific groups: those who have a paediatric background but who do not frequently manage critically ill patients and those who are used to
managing critically ill adults but have little paediatric experience. A good team will use the
skills and experience of both groups and allow them to work freely and calmly.
The following chapter aims to cover the factors that make the team looking after a
critically ill child function well.
Differences in paediatric emergencies

The major difference in the team composition compared to most other medical emergencies is the presence of paediatric doctors and nurses.
Some paediatricians will have spent a considerable amount of time on neonatal units as
part of their training. They will have acquired a lot of skills such as intubation and insertion
of arterial lines in babies. This can alleviate some of the pressure on anaesthetists who might
otherwise feel that the burden of stabilizing small children lies with them.
It should be remembered, however, that neonatal emergencies seen in ED may not be
similar to those in a maternity unit. The major difference in ED is that the children are less
well controlled in their presentation. A child may present at any point during their illness
(rather than after birth or on a neonatal intensive care unit (NICU)). In this respect the ED
doctor or anaesthetist will be more familiar with time-critical emergencies such as fulminant sepsis or trauma.
9
10
Organizing the team

Approaches to team management
Vertical management
In the past much has been made of vertical management systems when dealing with medical
emergencies (Figure 2.1). In this structure all information is relayed to the team leader, and
then disseminated to the team. The team leader is responsible for all decision-making and
tells the team members what to do at each stage.
Figure 2.1. Vertical management.
Vertical management has its place. In particular, it is useful when managing an inexperienced team, or when a child has a single life-threatening problem that needs immediate
attention.
Horizontal management
When compared to vertical management, horizontal management has greater responsibility
and involvement for all members (Figure 2.2). Team members make decisions, carry out
tasks and communicate with each other and the team leader in a controlled, calm fashion.
The advantage of the horizontal structure is that it recognizes that people within a team
have their own skills and abilities. It also recognizes that it is very rare for the management
of medical emergencies to occur in a stepwise fashion. The key to this management
structure is communication.
Figure 2.2. Horizontal management.
Chapter 2: Team approach and organization
11
Team leader
The team leader for a paediatric emergency should not be chosen purely on the basis of
seniority. Experience either within the required specialty or of managing emergency teams
should determine the best person to manage the situation.
This experience is essential as the team leaders role will involve core skills such as:
keeping an overview of the situation
integrating information as it feeds back
prioritizing treatments
keeping everyone aware of their tasks and overall plan.
Team member
As

well as having roles, team members have responsibilities. It is up to each member to:
work within their competencies
take appropriate decisions
listen
communicate clearly in a timely manner.
When a team member feels that they have a piece of information that needs to be raised
urgently, they need to remember that they are working within a team. Other members may
also have similarly urgent problems. Hence timely and concise relaying of information is
essential.
If at any point in the emergency someone has been allocated a role which has no
pressing problem, they should mention this at an opportune moment so that they can be
reassigned further tasks within the resuscitation.
Why mistakes happen

As mentioned in Chapter 1, preparation and practice are the best way to try to improve
team interaction. Errors occur for various reasons. Systems should be put in place to
minimize the potential for errors. However, human factors will still contribute to problems
if not addressed.
Technical skills
Technical procedures performed on small children are difficult especially if not done often.
Simulation and practical experience are important in order to stop fear of performing
procedures impacting on other aspects of the team interactions. Time-critical skills should
be performed by the most skilled person. The team leader also has a role in keeping the
emergency room calm, as anxiety will worsen performance.
Knowledge
Like technical skills, it is up to individual doctors to make sure that their knowledge is up to
date. Hospitals should facilitate relevant CPD in paediatric practice for appropriate staff
members.
12
Team dynamic
Communication is a two-way process. Each team member needs to feel comfortable raising
concerns with the team leader, while remaining clear and concise when reporting facts.
Mistakes happen within hierarchical teams when junior members feel unable to raise
concerns. This can be due to lack of confidence in their clinical ability or due to intimidation from the team leader.
When reporting a problem the team member needs to:
be clear and concise about what needs to be done
emphasize importance of corrective action
escalate the issue calmly if they feel that the point has not been addressed.
At all points group responsibility needs to be emphasized by the team member so as not to
seem confrontational. An example of raising a concern would therefore be:
We need to intubate this child urgently because the saturations are low and Im not
able to ventilate her with a mask
Differences of opinion
Differences of opinion between specialists are difficult to manage. Should a disagreement
arise the involvement of the most experienced physicians should be sought. Many PICUs
have a consultant on call or a retrieval team that could provide advice. At no point should
the situation be allowed to degenerate into an argument.
Before the child arrives

If the child is being brought in as an emergency it is likely that the emergency team will
assemble before the patient arrives. In this instance the time should be used to:
introduce each member of the team (name and background)
allocate roles appropriately
brief the team about any information given by the paramedics
give initial instructions and plan (the team leader)
check equipment.
The time available to perform these tasks may vary and in cases of urgency it should be kept
brief but thorough.
If the child is already present

Assembling a team
A pre-hospital emergency call from an ambulance crew enables swift team assembly. It is
often harder to assemble a team promptly for a patient who is already in hospital. If a
child deteriorates in ED, on a ward or in theatre there needs to be a clear policy within the
hospital on how to escalate the situation to have the relevant people present quickly. The
escalation policy needs to make certain that rapid escalation does not have to pass through
a chain of seniority. Instead, if a senior member is needed, they should be contacted as a
first line.
Chapter 2: Team approach and organization
13
A paediatric inpatient who is deteriorating rapidly needs the same level of attention as
the child brought in by ambulance. Each hospital needs to make sure that there is an
appropriate senior doctor available 24 hours a day who can make themselves available at
short notice.
Integrating the team

The health professional who put out the call to assemble an emergency team for an
inpatient should assume the role of the team leader initially. They will need to:
organize people as they arrive
give a brief overview of the child
describe what has happened since admission
explain the plan for ongoing management
hand over the team leader responsibility if appropriate.
Summary
The stress of infrequent paediatric emergencies can lead to teams underperforming. It is
important that all hospitals rehearse paediatric emergency scenarios, both illness and injury,
and have protocols and guidelines in place to ensure optimal management of sick and
injured children.
Section 1
Chapter
Equipment
Fiona Reynolds
Introduction
Having the correct equipment available in an emergency can make the management of a
sick child much more straightforward. This chapter does not aim to tell people which
particular pieces or brand of equipment to use. Instead it aims to highlight common
problems in using medical equipment in children. It also offers a guide to which sizes to
use in smaller children.
Airway and ventilation equipment

Airway
Endotracheal (ET) tubes
There are a number of formulae for the appropriate size of ET tube in children. The most
commonly used in children in the UK is:
Size of ET tube mm Age yrs=4 4
This, however, may either over- or underestimate the tube size so the size above and below
should be immediately available. A common mistake is not to upsize a tube which is too
small and has too great a leak.
Cuffed tubes have traditionally been used after puberty although more modern cuffed tubes
have proved safe in children as small as term babies. These smaller cuffed tubes tend only to be
available in childrens hospitals as there is little need for them in an emergency situation.
Children suffering from serious burns should have a cuffed ET tube inserted, if the appropriate size is available locally, as changing the tube will be difficult once facial swelling has occurred.
An uncut tube should also be used in this situation whether or not a cuffed tube is used.
Suction catheters for ET tube suctioning

Suction catheters should be available for ET tube suctioning. The appropriate size of suction
catheter can be calculated using the formula:
Suction catheter size Fr ET tube size mm 2
maximum size 14 Fr for an adolescent
14
Chapter 3: Equipment
15
Suction systems
Wall or portable suction may be used in children. For endotracheal suction a pressure of
6080 mmHg (810 kPa) is appropriate for neonates and up to 120 mmHg (< 16 kPa) for
older children. It should be used for the minimum period of time.
Tracheostomy
The care-givers of children with a tracheostomy are usually expert in the management of the tracheostomy. Emergency supplies of a change of tracheostomy tube,
including a smaller tube and suctioning equipment, usually accompany the child
wherever they go. Most children with tracheostomies will have uncuffed tubes
although occasionally a child may have a cuffed tube. Fenestrated tubes are rare
before adolescence.
The care of the child with a tracheostomy mirrors that of an adult, with the understanding that the smaller tubes are more likely to block with secretions.
Ventilation
Mechanical ventilators
Most ICU ventilators used to ventilate adult-sized patients are capable of ventilating even
the smallest of patients when used appropriately. An adult ventilator may be used with
pressure control mode of ventilation, titrating the pressure to the measured tidal volume
achieved or to the degree of chest movement and blood gases. Alternatively a volume
control mode may be used provided the software allows a small enough tidal volume to be
delivered (610 ml/kg).
The sedated, muscle-relaxed patient can be placed on a controlled mode of ventilation
and the mechanics of triggering and synchronization are less important. Weaning from
ventilation usually requires synchronization and triggering with the ventilator. Pressure
triggering in children is usually not successful. Flow triggering commonly used in adult
ICU is also used in paediatric practice (see Chapter 31).
Breathing circuits for ventilators

Breathing circuits come in two main sizes: 15 mm and 22 mm diameter tubing. The smaller
tubing is used in children less than 20 kg.
The compression volume lost in the 15 mm ventilator tubing is smaller, i.e. the
volume change of the circuit with each breath. However, some ventilators are able to
compensate for any lost compression volume. In this case the size of the tubing is less of
an issue.
The larger tubing often requires a catheter mount to connect from the Y piece of the
breathing circuit to the ET tube. The volume of the catheter mount contributes to dead
space. For a child over 20 kg on mandatory ventilation this is usually not significant as the
ventilator rate can be set to compensate. During the ventilator weaning phase when the
patient is improving the dead space from the catheter mount can be an impediment to
successful weaning.
The smaller 15 mm breathing circuits tend to have a single elbow connector. This
connects directly to the ET tube without the need for a catheter mount and the attendant
concerns about dead space.
16
Continuous positive airway pressure devices

Continuous positive airway pressure (CPAP) devices are commonly used for infants with
moderate respiratory distress who do not require urgent intubation. The most common
scenario is the neonate or infant with bronchiolitis. These babies may present with apnoea
or moderate respiratory distress, which often improves with CPAP.
A variety of devices are used for this purpose and for the most part the paediatricians are
usually familiar with the set-up in their own hospital. Anaesthetists and emergency department physicians should also make themselves familiar with the device used locally.
Most devices rely on an oxygen blender to titrate the percentage of inspired oxygen
between 21% and 100%. The blender supplies a constant flow of gas maintained at a near
constant pressure by an adjustable pressure valve. Alternatively the gas flow can be bubbled
through an adjustable head of water (bubble CPAP). CPAP devices for infants typically use
up to 15 l/min of gas and can generate pressures of up to 10 cmH2O.
Non-invasive ventilation
Non-invasive ventilation is used in children for the same indications as used in adults. The
same ventilators are used in children over 10 kg as are used in adult practice. Children
require smaller interfaces such as nasal, full face or face shield masks. It may be difficult to
move a child requiring non-invasive ventilation between hospitals without intubation.
Humidification
Active humidification is generally achieved by a warmed water bath evaporator/humidifier.
The principles and temperatures used are identical to those used in adults. In general
humidification aims at 100% relative humidity at body temperature for patients with an
ET tube in situ. The humidifier dead space and resistance are not relevant in a fully
ventilated patient or in the situation where a flow trigger is in use.
Passive humidification is achieved using a heatmoisture exchanger (HME). Care
should be taken to use an appropriately sized HME. This is usually indicated on the device
itself, giving a range of suggested tidal volumes where the device is suitable for use. An
HME provides a lower level of humidity than is available using active humidification.
Chest drains
Insertion of a chest drain follows the same principles as in adult practice. Smaller sizes are
used for draining a pneumothorax, with larger sizes used for draining fluid or blood (see
Table 3.1). Underwater seals used for adults are identical to those used for children. Current
guidance supports the use of ultrasound to guide the drainage of fluid.
Table 3.1. Guide to size of chest drain to use in a child.
Age
Newborn
Chest drain size (French)

812 Fr
Infant
1216 Fr
Child
1624 Fr
Adolescent
2032 Fr
17
Arterial and venous access

Intravenous access
Peripheral lines
Peripheral lines start at 26G, which is primarily used for premature neonates under 1 kg.
24G cannulae are commonly used for term neonates although it is usually possible to insert
a 22G cannula in the long saphenous vein at term.
The veins in the feet are commonly used in paediatric practice. Scalp veins are sometimes used in babies, making sure the temporal artery is not cannulated. In an emergency,
the external jugular vein may be useful to gain intravenous access.
Intraosseous access
If intravenous access cannot be obtained in a timely manner, intraosseous access should be
secured. The most common site used is the proximal tibia, although the distal femur and
proximal humerus may also be used. The hand-held needles are being replaced by battery
powered devices such as the EZ-IO as these are associated with a higher success rate. More
experienced clinicians are more likely to choose to use an intraosseous needle at an earlier
stage in a patients treatment.
Central lines
The main sites of central venous line insertion are the internal jugular and femoral veins.
Both should be accessed under ultrasound guidance using strict aseptic technique. The
subclavian route is rarely used in paediatric practice. In the neonate the jugular vein usually
measures up to three times the diameter of the femoral vein, but tends only to be used by
expert practitioners. Complications of femoral vein puncture are much rarer. Unlike adult
data there are no paediatric data to suggest a higher infection rate in femoral central lines.
The most commonly used central lines in children from 3 to 25 kg are 4.5 or 5 Fr triplelumen central lines, which are available from 5 to 12.5 cm in length. The 58 cm length can
be used for most situations where central venous access is required in an emergency. Above
a weight of 25 kg, a 7 Fr adult line is commonly used.
Arterial access
Arterial lines
Specific cannulae designed for use as arterial lines for adults may be used in children above
25 kg. Under 25 kg these lines may be too large in relation to the childs artery.
In smaller children, it is common practice to use cannulae intended for intravenous
insertion. Lines must be clearly marked to indicate the arterial nature of the line and any
side port used for injection must be occluded by covering over with a dressing to prevent
its use.
A 24G cannula is commonly used from 3 to 10 kg and a 22G above 10 kg. The radial
artery and posterior tibial arteries are commonly used, with the femoral artery being used
when more peripheral insertion sites prove impossible. The brachial and axillary arteries
can be used. However, their use should be avoided in preterm babies due to the lack of
collateral arterial flow.
18
Blood gas sampling devices

Arterial blood samples are taken in the same way as in adults. In-line sampling devices used
for adults may be used in children. It is common practice to return the dead-space sample
through either the inline sampling device or a syringe if an in-line sampling device is
not used.
The minimum volume of blood should be taken; most modern blood gas machines need
less than 1 ml per sample.
Monitoring and defibrillators

Monitoring
Minor modifications of the interfaces for physiological monitoring are necessary depending
on the size of the patient. These modifications are smaller ECG stickers, pulse oximeter
probes, blood pressure cuffs and temperature probes.
Invasive monitoring may rely on the insertion of smaller arterial and central lines but
the transducers used in paediatric practice are the same as used in adult practice. In children
less than 10 kg, many PICUs will use a flush of 1 ml per hour connected to a syringe driver
by a Y connector rather than the pressure transducers default 3 ml/h flush. In the
emergency situation the default 3 ml/h flush is entirely appropriate for a term baby or
older child.
Defibrillators
Manual defibrillators have a range of energy levels that are suitable for even the smallest
neonate. The energy required is dependent on the rhythm to be treated and is calculated
from the weight of the child.
Two sizes of hands-free stick-on pads are available: small for children less than 10 kg
and adult size for use in adults and children greater than 10 kg. The small pads are applied
in the same position used in adults. If the only pads available are adult pads, they may be
used in an emergency on the anterior and posterior chest wall using the same energy
calculated according to the weight of the child and rhythm to be treated.
External pacing
Many external defibrillators have an external pacing mode which may be used for bradyarrhythmias. External pacing uses the same principles as in adults. The capture current is
relatively lower and the paced rate should be higher according to the normal heart rate for
the age of child.
Other equipment
Nasogastric tubes
Nasogastric tube length is measured from the nose to ear then to the stomach. Insertion in a
child is usually simpler than in an adult. The site of placement must be confirmed by pH
testing or X-ray. The size of nasogastric tube is usually:
68 Fr in a neonate
1012 Fr in a toddler
1416 Fr in an adolescent.
19
A nasogastric tube may be used to deflate the stomach when ventilating a child using a face
mask and may be crucial to the ease of bagging in an emergency. For this reason, a
nasogastric tube should always be available when intubating a paediatric patient.
Cervical hard collars

A properly sized hard collar can be used for cervical spine immobilization along with blocks
and tapes for children over the age of 3 years. Single-piece hard collars used by the
ambulance service and EDs do not fit most children under the age of 3 years.
There are hard two-piece collars designed for this younger age group, e.g. Miami Jr
from Ossur, but these tend to only be available in childrens hospitals. In the absence of an
appropriately sized hard collar, manual in-line immobilization should be maintained. If a
small enough hard collar is not available for a toddler, after intubation and ventilation the
sedated and muscle-relaxed patient may be immobilized using blocks and tapes pending
admission to PICU. Care must be taken to minimize flexion and extension of the neck.
Summary
It is important that the right equipment is available to team members as they strive to
stabilize a child. Delays in finding the right equipment can make an already fraught
situation more stressful. As always, preparation and planning can make the process of
stabilizing a sick patient more efficient.
Golden rules
Most equipment used in adult practice is suitable for use with appropriate adjustments
for size
Equipment should be stored in a systematic way so that it is immediately available
Advice on equipment can be obtained from the local PICU
Section 2
Chapter
Clinical Conditions
Stabilization of the critically ill

child: the initial approach
Richard Skone
Introduction
Most acutely critically ill children present first to their local DGH. However, they still
remain a rare occurrence outside paediatric intensive care. The chances are slim
therefore of a child presenting to a team with regular experience of resuscitating
critically ill children.
There is plenty of knowledge, expertise and experience available amongst the team
members outside specialized centres that can be applied to managing the critically ill child.
This chapter aims to help to overcome some of the most common hurdles that adult
physicians face, and address the most common fears.
All doctors in a specialty that may manage sick children should already be familiar with
the treatment algorithms provided by resuscitation groups. This chapter will focus on the
emergency care provided by DGH ED physicians and anaesthetists.
Differences between children and adults

When sitting exams from medical school to postgraduate training the list of differences
between adults and children is usually learnt as in Table 4.1. In reality these differences
make little difference if you:
stick meticulously to the same gold standard principles of care delivered to adults, i.e.
optimize oxygen delivery, fluid balance, electrolytes and temperature
refer to reliable reference sources to remind you of normal values and drug doses
ask for help from regional PICU retrieval teams early.
21
22
Section 2: Clinical Conditions
Table 4.1. Classic list of differences between adults and children.
Common issues
Consequences
How do I sort it out?
Relatively large head
Trauma to head more common,

greater forces on neck
Airway positioning more
challenging
Be aware of potential for isolated

head injuries and position the
head as carefully as possible
Make sure that the head is
supported at all times
Large body surface

area for weight/volume
Temperature more difficult to

maintain
Use active warming when

needed
Give warmed fluids
Relatively deep
subcutaneous tissues/
small vessels
Cannulation technically difficult
Ask for help from paediatricians

Use sensible sizes of cannulas
Low threshold for IO needle
High metabolic rate

Small glycogen stores
Rapid use of reserves

Hypoglycaemia
Check blood sugars regularly

Dextrose should be included in
the maintenance fluid
Age-dependent normal
values
Impossible to remember
Have correct values printed and

attached to all areas that children
might be managed in
Congenital disease, e.g.

heart disease
Rare syndromes and
other diseases
Lack of familiarity with anatomy/

physiology/treatment
Seek advice from the PICU team
Upper airway anatomy
Obstruction
Difficult laryngoscopic view
Have an experienced anaesthetist

on all paediatric emergency
teams
Smaller airways
Resistance high increased work

of breathing
Effect of secretions or oedema
significant
Be aware that children tire quickly

Have a low threshold for CPAP or
intubation
Fewer alveoli
Reduced surface area for gas

exchange
Increased work of breathing
As above
High O2 consumption
Rapid use of reserves

Reduced time for intervention
Prepare and be thorough before

intubation
Do not persevere with
laryngoscopy if you cannot
intubate
(Bag and mask)
Compliant chest wall

Immature intercostal
and diaphragm
musculature
Energy wasted
Have a low threshold for CPAP

and intubation
Anatomy and physiology
Chapter 4: Stabilization of the critically ill child
23
Table 4.1. (cont.)
Common issues
Consequences
How do I sort it out?
Left ventricle less compliant
Less responsive stroke

volume
Tachycardia in an attempt to
increase cardiac output
Pay close attention to response

to fluid challenges
Optimize electrolytes (especially
calcium in neonates)
Myocardial failure/
vasoconstriction in response
to sepsis
Blood pressure not useful to

predict cardiac output
Vasodilatory agents
precipitate significant
hypotension
Same as in adults
Relatively high circulating

blood volume
Small total blood volume
Small losses have significant

cardiovascular effect
Fluid challenges form the

mainstay of assessing fluid
balance
Be aware of significance of small
volume losses
Cardiovascular
Emotional
Parents
History has to be obtained from

care-givers
Care-giver emotional upset affects
team
Paediatricians on team
will be useful
Emotional impact of
resuscitating sick child
Team performance may be affected

by fear and upset
Regular debriefing
sessions
By focussing on the differences between adults and children, many teams forget that they
have the skills at hand to manage children well. Working calmly and in an organized
fashion can compensate, to a degree, for a lack of familiarity. This is best achieved as
described in Table 4.2.
Table 4.2. Broad outline of approach to a sick child.
The essence of care for children is:

Dont make it more complicated than it has to be
Be systematic
Use basic principles
Use/listen to the parents/carers
Access help/expertise from your team and PICU
Acknowledge fear and upset in yourself and others then put it aside
Concentrate on making the team work remember human factors have a huge impact on
ability to perform
Prepare for eventualities (see Chapters 1 and 2)
Display visible normal values charts
Display charts for ET tube size/length and other parameters
Advanced paediatric resuscitation course staff up-to-date
Debrief each significant episode with learning points not just those with poor outcomes!
24
Assessing the critically ill child when to intervene

Approach to the child
How you approach a sick child will depend on the severity of their illness. If the child arrives
in cardiac arrest then a team approach where each member assesses, reports and acts on
their designated role should be used. It is always worth taking 5 seconds when the patient
arrives for the team leader to make a quick assessment, even if it is to confirm a lack of
cardiac output.
If a child comes in seriously unwell, but awake, a single person examining the
child may be appropriate in order to not upset the child. The team may arrive in a
staggered fashion and the approach can quickly fragment. It is worth the team leader
periodically repeating the management plan and allocation of roles in order to maintain
order.
Airway assessment
The narrow airway of a child makes obstruction more likely when conditions such as
inflammation and oedema of the trachea occur. If any of the signs seen in Table 4.3 occur
then it is important to find out whether it is normal for that child. If it is not then the
anaesthetist should be prepared to intubate the child (see Chapter 15).
Table 4.3. Signs of potential airway obstruction.

Stridor biphasic (inspiration and expiration) stridor being more serious

Stertor/snoring
Position of child the child may sit in a tripod position (leaning forward) to maintain
optimum airway position
Accessory muscle use in the presence of other upper airway signs
Tracheal tug indrawing of skin above sternal notch especially if stridor present
Indications for intubation

There are many indications for intubation in a child. Most are the same as in adults, but
some differ. Typical indications for intubation are detailed in Table 4.4.
Table 4.4. Indications for ventilation.

Respiratory failure
Cardiovascular instability fluid resuscitation greater than 40 ml/kg with no response should
prompt consideration
Active significant bleeding
An unconscious child (GCS < 9)
Head injury with a need for control of CO2/neuroprotection, etc.
Airway burns
Cardiac/respiratory arrest
Significant invasive procedures
25
It is usually necessary to intubate and ventilate small children if you are performing
invasive procedures, such as a jugular central line or chest drain insertion, as these are
tolerated less well. The threshold for ventilation usually favours earlier intubation.
Breathing assessment
Children have a very compliant chest wall, and respiratory muscles that fatigue quickly. Their
work of breathing, even at rest, is high compared to adults. The cartilaginous component of
the ribs in children means that there is less opposition to the chest wall elastance. This results
in a high closing capacity and a tendency towards airway collapse and atelectasis.
However, children are also very good at maintaining normal values, such as saturations
and PaCO2. They may not always look as if theyre about to decompensate. This might go
some way to explaining why paediatricians seem to have a low threshold for requesting
intubation and ventilation in their patients.
By the time children do look as if they are in need of support, their ability to continue to
maintain normal values including any semblance of cardiovascular stability may be
significantly compromised. Clinical signs that would make you worry about a childs
respiratory effort include those seen in Table 4.5.
Intercostal recession is a common finding in many children with chest infections. On its
own it may not point towards respiratory compromise. However, when present with some
of the other signs from Table 4.5 it may become significant.
Table 4.5. Clinical signs of significant respiratory pathology.

Respiratory rate tachypnoea is one of the most sensitive signs

Grunting an effort to generate positive end-expiratory pressure (PEEP)
Intercostal and subcostal recession
Appearing drowsy or tired
Apnoeas especially in babies
A slowing of the respiratory rate and bradycardia are potentially preterminal events.
Capillary gases can be used in the same way as arterial blood gases (ABG) when deciding
on management plans. The PaCO2 will equate to that of an ABG. See below for further
information about capillary gases.
When making a decision about intervening, waiting for a child to become sick
enough to need intubation can turn a straightforward procedure into a highly pressured
risky event.
Cardiovascular assessment
The circulating volume of small children is large relative to their size. However, the absolute
volume is easy to overestimate. Fluid losses (GI, skin, respiratory, urine, third-spacing) have
a significant impact. For example, a baby with a circulating volume of 80 ml/kg who weighs
4 kg will have a circulating volume of 320 ml. A loss of 80 ml equates to 25% of their
circulating volume.
Blood pressure is well maintained despite significant illness in children. It should always
be measured but never relied on as a single parameter to guide therapy. Capillary refill time
26
(CRT) is commonly used in paediatric practice to aid assessment of cardiovascular status.

As a single parameter it is not useful either however, CRT can be used along with other
parameters such as:
heart rate
pulse quality
blood pressure
lactic acidosis
response to therapies (e.g. volume bolus)
urine output.
Children have a huge capacity for vasoconstriction in the face of a low cardiac output.
When examining a child in shock, feel along the arms and legs. There is often a cut-off
point at which the limb becomes cool. The higher up the level, the more the child is
compensating for a poor cardiac output.
The cold shock response to sepsis seen in smaller children differs from the more usual
adult model of high output shock as it can occur very early in the disease process.
Myocardial failure and intense vasoconstriction is a difficult situation to manage. It needs
early recognition and aggressive management (see below and Chapter 5).
Table 4.6. Signs of a child with cardiovascular compromise.

Tachycardia a child may look well, but have an elevated heart rate (the cause of the
tachycardia needs to be treated)
Weak pulses
Cool peripheries the level at which a limb becomes cool may extend proximally as the
child becomes sicker
Mottling assuming the child is not cold
Hepatomegaly this is a sign of heart failure, e.g. congenital heart disease or severe sepsis
Decreased conscious level
Electrolytes and temperature

Electrolytes and blood sugar
The advent of blood gas machines that measure electrolytes and blood sugars in addition to
the usual parameters has made their management very straightforward. A separate blood
sugar measurement should be performed immediately on arrival for any sick child. It
should then be noted on every blood gas from there on, at regular intervals.
Temperature
Core temperature should also be measured on arrival and, as with blood sugars, should be
monitored at regular intervals. Again, management is straightforward; it is forgetting to
measure that forms the main stumbling block.
As well as hypothermia in sick children, pyrexia can cause significant problems. Pyrexia
can make children appear disproportionately unwell with lethargy and tachycardia. Pyrexia
can also worsen outcome in head injuries. It should be treated by:

27
removing the childs clothes

giving antipyretics such as paracetamol
keeping the environment cool
placing icepacks (head, axillae and over femoral arteries).
Carrying out the interventions what to do when you are the

specialist help
As mentioned above, anxiety can seriously affect performance. It is better to acknowledge
fear and to deal with it than to drown under the pressure of expectation. If you have been
called to manage a child, it is because you have the necessary skills that others in the team
do not.
The major advantage of paediatric emergencies is that they attract a large team of people
who are desperate to help. Use your team. Take a deep breath and manage the situation with
the same calm reasoning as you would an adult.
Airway
As children get older the airway becomes more and more familiar to those working in adult
medicine. By the time they reach the age of 12 years, the larynx is not as anterior, the
tongue relatively smaller and the FRC greater. For children closer to the neonatal age
paediatricians may become more helpful for practical procedures. Some will be very
familiar with intubating and siting arterial lines on neonates.
Bag-mask ventilation
When bagging small children, the biggest problem by far is the anxiety-driven airway grip.
Pressing on the soft tissue under the mandible easily obstructs the airways of children.
Managing the airway in babies should be done by fingertip pressure on bony prominences,
with the head in a neutral position.
The second biggest problem is gastric distension and is caused by even experienced
paediatric anaesthetists. Air forced into the stomach during bagging can compromise
ventilation. An early NG tube can make the situation much better. In fact it is
commonplace to put in an NG tube for induction of anaesthesia in critically ill
children.
Intubation
In small children, the occiput forms the biggest problem. The right size of roll underneath/between the shoulder blades can make a huge difference. If your intubation has
failed, stop early, bag the patient and reposition (as you would in adults). Always intubate
orally first. The retrieval team can make a decision on nasal intubation for transfer.
Important things to consider when intubating critically ill children are mentioned in
Table 4.7.
There are situations where a gas induction in theatre should be the method of choice for
induction. These are discussed in Chapters 15 and 16. Endotracheal intubation is covered in
Chapter 37.
28
Table 4.7. Tips for intubating a sick child.

If possible place a tube for gastric decompression prior to induction expect gastric
distension and high risk of aspiration
Have access secured (peripheral or IO under local anaesthetic)
Have volume already given and/or available to give during induction
Have inotrope attached and/or running (depending on cardiovascular status)
Consider carefully the choice of induction agents (ketamine 12 mg/kg is the default choice
for most retrieval teams)
Use a cuffed ET tube for burns, and if high airway pressures are anticipated, e.g. asthma
Be ready to abandon laryngoscopy and attempts to intubate at any stage and sooner
than expected have a timekeeper and agree time allowed for each attempt (usually 30
seconds)
Do not cut the length of an ET tube until its position has been checked on a CXR
Breathing
It has already been mentioned that smaller patients have to work harder to maintain a
normal state. They also fatigue more quickly. The relatively immature chest wall of small
children means that they have a high closing capacity and a tendency towards airway
collapse and atelectasis.
Pressure-control ventilation
Once intubated the settings for the ventilator need to be set. In general, pressure-control
ventilation will compensate for leaks around the ET tube. The pressures should be the
same as in adults. Because the tidal volume measurements become less reliable as children
become smaller, always look at the movement of the childs chest, to check that it is
sufficient (but not too much).
Positive end-expiratory pressure (PEEP)

The tendency towards airway collapse means that PEEP is essential when setting a ventilator. Start off with the same pressure as you would in adults (about 5 cmH2O) and titrate as
needed. The biggest problem in small children is that as the PEEP increases, the venous
return becomes compromised. If this occurs, first give a fluid bolus then back off the
pressure (back to 5 cmH2O).
Difficulty ventilating
If you are having difficulty in ventilating a child, the commonest problems are:
the ET tube is not in the trachea use end-tidal CO2 monitoring
the ET tube is too far in (very common) clinical examination and a CXR will confirm this
the ET tube is blocked pass a suction catheter
the ET tube is too small and there is a large leak
the child has significant lung pathology or fluid overload
there is a pneumothorax
the child has pulmonary hypertension.
29
Table 4.8. Setting a ventilator for a child.

Always use end-tidal CO2

Use lowest FiO2 necessary to achieve adequate oxygenation (check what is normal for the child)
Use lung protective strategies, aiming for tidal volumes of 610 ml/kg
Start inspiratory pressures at 1520 cmH2O then titrate to tidal volume
Use the same inspiratory pressure limits as used in adults
Use PEEP
If PEEP causes hypotension, give fluid before backing off
Inspiratory times may need to be long start with a minimum of 0.6 seconds for a neonate.
Use up to 1.0 s for older patients or young patients with difficult oxygenation
Permissive hypercapnoea should be used for lung protection where necessary with pH of
7.25 being acceptable (caution in pulmonary hypertension and neuroprotective strategies)
Perform a CXR
Use adequate sedation/analgesia and muscle relaxation to keep ET tube in situ and promote
best ventilation
As in adults, there are conditions where different ventilator strategies are applied. These
include asthma (Chapter 8) and bronchiolitis (Chapter 7). Ventilation strategies in children
with congenital heart disease are also covered (Chapter 6). However, even in these conditions,
the settings mentioned in Table 4.8 will be adequate as an initial set-up to be titrated.
Cardiovascular
It is regularly taught that small children cannot increase their stroke volume to compensate for
a low systemic vascular resistance (SVR). However, small babies who are unwell will present
with relative volume depletion. Therefore their stroke volume will increase as normovolaemia
returns. There will be a decrease in heart rate, and evidence of improved cardiac output.
Hypovolaemia should be treated aggressively, with boluses of 20 ml/kg given as quickly
as possible whilst maintaining very close observation of response (with the exception of
resuscitation in trauma 10 ml/kg). A good response to a fluid bolus should include:
a reduction in heart rate
improvement in quality of pulses and pulse pressure
improved capillary refill time
improved lactic acidosis
increased urine output.
How much fluid?

Many adult physicians struggle with changing measurement values when giving fluid, i.e.
using ml/kg rather than absolute volume. When working out whether you have given a lot
of fluid or not, there are two easy methods:
compare it to the circulating volume 40 ml/kg is already half of the circulating volume
multiply it by 70 to work out the equivalent volume in an average adult 40 ml/kg
would be 2.8 litres.
For children who are likely to receive a lot of fluid, e.g. severe sepsis, take a cross-match
sample early and request blood products as soon as possible. It is likely that you will need
blood products once you have reached 6080 ml/kg of fluid resuscitation (see Chapter 21
for giving blood products).
30
Sepsis and fluids

For sepsis, the evidence points strongly to early rapid fluid resuscitation being associated
with the largest reduction in mortality (accompanied with early antibiotic administration).
By the time the patient gets to PICU the timeliness of early fluid administration has
disappeared. If a child is septic, resuscitate quickly using crystalloid first (see Chapter 5
for further choice of fluids). Although it is not the norm, volumes in excess of 150 ml/kg of
fluid are sometimes given in severe sepsis.
Vascular access and IO needles

No one would argue that vascular access in children can be difficult. Intraosseous (IO)
access should not be seen as a failure. Instead it should be seen as a pressure-relieving
fallback that takes the stress out of gaining IV access. It is no coincidence that the more
senior the paediatric ED or PICU doctor, the quicker they resort to IO access.
Central lines and arterial lines are rarely life-saving procedures in children. The time is
often better spent concentrating on the management of the child. If you are planning to
place a central line, speak to the retrieval team first to help weigh up the pros and cons.
Remember that all drugs, including inotropes, can be given through an IO needle.
Table 4.9. Gaining vascular access.

Remove people whose presence is unhelpful

Have a timekeeper accurately record time spent attempting access
Use intraosseous access early one for vasoactive agents, one for everything else
Formalize access once the patient is more stable (volume resuscitated, vasoactive agents in
progress, intubated and ventilated) if there is time
Vasoactive agents
Inotropes and vasoactive drugs are chosen along the same principles as in adults.
Adrenaline is usually the first-line inotrope. Dopamine, noradrenaline and dobutamine
are used to achieve the same response as in adults. Adrenaline can be given peripherally if
used in a suitable dilution (see Chapter 36).
Other agents may be suggested by the PICU retrieval team, but should not be started as
a first line.
Sedation and muscle relaxants

Sedation for ventilated children is usually achieved with a morphine infusion 2040 g/kg/h.
Midazolam 24 g/kg/min is used from 3 months old (see Chapter 36 for how to make up
these infusions). Propofol is not routinely used in children due to the risk of propofol
infusion syndrome in critically ill children.
Insufficient sedation will confuse interpretation of haemodynamic parameters (such as
heart rate). If in doubt bolus the equivalent of an hours worth of both agents, i.e. if your
morphine is running at 2 ml/h, give a 2 ml bolus. Be careful with midazolam, as it can lead
to quite profound myocardial depression and hypotension.
For most paediatric emergencies a muscle-relaxant infusion is used. As well as aiding
ventilation, it helps to ensure safety of the ET tube, which can otherwise be easily coughed out.
31
Glucose
The blood sugar should be checked on arrival of a patient, and at regular intervals thereafter
(including with each blood gas). Many of the infusions, e.g. sedatives and inotropes, are
made up in dextrose. This alone can supply enough dextrose to maintain the blood sugar in
most cases. If not, then maintenance fluid (often 0.45% saline with 5% dextrose or 0.9%
saline with 5% dextrose) can be run alongside the fluid boluses that are given.
Hypoglycaemia
If the blood sugar is below 3.0 mmol/l give 2 ml/kg of 10% dextrose and increase the amount
given as an infusion, either by volume or by concentration of dextrose (up to 12.5% dextrose
can be given peripherally through a good cannula). A single bolus will only raise the blood sugar
level temporarily.
If the blood sugar remains low despite the increased maintenance consider whether it
can be attributable to:
severe sepsis
inherited metabolic disease
acute liver failure.
These should be discussed with the PICU retrieval consultant. It is worth calculating how
much dextrose the child is receiving before considering these diagnoses. Greater than
10 mg/kg/min would prompt concern.
Temperature
Aim for normothermia in any critically ill child and remember that small patients lose heat
quickly. Active rewarming should be easily available for all children who require it. As
mentioned above, treat pyrexia aggressively, and contact the PICU retrieval team to discuss
therapeutic hypothermia in cardiac arrest (see Chapter 23).
Care-givers
Parents are usually good sources of information, and may be able to remain nearby without
compromising resuscitation. They tend to respond well to information, however inadequate
and indefinite that information may be. Although an area of debate, there is some evidence
that parents cope better with bad outcomes if they are present at the resuscitation. Where
possible, a member of staff should be allocated to looking after parents.
Summary
Management of sick children needs a calm and structured approach. This chapter covers
many of the details that need to be covered when managing a sick child. Oxygen therapy
and fluid resuscitation form the mainstay of treatment along with attention to detail to
electrolytes and temperature. Figure 4.1 shows a brief summary of the commonly asked
management questions during resuscitation.
Golden rules
Do not wait for a child to get too sick before intubation
Have a low threshold for IO needle insertion
Do not be afraid of giving fluid boluses quickly, as long as the child is responding
Check and correct electrolytes and glucose
Pay close attention to temperature especially if you are performing procedures on a small child
32
Question
Do I need to intubate this child?
Consideration
Are any of the indications in Table 4.4 present?
Does the child appear to be tiring?
Are the blood /capillary gases worsening

(increasing PaCO2 or hypoxia refractory to
oxygen therapy)?
Would I intubate an adult in this situation?
IF THE ANSWER IS YES TO ANY OF THE ABOVE

POINTS THEN CALL FOR HELP AND PREPARE TO
INTUBATE
Do I need to go to theatre for a gas

induction?
Is there any sign of a narrowed airway? See

Table 4.3
Is the child likely to be a difficult intubation,
i.e. is there a syndrome that might indicate
one (ask the paediatrician and see Chapter 16)?
Is there time to move the child; are they
well enough to transfer to theatre?
IF THE ANSWER IS YES TO ANY OF THE ABOVE

POINTS THEN CALL FOR HELP AND CALL THEATRES
TO PREPARE EQUIPMENT
Do I need to use an IO needle?
Have you had more than two attempts?

Have you taken longer than 2 minutes?
Does the child need immediate access for
fluids or drugs e.g. adrenaline?
Have you ruled out contraindications e.g.
fracture of bone to be accessed, overlying
infection?
IF THE ANSWER IS YES TO ALL OF THE ABOVE
POINTS THEN INSERT AN IO NEEDLE
How much fluid should I give?
Which inotropes should I use?
Give up to 40 ml/kg then cross-match (if not

done already)
After 6080 ml/kg start giving blood products
(FFP and packed red cells)
Keep giving fluid as long as you get a
response (improved heart rate, blood pressure,
etc.)
When the patient stops responding to fluid,
start inotropes
If the child is in extremis and the diagnosis
uncertain start peripheral strength
adrenaline (Chapter 36) or normal strength through
an IO needle
If you have IO or CVC access:
Is the child warm with bounding
pulses? noradrenaline
Is it primarily a cardiac problem?

consider dobutamine
Is there sepsis and myocardial

dysfunction?
dopamine/dobutamine +
noradrenaline, or adrenaline
Figure 4.1. Timeline for commonly asked questions during resuscitation.
Section 2
Chapter
Clinical Conditions
The child with sepsis

Matthew D. Christopherson and Paul Baines
Introduction
Infections in children are common. Around 1 in 14 of all infants will be hospitalized with an
infectious disease. The majority of infections are mild and managed with careful observation, antibiotics and fluid management before discharge. Severe sepsis is uncommon, with
about 0.6 cases per 1000 children in the USA and causing about 5% of UK PICU
admissions. Sepsis affects the whole paediatric age range, peaking in the second month of
life. Infections have a peak incidence over the winter months.
Sepsis in children
Severe sepsis is defined as systemic inflammatory response syndrome (SIRS) in the presence
of or as a result of suspected or proven infection. The definition of paediatric SIRS reflects
the normal age-dependent physiological parameters (Table 5.1).
Table 5.1. Criteria for SIRS in children.
Temperature (rectal)
< 36 C or > 38 C
Heart rate (beats per minute)
Neonates (< 4 weeks)

Infants (1 month1 year)
Children 15 years
Children 612 years
Children 1318 years
< 100 or > 180

< 90 or > 180
> 140
> 130
> 110
Respiratory rate (breaths per minute)
Neonates (< 4 weeks)

Infants (1 month1 year)
Children 15 years
Children 612 years
Children 1318 years
> 50
> 34
> 22
> 18
> 14
White blood cell count
< 4 109/l or > 12 109/l
Modified from Goldstein B, Giroir B, Randolph A, et al. International pediatric sepsis consensus conference:
Definitions for sepsis and organ dysfunction in pediatrics. Pediatr Crit Care Med 2005;6:28.
33
34
The pathophysiological process of sepsis in children is broadly similar to that of adults. The
complex interaction between pathogen and host provokes the release of pro- and anti-inflammatory
mediators. These mediators have broad-ranging effects on myocardial function, vascular tone,
permeability and coagulation, leading to hypotension, capillary leak and coagulopathy.
The pathogen causing sepsis varies with age. In neonates the main pathogens include:
group B Streptococci
Escherichia coli
herpes simplex
enterovirus.
In

older children the main causes of sepsis are likely to be:

Neisseria meningitides
Haemophilus influenzae
Staphylococcus sp.
Streptococcus sp.
Fungal sepsis and other viruses such as adenovirus may present in the immunocompromised child. The general principles of managing severe sepsis in children are the same
regardless of pathogen, with antibiotic therapy directed at the commonest causative organisms in the age group of the affected child. Care should be taken with chronically unwell
children who may be colonized with Pseudomonas or Klebsiella species.
Around 10% of deaths in children younger than 4 years are as a result of infection and
approximately 20% of the children admitted to UK PICUs with severe sepsis die. Higher
mortality is associated with multi-organ dysfunction. Four or more affected organ systems
increase the reported mortality to 50%; however, total mortality is declining.
Differences between paediatric sepsis and adult sepsis

Susceptibility to infection
Children are more susceptible to infection than adults for a wide variety of reasons. At
birth, the skin and mucous membranes provide less effective passive protection because
colonization with commensal flora is incomplete.
Neonates possess all the components of the immune system to mount an immune response;
however, they are pathogen-naive. At birth, there is an absolute IgA and IgM deficiency, with
levels rising over the first 6 months. Passive immunity is afforded by transplacentally acquired
maternal IgG. This IgG declines rapidly over the first few weeks of life. To coincide with lasting
antibody formation, the childhood immunization schedule commences at 2 months of age,
with only high-risk infants being immunized against tuberculosis and hepatitis B prior to this.
Childrens immune function matures as they grow. Some children remain at risk. These
include:
infants born prematurely
infants with chronic lung disease (respiratory tract infections)
children with short gut syndrome (parenteral nutrition through an indwelling vascular
access device and may lead to line sepsis)
children with hydrocephalus (infected ventriculo-peritoneal shunts)
congenital or acquired immunodeficiency e.g. DiGeorge syndrome
children with sickle cell disease (Salmonella and Streptococcus).
Chapter 5: The child with sepsis
35
In older children, it is those with neurological disorders or those receiving immunosuppressive therapy who have an increased risk of sepsis.
Presentation
The presentation of sepsis is variable and remarkably non-specific. The child may be
unsettled with muscle aches, listlessness or a reluctance to be separated from the main
care-giver. Frequently, children refuse to eat or drink or may be vomiting, leading to
dehydration and decreased urine output or number of wet nappies. Examination of the
fontanelle may show it to be sunken in dehydration or bulging in meningitis. Despite
presentation with mild symptoms, all children are at risk of rapidly deteriorating to
complete collapse within a short period of time.
Variation in presentation
Pyrexia is one of the first clues to infection in children but neonates and infants may be
hypothermic, especially in severe sepsis. Tachypnoea may be present as the child attempts to
compensate for metabolic acidosis, but neonates and infants often suffer from apnoeas.
Hypoglycaemia is a reasonably frequent finding in infants with sepsis. Maintenance
fluid with an adequate dextrose concentration should be commenced until enteral feeds
start. Some septic children become hyperglycaemic, which may be due to relative insulin
deficiency. Very high glucose levels are associated with increased mortality.
Clinical presentation of sick children can be misleading because:
they compensate well until they suddenly collapse
blood pressure measurements can be unreliable and hypotension is a late sign of
impending collapse
tachycardia is a useful indicator of illness but the heart rate may also be high in children
who are not sick if they are pyrexial or distressed.
Blood tests
A full blood count can show either raised white cell counts (WCC > 12 109/l) or low
white cell counts (< 4 109/l). Raised white cell counts are more likely in children who
mount a normal response to infection. Very high white cell, especially lymphocyte, counts
(> 50 109/l) suggest infection with Bordetella pertussis though the first presentation of
leukaemia should be borne in mind.
Infants under 3 months have reduced neutrophil stores and tend to drop their white cell
count when septic. Bone marrow suppression may be found as part of a multiorgan
dysfunction syndrome with anaemia, leucopenia and thrombocytopenia on investigation.
Other markers of inflammation including C-reactive protein, erythrocyte sedimentation
rate, procalcitonin and IL-6 can indicate sepsis; however, investigations are unreliable and
may be normal, especially early on in overwhelming disease.
Cold shock
In septic adults cardiac output is usually maintained despite myocardial dysfunction
because of reduced systemic vascular resistance. There is a decreased ejection fraction but
maintenance of cardiac output via ventricular dilatation and an increased heart rate.
Hyperdynamiclow systemic vascular resistance sepsis or warm shock is frequently
36
encountered in adults. Septic children, however, most frequently present with a low cardiac
outputhigh systemic vascular resistance or cold shock picture.
Children do not develop the ventricular dilatation seen in adults that maintains cardiac
output and, whilst tachycardia contributes to compensatory mechanisms, the proportional
increase in heart rate seen in adults is not sustainable in children. Septic children with low
cardiac output have greater mortality than those with a cardiac index between 3.3 and
6.0 l/min/m2. Because vasomotor regulatory mechanisms usually remain intact, the mainstay of treatment is aggressive fluid resuscitation and positive inotropes.
Monitoring degree of vasoconstriction

It is possible to observe the changing vascular tone by feeling the coreperipheral temperature gap and monitoring the progression from distal to proximal sites, e.g. from the feet
upwards. Increased coreperipheral temperature gap is a marker of poor peripheral perfusion and one can use this clinical assessment alongside heart rate, capillary refill time and
blood pressure to guide fluid resuscitation whilst invasive monitoring is established.
Continual assessment is required as the progression of sepsis evolves. Response to
interventions can be variable and the symptoms may switch between those of cold and
warm shock.
What are the warning signs that a child is really sick?

The presentation of sepsis can be misleading. Despite signs of hypotension and delayed capillary
refill time, the child may remain alert. Children who lie still and passively endure interventions
give cause for concern and children who seem to be good or cooperative may just be very ill.
Temperature
Infants who present with hypothermia often represent the more severe end of the spectrum
of disease and present a challenge in the resuscitation room as vascular access can be
difficult in a cold shut-down child. Whilst it is essential to remove clothes to allow
examination of the whole child for signs of rash and the placement of lines, it is imperative
that the child is kept warm in a ward room or by using a radiant heater.
Respiratory signs
Respiratory distress may be present in severely ill children. Tachypnoea may be respiratory
compensation for metabolic acidosis. As exhaustion ensues, the child may start grunting.
Apnoeas, if present, require intervention, as they suggest impending collapse.
Cardiovascular signs
Persistent tachycardia is a useful indicator of impending cardiovascular collapse in sick
children. The pulse rate can be used to monitor the response to fluid resuscitation with the
rate falling as adequate filling has been reached.
Neurological signs
If there is decreased conscious level or intractable seizures, intubation is necessary to protect
the airway and facilitate transfer for neuroimaging. Signs of meningitis or raised intracranial
pressure require intubation for neuroprotective management strategies (see Chapter 19).
37
Specific conditions
Meningitis
The presentation of meningitis in neonates and infants can be non-specific. A bulging, tense
fontanelle or focal neurological signs may be present on examination; however, apnoeas and
bradycardias may be the only feature. Children older than 1 year may appear generally
unwell, with headache, neck stiffness, vomiting and photophobia. In all children, signs of
raised intracranial pressure are features that require prompt intervention.
Diagnosis is made clinically with microbiological confirmation of cerebrospinal fluid
(CSF) cultures. The decision to perform a lumbar puncture must balance the benefits of
making a diagnosis, identifying the organism and obtaining antibiotic sensitivities against
the risks of coning, bleeding and introducing infection. Lumbar puncture should not be
performed in children who:
are cardiovascularly unstable
have decreased conscious level
show focal neurological signs
demonstrate a coagulopathy.
The responsible organism can be identified by blood cultures or polymerase chain reaction
(PCR), or CSF PCR and CSF cell counts obtained when the child has improved.
Antibiotic therapy is initially broad-spectrum, guided by the age of the child and
rationalized when the organism is identified. As a general rule, the organisms that cause
meningitis may also cause sepsis. Here supportive management should be directed to
maintaining normal parameters with neuroprotection (see Chapter 19).
Neonates with meningitis should receive antibiotics according to local policy, e.g.
amoxicillin and cefotaxime. Aciclovir should also be considered. Infants and older children
with meningitis should receive cefotaxime or ceftriaxone.
Dexamethasone 0.15 mg/kg (maximum dose 10 mg) 6 hourly in children over 3 months
of age reduces the incidence of neurological sequelae.
Group B streptococcus
Group B streptococcus (GBS) is a normal commensal bacterium found in the rectum and
vagina of around one-quarter of pregnant women. Approximately 1 in 2000 neonates
develop invasive GBS, which presents either early (within the first week of life) or late
(1 week to 90 days of age).
Early-onset GBS carries a risk of mortality of 515% in term infants and causes either
meningitis or sepsis. The incidence of late-onset GBS has not reduced with intrapartum
antibiotics. Presentation is similar to early-onset disease but with a mortality of less than 3%.
E. coli
Escherichia coli meningitis and sepsis can be particularly severe in neonates.
Listeria
Listeria monocytogenes is a rare cause of neonatal meningitis and sepsis. The organism is
able to cross the placenta, infecting the fetus. Mortality is 80%; however, Listeria is sensitive
to amoxicillin and prompt treatment may modify the disease course.
38
Pneumococcal meningitis
Pneumococcal meningitis is associated with significant neurological morbidity and mortality. Carriage of Streptococcus pneumoniae occurs in around half of all pre-school children.
The organism reaches the meninges by haematological spread or direct transmission
through a communication in sinusitis or otitis media.
Haemophilus influenzae type B

Haemophilus influenzae type B (Hib) meningitis is uncommon due to the inclusion of the
conjugate vaccine in the immunization schedule. Mortality from this disease is around 5%;
however, around 30% have neurological sequelae.
Meningoencephalitis
A number of other organisms may cause a meningoencephalitis-type picture (combined
picture of meningitis and encephalitis). Bacterial infection with Mycoplasma pneumoniae is
amongst the differential diagnoses and a macrolide antibiotic should be introduced if the
history is not typical of one of the more common causes. Viruses including enterovirus and
herpes simplex are uncommon causes of meningoencephalitis. Herpes simplex should be
considered if there is recent contact with the virus or in the presence of herpetic lesions, and
treatment with aciclovir commenced.
Meningococcal disease
Neisseria meningitides or the meningococcus is the organism most frequently associated
with meningitis. Carriage occurs throughout the adult population; however, invasive
disease mainly affects children. Recent influenza A infection, exposure to tobacco smoke
and close-quarter living are known risk factors. Progression of the disease is rapid and, with
sepsis, death can occur within hours. There is frequently a prodromal period with nonspecific symptoms. Muscle aches and pains may be an early indicator. Invasive disease may
present with a blanching pink macular rash or the more characteristic purpura; however,
absence of a rash does not exclude meningococcal infection. Some children present with
just signs of meningitis or sepsis but in the main it is a mixed picture. Children who have
sepsis without evidence of meningitis carry a poor prognosis.
Table 5.2. Poor prognostic signs in meningococcal sepsis.
Low white cell count

Thrombocytopenia
Deranged clotting
Persisting shock
Rapidly progressing purpura
No evidence of meningitis
Management is with antibiotic treatment and supportive measures. Electrolyte disturbance is common, with hypokalaemia, hypocalcaemia and hypomagnesaemia all potentially
correctable. Myocardial dysfunction in meningococcal disease is common and high doses
39
of inotropes may be necessary to support the circulation. Despite intensive care, the
outcome may remain poor, with up to 5% dying. Immunization against serotype C has
reduced the UK incidence and serotype B is now the dominant strain. Nasal carriage
persists with cefotaxime therapy and carriage should be eliminated with ciprofloxacin or
ceftriaxone.
Toxic shock syndrome

Infections with Streptococcus sp. and Staphylococcus sp. can cause SIRS secondary to toxin
production. Streptococcal infections are usually mild, with pneumonia, impetigo and pharyngitis being common. Group A streptococcus can have serious effects when it becomes invasive
(iGAS). Risk factors for iGAS are recent chicken pox (varicella zoster) or ibuprofen use.
Staphylococcus aureus may cause sepsis from chest or urinary tract infections or toxic
shock syndrome, with a widespread fine erythematous rash, particularly on the soles of the
hands and feet. Treatment is with flucloxacillin or cefuroxime with clindamycin.
Less commonly, children may present with methicillin-resistant Staphylococcus aureus
(MRSA) or coagulase-negative Staphylococcus sepsis. Usually, these children have indwelling catheters or a history of prolonged hospital admissions. Intravenous immunoglobulin
G is thought to reduce sepsis-related organ dysfunction and mortality in toxic shock
syndrome and its use is recommended in this situation.
Neutropenic sepsis
Children with neutropenia develop sepsis from not only the common childhood causes but
also a number of more unusual organisms. Pseudomonas, Streptococcus viridans, adenovirus and fungal infections can cause overwhelming sepsis in these patients. Cultures must
be taken before the administration of antibiotics as isolation of pathogens dictates treatment. Oncology departments in conjunction with infectious disease specialists have often
written a febrile neutropenia policy that guides treatment based on local infection patterns
and antibiotic susceptibility. In the absence of a local policy, dual therapy with gentamicin
and piperacillin/tazobactam (Tazocin) is indicated and aciclovir added for herpes or
varicella infection.
Targeted treatments
When faced with a septic child, it is important to place particular emphasis on assessing the
child and making appropriate interventions quickly prior to collapse. Failure to apply the
correct normal range to physiological observations is a recognized failure of care that
continues to contribute to morbidity and mortality.
Figure 5.1 shows a timeline of specific interventions for sepsis in children. The first few
minutes are critical once a child is recognized as being critically ill. When cannulating a
child blood samples should be sent for:
glucose
full blood count
clotting screen
biochemistry
blood cultures and PCR
cross-match.
40
0 min
Recognize Shock
Begin high flow oxygen
Establish IV/IO access
5 min
Initiate Fluid Resuscitation

20 ml/kg 0.9% saline then 20 ml/kg colloid
Commence antibiotics
Correct hypoglycaemia 2 ml/kg 10% dextrose
Shock not reversed
15 min
Fluid Refractory Shock

Begin dopamine IV/IO 515 g/kg/min
Anaesthetize for ventilation and invasive monitoring
Goal-Directed Therapy
Normal tissue perfusion, CRT< 2 seconds, CVP 1015
Normal blood pressure
Urine output > 1 ml/kg/h
Haemoglobin > 10 g/dl
Intermittent ScvO2 > 70%
Normal glucose and electrolyte concentrations
Cold shock with

normal blood
pressure
Cold shock with

low blood
pressure
Warm shock
with low blood
pressure
Titrate fluid
Add adrenaline
Add vasodilator
Titrate fluid
Add adrenaline
Add noradrenaline
Titrate fluid
Add noradrenaline
Add adrenaline
Shock not reversed
60 min
Catecholamine-resistant shock
Begin hydrocortisone 1 mg/kg 6 hourly if at risk of adrenal
insufficiency
Shock not reversed
Discuss with PICU for consideration of experimental therapy

Figure 5.1. Goal-directed therapy in paediatric sepsis the first hour.
41
Goal-directed therapy
Reductions in mortality are associated with early aggressive fluid resuscitation in children.
Goal-directed therapy has reduced mortality from sepsis in adults.
Just as in adults, the aim of treatment is to restore tissue perfusion and improve oxygen
delivery. Treatment is directed at normalizing:
tissue perfusion
pulse rate (which may remain high indicating severe disease)
blood pressure
urine output (> 1 ml/kg/h in children)
central venous oxygen saturations (ScvO2 > 70%)
haemoglobin concentration (> 100 g/l).
ScvO2 is used as an assessment of oxygen delivery and consumption, with low results
reflecting increased tissue oxygen extraction through an inadequate cardiac output.
Improving cardiac output and optimizing oxygen delivery by increasing haemoglobin
concentrations can increase the ScvO2. Resuscitation targeting improved ScvO2 has been
associated with improved survival.
Raised serum lactate concentrations can also reflect poor tissue perfusion. High levels
may take longer to fall in improving children, compared to adults, due to physiological
differences in hepatic lactate metabolism.
Fluid resuscitation
Principles of fluid therapy in severe sepsis in children
Severe sepsis needs rapid fluid resuscitation
Each hour passing without reversal of shock doubles the risk of death
20 ml/kg aliquots should be infused over 5 min and the child reassessed for signs of
improvement in perfusion or fluid overload
Resuscitation fluid volumes of 60 ml/kg administered within the first hour improve
outcome with no increased risk of ARDS or pulmonary oedema
Fluid overload in this situation is uncommon
Have blood products ready (see below)
Pulmonary oedema occurring early in children with septic shock is likely to be due to
capillary leak and should be treated with ventilation with high positive end-expiratory
pressure (PEEP). Over the first 24 hours, it is not uncommon for severely ill, septic children
to receive between 150 and 200 ml/kg or more of fluid in addition to their maintenance
requirement.
During the initial management give 20 ml/kg crystalloid followed by 20 ml/kg aliquots
of 4.5% human albumin solution (HAS) for all subsequent boluses; however, resuscitation
should not be delayed if HAS is unavailable (continue with crystalloid or other colloids).
Central venous pressure (CVP) can be monitored to guide fluid therapy with a target range
of 1015 mmHg.
42
Blood products
Resuscitation with large volumes of crystalloids will reduce the haematocrit. Current
guidance suggests maintaining the haemoglobin concentration above 10 g/dl to improve
oxygen delivery to tissues. Coagulopathies are to be anticipated not only as part of the
consumptive pathology but secondary to haemodilution. Infusions of cryoprecipitate and
fresh frozen plasma are used to treat the clotting abnormalities and should be infused even
with no active bleeding.
Platelets should be given to all children with a count of less than 10 109/l, or 1040 109/l
and a risk factor for bleeding. Platelet levels should be maintained at more than 50 109/l for
invasive procedures (see Chapter 21). It is unusual for the platelet count to fall in the early
hours of sepsis and it may be an indicator of an additional underlying disease process. Blood
products may also act as colloid within the fluid resuscitation process; however, fluid
administration should not be delayed until these products become available.
Vasoactive drugs
The majority of septic children respond well to fluid; however, some need cardiovascular
support. The aim is to counteract the myocardial dysfunction present in septic children.
If the cause of the collapse is uncertain adrenaline should be considered first-line inotropic
therapy.
Dopamine infusion at 510 g/kg/min to a maximum of 15 g/kg/min is the first-line
agent for both cold and warm shock in paediatric sepsis (see Chapter 35 for infusion
strength). The positive inotropic and chronotropic effects increase cardiac output in both
scenarios. Paediatric studies do not support the inferiority of dopamine when compared to
noradrenaline and, as such, it remains the first-line inotrope in paediatric sepsis.
In warm shock, noradrenaline plays a similar role to that in adult practice and can be
used as single-agent vasopressor therapy where central access is immediately available. The
safety and efficacy of vasopressin infusion in paediatric sepsis is currently under investigation and its routine use is not currently recommended.
For dopamine/dobutamine-resistant cold shock, adrenaline (0.11 g/kg/min) is the
next inotrope to start (Figure 5.1). Dobutamine may cause vasodilatation, with further
fluids required to prevent hypotension.
Type III phosphodiesterase inhibitors, e.g. milrinone, provide a theoretical advantage in
cold shock, adding inotropic effects to vasodilatation independently of the catecholamine
pathway. In reality, the steady state may take several hours to achieve so these agents may
only have a place in the ongoing intensive care environment.
Endotracheal intubation and mechanical ventilation

The decision to intubate and ventilate needs to be made early in the resuscitation process.
Indications for ventilation include:
decreased conscious level
respiratory failure or apnoeas
persisting shock despite 40 ml/kg fluid resuscitation over a short period of time should
also act as a trigger to prepare for intubation.
43
In reality, if further fluid resuscitation is required, central venous access is necessary to

monitor CVP, measure ScvO2 and reliably deliver vasoactive drugs. Such procedures are
painful, require a cooperative child and therefore are most safely achieved under
anaesthesia.
Mechanical ventilation has additional benefits in sepsis. Ventilation will relieve the work
of breathing, which may be higher in septic shock. Children with low cardiac output and
high systemic vascular resistance may receive benefit from the improved left ventricular
function associated with with increased intrathoracic pressure. Pulmonary oedema may
develop and can be treated with high PEEP.
Steroids
The role of steroids in paediatric sepsis is controversial. Some septic children have low
steroid concentrations and those children are more likely to die. Current guidelines
suggest the administration of hydrocortisone to children with sepsis and catecholamineresistant shock, who are at risk of absolute adrenal insufficiency or adrenal pituitary axis
failure, for example those with purpura fulminans, congenital adrenal hyperplasia or
recent steroid use.
In reality, the practicalities of demonstrating cortisol deficiency prevent formal investigations in the acute situation and as such, if the child is deteriorating or on escalating
catecholamines, give hydrocortisone at 1 mg/kg 6 hourly. A random cortisol can be
requested on a sample taken before steroids were commenced.
What do I need to do pre/post intubation?

Intubation
When preparing to intubate a septic child, consideration should be given to the potential
pitfalls. Pulmonary oedema bubbling up the trachea following induction may obscure the
laryngeal inlet. An appropriately sized bougie can be useful to place through the cords to
guide intubation. If there is pulmonary oedema use a high PEEP and consider asking PICU
to prepare for high-frequency oscillatory ventilation. Hand ventilation in the presence of
significant pulmonary oedema may also be difficult due to the inability to maintain a
constant PEEP. Placing the child on a ventilator may improve the situation.
A rapid-sequence induction is usually required; however, giving thiopentone to shutdown and effectively hypovolaemic children can lead to loss of cardiac output. Ketamine
(12 mg/kg) theoretically provides more cardiovascular stability but hypotension is not
unknown. The change in physiology from negative to positive intrathoracic pressure on
invasive positive-pressure ventilation exacerbates the fall in cardiac output. Continuous
infusion of volume along with an inotrope infusion running may prevent arrest on
induction.
Post intubation
Post intubation, arterial and central venous access should be placed, thus facilitating further
stabilization. Insert a urinary catheter to monitor urine output and obtain a sample of urine
44
to send for microbiological testing. If not done already, site a nasogastric tube to deflate the
stomach and empty any contents.
At this point the child should be reassessed with particular attention paid to:
correction of blood glucose and electrolytes
continued fluid resuscitation
regular temperature monitoring.
Parents are frequently asked to wait in the parents room during the initial stabilization and
intubation process. When invasive procedures are complete the parents should be informed of
what has happened since they left their child and invited to return to their childs side.
Appropriate support needs to be provided to explain their childs status and appearance.
A further more detailed history can often be taken at this point.
What do I need to have done/organized before transfer?

Prior to transfer to PICU, a number of routine tasks should be completed whilst continuing
to deliver intensive care (Figure 5.2). The checklist for transfer in Chapter 24 can be used to
guide preparation of the child for transfer in order to speed up the process.
Airway
Nasal endotracheal tube caution in coagulopathy, may cause epistaxis
Endotracheal tube secured appropriately for transfer
Nasogastric tube placed to decompress the stomach
Chest X-ray demonstrating position of endotracheal tube and nasogastric tube
Inclusion of a heat moisture exchanger within the circuit
Breathing
Placed on ventilator with clear settings and blood gas monitoring
Positive end-expiratory pressure (PEEP) maintained as appropriate
High PEEP in pulmonary oedema
Circulation
At least one good peripheral venous cannula
Multi-lumen central venous catheter (CVC) sited and secured
Position confirmed on X-ray if internal jugular line
Measurement of the central venous pressure (CVP)
Arterial line sited, secured and transduced
If placing in the groin, preferably the same side as the CVC to keep the other
side free for renal support catheter
Inotrope infusions running via the CVC
Urinary catheter
Investigations
Blood cultures
Full blood count, coagulation studies
Urea and electrolytes, calcium, magnesium, lactate, C-reactive protein
Blood glucose
Arterial blood gas and intermittent ScvO 2
Urine dipstick and cultures
Drugs
Antibiotics prescribed with times administered
Consider steroids if meningitis or escalating inotrope requirement
Request random cortisol
Maintenance infusion containing dextrose to maintain normal blood glucose levels
Communication
Parents
Outline diagnosis, management and prognosis
With the transfer team
Copies of notes, X-rays, drug charts, management pre transfer
Receiving PICU
History, current management, requests for specialist equipment (e.g. HFOV)
Figure 5.2. Beyond the first hour until transfer.
45
46
History
A detailed account of the history, initial examination, anaesthetic difficulties and interventions should be written. In particular, the timing of administration of antimicrobials, fluids,
paracetamol and steroids should be recorded. Treatment can then safely be continued on
PICU. A list of microbiological specimens sent and copies of radiological and blood
investigations need to accompany the child upon transfer.
Blood products
Blood products are strictly regulated and the transfer of products between hospitals requires
organization. With sepsis, coagulopathies can be expected due to the large fluid volumes
administered during resuscitation along with the consumptive processes. Intensive care
continues during ambulance transfers and the infusion of either blood or clotting products
may become necessary.
Notification of disease
There is a statutory requirement to notify many causes of paediatric sepsis, including
bacterial and viral meningitis, invasive group A streptococcus infection and haemolytic
uraemic syndrome, to the local public health team. They arrange contact-tracing and
chemoprophylaxis for those at high risk of infection. In meningococcal disease, chemoprophylaxis should be provided to household contacts and those exposed to a large amount of
droplets from the respiratory tract around the time of admission.
What are the pitfalls in this kind of child?

Common pitfalls in managing paediatric sepsis
Failing to appreciate severity of illness
Not using age-appropriate values (see Chapter 35)
Not exposing a child to see rashes
Not giving fluids early use an IO needle if necessary
Not giving enough fluids
Not intubating early enough
Not preparing for unstable intubation
Not cross-matching blood products early enough
Allowing the child to become hypothermic with procedures
Summary
During the time following initial stabilization until the child is transferred to PICU it is
imperative that the optimal management guidelines are followed. Advice for ongoing
treatment can be obtained from the transport service or receiving PICU. Persisting shock
on arrival at the PICU is associated with an increased risk of mortality. It has been shown
that these children frequently do not receive the fluid and inotrope therapy they require.
Continual assessment and early intervention thus maintaining our treatment goals should
improve the outcome for this critical group of children in the future.
Further reading
Brierley J, Carcillo JA, Choong K, et al. Clinical
practice parameters for haemodynamic
support of pediatric and neonatal septic
shock: 2007 update from the American
College of Critical Care Medicine. Crit Care
Med 2009;37:666688.
47
Goldstein B, Giroir B, Randolph A, et al. International pediatric sepsis consensus conference:

definitions for sepsis and organ dysfunction in
pediatrics. Pediatr Crit Care Med 2005;6:28.
Meningitis Research Foundation website. http://
www.meningitis.org/health-professionals.
Clinical Conditions
Section 2
Chapter
The child with cardiac disease
John Smith, with illustrations by Mazyar Kanani
Introduction
Abnormalities in the development of the heart and great vessels are the commonest
congenital abnormalities, with a frequency of 5.2/1000 live births. Whilst many are
not life-threatening and may require only conservative therapy there are a number that
present in the newborn period or early infancy that require stabilization and urgent
treatment.
In this chapter two different scenarios will be covered. The first is the collapsed
neonate admitted to ED with possible congenital heart disease. The second is the child
with known congenital heart disease (CHD).
Neonatal presentation of cardiac disease

The final switch from a fetal to an adult circulation by closure of the ductus arteriosus (DA)
can unmask CHD. It is the most likely situation for an anaesthetist/ED doctor to encounter
in the context of a baby with CHD returning to ED in extremis within 2 to 3 weeks of birth
as the DA closes.
Suspecting CHD
The collapsed neonate presenting to ED is unlikely to have an obvious diagnosis of CHD. It
is more likely that the child will present with an unknown cause of their illness where CHD
may or may not be the underlying cause. For this reason CHD should always be kept in the
differential diagnosis of a collapsed baby.
It would be reasonable to expect that the presentation of the sick child would depend on
the anatomy of the cardiac lesion, i.e. low saturations in conditions with poor lung blood
supply or systemic hypoperfusion/weak pulses in those with aortic outflow obstruction.
However, in reality, a child brought back in to a DGH after duct closure is likely to present
with a mixture of signs:
low saturations that are refractory to oxygen therapy
poor peripheral perfusion
48
Chapter 6: The child with cardiac disease
49
weak pulses (especially if normal upper limb but poor lower limb)
high lactate
cardiogenic shock.
Other potential diagnoses need to be considered. Most children are treated empirically with
broad-spectrum antibiotics to cover sepsis.
Confirming CHD as a cause of collapse

Peripheral pulses
Peripheral and central pulses usually provide the first sign of CHD. Poor peripheral pulses
and capillary refill would suggest that a bolus of fluid (20 ml of crystalloid) should be
administered and the response assessed. However, if the femoral pulse is impalpable in the
face of a good right brachial pulse then some form of left heart obstruction is likely.
Palpable femoral and foot pulses are reassuring.
Saturations
A difference in saturation between the right arm and lower limbs suggests CHD (although
not the exact lesion). The reason for the difference is that the DA joins the aorta close to the
origin of the left subclavian artery (see Figure 6.1).
If there is a complete obstruction to flow from the left ventricle, any blood flowing in the
aorta will be both anterograde and retrograde from the DA and pulmonary artery. It will
therefore be deoxygenated equally as measured by the saturations in the hand and foot.
This diagnostic sign is not without its problems, as a shocked child coming into the ED
may not have enough peripheral perfusion to give readable saturations.
Hepatomegaly
As with adults, right heart failure leads to increased inferior vena cava pressure. The liver
can then become distended and easily palpable. It is possible to get false positives if a child
has been given large volumes of resuscitation fluid or if they are ventilated with high airway
pressures.
Chest X-ray
Pulmonary oedema has the same characteristics on X-rays to that of adults. Cardiomegaly
should also raise the suspicion of CHD. Again, false positives from fluid resuscitation are
possible.
Echocardiography
Some hospitals may have the facility to perform a cardiac ultrasound scan. This may
help define whether there is a one- or two-ventricle pattern to the heart, whether there is
a ventricular septal defect (VSD) or a duct present. However, complex lesions cannot
always be excluded, especially if the practitioner in question is used to adult scans, or the
correct scanner is not available.
50
The ductus arteriosus (DA)

Figure 6.1. Ductus arteriosus. Note the DA arising
from the bifurcation of the pulmonary artery and
passing onto the inferior aspect of the aortic arch,
opposite the left subclavian artery.
The DA is one of the two links in the fetal circulation that allow blood to bypass the highresistance pulmonary circulation (the other is the foramen ovale). Once the lungs inflate
and pulmonary vascular resistance drops the DA ceases to have a function in healthy babies.
After birth the duct usually closes in two stages. First, it undergoes functional closure at
about 15 hours, by smooth muscle contraction. This is triggered by a rise in blood oxygen
levels, and a drop in maternal prostaglandins. The second stage is true anatomical closure,
which can take a couple of weeks.
Persistence of the DA causes long-term problems such as pulmonary hypertension or
heart failure. This is because the DA directly connects the high-pressure aorta with the
pulmonary arteries.
The initial management of CHD relies on the fact that the duct can be re-opened
temporarily (after functional closure) by the use of prostaglandin E1 or E2 (PgE). After
true anatomical closure PgE will not be effective. However, CHD which is duct-dependent
may prolong the amount of time that the DA remains open, and therefore allow the DA to
re-open in response to PgE infusion up to 34 weeks after birth.
Prior to its closure the DA can mask the presence of CHD in one of three ways:
supplying blood to the lungs from the aorta if there is an interruption to the pulmonary
artery, e.g. pulmonary atresia (left to right shunt across the DA)
supplying blood to the systemic circulation from the pulmonary artery if there is an
interruption or obstruction to the outflow of the left heart, e.g. severe coarctation (right
to left shunt across the DA)
providing a mixing point where oxygenated blood can get from the pulmonary to the
systemic side, e.g. transposition of the great vessels.
51
Specific conditions affecting neonates

Conditions with duct-dependent pulmonary blood flow
Critical pulmonary stenosis/pulmonary atresia/tricuspid atresia
The primary problem is a restriction to blood flow out of the right ventricle into the
pulmonary artery (PA) (Figure 6.2). The DA, from the aorta, then becomes the main blood
supply to the PA. Without reasonable blood flow through the DA these children will have
very low saturations. Most conditions will have a second mixing point (atrial septal defect
(ASP) or VSD) to allow blood to pass from the pulmonary to the systemic side, which will
allow for a cardiac output to be maintained. This is essential as it would not be possible to
maintain a circulation in conditions such as pulmonary atresia.
Figure 6.2. Pulmonary atresia. Note the very small
(atretic) pulmonary artery (PA) and branch Pas. The VSD
allows blood to escape from the right ventricle into the
left ventricle. This diagram shows MAPCAs, which are
anomalous blood vessels which supply the lungs
directly from the aorta, under high pressure.
Conditions with duct-dependent systemic blood flow

Coarctation of the aorta
The classic presentation is of an infant who presents with feeding difficulties, breathlessness
or collapse within the first week of birth. The contraction of ductal tissue within the distal
aortic arch narrows the lumen of the aorta (see Figure 6.1 for point of insertion of DA on
the aorta) and makes the lower half of the body dependent upon the flow of desaturated
blood from pulmonary artery, via the DA. The closure of the duct then precipitates the
presentation. Physical examination will reveal weak or absent femoral pulses as blood fails
to travel past the narrowing (coarctation) in the aorta, while managing to supply the upper
part of the body.
Once the diagnosis is suspected, treatment should include appropriate resuscitation,
PgE1/PgE2 (which may or may not be effective depending on the age at presentation) and
assessment for surgical repair.
Hypoplastic left heart syndrome (HLHS)

This condition used to be lethal in the neonatal period. The clinical presentation is similar to
that of coarctation of the aorta. Mitral atresia (failure of development of the mitral valve)
52
Figure 6.3. Hypoplastic left heart syndrome. Note

the very small aorta passing around the pulmonary
artery. The aorta enlarges at the point of insertion of
the DA. This diagram does not show the small left
ventricle or the fact that the coronary arteries arise
from the small aorta.
leads to a condition where the left ventricle and first part of the aorta fail to develop properly
(see Figure 6.3). As a result, blood supply to the systemic circulation is dependent on the DA.
Blood passing out of the right ventricle and into the PA can flow either along the branch
pulmonary arteries to the lungs, or down the DA to the systemic circulation. The direction
of flow will be decided by the balance between pulmonary and systemic vascular resistance.
Aiming for high systemic arteral oxygen Saturation (SaO2) in these children may result
in a low cardiac output or cardiac arrest.
A high PaO2 will reduce pulmonary vascular resistance (by abolishing hypoxic pulmonary vasoconstriction). Blood will then flow along the path of least resistance, into the lungs,
leaving very little flow to the systemic circulation, compromising blood flow down the
coronary arteries, and may cause cardiac arrest.
The key to adequate resuscitation is to ensure that the aims are conservative, i.e. SaO2
of 75% with a normal or slightly high PaCO2 so that the pulmonary vascular resistance is
not reduced. Heart rate and cardiac function need to be optimized with volume boluses and
inotropes if oxygen delivery is compromised. Once stabilized and appropriately assessed
these children undergo early surgery.
Conditions with duct-dependent mixing of pulmonary and

systemic blood
Transposition of the great arteries (TGA)
In this condition the systemic and pulmonary circulations exist as two parallel circuits and
cyanosis is present from birth (Figure 6.4). Blood mixes between the two otherwise independent
circuits by passing across a patent arterial duct or across the foramen ovale. A VSD, if present, is
53
another point at which mixing can occur. The amount of mixing will depend on the patency of
the DA (or septal defects) and the relative pressures in the pulmonary and systemic circulations.
A single mixing point, such as an ASD, may not be enough to sustain a child with a
TGA. PgE2 should be started in these children. If it does not lead to an increase in the
arterial saturations then the usual strategies of assessing and treating cardiovascular filling,
the administration of inotropes and controlling ventilation should be instituted before
emergency balloon atrial septostomy (BAS) to improve mixing.
Lungs
Body
Figure 6.4. Transposition of the great arteries (with

connecting DA).
Left ventricle
Right
ventricle
M = Mixing point i.e. ductus arteriosus and PFO/ASD
Duct-independent condition causing cyanosis

Total anomalous pulmonary venous connection (TAPVC)
The pulmonary veins drain, either partially or fully, into the systemic veins, e.g. inferior
vena cava. There is an absence or much reduced pulmonary venous connection to the left
atrium. The consequences are reduced blood flow to the left heart with a subsequent
increase in pulmonary blood flow. This results in pulmonary hypertension, breathing
difficulties secondary to the volume of blood in the pulmonary circulation and right heart
failure. If the pulmonary venous drainage is obstructed the condition is a true surgical
emergency. This is because there is very little flow to the left atrium and the lungs become
very oedematous secondary to pulmonary venous hypertension. This in turn leads to
compensatory pulmonary arterial hypertension. Despite the need for urgent surgery proper
resuscitation prior to urgent transfer to theatre is needed.
Conditions presenting with signs of poor cardiac function in

infants and older children
Congenital heart disease may present when the child is no longer a neonate. The presenting
symptom is more likely to be breathlessness on a background of poor feeding and faltering
growth. Fluid overload of the right ventricle, e.g. from a VSD, will lead to pulmonary hypertension and right heart failure. Obstructive conditions such as tetralogy of Fallot or subclinical
coarctation of the aorta will cause right and left ventricular hypertrophy respectively.
Heart failure
An infant or older child who presents with fluid retention, breathlessness and signs of a low cardiac
output may well be suffering from cardiac failure. Causes of heart failure in children include:
large left to right shunts, e.g. VSD or persistent DA
chronic arrhythmias, e.g. supravertricular tachycardias (SVTs) from channelopathies
primary cardiomyopathy, e.g. dilated cardiomyopathy
outflow tract obstruction, e.g. undiagnosed coarctation of the aorta
54
cyanotic CHD, e.g. truncus arteriosus (although this is rarely undiagnosed)

myocarditis.
A very brief history of breathlessness with symptoms and signs of heart failure, but without
a very enlarged heart on chest X-ray, should raise concerns about fulminant myocarditis.
Although, as mentioned above, classic presentations may not occur in CHD.
Left to right shunts

An infant who presents with breathlessness, signs of fluid retention and a murmur is likely to have a
lesion with a large left to right shunt, e.g. VSD, complete atriovertricular septal defect (AVSD) or
truncus arteriosus. These children do not present when the DA closes but present some weeks after
birth when the pulmonary vascular resistance (PVR) falls (it does not achieve adult levels until
approximately 6 months of age).
When the PVR drops, blood flows freely across the VSD and back into the pulmonary
circulation. This increases pulmonary blood flow and lung water while the relative underfilling of the systemic circulation causes fluid retention. This results in an increase in the
work of breathing and heart failure.
Tetralogy of Fallot (ToF)

The main problem in the tetralogy of Fallot (ToF) is obstruction to the right ventricular
outflow tract (RVOT) as it passes into the pulmonary artery (see Figure 6.5). It is the main
reason that the right ventricle hypertrophies. The obstruction to the RVOT is dynamic in that
it worsens when the infundibulum at the base of the pulmonary artery goes into spasm. With
increased resistance to blood passing out of the right ventricle, it passes instead through a VSD
into the left ventricle. This can cause profound cyanosis. Giving oxygen may not correct the
hypoxia, as this is a true shunt. Giving oxygen is important though as it will optimize
oxygenation of the blood that does pass through the lungs. Severe desaturations associated
Figure 6.5. Tetralogy of Fallot. Note the VSD (with
the root of the aorta shown overriding the VSD). There
is also pulmonary stenosis and a hypertrophied right
ventricle.
55
with right ventricular outflow tract obstruction are known as spells. The steps in managing a
child who is spelling are detailed in Figure 6.7.
Hypertrophic ventricles
As mentioned previously ToF leads to right ventricular hypertrophy whereas coarctation of
the aorta causes the left ventricle to hypertrophy. Children with hypertrophic ventricles
have a similar problem to adults. The decreased compliance means that a greater pressure is
required to achieve the same ventricular filling. Also, the myocardial oxygen requirement
can outstrip the coronary blood supply. The principle of aiming for a low normal heart rate
with adequate coronary perfusion is the same.
Arrhythmias
An arrhythmia is usually well tolerated in children. However, they may be associated with
collapse or dyspnoea. It is often not easy to distinguish between a physiological tachycardia
and a pathological rhythm. A good-quality ECG and a long rhythm strip are essential for
diagnosis.
The arrhythmias can be caused by:
electrolyte abnormalities
channelopathies
conduction pathway abnormalities, e.g. re-entrant tachycardias
structural abnormalities, e.g. hypertrophic ventricle
postoperative, e.g. AVSD repair causing complete heart block.
The principles of treatment of arrhythmias in children are the same as in adults and are
detailed below under Problems with known/treated/palliated CHD. Advice can be sought
from the local cardiac centre.
Child with known CHD (post surgery)

As paediatric cardiac surgery becomes more successful, the prevalence of children with
CHD increases. It is not uncommon for children with known cardiac disease to present to a
local emergency department with any of the common intercurrent infections or other
diseases of childhood. They may also be suffering from an acute deterioration of their
cardiac problem. The management of these conditions is described below under Problems
with known/treated/palliated CHD.
Managing the neonate with CHD

The collapsed neonate with CHD
The question in neonates is often not what type of CHD does the child have? but does this
child have CHD? The initial assessment may not reveal the diagnosis and therefore a
structured approach is essential. First manage the collapsed child as the initial approach
recommends in Chapter 4 and in Table 6.1.
Once a diagnosis of CHD is suspected, it is important to continue to stabilize the patient
rather than spend time attempting to diagnose the type of CHD. A child with an acyanotic
heart condition will be cyanotic if they are sick enough.
56
Table 6.1. Initial management of a neonate with suspected cardiac disease.
Phase of
management
Assessment
Action
Initial phase
On presentation of
the child
(see Chapter 4 for
initial
management)
Assess need for intubation

or CPR
Give oxygen
Ventilate by hand if needed
Gain IV access
IO if more than three IV attempts
Give fluid bolus
20 ml/kg of crystalloid
Take blood including ABG, clotting
and cross-match
Treat for other pathologies if uncertain of
cause, e.g. give antibiotics
After initial
assessment
Once imminently
life-threatening
risks have been
addressed and
CHD is suspected
Consider CHD and look for

confirmation
Pulses, peripheral and
central
Differential saturations
pre- and post-ductal
Poor peripheral perfusion
Organomegaly
Reassess after starting PgE2
Start PgE2
Airway
Consider intubation if PgE2 dose rising
Breathing
Do not aim too high with SaO2 (> 75%
is often adequate)
Circulation
Continue with fluid boluses
Consider inotropes
Further
stabilization
Not immediately
lifesaving
procedures
Continue to reassess clinical

condition and blood results
Definitive plan (transfer vs.
operate)
Decide on further monitoring
Contact tertiary centre for advice, e.g. via

regional retrieval team
Arterial line (preferably right radial artery)
especially when:
Starting inotropes
Repeated blood gases required
CVC if:
More definitive access needed
Starting inotropes
Giving high-concentration drugs
Correct coagulopathy
Prostaglandin E
Prostaglandins are rapidly metabolized and therefore given by IV infusion. PgE2 is made up
as a solution with a concentration of 1 g/ml and given at a dose of 520 ng/kg/min. When
given to a child with CHD the response is rapid, within minutes. SaO2 and clinical signs of
cardiac function should be assessed regularly once treatment has begun in order to measure
the response.
Side effects of prostaglandin E treatment include hypotension, apnoea, fever, tachycardia, bradycardia and hypoglycaemia. An increasing dose of prostaglandin is an indication
for intubation in otherwise stable babies with CHD, as apnoeas may become significant
with higher doses.
Once the question has been raised of potential CHD in a sick child, the default position
should be to start PgE2 treatment (and monitor response). Table 6.2 gives a broad outline of
when to start PgE.
57
Table 6.2. Should I start PgE?
Findings that would prompt a trial of prostaglandin therapy in a neonate

Weak femoral pulses (especially if brachial pulse good)
Low saturations (< 90%) on maximal oxygen therapy
Pre-ductal (right arm) saturations higher than post-ductal (lower limb)
Inotropes
The need for fluid resuscitation in excess of 40 ml/kg, on the basis of poor pulses or
capillary refill, or a metabolic acidosis should prompt ventilation and inotrope therapy.
Inotropes should be started early if CHD is suspected. The choice of inotrope follows a
similar rationale to that in adults. In the case of conditions where inotropy and reduced
SVR are ideal, e.g. heart failure or conditions with excessive left to right shunts, dobutamine and milrinone can be considered. However, for a sick child who presents in a state
of shock, adrenaline is also a reasonable choice as a first-line inotrope. Remember that
inotropes may be administered through an IO needle if necessary.
A regional PICU retrieval team should have been contacted both for advice and to
initiate the transfer of the child by this point.
Invasive monitoring
Once the child is ventilated, an arterial line can be placed, preferably in the right arm
(radial rather than brachial) or a femoral artery and a blood gas and lactate obtained. This
should be monitored continuously for blood pressure with regular intermittent blood
gases.
A central line is desirable, but, as with other children, should only really be attempted if
the practitioner is confident of their skill in the procedure in small children.
Aims of management of neonatal CHD

The aim of managing neonates with CHD is to have:
adequate cardiac output
adequate blood flow to the pulmonary and systemic circulations
adequate oxygen saturations to meet demand.
When deciding on oxygen delivery remember that neonates will usually have a higher
haemoglobin concentration than adults (if not, early transfusion is indicated).
Cardiac output
Achieving a good cardiac output involves the basics discussed in Chapter 4, i.e. adequate
oxygenation and fluid resuscitation. Inotropes can be used as discussed above.
Balancing circulations directing blood flow to the correct side of the circulation
The DA provides an unregulated, low-resistance path for blood to flow between the
systemic and pulmonary circulations. As a result, the direction of blood flow will depend
58
on the balance between SVR PVR. A similar situation can occur in conditions such as
truncus arteriosus (TA) when both main arteries effectively come from a common outlet,
i.e. blood will flow via the path of least resistance.
Managing SVR is familiar to most adult physicians. Managing PVR on the other hand
may be a bit less familiar. Once intubated, managing the PVR of a child is straightforward.
Factors that will increase PVR include:
raised PaCO2 allowing the CO2 to rise will increase PVR
hypoxia hypoxia increases PVR, by means of hypoxic pulmonary vasoconstriction
acidosis acidosis will increase PVR.
On the other hand PVR can be reduced by:
low CO2 hyperventilation will reduce the PVR
high FiO2 hyperoxia will reduce PVR
nitric oxide inhaled nitric oxide will reduce PVR
drugs nitrates, beta-agonists and phosphodiesterase inhibitors will lower PVR.
In a situation where too much of the cardiac output is being diverted to the pulmonary
circulation and poor peripheral perfusion exists, relative hypoventilation with an FiO2
titrated to keep SaO2 at 7580% maximum may help balance the blood flow towards an
improved systemic output (by raising PVR).
Oxygen saturations
If a child has a true right to left shunt then no amount of oxygen will improve their SaO2.
Adaptations in utero, such as fetal haemoglobin, allow SaO2 of 7080% to be tolerated in
neonates with cyanotic heart disease. If the oxygen delivery does not match the babys needs
then markers of anaerobic metabolism such as lactate will increase.
The stable neonate with CHD

If a neonate with CHD is assessed and found to be stable it is reasonable not to chase the
saturations. There is no need to ventilate to improve the SaO2 alone; SaO2 above 70% can
be tolerated in these circumstances until diagnosis. The SaO2 should be measured in the
right arm (pre-ductal) as well as in the legs (post-ductal). A difference between the two
readings (lower SaO2 in the lower limbs) suggests a significant shunt but not a precise
diagnosis.
Managing the older child with CHD

Infants or young children with cardiac conditions can present with breathlessness,
drowsiness, oedema or nausea. Cardiac diseases can also mimic gastrointestinal
problems (abdominal pain and poor feeding in infants and children). Other signs of
heart failure are similar to those seen in adults, e.g. added heart sounds and distended
veins. Although cardiac problems are a rare cause of breathlessness in the general
paediatric population, shortness of breath is a common presentation for heart failure.
59
The initial management of sick children with CHD follows the pattern set out in
Chapter 4.
Initial management of the older child with CHD

It should come as no surprise that, even though the causes are different, the management
of cardiac disease in children is very similar to that in adults. It follows the same
principles:
give oxygen, aiming for SaO2 > 90%, unless the child is known to normally have lower
SaO2 saturations
check for arrhythmias
induce a diuresis, e.g. with furosemide 0.51 mg/kg
vasodilate and offload the heart.
The examination may reveal signs such as bilateral fine crackles, distended neck veins, a
gallop rhythm and an enlarged liver. An ECG and chest X-ray should be performed at the
earliest possible opportunity. The chest X-ray may confirm the size of the heart and the
presence or absence of any pleural effusions.
Further interventions such as intubation or inotropes will be required if:
inappropriate cyanosis persists in the face of high flow oxygen
there are poor peripheral pulses
tachycardia persists
a metabolic acidosis persists.
Inotropes
Often on PICU dobutamine or milrinone is used as a first-line therapy for heart failure.
However, given that many children will present to ED in extremis it is reasonable to use
adrenaline as a first-line inotrope. The choice of agent will need to be decided based on the
presentation of the child.
The inotropes should ideally be given through a second intravenous line (preferably an
IO or central line for adrenaline).
Management of arrhythmias
Tachycardias
Most tachyarrhythmias are variations of SVR due to aberrant intracardiac conduction
pathways that allow self-sustaining circuits to override the usual conduction pathway. It is
crucial to decide whether the rate is well tolerated physiologically or not. Then distinguish
between a narrow and a broad complex tachycardia. The former is always supraventricular
in origin, the latter may be supraventricular or ventricular in origin. A QRS complex
longer than 0.12 seconds is considered broad.
The first line of management should involve the initial management detailed in
Chapter 4.
The flow chart in Figure 6.6 gives an appropriate management path.
60
Bradycardia
A

bradycardia is a rate of:

slower than 90 bpm in an infant
slower than 80 bpm between the ages of 5 and 11
slower than 60 bpm in an older child.
The first stage in treating a bradycardia is to check that it is not secondary to a systemic
illness or a sign of imminent collapse. Bradycardia is an agonal rhythm in the critically ill or
hypoxic child. Other causes of bradycardia include hypertension, raised intracranial pressure or physical training in the teenager.
As with adults, the blood pressure and conscious level of the child should be assessed in
order to guide the next step. Once causative factors have been addressed atropine (20 g/kg)
should be given if the heart rate does not improve. Adrenaline 10 g/kg may be used in
extremis.
Heart block
The classification of heart block is the same for children as it is in adults. It is rare as a
primary condition and most children who present will have a history of cardiac
intervention or drug therapy. Assessment of the adequacy of the circulation is of
prime importance in making decisions. Symptomatic heart block is treated temporarily
with intravenous isoprenaline or external pacing if the equipment is available. Consultation with a paediatric cardiologist is essential to guide management in these
children.
The spelling child with tetralogy of Fallot

Spelling occurs when the infundibulum goes into spasm. The spell may be secondary to
fever, dehydration or another intercurrent illness. It can occur in children from birth until
they undergo surgical repair. Children with ToF typically present to their local hospital with
an exacerbation of cyanosis. These episodes of acute desaturation may get worse as the child
gets older.
The mainstay of treatment is to relieve anxiety and try to decrease the quantity of blood
shunted (right to left) across the VSD. This is done by:
trying to relax the muscle of the infundibulum
increasing right ventricular filling
improving the balance between SVR and PVR to reduce the shunt across the VSD.
Oxygen, the kneechest position, morphine, fluid loading and vasoconstrictors are
the mainstays of treatment. In extremes, ventilation, vasoconstrictor infusions and
cooling (to minimize oxygen consumption) are used prior to emergency surgery
(see Figure 6.7).
Problems with known/treated/palliated CHD

The parents of children in the cardiac outpatient population will have a good idea of their
childs usual SaO2 and activity level. The children are likely to present to ED with a
Ventricular
Broad complex >3 beats together
rate 150300 bpm
Fusion beats
Rate 110300 bpm
Supraventricular
Rate 110300 bpm
Stable
Hypotensive/shock
VT sustained > 30 seconds
Stable
Vagal manoeuvres
Adenosine
50400 g/kg
No conversion
Unstable
Cardioversion 0.52.0 J/kg

Synchronized shock
Repeat 2j/kg
Consider amiodarone
Repeat 2j/kg shock
Review diagnosis
Most likely to be SVT
No conversion
Unstable
Conversion
Stable
No conversion
Stable
Conversion
Stable
Discuss with cardiologist
Chronic management
Refer to cardiology
with investigations
Seek expert opinion
Figure 6.6. Management of tachycardia.

Initially manage as
chapter 4
Give oxygen
Bring knees up to chest
Give morphine
0.1 mg/kg
Clinically improving
Give fluid 20 ml/kg
Reassess
Give intravenous
vasoconstrictor
Prepare to intubate
Consider cooling to
35C
Figure 6.7. Management of a child with ToF who is spelling.
62
common paediatric illness which has been made more severe by their underlying CHD.
The child with CHD may also present with worsening of their underlying cardiac
condition.
Worsening cyanosis
This may be unforeseen or develop ahead of the cardiologists expectations. A full physical
examination should be performed in order to exclude intercurrent pulmonary or other
diseases. In the absence of a clear secondary cause of the cyanosis, referral back to the
cardiac centre is appropriate. The finding of a large pleural effusion in this context may
present difficulties as drainage will be required to improve respiratory function but the
sedation or anaesthesia required for drainage may cause severe instability. Beyond the
neonatal period PgE has no role.
Heart failure
This may be a consequence of either an arrhythmia or myocardial decompensation after
previous surgical treatment. The assessment of cardiac function and related arrhythmias
needs to be thorough. The management should be as above, with advice from the childs
cardiac centre.
Syncope
This may be a consequence of a tachycardia/bradycardia or worsening cardiac function. It
may be the result of the progression of disease or the complications of surgery.
Palpitations
This may be the symptom of an arrhythmia that can be detected clinically. It might be an
anxiety symptom but should be investigated in the usual way and will merit referral back to
the cardiology clinic.
Infective endocarditis
Non-specific symptoms in a child with a known structural cardiac disease should alert
the admitting physician to the possibility of infective endocarditis. Clinical assessment of the circulation will guide the intensity of therapy but crucial in the management of such a child is a microbiological identification of the infective organism.
Unless there is a clear reason for urgent treatment, antibiotics should not be started
empirically.
Summary
Managing children with CHD can be an intimidating task. Although this chapter has
explained the details of their management, it should not be forgotten that the initial
management is the same as for all other emergency conditions. The main difference is that
prostaglandin therapy should always be considered in a sick neonate and a low threshold
should be held for its use.
63
Golden rules when managing children with CHD

Ask the parents what the saturations are normally aim for the saturations given
Children with CHD still suffer from common illnesses
When other factors have been ruled out consider cardiac decompensation
Always perform an ECG in a child with CHD to look for arrhythmias
Further reading
Biarent D, et al. European Resuscitation
Council Guidelines 2010, Section 6.
Paediatric life support. Resuscitation
2010;81:13641388.
Marino B, Fine K, McMillan J. Blueprint

Paediatrics. Blackwell, 2006.
Wren C, OSullivan JJ. Survival with congenital
heart disease and need for follow up in adult
life. Heart 2001;85:438443.
Section 2
Chapter
Clinical Conditions
The child with shortness of breath

Brian Shields and Benjamin Stanhope
Introduction
Respiratory disease makes up approximately 35% of all unplanned PICU admissions in the
UK. The commonest causes are:
bronchiolitis (41%)
pneumonia/lower respiratory tract infection (LRTI) (21%)
croup (6.8%)
asthma (6.5%).
Shortness of breath can be the presenting feature of a host of problems. This chapter
primarily aims to cover the pulmonary causes. Asthma is covered in Chapter 8.
How respiratory pathology differs in children

Children have a greater initial capacity to respond to physiological stress than adults.
Exceptions to this include small neonates and children with chronic illness. When children
become exhausted they can decompensate suddenly and precipitously.
Pathology
Children are more prone to virally mediated chest problems such as bronchiolitis and viralinduced wheeze than adults. This is due to:
mucous plugging
inflammation of their much smaller airways having a greater impact
relative immaturity of immune development.
Increasingly, children with an extensive history of chronic lung disease due to prematurity and mechanical ventilation are presenting to the ED. These children are particularly
susceptible to all forms of chest infection.
Physiology
There is evidence to suggest that children younger than 1 year old with bronchospasm do
not respond to standard medications such as 2-agonists, presumably due to lack of airway
receptor development.
64
Chapter 7: The child with shortness of breath
65

To doctors unfamiliar with managing children, it can be very difficult to differentiate those
with a simple chest infection from those who are critically ill. For many children the results
of giving fluid, paracetamol and cooling by removing clothes can be dramatic. However,
Table 7.1 lists some signs that should cause concern in any child.
Table 7.1.
Intermediate risk
High risk
Respiratory rate (RR)
Age < 12 months and RR > 50

breaths/min
Age > 12 months and RR > 40
breaths/min
RR > 60 breaths/min
Other respiratory features
Nasal flaring
Crepitations (pneumonia or
bronchiolitis)
SaO2 < 95% in air
Apnoea
Grunting
Moderate or severe chest
indrawing
Head bobbing
SaO2 < 90% on air
Colour
Pallor reported by parent/carer
Activity
No response to normal social cues

Wakes only with prolonged
stimulationa
Decreased activitya
Pale/mottled/ashen/blue
a
No response to social
cuesa
Unable to be roused, or if
rousable does not stay
awakea
Appears ill to healthcare
professionala
Confusion/altered mental
state
Unable to speak in
sentences
Hydration/feeding
Poor feeding in infants (less than

50% of normal fluid intake in
preceding 24 hours)
Dry mucous membranes
Reduced urine output
Capillary refill time > 3 seconds
Reduced skin turgor

Unable to feed
Temperature
Fever > 5 days duration
Age < 3 months and

temperature > 38 C
Age 36 months and
temperature > 39 C
Activity and appearance are highly subjective and are considered poor markers of severity by some experts.
Data adapted from: British Thoracic Society, 2002; SIGN, 2006; NICE, 2007. http://www.cks.nhs.uk/
cough_acute_with_chest_signs_in_children/management/detailed_answers/assessment.
66
Specific conditions causing shortness of breath

Bronchiolitis
Bronchiolitis is a condition caused by inflammation of the small airways (bronchioles) of
the lungs. It is most common in children aged 26 months, and is uncommon in children
over 1 year old. It is usually caused by respiratory syncytial virus (75%), but can also be
caused by parainfluenza, influenza and adenoviruses.
Bronchiolitis usually has a gradual onset with coryzal symptoms and mild fever followed
by cough and increasing respiratory distress. Disease severity peaks at around 45 days and
then gradually improves. Approximately 9% of all hospital admissions with bronchiolitis
require admission to PICU.
In bronchiolitis, the bronchiolar epithelial cells are invaded by virus, resulting in:
ciliated cell death
sloughing of dead cells into the airway
increased mucus production
proliferation of non-ciliated cells.
This results in reduced mucus removal, air trapping and reduced gas exchange. The end
result is an obstructive airway disease similar to asthma.
Children who are admitted to hospital will initially receive supportive care with
humidified oxygen, nasogastric or IV fluids, or observation. They can usually be managed
on general paediatric wards with continuous pulse oximetry monitoring and regular
observations.
Identifying the sicker children with bronchiolitis

Although many children with bronchiolitis will look quite unwell, Table 7.2 highlights the
main signs that should prompt admission to hospital. Table 7.3 lists patients who are at
high risk of getting severe bronchiolitis and in whom a lower threshold for admission
should be practised.
Table 7.2. Signs of bronchiolitis that should warrant admission to hospital.
Poor feeding (< 50% of usual fluid intake in preceding 24 hours)

Lethargy
Apnoea
Respiratory rate > 60/min
Nasal flaring and/or grunting
Severe chest wall recession
Cyanosis
Oxygen saturation 94% in air
Uncertainty regarding diagnosis
High-risk factors (see above)
67
Table 7.3. High-risk patients with bronchiolitis.
Prematurity < 35 weeks

Chronic lung disease (cystic fibrosis, bronchopulmonary dysplasia)
Age less than 12 weeks
Congenital heart disease
Hypotonia (e.g. trisomy 21 or neuromuscular disease)
Immunocompromised
Anatomical airway abnormalities
Signs of a deteriorating child

Children who appear to be worsening should receive regular capillary blood gases. As the
childs work of breathing becomes greater, and they tire, the PaCO2 will rise. If there is a
trend towards a rising PaCO2 (especially if the child is on CPAP see below) then
intubation is likely to be needed.
Apnoeas are relatively common in younger babies suffering from bronchiolitis. The
majority are brief (less than 5 seconds) and self-terminating. However, the signs that a child
having apnoeas might need intubation include:
prolonged apnoeas
needing stimulation to terminate apnoeas
increasing frequency of apnoeas
apnoeas associated with episodes of hypoxia and bradycardia.
Targeted treatments for bronchiolitis

Oxygen
Humidified oxygen is usually delivered via an appropriately sized headbox, with adequate
gas flow to avoid CO2 build-up inside the box. The oxygen needs to be humidified because
of the build-up of secretions that would otherwise dry up and worsen the condition. Face
masks or nasal prongs may be used although they are sometimes poorly tolerated.
Fluid management
Children who are unable to feed effectively should receive nasogastric feeds. Those who
have severe respiratory distress may require intravenous fluids instead.
Intravenous fluids should be restricted to two-thirds of usual maintenance in severe
bronchiolitis (see Chapter 32). This is because of the risk of syndrome of inappropriate
antidiuretic hormone secretion (SIADH) and hyponatraemia (which can be severe enough
to cause convulsions). However, some children may be hypovolaemic due to reduced intake
and may need fluid boluses. Clinical assessment will dictate the appropriate management.
Ventilatory support
Continuous positive airway pressure (CPAP) or mechanical vertilation (IPPV) may be
needed for infants exhibiting:
apnoeas (see above)
hypoxaemia refractory to oxygen therapy
68
hypercapnia
moderately increased work of breathing.
CPAP in bronchiolitis aims to improve gas exchange by preventing alveolar collapse. Because
of the obstructive nature of the pathology, CPAP actually reduces the PaCO2 in bronchiolitis
by keeping airways open and decreasing the work of breathing. CPAP also helps to minimize
apnoeas by stimulating breathing in children.
IPPV in bronchiolitis initially requires high inspiratory pressures and long inspiratory
times. Once alveoli are recruited pressures can be gradually weaned. When ventilating bear
in mind similar principles to those of ventilating a child with asthma (see Table 7.4).
Table 7.4. Ventilation strategy in bronchiolitis.
Use a protective lung strategy (tidal volume of 510 ml/kg)

Allow enough time for expiration to avoid gas trapping
Keep peak inspiratory pressure below 30 cmH2O (or plateau pressure depending on
measurement)
Use high PEEP values (up to 10 cmH2O) to overcome airway collapse titrated to optimize
compliance
Use longer inspiratory times to allow air to flow through the high resistance airways (0.751
second, depending on size and age)
Tolerate moderate hypercapnoea (to a pH of 7.2)
Suction the ET tube regularly as secretions may build up rapidly
Antibiotics
Secondary bacterial infection in bronchiolitis may affect as many as 40% of patients who
require intubation. Patients at high risk of severe bronchiolitis are also at increased risk of
secondary bacterial infection. Antibiotics should be considered in high-risk patients, or if there
is strong clinical suspicion of bacterial infection (high temperature or lobar changes on CXR).
2-Agonists, adrenaline and corticosteroids

Use of these agents is not supported by the current literature.
Potential pitfalls when managing children with bronchiolitis

Apnoea can occur without preceding signs of severe respiratory distress.
Symptoms will peak at around 45 days so be aware that children in the first few days of
illness are likely to get worse.
Bronchodilators are ineffective.
Secondary bacterial infections or collapse/consolidation can complicate bronchiolitis
and prolong PICU stays.
Wheeze in pre-school children ( 5 years)

Wheeze in pre-school children is a common presentation. It should be distinguished from
wheezing episodes in older children as the majority of pre-school wheezers will not go on to
develop asthma. Wheeze in pre-school children has been described in two main categories:
69
episodic (viral) wheeze wheeze following upper respiratory tract infection (URTI)
lasting 24 weeks but the patient is otherwise well
multiple trigger wheeze wheeze following URTI plus interval symptoms and/or
wheeze due to other triggers. There may be a personal or family history of atopy.
The vast majority of these children will not have severe problems unless they have a past
history of chronic lung disease or atopy.
Targeted treatments for pre-school wheeze

Inhaled 2-agonists
These form the mainstay of treatment of acute wheeze, although inhaled salbutamol has
been shown often to have no effect in children less than 1 year of age.
Inhaled steroids
These are not effective in the acute setting. However, long-term inhaled corticosteroids may
reduce severity of future wheeze episodes in children with multi-trigger wheeze.
Oral steroids
Steroids have been shown to be ineffective in non-atopic children with episodic (viral)
wheeze. In atopic children they may be of some benefit.
Severe wheeze
Intravenous salbutamol, aminophylline and magnesium sulphate can all be considered in
severe cases. They can be used for the same recommendations and dosing regimens as for
asthma. The indication and strategies for ventilation in these children will be similar to
those for asthma also (see Chapter 8).
Potential pitfalls when managing pre-school children with wheeze

Always reassess the child after their initial treatment to ensure that the bronchodilators are
effective. Symptoms can return suddenly after initial improvement, so patients should be
monitored carefully.
Pneumonia
Causes
The most common bacterial organism that causes community-acquired pneumonia (CAP)
in children is Streptococcus pneumoniae, with Mycoplasma pneumoniae and Chlamydia
infection being less common. Bordetella pertussis infection can occur in non- or partially
immunized children.
Respiratory syncytial virus is the commonest viral pathogen in paediatric pneumonia.
Other viruses that cause CAP include parainfluenza, adenovirus, rhinovirus, varicella zoster
virus, influenza, cytomegalovirus, herpes simplex virus and enteroviruses. Viruses account
for 1435% of CAP cases in childhood. There may be mixed bacterial/viral infection in up
to 40% of cases.
Viral causes are more common in pre-school-age children.
70
Clinical features
Symptoms and signs of pneumonia may include:
tachypnoea
tachycardia
use of accessory muscles
nasal flaring
subcostal/intercostal recession
cough with sputum production
fever
chest pain
dyspnoea.
Bacterial pneumonia should be considered in children aged up to 3 years when there is a
combination of:
fever of > 38.5 C
chest recession
respiratory rate of > 50 breaths/min.
If wheeze is present in a pre-school child, primary bacterial pneumonia is unlikely. For
older children a history of difficulty in breathing is more helpful than clinical signs.
Targeted treatments for pneumonia

When managing children with severe pneumonia, there is significant overlap with that of
sepsis (see Chapter 5).
Antibiotics
Amoxicillin is the first-choice antibiotic therapy in children under the age of 5 years
because it is effective against the majority of pathogens that cause CAP in this group. Local
guidelines should be followed for any other choices. Macrolide antibiotics may be used as
empirical treatment in children above 5 years of age because of the higher incidence of
Mycoplasma pneumoniae infection.
Physiotherapy
There is no evidence to support the use of physiotherapy for pneumonia in otherwise
healthy children. Physiotherapy may be of benefit to children with neuromuscular or
musculoskeletal disease who have difficulty clearing secretions on their own.
CPAP/ventilation
The decision of when to ventilate children with pneumonia will depend on similar factors to
all those mentioned above, i.e.:
hypoxia refractory to oxygen therapy
rising PaCO2
a child who appears to be tiring/confused/irritable
large fluid requirement and sepsis.
71
As a guide to whether a child is tiring, if they allow you to put in a cannula without crying
or making a fuss, it is likely that they are very unwell.
CPAP is used in pneumonia in the same way as it is in adults. The major caveat to the
use of CPAP is to not let a child deteriorate on it. If they do not respond quickly (within 30
minutes) then delaying intubation can be dangerous.
If a decision is taken to ventilate a child with pneumonia, be aware that they will
desaturate very quickly on induction. Preoxygenate the child as much as possible, ventilate
the patient with a bag and mask while waiting for the muscle relaxant to work, and do not
take too long before abandoning an attempt at intubation if you struggle.
Pleural effusion and empyema

Infective causes of pleural effusion
The most common cause of empyema in the UK is Streptococcus pneumoniae. Other
organisms include S. pyogenes, Haemophilus influenzae type b, Mycoplasma pneumoniae,
Pseudomonas aeruginosa and other streptococcal species. In developing countries Staphylococcus aureus is the main cause and Gram-negative bacteria are more common.
There is a constant production and reabsorbtion of fluid within the pleural space. In
pneumonia, parenchymal inflammation can lead to an increase in pleural fluid (effusion). If
not reabsorbed, there will be an increase in inflammatory cells resulting in pus (empyema)
and fibrin deposition causing loculations. If left untreated, the loculations will develop into
thick and non-elastic bands, which may impair lung function and cause chronic problems.
Non-infective causes of pleural effusion

Rarely, an effusion is the presenting sign of an underlying malignancy in a child. Many of
the other secondary causes of pleural effusion occur in children with a known underlying
condition such as congenital heart disease, renal disease, connective tissue disorders and
trauma, which includes post-cardiothoracic surgery.
Differences between adults and children

It is uncommon for children to have pre-existing lung disease, therefore the final outcome
of pleural effusion and/or empyema is almost always excellent. This is in contrast to
adults, where empyema is a cause of significant morbidity and mortality (20%) due to
co-morbidities.
Targeted treatments of pleural effusion/empyema

Antibiotics without chest drain
If the effusion is small and the child is well then conservative antibiotic therapy can be
given, coupled with close observation. Signs of deterioration or failure to improve should
prompt urgent consideration of drainage of the effusion (see Table 7.1).
Antibiotics with chest drain

Early intervention with drainage of effusions/empyemas has been shown to reduce length of
stay. There is no evidence to indicate that large-bore chest drains confer any advantage over
smaller. As with adults, remember the basic principles of chest drain management.
72
All chest tubes should be connected to a unidirectional-flow drainage system (such as an

underwater seal bottle) which must be kept below the level of the patients chest at all times.
A bubbling chest drain should never be clamped.
A clamped drain should be unclamped immediately if a patient complains of
breathlessness or chest pain, or deteriorates whilst being ventilated.
The drain should be clamped for 1 hour after 10 ml/kg of chest fluid has drained as
re-expansion pulmonary oedema is a potential problem.
Potential pitfalls when managing children with pleural collections

General anaesthesia and large-volume chest fluid aspiration pose a significant risk of
sudden death in children with superior mediastinal obstruction related to malignancy.
Aspiration of pleural fluid should therefore be of small volume (e.g. 5 ml) for diagnostic
purposes only and general anaesthesia/sedation avoided under such circumstances.
Non-pulmonary causes of shortness of breath

Cardiac
Heart failure may present with tachypnoea. Patients would also have other signs of failure
such as tachycardia, pallor, sweating, weak pulse and hepatomegaly. This may occur in
children with a past history of cardiac disease or acutely in children with undiagnosed
conditions such as large ventricular septal defect or viral myocarditis (see Chapter 6).
Metabolic
Hyperammonaemic states with associated, often profound, metabolic acidosis secondary to
inherited metabolic conditions, such as urea-cycle disorders or organic acidaemias, can act
as a respiratory stimulant and cause tachypnoea (see Chapter 12 for management).
Diabetic ketoacidosis (Chapter 11) and, less commonly in children, salicylate poisoning
also present with a metabolic acidosis-driven tachypnoea.
If any of these conditions are suspected, initial investigation should include a blood gas,
lactate and ammonia.
Monitoring and investigations

Pulse oximetry, ECG, blood pressure, temperature and blood glucose should be monitored
in every child admitted to hospital. The tests below may aid in making a diagnosis and
assessing the severity of disease.
Blood tests
Acute-phase reactants (full blood count (FBC), C-reactive protein(CRP),
erythrocyte sedimentation rate (ESR))
These markers distinguish poorly between bacterial and viral infections in children and
therefore should not be measured routinely. However, they may be helpful in monitoring
response to treatment in severe bacterial infections and empyema (particularly CRP).
Blood gas measurement

Capillary blood gas measurement gives an acceptable indication of PaCO2 and pH. It is less
reliable in children with impaired peripheral perfusion (see Chapter 37).
73
Blood gases should be measured in children with signs of severe respiratory distress,
apnoea or exhaustion. Ex-preterm infants with chronic lung disease may have a
chronically raised PaCO2 with metabolic compensation (similar to adults with severe
COPD).
Sampling blood gases should not delay the institution of respiratory support in children
with signs of severe respiratory distress, apnoea or exhaustion.
Serum electrolytes
These should be measured in children with severe respiratory distress or LRTI when
SIADH cannot be excluded. Serum potassium should be monitored in children requiring
IV salbutamol or aminophylline, or prolonged, frequent nebulized salbutamol.
Radiology
Chest X-ray
Chest radiography should not be performed routinely in children with mild uncomplicated acute LRTI or bronchiolitis. Instead it should be considered in:
patients who are high risk
patients not following an expected clinical course
patients possibly requiring additional ventilatory support.
When interpreting paediatric CXRs, findings are poor indicators of aetiology; however,
hilar consolidation is more likely to be associated with severe disease. A large effusion
causing whiteout can be difficult to differentiate from severe collapse/consolidation
on X-ray.
Ultrasound
Ultrasound can be helpful for:
determining depth of an effusion
differentiating effusion from consolidation in whiteout
identifying loculations and density of fluidsuggesting empyema
guidance when inserting chest drains.
CT scan
If a CT scan of the thorax is indicated in a child, it is worth discussing this with your local
tertiary centre respiratory or PICU team, as small children may need an anaesthetic for the
procedure. It may be possible, depending on how unwell the child is, to transfer them
unintubated in order to have the scan performed in a specialist centre.
Microbiology
Blood cultures should be performed in all critically unwell children. Any pleural fluid
obtained should be sent for Gram stain and culture. An anti-streptolysin O (ASOT) test
should be requested for all patients with pleural effusion.
74
Nasopharyngeal aspirate (NPA)

Rapid direct immunofluorescent antibody testing can identify the causative virus in
bronchiolitis. Whilst not necessarily affecting management, this information may be useful
when nursing children in cohorts during winter epidemics of the disease.
Post-intubation management
Removing the work of breathing and applying PEEP can often improve the situation
markedly in children with an isolated chest infection. In the absence of cardiovascular
instability insertion of invasive monitoring such as a central or arterial line is not necessary.
A repeat CXR after intubation is essential in order to check ET tube placement.
If a child is failing to respond to ventilation and remains hypoxic then it is worth
considering whether there is a mechanical problem or whether the diagnosis is correct.
Mechanical
The most common cause for a child to be difficult to ventilate is endobronchial intubation. This
should be checked on a CXR as soon as possible. Another cause is mucus plugging. A suction
tube should be passed down the ET tube with saline wash in order to remove any plugs.
Diagnosis
Failure to respond to therapy should raise suspicion about your diagnosis. Consider a
pneumothorax (especially if a neck line has been sited, or high pressures have been used to
ventilate). Also think about congenital cardiac disease (especially in neonates).
Other considerations
Once the child has been intubated and is stable attention should move to:
optimizing ventilation (titrate to the blood gases)
checking glucose and electrolytes
checking temperature
gaining further IV access (if needed).
Preparing for transfer

As with other chapters, preparation for transfer will need detailed, written, documentation
of the history, clinical findings and lab results. Other items which should be arranged prior
to transfer include:
copies of any investigations/images to be taken or sent to the receiving hospital
pre- and post-intubation CXR should be available
a copy of all of the blood gases taken throughout the episode
ensure that the parents are aware of any plans why/and to where the child is being
transferred.
Summary
Shortness of breath is a common mode of presentation for children. A methodical approach to
its management is needed, as well as a decisive plan. If there is any doubt about the management
of the patient, it is best to get the input of a PICU retrieval team sooner rather than later.
Golden rules
The work of breathing for children is higher than that for adults
Children can compensate for respiratory disease
They also decompensate quickly
Children with chronic disease will need closer monitoring and earlier intervention for
common illnesses
Further reading
British Thoracic Society. 2005. Guidelines for
the management of pleural infection in
children.
managed? Arch Dis Child 2007;

92:394398.
Yanney M, Vyas H. The treatment of
bronchiolitis. Arch Dis Child 2008;
93:793798.
Clark JE. Children with pneumonia: how

do they present and how are they
75
Section 2
Chapter
Clinical Conditions
The child with asthma

Josephine Langton and Stuart Hartshorn
Introduction
The UK has one of the highest prevalence rates of childhood asthma worldwide; it affects
1.1 million children in the UK, equating to one in ten children.
Because of improved management, hospital admission rates for childhood asthma have
decreased over the past 20 years. However, in 2009 12 children under 14 years of age died
from asthma in the UK. A diagnosis of asthma is much more likely in young children, but
fatal or life-threatening exacerbations are more common in adolescents.
Much of this chapter makes reference to sections of the British Thoracic Society (BTS)/
Scottish Intercollegiate Guidelines Network (SIGN) guidelines pertinent to the assessment
and management of acute exacerbations of asthma in children.
Pathophysiology
Asthma is a chronic inflammatory condition associated with variable and reversible airway
obstruction. It is characterized by recurrent episodes of cough, wheeze, chest tightness and
difficulty in breathing. Asthma is known to be associated with a family history of atopy,
exposure to certain environmental factors and intrinsic airway hyper-reactivity.
Exposure to specific triggers (e.g. viral upper respiratory tract infections, the cold,
exercise or smoke) causes:
airway oedema
excess mucus production
bronchoconstriction.
How asthma differs in children

Although asthma is a condition that affects both children and adults, there are age-related
differences in presentation, investigation and management. The prevalence of asthma is
much higher in children, and they are much more likely to present with acute
exacerbations.
Assessing children with asthma

Asthma is a clinical diagnosis. A clinical assessment is, therefore, the best method to
determine severity of an exacerbation. When assessing a child it is important to use age
76
Chapter 8: The child with asthma
77
specific parameters (Table 8.1). Tachypnoea and tachycardia are suggestive of respiratory
distress. Bradypnoea and bradycardia suggest fatigue and imminent collapse.
Table 8.1. Age-specific normal values.
Age
Respiratory rate range

(breaths per minute)
Heart rate range

(beats per minute)
Systolic blood pressure

range (mmHg)
< 1 year
3040
110150
7090
15 years
2035
90120
80100
512 years
1525
80120
90110
> 12 years
1520
60100
100130
Recession
Features of respiratory distress include intercostal, subcostal and sternal recession; if this is
present in an older child (over 7 years) it suggests severe respiratory distress. Infants may
also head bob and have nasal flaring. As children become tired from their increased
respiratory effort, these clinical signs can become less apparent.
Wheeze
Assessing the amount and character of the wheeze is important; biphasic wheeze suggests
increasing obstruction, while a silent chest indicates that the exacerbation is life-threatening.
Conscious level and speech

Inability to talk or a decrease in conscious level also signifies a severe or life-threatening
exacerbation.
Regular review during their admission is essential as children who have previously
appeared to be sick but stable can rapidly deteriorate. Clinical features for assessment of
severity are listed in Table 8.3.
Presentation of acute asthma exacerbations

Children with an acute exacerbation of asthma typically present with a history of
wheeze, worsening cough, chest tightness and increasing difficulty in breathing. Parents
will often use the term wheezing to describe any abnormal noise associated with
breathing. It is therefore important to clarify exactly what they mean in order to rule
out other diagnoses.
In order for children to be managed appropriately, the severity of their symptoms must be
accurately assessed. When assessing the severity of the exacerbation it is useful to find out about:
symptom duration
exposure to known triggers
medication used at home
recent increase in use of their reliever.
Table 8.2 lists risks factors that would put a child at risk of having a severe exacerbation of
asthma.
78
Table 8.2. Risk factors for severe exacerbations (adapted from BTS/SIGN guideline).
Previous life-threatening asthma, e.g. PICU or HDU admission

Previous admission to hospital for asthma within the last year
Repeated ED attendances for asthma within the last year
Treatment regimen consisting of three or more classes of asthma medication
Repeated use of 2-agonist
Brittle asthma multiple sudden severe exacerbations, poorly responsive to therapy
Non-compliance with treatment
Investigations
Two quick, non-invasive, investigations that can be used to provide information about the
severity of the exacerbation are:
pulse oximetry
peak expiratory flow (PEF) compared to patients own best value or best predicted value
(in children over 6 years).
Chest X-ray
Chest X-rays are rarely useful as they typically show hyperinflation and atelectasis and tend
not to affect patient management. They should be reserved for life-threatening cases not
responding to treatment, or when there is persistent asymmetry of chest signs.
Blood gases
Blood gas analysis (typically capillary in children) should be reserved for those with severe
or life-threatening symptoms or when ventilatory support is being considered. Blood gas
abnormalities should not themselves be used as absolute indicators for intubation and
ventilation; however, trends over time provide valuable additional information about the
response of the patient to treatment.
Blood lactate level can signify severe hypoxia or poor perfusion. It can also increase with
aggressive adrenoceptor stimulants such as salbutamol.
79
Table 8.3. Clinical features for assessment of severity (reproduced from BTS/SIGN guideline).
Moderate asthma
exacerbation
Acute severe
asthma
Life-threatening asthma
Talking
Able to talk in
sentences
Cant complete
sentences in one
breath or too
breathless to talk or
feed
Confused, exhausted
Oxygen
saturations
92%
< 92%
< 92%
Peak flow
50% best or
predicted
3350% best or
predicted
< 33% best or predicted
Heart rate
140/min in children
(aged 25 years)
125/min in children
(aged > 5 years)
> 140 in children

(aged 25 years)
> 125 in children
(aged > 5 years)
Hypotension
Respiratory rate
40/min in children
(aged 25 years)
30/min in children
(aged > 5 years)
> 40 breaths/min
(aged
25 years)
> 30 breaths/min
(aged > 5 years)
Poor respiratory effort
Examination
Silent chest
Cyanosis
Management
Initial management
For acute exacerbation of asthma, the aim of treatment is to reverse bronchoconstriction
and promote oxygenation. Current management of asthma is based on the BTS/SIGN
guideline (Table 8.4). Initial therapy is well established with a large amount of evidence
documenting both safety and efficacy.
Table 8.4. Management of acute asthma (reproduced from BTS/SIGN guideline).
Age 25 years
Age > 5 years
Assess asthma severity
Assess asthma severity
Moderate asthma
SpO2 92%
No clinical features
of severe asthma
NB: If a patient
has signs and
symptoms across
categories,
always treat
according to their
most severe
features
2-Agonist 210
puffs via spacer
facemask [given
one at a time single
puffs, tidal
breathing and
inhaled separately]
Increase 2-agonist
dose by 2 puffs
every 2 minutes up
to 10 puffs
according to
response
Severe asthma
SpO2 < 92%
Too breathless to talk
or eat
Heart rate > 140/min
Respiratory rate > 40/
min
Use of accessory neck
muscles
SpO2 < 92% plus any of:
Silent chest
Agitation
Altered consciousness
Cyanosis
Moderate asthma
SpO2 92%
PEF > 50% best or
predicted
No clinical features of
severe asthma
NB: If a patient has
signs and symptoms
across categories,
always treat
according to their
most severe features
Oxygen via face mask/nasal prongs to achieve

SpO2 9498%
Nebulized 2-agonist:
2-Agonist 10 puffs
via spacer facemask
salbutamol 2.5 mg or
or nebulized
terbutaline 5 mg plus
salbutamol
ipratropium bromide
2.5 mg or terbutaline
0.25 mg nebulized
5 mg
Oral prednisolone 20
mg or IV
Soluble prednisolone

hydrocortisone 4mg/
20 mg or IV
kg if vomiting
hydrocortisone 4 mg/
Discuss with senior
kg
clinician, PICU team
Repeat 2-agonist up
or paediatrician
to every 2030
minutes according to Repeat
bronchodilators every
response
2030 minutes
Severe asthma
SpO2 < 92%
PEF 3350% best or
predicted
Heart rate > 125/min
Respiratory rate > 30/
min
Use of accessory neck
muscles
SpO2 < 92% plus any
of:
PEF < 33% best of
predicted
Silent chest
Poor respiratory
effort
Altered
consciousness
Cyanosis
Oxygen via face mask/nasal prongs to achieve

SpO2 9498%
2-Agonist 210 puffs 2-Agonist 10 puffs
Nebulized 2via spacer
via spacer or
agonist: salbutamol
nebulized salbutamol
5 mg or terbutaline
Increase 2-agonist
2.55 mg or
10 mg plus
dose by 2 puffs every 2
terbutaline 510 mg
ipratropium
minutes up to 10 puffs
bromide 0.25 mg
according to response Oral prednisolone 30
nebulized
40 mg or IV
Oral prednisolone 30
hydrocortisone 4 mg/ Oral prednisolone
40 mg
kg if vomiting
3040mg or IV
Reassess within 1
hydrocortisone
If
poor
response
hour

4mg/kg if vomiting
nebulized ipratropium
Discuss with
bromide 0.25 mg
senior clinician,
Repeat 2 agonist and
PICU team or
ipratropium up to
paediatrician
every 2030 minutes
according to response
Consider soluble

oral prednisolone
20 mg
Reassess within 1
hour
If poor response add

0.25 mg nebulized
ipratropium bromide
Repeat
bronchodilators
every 2030
minutes
Assess response to treatment
Assess response to treatment
Record respiratory rate, heart rate and oxygen saturation every 14 hours
Record respiratory rate, heart rate, oxygen saturation and PEF/FEV every
14 hours
Responding
Continue bronchodilators 14
hours prn
Discharge when stable on 4
hourly treatment
Responding
Not responding
Continue bronchodilators 14 hours Continue 2030 minute
prn
nebulizers and arrange HDU/
PICU transfer
Discharge when stable on 4 hourly
treatment
Consider: Chest X-ray and blood
gases
Continue oral prednisolone 3040 Consider risks and benefits of:
mg for up to 3 days
Bolus IV salbutamol 15 g/kg
At discharge
if not already given
Ensure stable on 4 hourly inhaled
Continuous IV salbutamol
treatment
infusion 15 g/kg/min
(200 g/ml solution)
Review the need for regular
treatment and
IV aminophylline 5 mg/kg
the use of inhaled steroids
loading dose over 20 minutes
(omit in those receiving oral
Review inhaler technique
theophyllines) followed by
Provide a written asthma action
continuous infusion 1 mg/kg/
plan for
hour
treating future attacks
Bolus IV infusion of
Arrange follow up according to
magnesium sulphate 40 mg/kg
local policy
(max 2 g) over 20 minutes

Not responding
Arrange HDU/PICU transfer
Consider:
Chest X-ray and blood gases
Continue oral prednisolone for up

to 3 days
At discharge
Ensure stable on 4 hourly inhaled
treatment

Review the need for regular
treatment and the use of inhaled
steroids
Review inhaler technique
Provide a written asthma action
plan for treating future attacks
Arrange follow up according to
local policy
IV salbutamol 15 g/kg bolus over

10 minutes followed by continuous
infusion 15 g/kg/min (dilute to
200 g/ml)
IV aminophylline 5 mg/kg loading
dose over 20 minutes (omit in those
receiving oral theophyllines)
followed by continuous infusion
1 mg/kg/hour
82
Oxygen
Oxygen should be given when SaO2 is less than 94%.
Inhaled bronchodilators
Inhaled 2-adrenoceptor agonists, such as salbutamol or terbutaline, are the first-line
therapy irrespective of severity. 2-agonists can be administered via an inhaler, a spacer
or a nebulizer. Ten puffs of an inhaled bronchodilator given via a spacer has the same
therapeutic effect as the nebulized route, as long as the patient is able to use the inhaler
properly. Nebulized treatment should be reserved for those with severe or life-threatening
exacerbations.
Ipratropium bromide, when used in combination with 2-agonists, has been shown to
reduce the need for hospital admission in those with severe exacerbations.
Steroids
Systemic corticosteroids are important and should be given early. Evidence shows that this
will reduce the need for admission and prevent symptom relapse. Oral and IV steroid
therapy have similar efficacy, so IV treatment should only be used in children who are
vomiting or unable to swallow.
In patients failing to respond to optimal first-line treatment or those with features of lifethreatening asthma, it is crucial to involve the intensive care team early.
In contrast to the extensive evidence about initial management of acute asthma, there is
relatively little guidance for second-line therapy in those who are not responding. The
options available are detailed below, with the treatment of choice often determined by local
hospital guidelines.
Intravenous salbutamol
This should be considered when there is poor response to inhaled 2-agonists as there is
evidence to suggest that it reduces the duration and severity of severe exacerbations of
asthma. It should be given as below:
loading dose of 15 g/kg over 10 minutes
followed by a continuous infusion of 15 g/kg/min, dose-adjusted according to
response and heart rate.
Aminophylline
This should only be used in severe or life-threatening exacerbations that are failing to
respond to conventional therapy. There is limited evidence for its efficacy. It may improve
lung function, but there is no evidence to confirm a reduction in acute symptoms or need
for intubation. Evidence about impact on duration of admission is unclear.
There is a significant risk of vomiting and cardiac arrhythmias. Cardiac monitoring is
essential.
Loading dose of 5 mg/kg over 20 minutes (omit if on oral theophylline).
Followed by a continuous infusion of 1 mg/kg/h.
83
Magnesium sulphate
Evidence confirms that this is a safe therapy in asthma but the therapeutic benefit remains
unclear. It may reduce the rate of intubation and ventilation. Studies do suggest that in
severe exacerbations there is a reduction in admission rate and improved lung function.
Doses of up to 40 mg/kg (maximum 2 g) over 20 minutes.
Hypotension can be a complication.
Intubation and initiating intensive care

Most children with acute exacerbations of asthma respond to early and aggressive medical
treatment, but those who fail may require intubation and ventilation. Prior to considering
intubation all other therapies should be maximized.
Indications for ventilation

The decision to ventilate a child in status asthmaticus is a clinical one. The mortality in
ventilated children is approximately 4%. It remains unclear whether ventilation contributes
to this mortality rate, or whether it simply represents the chance of dying in children with
asthma severe enough to warrant ventilation.
Failure to initiate ventilation in a timely manner may put a child at undue risk.
Indications for mechanical ventilation in children with status asthmaticus include:
altered sensorium
progressive exhaustion
severe hypoxia despite maximal oxygen therapy
failure to reverse a respiratory acidosis
a silent chest
cardiac or respiratory arrest.
Induction of anaesthesia
As with any of the conditions detailed in this book, intubation is a risky phase of
management. When intubating a child in status asthmaticus it is important to anticipate:
rapid desaturation
reflux/vomiting (especially if on aminophylline)
cardiovascular instability
the potential to cause a pneumothorax as positive-pressure ventilation is initiated.
Ketamine is routinely used on PICU as an induction agent (13 mg/kg depending on the
state of the child). It is ideal for asthma due to stimulation of the sympathetic system and
bronchodilator effect. Suxamethonium or rocuronium are used as first-line muscle
relaxants.
Choice of ET tube
A cuffed ET tube can be used, even in small children, in order to manage the high airway
pressures used for ventilation. A cuffed ET tube will obviate the need for a tube change to
minimize a leak.
84
Sedation
Morphine and midazolam are still used as sedative agents in status asthmaticus, although in
severe cases a ketamine infusion can be used (instead of morphine) in order to promote
bronchodilatation. Ketamine is given at a rate of 0.52 mg/kg/h.
All patients ventilated for asthma should receive a muscle relaxant infusion, e.g.
rocuronium at 1mg/kg/h.
Factors complicating ventilation in asthma

Two principal factors that influence ventilation in asthma are gas trapping and
auto-PEEP.
Gas trapping (breath stacking)

If the ventilator is set with too short an expiratory time (e-time) then the resistance of the
airways will not allow the air blown in during the inspiratory phase to escape during
expiration. If there is a part of the tidal volume left behind with each breath, the lung
volume at end-expiration will increase with each cycle.
Eventually this will reach a point where the lung volume at end-expiration will reach the
plateau portion of the lung compliance curve. The ventilator will continue to deliver
positive pressure, and the intrathoracic pressure will increase. This can lead to severe
cardiovascular compromise.
Auto-PEEP
The gas that remains in the alveoli from gas trapping will exert its own positive pressure,
above atmospheric pressure. This is auto-PEEP.
Setting a ventilator in asthma

Ventilation strategies for children with asthma follow the same principles as for adults. Asthma
is an obstructive lung pathology that restricts the movement of gases. It is reasonable to
start ventilation with a lung-protective strategy (see Chapter 4 for settings), but rapid
titration will be needed which takes into account the need for:
long expiratory times to allow air to move out and prevent gas trapping
long inspiratory times to allow adequate gas exchange
high airway pressures much of which will not be transmitted to the alveoli
positive end-expiratory pressure to overcome the tendency of airways to collapse.
Inspiratory pressure
Inspiratory pressures in children are the same as adults. A pressure of 30 cmH2O is
considered the maximum, which should only be exceeded if there is no other option. The
pressure should be titrated to deliver a smaller tidal volume of 57 ml/kg, if possible.
Inspiratory and expiratory times

Long inspiratory times (i-times) will be needed to allow the pressure of ventilation to
overcome the resistance of the airways. Teenagers should have settings similar to those of
adults (assuming they are a normal size). Infants will need i-times set at 0.751.0 second
initially. This can be increased as necessary.
85
The limiting factor for the e-time is the respiratory rate. Most children can tolerate a
relatively low respiratory rate of 1012 breaths per minute. This is because a paralysed child
will produce considerably less CO2 than an awake child.
Ventilators capable of displaying the ventilation flow loops are ideal for demonstrating
that a child is managing to breathe out completely. If the expiratory flow does not return to
a baseline, then the e-time should be lengthened (respiratory rate lowered).
Positive end-expiratory pressure (PEEP)

There are two schools of thought regarding the use of PEEP in asthma. Most people hold
with the theory that the PEEP should be set to match the patients own intrinsic PEEP
(auto-PEEP). This will optimize the airway opening and allow the greatest degree of gas
transfer.
It can be found by gradually increasing the PEEP until the tidal volume no longer
increases with each breath.
Others opt not to use any PEEP on the grounds that it allows for a larger difference
between inspiratory and expiratory pressures (thus increasing the tidal volume).
Carbon dioxide
Permissive hypercapnoea is practised in status asthmaticus. A much higher than normal
PaCO2 may have to be tolerated (seek expert advice at this point). Treating the acidosis with
sodium bicarbonate is counter-productive due to the increased carbon dioxide load.
Potential problems with ventilation

Hypoxia
If hypoxia persists on the ventilator, increase the FiO2 and request an urgent CXR. Examine
the child, looking for signs of:
pneumothorax
endobronchial intubation.
In the absence of either, increase the drug regime, i.e. salbutamol and ketamine, and
consider the use of volatile agents.
Cardiovascular instability
If a childs blood pressure drops while being ventilated, give a fluid bolus and rule out a
tension pneumothorax clinically.
Preparing the child for transfer

As with other chapters, the mainstay of preparing a child for transfer is first to stabilize the
child, then ensure that the child is optimized, i.e. adequate intravenous access, and a secured
airway.
Details of the treatment given and history should be documented, and all radiological
investigations available for transfer. Blood gases should all be available and labelled relative
to the childs condition at the time of taking, i.e. pre-intubation or after starting
aminophylline.
86
Summary
Asthma still carries a significant mortality rate. It should never be underestimated.
Although the majority of children improve with medication, it is still necessary to ventilate
the sickest children. If in doubt, contact the local PICU retrieval team for advice at any
point along the treatment algorithm.
Golden rules
A child who is getting quieter may be becoming sicker
Regular repeated observations are essential
Don't be afraid to ventilate a child if they fail to respond to medical management
Difficulty in ventilating a child may be due to a pneumothorax
Discuss any child that you are concerned about with PICU
Further reading
British Guideline on the Management of Asthma.
A national clinical guideline. May 2008,
revised January 2012: British Thoracic
Society/SIGN.
Browne G, Lam L. Single-dose
intravenous salbutamol bolus for
managing children with acute severe
asthma in the emergency department:
reanalysis of data. Pediatr Crit Care Med
2002;3(2):117123.
children with moderate to severe acute

asthma. Arch Pediatr Adolesc Med
2000;154:979983.
Mitra A, et al. Intravenous aminophylline
for acute severe asthma in children
over two years receiving inhaled
bronchodilators. Cochrane
Collaboration 2009.
Partridge R, Abramo T. Acute asthma in the
pediatric emergency department. Pediatr
Emerg Med Pract 2008;5(11).
Cheuk DKL, Chau TCH, Lee SL. A metaanalysis on intravenous magnesium sulphate
for treating acute asthma. Arch Dis
Child 2005;90:7477.
Roberts G, et al. Intravenous salbutamol bolus

compared with an aminophylline infusion in
children with severe asthma: a randomised
controlled trial. Thorax 2003;58:306310.
Ciarallo L, Brousseau D, Reinert S. Higher-dose

intravenous magnesium therapy for
www.asthma.org.uk.
Section 2
Chapter
Clinical Conditions
The child with decreased

consciousness and coma
Mark D. Lyttle
Introduction
Consciousness is a measure of a childs ability to interact with their environment and other
people. Decreased consciousness may be due to any disease in its most severe form.
Although non-traumatic causes are less frequent, they carry a higher mortality rate of
almost 50%.
The annual incidence according to cause is:
338/100 000 in children 015 years old
trauma
160/100 000 in the first year of life
non-traumatic
30/100 000 in all children
non-traumatic
The aim of immediate management is to minimize any neurological damage whilst making
a definitive diagnosis. Therefore, therapeutic measures must take place at the same time as
diagnostic procedures.
After initial assessment and stabilization, management centres on determining the
specific diagnosis. However, 14% of children presenting with coma remain undiagnosed
after hospital discharge or post-mortem examination. With appropriate assessment and
investigation, the risk of missing important diagnoses is reduced, and potentially lifesaving
therapy can be initiated without delay.
How decreased consciousness differs in children

Assessment
An identical stimulus will cause a range of responses in different children, and reactions are
often different from those seen in adults. A modified version of the GCS is used in younger
children in order to take into account their developmental abilities (see Chapter 35).
Assessment is most difficult in preverbal children and infants, as the symptoms and signs
are often non-specific, and poor differentiators of the underlying cause. In babies there may
be excessive sleepiness, decreased feeding or irritability.
Many children have an underlying condition causing a baseline conscious level that
would not be seen as normal in other children. While clinicians will be able to detect gross
abnormalities, care-givers will appreciate subtle variations, and they should be involved in
the assessment of such children if possible.
87
88
Causes
The incidence of head injury increases throughout childhood, peaking in adolescence. Nontraumatic coma has a bimodal distribution, being most common in infants and toddlers,
with a smaller peak in adolescence. It is secondary to a diffuse metabolic insult in 95% of
cases, and structural lesions in 5%. Some conditions are much less common in children
than in adults, notably hypertensive encephalopathy and cerebrovascular accidents.
The features of each condition are discussed later in this chapter. Table 9.1 outlines
some of the more common causes by age of presentation
Table 9.1. Common causes of decreased consciousness by age of presentation.
Age range
Cause
Additional information
Infant
(01 year)
Infection
More common in infants

Nonspecific presenting features
Non-accidental injury
(NAI)
Consider in all cases of decreased consciousness with

no clear cause
Hypoglycaemia
May be due to congenital cause, poor feeding, or

underlying cause, e.g. infection
Raised intracranial
pressure
May be due to congenital malformations

Often slowly increasing, head circumference
expanding due to open sutures
Inherited metabolic
disease (IMD)
Present with first illness
Infection
Compared with infants, classic features of infection

tend to be present
Traumatic causes
Usually due to exploratory behaviour

Consider NAI
Toxin ingestion
Usually accidental due to exploratory behaviour
Raised intracranial
pressure
Hypoglycaemia
More acute than infants as sutures closed
Young child
(15 years)
May be lack of substrate

May be inherited metabolic disease
Inherited metabolic
disease
May never have had illness as infant
Older child
(512 years)
Infection
Classic signs of infection generally present
Metabolic/endocrine
disease
First presentation of endocrine disorders
Adolescent
(over 12 years)
Traumatic causes
Risk-taking behaviour
Multisystem and isolated head trauma
Diabetic ketoacidosis
Most commonly presents in adolescence

Often due to poor compliance
Toxin ingestion
Often deliberate
May be deliberate self-harm or recreational
Chapter 9: The child with decreased consciousness and coma
89
Investigations and management

Overarching principles
As mentioned above, history-taking, examination, investigation and therapeutic measures
must occur simultaneously. Many of the conditions in Table 9.1 require early, and aggressive, intervention.
Treating life-threatening causes concurrently at the beginning of the clinical course
while simultaneously conducting investigations to determine the diagnosis is often the
best management strategy, as no individual cause is more important, and several may
coexist in the same child.
Early liaison with specialist teams at a tertiary centre is often important to optimize care,
especially if a particular diagnosis becomes likely. This may involve contacting general
paediatricians, intensivists, neurologists, specialists in metabolic medicine, endocrinologists, cardiologists or toxicologists.
Investigations
Several causes of decreased consciousness may be recognized clinically but require further
investigation, whereas some diagnoses can only be made through laboratory testing. The
rarity of many of the causes adds to the diagnostic difficulty. In children in whom the
cause is clear, including those with trauma or recurrent episodes of decreased consciousness, it is acceptable to perform directed investigations. In children who are post-ictal it is
acceptable to adopt a watch-and-wait approach for the first hour provided they have a
normal blood sugar.
Initial investigations for all children

Children with decreased consciousness of unknown cause should have the investigations listed in Table 9.2 performed at the earliest opportunity. These will identify all
immediately treatable problems, and include those samples that can aid diagnosis at a
later stage.
Table 9.2. Initial investigations in a child with decreased consciousness.
Capillary and laboratory blood glucose

Blood gas
Urea and electrolytes
Liver function tests
Plasma ammonia
Full blood count and film
Coagulation studies
Blood culture
12 ml of plasma to be separated, frozen and stored for later analysis
12 ml of plain serum to be saved for later analysis
Urinalysis for ketones, glucose, protein, nitrites and leucocytes
10 ml of urine to be saved for later analysis
ECG
90
Further investigations
The additional tests in Table 9.3 should be requested if the cause remains unknown after the
initial investigations or if confirmation of a suspected diagnosis is needed.
Table 9.3. Further investigations to consider in a child with decreased consciousness.
CT brain
Especially if the working diagnosis is raised intracranial pressure (ICP), intracranial abscess, or
cause unknown
Lumbar puncture (check for contraindications first)
Microscopy, Gram stain, culture and sensitivity, glucose, protein
PCR for herpes simplex with/without pneumococcus/meningococcus, other viruses
Other tests may be performed in individual cases
Urine and blood toxicology
Urine organic and amino acids
Plasma lactate
Advanced investigations
If the cause remains unknown after the initial investigations, CT brain and lumbar
puncture results, the following should be performed:
EEG for non-convulsive status epilepticus
acyl carnitine profile and plasma amino acids
ESR and autoimmune screen
thyroid function test and thyroid antibodies.
General management
Attention should be given to the basics of resuscitation prior to establishing the underlying
cause of the decreased consciousness (see Golden rules at the end of the chapter and
Chapter 4). This will ensure that if the cerebral insult is due to reduced cerebral blood flow
or hypoxia it will not be worsened. Some simple interventions may improve the conscious
level. Decreased consciousness usually represents disease in its most severe form. Senior
assistance should be sought immediately in order to intervene early to manage problems
such as hypovolaemia, hypoglycaemia and raised ICP.
Intubation
The selection of drugs for induction should be based on the underlying condition and preexisting pathology, and they should be given judiciously. Some children will present a difficult
airway, whether expected or unexpected, and this should be anticipated. Even with these
precautions, the patient may deteriorate rapidly, especially if they have elevated ICP or severe
sepsis. It is, therefore, sensible to have resuscitation drugs prepared.
In patients selected for intubation it is vital to clearly document both the neurological
assessment immediately prior to intubation, and the progression of conscious level in the
time preceding intubation. It will be possible to continue to monitor the response to
treatment in certain underlying conditions such as hypovolaemia, but others, such as
intractable seizures, may require additional equipment. Patients will still require adequate
sedation in the normal manner once intubated.
91
Abbreviated neurological assessment

If time permits, a full neurological assessment should be performed. However, in an emergency, important signs that can be assessed quickly include pupil size and symmetry and
response to stimulus. Supraorbital pressure should be used as the painful stimulus as this is
Table 9.4. Management of a child with decreased conscious level.
Phase of
management
Assessment
Action
Initial phase
On presentation of
the child
Also see Chapter 4
Manage airway and assess for need to

intubate
GCS/pupil size and symmetry
Hypoxia refractory to oxygen
Rising CO2
Inability to maintain airway
Concerns about raised ICP
Cardiovascular shock
Assess circulation for:
Signs of shock
Arrhythmias
Hypovolaemia
Check blood glucose
Give oxygen
After initial
assessment
Once imminently
life-threatening
risks have been
addressed
Assess response to phase 1

Assess neurology
Supraorbital painful stimulus
AVPU (alert, responds to
voice, responds to pair,
unconscious) / GCS
Record GCS every 15
minutes if < 12
Every hour if > 12
Consider antibiotics
Repeat blood gases to assess:
Ventilation
Electrolytes
Acidosis
If raised ICP suspected manage
as in Chapter 19
Further stabilization
Not immediately
lifesaving
procedures

Contact retrieval team

Document and review results as
they arrive
Arterial line if:
Haemodynamic instability
Starting inotropes
Repeated blood gases
CVC if:
Starting inotropes
Giving high concentration
drugs
Search for cause according

to investigations mentioned above
Definitive plan (transfer)
Gain IV access
Give fluid bolus
Take blood including ABG,
clotting and cross-match
If situation allows collect a
hypoglycaemic screen
92
replicable between observers, and avoids any confusion caused by paraesthesia or hemiplegia.
During initial assessment AVPU or modified GCS assessments are appropriate. For subsequent assessments the modified GCS will reflect more subtle changes in consciousness
Hypoglycaemia
The blood glucose concentration should be determined immediately in all cases. Even if
present, hypoglycaemia may not be the sole cause of decreased consciousness. It should
therefore be corrected quickly and the patient should be reassessed once corrected.
If the blood glucose is less than 2.6 mmol/l the following should be performed:
give 2 ml/kg of 10% dextrose
maintain blood glucose between 4 and 7 mmol/l with an infusion of 10% dextrose
request the following bloods as part of a hypoglycaemic screen:
lactate
insulin
cortisol
growth hormone
free fatty acids
-hydroxybutyrate
acyl carnitine profile from the stored samples taken with initial investigations.
If the blood glucose is between 2.6 and 3.5 mmol/l then the laboratory glucose result from
initial investigations should be reviewed urgently.
Management of specific conditions

As decreased consciousness is the end point of many disease processes, the individual causes
are covered in detail elsewhere in this book. However, a specific cause is sometimes
identified based on the clinical features. Targeted management should then be instituted.
The conditions below are mentioned in detail elsewhere in the book. Should the
conditions be suspected please consult the relevant chapter.
Infection
Decreased consciousness may be secondary to central nervous system or systemic
infection. Investigations should consist of those listed above, with a lumbar puncture
if there are no contraindications. Because infection is common and treatable, commencement of broad-spectrum antibiotics with or without acyclovir should be considered in all children presenting with decreased consciousness with non-specific
features, especially if there is:
temperature > 38 or < 35.5 C
tachycardia or tachypnoea
white cell count > 15 000 mm3 or < 5000 mm3
non-blanching rash.
When examining these patients older children tend to present with classical features;
however, infants may only display non-specific features such as floppiness, poor feeding,
lethargy, irritability. They may not be pyrexial either. See Chapter 5.
93
Toxin ingestion
It should be routine to ask family or friends about medicines, essential oils or recreational
drugs kept in the house. In older children direct questioning may also reveal the problem.
In young children naloxone should be given if there are opiates in the home, or signs of
toxicity present. See Chapter 13.
Diabetic ketoacidosis (DKA)

Decreased consciousness may be the first presentation of diabetes mellitus. As well as the
blood sugar and pH, urinary ketones must be checked if DKA is suspected. Drowsiness in
children with DKA is a very serious sign of potential disaster as it is caused by cerebral
oedema and raised ICP (as well as venous sinus thrombosis). See Chapter 11.
Seizures
Seizures may be subtle, especially if only autonomic signs are present. The worry in children
with decreased conscious level is determining between the post-ictal state and ongoing
seizures. The investigations should be carried out as above if the seizure is prolonged and
there is no previous history of epilepsy. See Chapter 10.
Arrhythmias
Decrease or loss of consciousness may be the first presentation of cardiac disease or occur in
children with a known background of cardiac disease. The arrhythmia may also be
precipitated by electrolyte imbalance or toxin ingestion. An ECG should be performed in
all children with decreased consciousness. See Chapter 6.
Hypertensive encephalopathy
Hypertension is defined as a systolic blood pressure above the 95th centile on two
separate readings. In children it may be due to a number of pathologies. However, the
most common cause is renal disease. Distinguishing hypertensive encephalopathy from
hypertension secondary to raised ICP is crucial as it will guide management. Treating
hypertension secondary to raised ICP can cause deterioration due to decreased cerebral
blood flow. When intracranial causes have been ruled out, the hypertension should be
corrected in a controlled way, especially if there is a longstanding history of
hypertension.
Inborn errors of metabolism

These often present in infancy when precipitated by the childs first illness. A high index of
suspicion is needed in order to detect them early. Once suspected, it is vital to liaise with the
regional metabolic team. See Chapter 12.
Transfer
Even at the point of transfer, some children will not have a diagnosis. It is important to
continue to improve their clinical status where possible, often treating multiple remediable
causes simultaneously. Respiratory and cardiovascular status should be optimized, and
94
normal temperature should be maintained. Normoglycaemia and electrolyte balance

should be achieved by using isotonic solutions containing dextrose. The results of any
investigations should be made available to the retrieval team in an organized manner.
A decision should be made regarding who is responsible for following up outstanding
investigations and informing the receiving team. In the case of neonates, if the mother is
too unwell to travel, a sample of her blood should be sent in case it is required for analysis
or cross-match.
Summary
Treating a child with a decreased conscious level is challenging. Performing the basics well
and correcting remediable causes may be the sum total of management before the retrieval
team arrives. If time permits, all of the above investigations should be performed, where
indicated. They should also be written clearly in the notes, with any late results phoned
through to the relevant team at the referral centre.
Golden rules

Begin assessment and treatment concurrently, not sequentially

Check for and treat hypoglycaemia and remember to start a dextrose infusion after the
bolus
When putting in a line or taking bloods, perform all investigations concurrently
Lumbar puncture is useful, but ensure no contraindications are present
Have a very low threshold for commencing antimicrobials
Ensure hypertension is not secondary to raised ICP before aggressive treatment
Remember NAI, especially in those < 1 year, and those with no obvious cause
Have a low threshold for giving a trial of naloxone
Further reading
Advanced Life Support Group.
Advanced Paediatric Life Support:
The practical approach, 5th edition.
Wiley-Blackwell 2011.
Avner JR. Altered states of consciousness.
Paediatr Rev 2006;27(9): 331338.
Kirkham FJ. Non traumatic coma in
children. Arch Dis Child 2001;
85:303312.
NHS Health and Social Care Information
Centre. Hospital episode statistics.
Department of Health. 2005. London.
www.hesonline.nhs.uk.
Paediatric Accident and Emergency Research

group. The management of a child
(018 years) with a decreased conscious level.
An evidence based guideline for health
professionals based in the hospital setting.
Available at: http://www.rcpch.ac.uk/childhealth/standards-care/child-healthguidelines-and-standards/guidelinesendorsed-rcpch-subspe-0#PAERG_decon.
Wong C, Forsyth R, Kelly T, Eyre J. Incidence,
aetiology, and outcome of non-traumatic
coma: a population based study. Arch Dis
Child 2001;84(3):193199.
Section 2
Chapter
10
Clinical Conditions
The child with seizures

Katie Z. Wright
Introduction
A fitting child is frightening for parents and carers. The management of a fitting child is
also a challenge for health professionals, requiring a calm and structured approach with
timely and specific interventions. This chapter covers the causes of fitting in different age
groups. It also looks at specific points to elicit in the history and examination as well as the
practical management of a fitting child.
Definitions and causes in children

A seizure can be defined as abnormal electrical activity of the cerebral cortex giving rise to
signs and symptoms. Children presenting with seizures account for 5% of paediatric
attendances to emergency departments.
Status epilepticus
Status epilepticus (SE) is divided into convulsive status epilepticus (CSE), which accounts
for the majority of presentations, and non-convulsive status. SE is defined as a generalized
convulsion lasting 30 minutes or longer, or when a patient fails to regain consciousness
between successive fits over 30 minutes or longer. CSE is a life-threatening neurological
emergency. It must be identified quickly and treated as it carries significant morbidity, and
a mortality of approximately 4%. Neurological sequelae of SE include epilepsy, learning
difficulties, behavioural problems and motor deficits. They occur more often in those under
1 year and can be devastating.
The first prospective population-based study on CSE was published in 2006. The north
London study found the incidence of CSE in childhood to be 1820 per 100 000 per year,
nearly four times higher than reported in adults.
Non-convulsive SE, while alarming and in need of treatment, does not pose the same
threat of immediate injury, specifically airway compromise. It is not treated with the
same pathway. The priority for patients with prolonged absence or partial seizure activity
is to seek expert advice from a paediatrician or paediatric neurologist as to which anticonvulsant agent to use.
Causes of seizures at different ages

Different aetiologies are more likely in different age groups; some common causes with age
ranges are listed in Table 10.1. The treatment for CSE, however, remains constant.
95
96
Table 10.1. Causes of fitting at different ages.
Age
Possible cause of fitting
Neonate
(up to 28 days)
Hypoglycaemia
CNS infections,a predominantly meningitis
Non-accidental injury (NAI)
Metabolic conditions
Electrolyte imbalance
Pyridoxine deficiency
Infant
Hypoglycaemia
CNS infectiona
Febrile illness
Epilepsy
NAI
Metabolic condition
Infantile spasms
Toddler
Hypoglycaemia
Head injury
Febrile illness
Malignancy
CNS infectiona
Accidental ingestion
Epilepsy
NAI
Child
Hypoglycaemia
NAI
Epilepsy
Head injury
Febrile illness
Malignancy
CNS infectiona
Accidental ingestion
Deliberate overdose
Adolescent
Epilepsy
CNS infectiona
Deliberate overdose
Head injury
Malignancy
Alcohol intoxication or withdrawal
Central nervous system (CNS) infection includes meningitis, encephalitis and cerebral abscess.
Neonates
The incidence of seizures is higher during the first 28 days of life than at any other time of
life. During this period, seizures are likely to have an underlying pathology. They often do
not present with the generalized tonicclonic movements seen in older children and adults.
The seizures may be subtle, with a combination of signs and symptoms, which may be
motor, autonomic or behavioural. First-line management in this age range is phenobarbital
with an IV loading dose of 2040 mg/kg.
Chapter 10: The child with seizures
97
Fitting is seen with significant hypoglycaemia, usually when the blood glucose is less
than 2 mmol/l. In neonates, this may be due to feeding difficulties, sepsis, or an underlying
metabolic disorder. In these cases, rapid identification of the low blood sugar and its
correction should prevent a seizure becoming prolonged. Treatment is with 2 ml/kg of
10% glucose intravenously (2.5 mmol/l for neonates), followed by a constant supply of
glucose, i.e. maintenance fluid or NG feeding (see Chapter 32).
In neonates and babies, fitting may be the first indicator of a damaged brain from nonaccidental injury (NAI). Subdural haematomas are not infrequently seen, as well as subarachnoid blood, cerebral contusions and diffuse axonal injury. There may be no outward
signs of injury or abuse on initial assessment.
Febrile seizures
Febrile seizures (FS) are the most common cause of childhood seizure, affecting between 2%
and 5% of all children. They can occur from 6 months to 6 years of age, with a peak
incidence of a first febrile fit at 18 months of age. They are usually self-limiting, lasting less
than 15 minutes. Up to 5% of children with febrile seizures will present with convulsive
status epilepticus, and should be managed as detailed below (Figure 10.1).
Patients who have a tendency towards seizures will have a lower seizure threshold if they
have an infection or fever, sometimes making the distinction between epilepsy and febrile
convulsion difficult.
Infection
Meningitis is the most frequently seen central nervous system (CNS) infection in children.
It must be considered in those with both a short-lived febrile fit and febrile CSE. If
suspected, treatment should be according to local antibiotic policy, including cover for
listeria in those younger than 3 months of age.
Poisoning and ingestion

Always consider ingestion and poisoning in children with a seizure of unknown cause,
especially if they have become acutely unwell without a fever. Be persistent when questioning what medicines are in the household, both legal and otherwise, and how accessible they
may be.
Epilepsy
Epilepsy is a term that covers a large collection of disorders of brain function, each
manifesting with repeated and unprovoked seizure activity. While epilepsy is an important
cause of seizures, not everyone with seizures has epilepsy. The overall incidence of epilepsy
in the population is 0.5%, whereas it is estimated that up to 10% of individuals may
experience at least one seizure in their lifetime.
In those with a diagnosis of epilepsy who are receiving anti-epilepsy drugs (AEDs) it is
worthwhile checking compliance with medication, and that the dose is weight-appropriate.
Fitting may be because they have had a recent change or withdrawal of medication, or the
child may have outgrown their current dose.
98
Complex children
Children with refractory seizures, or with complex care needs and seizure disorders, may be
well known to their local emergency department and paediatric team. Some have a written
management plan including details of seizure pattern, expected duration, recommended
treatment and any side effects of medications. These may travel with the child or be held at
the local ED. It is worth following the childs personal seizure treatment plan as they are
often written by a process of iteration from previous episodes. If it does not work then a
consultant neurologist should be contacted and intubation considered.
Whatever the cause of a generalized fit affecting the conscious level, if the seizure
activity lasts longer than 5 minutes the emergency management is the same (Figure 10.1).
Management of a fitting child

After 30 minutes of seizure activity, the cerebral arterial autoregulation is lost and blood
flow to the brain may become compromised. A vicious circle of anaerobic metabolism and
lactic acidosis with cell death and oedema then develops, resulting in increased intracranial
pressure. This in turn further reduces cerebral perfusion, exacerbating cell death. Systemic
effects of prolonged seizures include leucocytosis, acidosis, disseminated intravascular
coagulation, rhabdomyolysis and cardiac compromise with pulmonary oedema.
Many seizures are short-lived, resolving spontaneously within a few minutes. The
treatment regime starts 5 minutes after the onset of the fit, to reduce the risk of medicating
those who do not need it. For those who have stopped having seizures, airway management
remains a priority.
For those seizures that continue beyond 5 minutes, many will continue for 20 or
30 minutes, and treatment should be started to stop the fit and prevent SE. Unless the
child started fitting in the hospital, the first 5 minutes is likely to have passed at home or
with the transporting ambulance crew.
History and examination

One of the challenges in managing a fitting child is taking a focussed history from those
with the child while simultaneously assessing and managing airway, breathing and circulation. Ambulance crews will give a handover, note the details of any drugs given in the
pre-hospital setting and the time of administration. SE in children is most commonly
seen in those with a known diagnosis of seizure disorder. For children who fit regularly,
teachers, carers and parents may have given rectal diazepam or buccal midazolam. Always
ask about allergies or drug reactions.
While the childs seizure is being managed, a quick and directed examination for signs
of meningism, rash, fever, head trauma or signs of other injuries should be performed.
A full and thorough examination can be completed after the patient has been stabilized.
This should include looking for a source of infection and full neurological assessment
including fundoscopy. Any injuries should be documented, including photography if
possible, and communicated to the admitting team.
Management of the seizure

While talking to the family or paramedic crew, airway assessment is the first priority. If not
patent, use airway opening manoeuvres and adjuncts. The nasopharyngeal (NP) airway is
particularly useful in this situation (unless the seizure is secondary to head injury), as:
99
it can be used even if the teeth are clenched

it is better tolerated than an oropharyngeal airway as the patients conscious level
improves.
If the airway remains compromised, or there is obvious respiratory depression that is not
improving, consider early intubation.
While airway management is ongoing, another team member should simultaneously
gain intravenous access. Blood samples should be drawn. The priority is a venous blood gas
in order to measure blood glucose and electrolytes. A mixed metabolic and respiratory
acidosis is the most common abnormality seen during or shortly after seizure activity. This
should improve with cessation of seizure activity and a return to normal respiration. In
addition measure the blood glucose with a bedside testing kit.
If IV access is proving difficult, consider IO access early.
Drug treatment
The updated, evidence-based consensus algorithm for the management of convulsive status
epilepticus was published in the fifth edition of the Advanced Paediatric Life Support
Manual in 2011 (Figure 10.1).
100
Advanced
Life
Support
Group
Airway
High-flow oxygen
Dont ever forget glucose
A charity dedicated to saving life

by providing training
5 minutes after convulsion started
Yes or can
be established quickly
S
T
E
P
1
No
Vascular Access?
Midazolam (buccal)
0.5 mg/kg or
Diazepam (rectal)
0.5 mg/kg
Lorazepam
0.1 mg/kg IV/IO
If seizure is continuing 10 mins after start of step 1

S
T
E
P
2
Lorazepam
0.1 mg/kg IV/IO
Call for senior help
Prepare phenytoin
If seizure is continuing
10 mins after the start of step 2
reconfirm it is an epileptic seizure
S
T
E
P
3
Senior help is now needed

Seek anaesthetic/ICU advice
Phenytoin 20 mg/kg IV/IO over 20 min
Or if already on phenytoin give phenobarbitone 20 mg/kg IV/IO over 5 minutes
If seizure is continuing
20 mins after the start of step 3 (start of infusion)
an anaesthetist MUST be present
S
T
E
P
4
RSI with Thiopental (Thiopentone)
Figure 10.1. Status epilepticus treatment algorithm (reproduced with kind permission of the Advanced Life
Support Group).
101
Following the algorithm

Steps 1 and 2
If the seizure started 5 or more minutes ago, start at step 1 and proceed.
If one initial dose of benzodiazepine (step 1) has been given before hospital, start at
step 2 (assuming seizure activity continues past the 10 minutes).
If lorazepam is not available use IV or IO diazepam as a substitute at 0.25 mg/kg.
No more than two doses of benzodiazepine should be given, which includes any prehospital medication. This should be enough to stop seizure activity in the majority of
children.
Step 3
The majority of children (80% in some studies) should show a response to phenytoin within
20 minutes, and the anticonvulsant effect is relatively long-lasting; phenytoin has an average
half-life of 24 hours.
The longer a fit continues, the more difficult it can be to terminate. In practical terms,
this means the further down the algorithm you proceed without successfully stopping the
fit, the more likely you are to need to intubate the child. Thinking ahead and having
everything, and everyone, at hand is essential. This will include calling for senior anaesthetic
help sooner rather than later, certainly at or before step 3. Colleagues will be happier to
attend early and find a child who has stopped fitting than arrive after being called to a
compromised, fitting child with evolving complications.
If the child is already on maintenance phenytoin, give phenobarbitone 20 mg/kg over
5 minutes for step 3 instead. Other alternatives if already taking phenytoin are levetiracetam
(Keppra) or sodium valproate, both of which can be used intravenously.
Rectal paraldehyde may also be used during step 3 with senior advice, at a dose of
0.4 ml/kg mixed with an equal volume of olive oil. It should not be left in a plastic syringe
for more than a few minutes.
If 20 minutes after the start of the phenytoin infusion the seizure is persisting, proceed
to a rapid-sequence induction (RSI) with thiopentone.
Intubation of the child with seizures

Intubation may be required for a number of reasons, including:
airway obstruction
control of seizures with thiopentone
prolonged respiratory depression as a result of benzodiazepines.
If steps 13 of the algorithm and all other measures have failed to control seizure activity
then a rapid-sequence induction (RSI) with thiopentone at 25 mg/kg is the fourth and final
step of the treatment algorithm (Figure 10.1).
Ongoing sedation of the child

The child should be sedated with morphine and midazolam. In this instance it is reasonable
to use midazolam in younger babies, but care should be taken as it may lead to significant
102
hypotension. A short-acting muscle relaxant should be used initially in order to minimize

the chance of missing movement associated with seizures. An infusion can always be started
by the retrieval team.
The main problem with looking after a child who is ventilated is the false reassurance
gained from muscle relaxants. Seizures may continue, or recur, even after the administration of thiopentone. Sudden changes in heart rate, blood pressure or pupil size may be
the only signs of seizures.
If the child continues to fit after intubation a discussion should be had with your local PICU.
They may suggest repeat doses of some of the drugs or a change of anticonvulsant. However, in
the meantime give a bolus 0.5 mg/kg of midazolam and double the rate of the infusion.
Reassess the child

Once a child who has been in CSE is intubated the main aim of management is to reassess the
patient and correct any factors that may have triggered or exacerbated seizures. This will include:
treating any underlying infections
correcting electrolyte or glucose abnormalities
deciding on further investigations, e.g. CT scan
ensuring adequate sedation
sending off any investigations not done so far, e.g. blood cultures
passing a NG tube.
The same measures for prevention of secondary brain damage in trauma can be employed
for those intubated due to CSE (see Chapter 19). The main aims are:
ensure normothermia
aim for normocarbia
aim for normoglycaemia
maintain a 30-degree head-up position.
Once the seizures have been stopped the child should be relatively stable. If there are
cardiovascular problems consider another diagnosis as a cause of seizures, e.g. sepsis or
NAI. If there is any problem with ventilation perform a CXR. Check the ET tube position
and look for signs of aspiration.
Extubating a child
If the child is known to have a seizure disorder or a cause of fitting which is easily corrected
it may be considered best for the patient and their family to extubate them in the referring
hospital rather than transfer them to another hospital. This should be done after discussion
with the tertiary hospital.
In order to wake a child up and extubate them the team need to be certain that they are
happy to do so. This will include factors such as:
the age of the child
the cause of the seizure
the ease of intubation
an appropriate ward for looking after a child who has had an anaesthetic
medical cover out of hours.
103
Preparation for transfer

As with many of the other conditions the child should be prepared in a way that minimizes
delays to transfer. This will mean making sure that there is a thorough written history of
what has happened, including timings of the seizure. There also needs to be documentation
of test results and radiological images should be available to transfer.
Summary
Managing seizures in children has become highly protocolized. Once the protocol has been
followed it is important to get advice from your local PICU retrieval team. Decisive and
timely intervention is important in order to prevent long-term injury. Dont ever forget the
glucose.
Golden rules

The management of seizures is highly protocolized

Children with repeated seizures may have personalized protocols
Perform a blood gas early and correct simple pathologies such as hypoglycaemia
Further reading
Advanced Life Support Group. Advanced
Paediatric Life Support: The Practical
Approach, 5th edition. Wiley-Blackwell.
2011.
Convulsive status epilepticus. Arch Dis

Child 2007;92:28.
Stephenson JBP. Incidence, cause, and shortterm outcome of convulsive status epilepticus
in childhood: prospective population-based
study. Lancet 2006;368:222229.
Clinical Conditions
Section 2
Chapter
The child with diabetic ketoacidosis
11
Ian Jenkins
Introduction
Diabetic ketoacidosis (DKA) is defined biochemically as:
ketonaemia and ketonuria
hyperglycaemia (> 11 mmol/l)
venous pH < 7.3.
The peak age for diagnosis of type 1 diabetes mellitus (T1DM) in the UK is 1014 years old,
although this peak is moving towards occurring younger (there has been a steep rise in
patients under 5 years old presenting with DKA).
In the USA, 3040% of new T1DM patients present with DKA. The mortality rate for
these patients is 25%. Perhaps surprisingly, 525% of children with type 2 DM present
with DKA at first diagnosis.
This chapter focusses on assessment and management of the infant or child
suffering from DKA. It also aims to highlight the difference in management from
that of adult DKA.
How does DKA differ in children?

The occurrence of cerebral oedema is the stand out complication that makes this condition
different in children compared to the presentation in adults. Cerebral oedema is more
common in children under 5 years old, with its incidence decreasing markedly after
puberty.
Cerebral oedema occurs in up to 1% of all childhood DKA and accounts for about half
of the DKA-related mortality. It has a bi-modal appearance after DKA presentation; the
first peak is at 36 hours, the second at 915 hours.
The approach to the management of childhood DKA has evolved quite distinctly from
that of adults, particularly in respect of insulin and fluid administration. It may seem
uncomfortable for most physicians, but the replacement of fluid in children with DKA
(in the absence of significant shock) should occur over 4872 hours. This reflects the fact
that onset of DKA is a gradual process.
The aim of treatment is to correct the metabolic derangement over a similar period
to which it came on. The British Society of Paediatric Endocrinology and Diabetes
104
Chapter 11: The child with diabetic ketoacidosis
105
(BSPED) have a website with an interactive page that guides doctors through each
step of management.
Why does cerebral oedema occur?

The evidence for any one factor causing cerebral oedema in children is circumstantial.
Some of the mechanisms listed below have been considered as mechanisms for cerebral
oedema in DKA; however, the presence of cerebral oedema in these children before the
initiation of treatment effectively rules many out. It is probably worth considering the
clinical findings below as risk factors for cerebral oedema rather than causative
mechanisms.
Aggressive use of insulin

Insulin is necessary for sodium pumps to work at the bloodbrain barrier. In animal
models giving insulin exacerbates cerebral oedema.
Low PaCO2
Hypocapnoea at presentation suggests greater derangement of physiology and thus greater
propensity to further complications.
High serum urea nitrogen

Although this is associated with poorer outcomes, it may be just a marker of the underlying
derangement.
Treatment with sodium bicarbonate

Sodium bicarbonate administration has been associated with increased mortality in
DKA, but it is unclear whether the perceived need to use this simply makes it a marker
of severe metabolic derangement. Acutely raising the pH has the same deleterious effect
as hyperventilation on cerebral blood flow and haemoglobin dissociation; for this reason
bicarbonate is rarely used and only in cases where myocardial function appears to be
compromised.
Slow rate of rise of sodium

As the management of DKA progresses the serum sodium concentration should increase.
Failure to observe this may be due to giving hypotonic fluids or the release of stress
hormones (antidiuretic hormone is released in DKA). This is one reason why rehydration
should be gradual and comprise 0.9% saline.

Observations should be carried out as detailed in Table 11.1.
106
Table 11.1. Timing of observations in DKA.
Item
Hourly
Fluid balance
Heart rate (HR), blood pressure (BP), respiratory rate
Neurological observations (see text)
Capillary glucose
24 hourly
Electrolytes (Na, K, Cl, urea, Ca, Mg, PO4)
Blood gases and lactate
Blood and urine ketones
In the early phase, e.g. the first 24 h, the intervals above would be maintained. With resolution of the childs
ketoacidosis, items marked as * could be put back to 2 hourly, items marked could go 4 hourly. Note that raised
intracranial pressure (ICP) can be a delayed phenomenon (second peak at 915 h).
Contacting PICU
The British Society of Paediatric Endocrinology and Diabetes recommend discussing with
PICU any child who is younger than 2 years of age, or who demonstrates:
a pH of less than 7.1, along with marked hyperventilation
severe dehydration with shock
depressed sensorium with risk of hypoventilation or aspiration.
Although the child may not be transferred to PICU it is worth discussing these cases in
order to speed up any future involvement should the child develop neurological signs.
Lack of response to 30 ml/kg fluid boluses

This implies circulatory failure that may not be explained by DKA alone. There may be
coexisting pathology, such as sepsis, and the child may need inotropes, together with
invasive monitoring and central venous access.
Signs of raised ICP

All patients with DKA should be monitored using age-appropriate Glasgow coma scoring
(hourly during the initial phase). This should not be used as a sole guide of management
though. Other markers of raised ICP include:
symptoms such as irritability, drowsiness, headache, incontinence
specific neurological signs (cranial nerve palsies, abnormal posturing)
rising blood pressure and slowing heart rate.
Targeted treatments in the management of DKA

The management of DKA in children can be found described in prescriptive form on
the British Society of Paediatric Endocrinology and Diabetes (BSPED) website. This will
help guide management in a step-by-step fashion. The main points of management are
detailed below.
107
Fluid therapy
Slow rehydration over 48 to 72 hours
Historically, the need to maintain or increase serum osmolality was not as well appreciated
and rapid fluid replacement was the norm.
Rehydration is perhaps a misleading term, implying a water shortage, whereas in fact
significant whole body shortages of electrolytes such as sodium, potassium, chloride and
phosphate exist along with water.
Gradual rehydration has not been shown experimentally to be associated with decreased
incidence of cerebral oedema. Nonetheless, as part of the general approach to managing
childhood DKA it is advised that rehydration should take place over 48 hours (or 72 hours
in the more severe cases).
Treat shock
Use 10 ml/kg boluses of normal saline up to a maximum of 30 ml/kg. If you feel more
should be given perhaps there is underlying sepsis and consideration should be given to
better access and inotropes.
Any resuscitation fluid should be deducted from the calculated deficit (see below). This
is not true of the International Guidelines (see Further reading below.)
Steps in calculating fluid replacement

1. Calculate the percentage dehydration, using 8% as the maximum (see BSPED website).
Use the equation below to calculate fluid deficit:
10 % dehydration body weight kg total fluid deficit in ml:
e:g: for a 10 kg child at 5% dehydration, this 500 ml:
2. Calculate maintenance fluid requirement (see BSPED website).
3. Add the total maintenance for 48 hours (or 72 hours depending on condition) to the
deficit.
4. Subtract the resuscitation fluid already given. This volume should then be replaced over
48 (or 72) hours.
The initial fluid of choice is normal saline (with potassium 40 mmol/l).
When to change fluid

When glucose levels do drop, avoid the temptation of decreasing the insulin rate below 0.05
units/kg/h. Insulin is the key therapeutic agent and is needed to clear ketones.
Glucose (5%) should be added to the IV fluids when the glucose drops to 14 mmol/l.
Pre-prepared fluids of 5% glucose in 0.9% saline should be available in hospitals with
paediatric units.
Insulin
Continuous insulin infusion
Insulin is started at a maximum of 0.1 units/kg/h (research indicates that it might not be
necessary to exceed 0.05 units/kg/h).
108
When the blood sugar comes down to 14 mmol/l then the lower rate of 0.05 units/kg/h
should be employed.
Insulin should not be stopped when the blood glucose normalizes. Instead, dextrose
should be added to the maintenance fluid.
Electrolytes and osmolality

Managing sodium and osmolality
Keeping the serum sodium concentration elevated is considered to be neuroprotective, as is
maintaining serum osmolality.
There are important considerations in the interpretation of the laboratory level of
the measured serum sodium in DKA. The measured sodium level is depressed by the high
level of glucose that draws water into the extracellular space. The patients true sodium
concentration will be higher than that seen on a blood gas or lab sample when the glucose
level is corrected and returns to normal. The corrected sodium level can be calculated as
below:
corrected Na measured Na 2plasma glucose 5:6=5:6 mmol=l
effective osmolality (eOsm) can be calculated as:
eOsm 2 Na K plasma glucose urea mOsm=kg
Potassium must be supplemented from the outset at 40 mmol/l in all non-bolus IV fluids
(maintenance and replacement) as treatment may precipitate hypokalaemia.
Although phosphate levels do drop (as a consequence of renal and metabolic depletion)
replacement has not been shown to benefit children, and doing so may cause inadvertent
hypocalcaemia.
Intubation
The advantage of not intubating a child with DKA is that the neurology can be assessed at
any point. However, there are occasions when intubation must be performed. They include:
need for neuroprotection obvious signs of raised ICP (see above and Chapter 19)
a child who is tiring of the massive ventilatory effort
protection of the airway in a drowsy child.
Treatment of raised ICP in DKA

A careful balance in DKA has to be struck between keeping the patient awake for careful
neurological monitoring and sedating and ventilating them for neuroprotection and airway
safety. Once a decision has been made to ventilate a child with raised ICP, management
follows similar principles to that of head injuries (see Chapter 19).
Intervention for acutely raised ICP

Once all of the simple manoeuvres for minimizing ICP detailed in Chapter 19 are employed
osmotic therapy can be used in DKA:
mannitol 0.51.0 g/kg over 20 min
3% hypertonic saline 5 ml/kg over 10 min.
109
If ICP becomes a major problem then fluids should be restricted even further by halving the
maintenance rate and extending the correction of the deficit to 72 hours. This means that
inotropes or vasopressors may become necessary to maintain the cerebral perfusion
pressure.
Radiological imaging
If a child begins to show signs of neurological deterioration, then a CT scan of the head
should be arranged as an emergency. The aim of the scan is to detect any coexisting
pathology, which may be incidental or related to the hypercoagulable state, e.g. venous
sinus thrombosis.
Other treatments to consider during management

Heparin therapy (prophylactic)
The BSPED guidelines (see Further reading) recommend anticoagulation due to the dehydrated and hyperviscous state of the patients blood, especially if femoral lines are used.
Local guidelines should be used to guide systemic heparinization, e.g. > 20 units/kg/h, if the
risk of thrombosis is deemed to be significant.
Antibiotics if sepsis present or suspected

Take cultures of blood, urine and any other sample as indicated (including tracheal aspirate
if intubated). If meningitis is suspected, treat accordingly, deferring dural puncture until the
child has recovered from the DKA, when CSF PCR can be performed.
What do I need to do after intubation?

Neuroprotection
Once the child is intubated, assessing progress of the ICP becomes problematic. However,
regular neurological observations (see Table 11.1) should be undertaken, as rapid deterioration and coning can occur (see Chapter 19 for further management).
Hyperventilation
There is evidence that in DKA cerebral autoregulation is diminished and keeping the
PaCO2 at pre-intubation levels will cause least disturbance. This is a contentious subject
because hyperventilation will cause further cerebral vasoconstriction and ischaemia,
whereas hypoventilation can cause cerebral vasodilatation and a rise in intracranial
pressure.
A lung-protective strategy may make keeping the PaCO2 at pre-intubation levels
impossible. The result is that a compromise has to be struck between causing lung damage
and managing the ICP (depending on how severe each problem is at the time). The target
PaCO2 should be discussed with PICU before intubation.
Imaging
Unless already done a CT scan should be considered immediately after intubation, mainly
to exclude venous sinus thrombosis, infarction or haemorrhage.
110
Measuring blood ketone levels

This has become recommended in the last few years as a good marker of whether the
pathological ketogenic process is being switched off. In the absence of this assay, urinary
ketones should be monitored.
Preparing a child for transfer

The decision of whether a child should be retrieved by the retrieval service or transferred
by the local team should be taken in conjunction with the PICU retrieval team (see
Chapter 24).
The preparations for acute transfer should include everything detailed in Chapter 24,
with the additional considerations of:
frequent blood monitoring ideally an arterial line or CVC should be sited to aid with
regular blood sugar and electrolyte checks
because of the bi-modal presentation of cerebral oedema there is a significant risk of an
acute change in ICP, therefore hypertonic saline or mannitol should be available for the
transfer
10% glucose should be available as a rescue treatment should the blood glucose level
drop unexpectedly.
Documentation should be prepared before transfer, and should include:
a detailed history including:
any precipitating factors: stress, infection, poor compliance
investigations to date
previous treatment regimen if a known diabetic
a timeline of treatment including:
when the child presented
when insulin was started
exactly how much fluid has been given
whether it was incorporated into the calculated deficit
blood results, including timing of samples, in order to track the rate of change.
As with all other conditions diagnostic imaging should be available for transfer, either as
hard copies or via secure link.
Summary
Administration of fluids to children suffering from DKA can cause a lot of problems. The
risk of cerebral oedema is sufficiently high to warrant developing different management
protocols for children. Whereas early, aggressive therapy is the mainstay for most conditions this is one of the few disease processes that benefits from more controlled patient
resuscitation.
Golden rules
Discuss early with PICU
Avoid bicarbonate administration for acidosis

When blood sugars drop, add in glucose, do not stop the insulin infusion
ICP can increase suddenly, even hours after treatment starts
Avoid hypotonic fluids
Be aware that intubation will mask signs of raised ICP
Be careful to avoid missing sepsis as a cause of shock
Further reading
BSPED. British Society of Paediatric
Endocrinology & Diabetes DKA Guideline
2009. http://www.bsped.org.uk/professional/
guidelines/docs/DKAGuideline.pdf (accessed
17 June 2011).
Levin DL. Cerebral edema in diabetic

ketoacidosis. Pediatr Crit Care Med
2008;9:320329.
Wolfsdorf J, Craig ME, Daneman D, et al.
Diabetic ketoacidosis. Pediatr Diabetes
2007;8:2843.
111
Section 2
Chapter
12
Clinical Conditions
Inherited metabolic conditions

Saikat Santra
Introduction
Inherited metabolic disorders (IMD) are a diverse group of conditions caused by inherited
deficiencies in one or more enzymes of key metabolic pathways in the body. Some
conditions present very early in the newborn period whilst others lead to more slowly
progressive disease. While some conditions have no effective treatment, others are manageable with appropriate medical and dietary interventions.
Many IMD conditions put the child at risk of serious and rapid metabolic decompensation, particularly when the body is already stressed, for example during infections. It is these
conditions that will be concentrated upon in this chapter.
The acute management of most metabolic conditions is very similar and a systematic
approach can be followed for most children in this situation.
How the condition affects children

At their simplest, inborn errors of metabolism can be thought of as a problem in converting
one chemical (the substrate) into another chemical (the product). Disease can occur because
of the accumulation of toxic substrates or metabolites, deficiency in the product of a
reaction, or both (Figure 12.1).
Figure 12.1. Potential ways in
which metabolic pathway errors
can lead to disease.
112
Chapter 12: Inherited metabolic conditions
113
Examples of accumulating toxic metabolites include:

ammonia
amino acids such as leucine
organic acids
large molecules such as mucopolysaccharides.
Examples of product deficiencies include:
hypoglycaemia
energy deficiency.
The effects of IMDs are usually multisystem and often affect metabolically active organs.
Features include encephalopathy, liver dysfunction/failure and cardiomyopathy. Mildly
affected children, as well as those who are more severely affected but have survived the
neonatal period, typically present to the ED during periods of stress to the body. These
can include infections, pregnancy, prolonged fasting or high protein loads (for example
GI bleeds). Sometimes the trigger is not always apparent. Gastroenteritis is a frequent
trigger in childhood, as it causes increased energy demand and reduced nutrient
absorption.
Specific inherited metabolic conditions

It is beyond the scope of this chapter to cover the presentations of all inborn errors of
metabolism and at all ages. However, the commoner conditions in European populations
that present acutely are detailed below.
Amino-acid-based conditions
Urea cycle disorders
The urea cycle is a pathway that removes excess nitrogen from amino acids by converting
ammonia into urea, which is water-soluble and easily excreted.
The hallmark of all these conditions is an elevated ammonia level although the degree to
which this occurs is variable between conditions and even between patients with the same
condition. Severely affected infants typically present in the first week of life with severe
hyperammonaemia and encephalopathy with plasma ammonia levels sometimes exceeding
1000 mol/l.
Branched-chain organic acidaemias

Other steps in the breakdown of the branched-chain amino acids lead to one of the organic
acidaemias namely:
propionic acidaemia (PA)
methylmalonic acidaemia (MMA)
isovaleric acidaemia (IVA).
114
Severely affected infants typically present with encephalopathy and a severe metabolic
acidosis from the accumulation of these organic acid intermediates. Hyperammonaemia
is also seen as the organic acids cause a secondary inhibition of the urea cycle. Acute
pancreatitis is a well-recognized complication of all three conditions and should be
suspected if the child is particularly shocked or has severe abdominal pain. Metabolic
strokes, especially affecting the basal ganglia, are a feared complication of
decompensation.
Maple syrup urine disease (MSUD)

This condition is caused by an inherited deficiency of the enzyme complex which catalyses
the second step of the breakdown of the three branched-chain amino acids: leucine,
isoleucine and valine. Severely affected infants present with a progressive encephalopathy
caused by the accumulation of leucine within the first 2 weeks of life. MSUD may lead to
cerebral oedema (with a bulging fontanelle), seizures, cycling movements of the lower limbs
and apnoeas.
There is no hyperammonaemia and the accumulation of ketoacids rarely causes metabolic acidosis but does cause ketonuria, which should raise suspicion of an inborn error of
metabolism in a neonate. It is these ketoacids which also lend a characteristic odour of
burnt sugar to the urine.
Enteral feeding (e.g. by nasogastric tube) with the appropriate amino acid mixture is
preferable even in an emergency in children with MSUD. The amino acid mixture, in
addition to carbohydrate, will enable the body to incorporate the excess branched-chain
amino acids into protein (reducing the leucine levels in the brain).
Glutaric aciduria type 1

This condition, which is also an organic aciduria, occurs due to deficiency of one of the
enzymes in the breakdown pathway of the amino acids lysine and tryptophan. These
children are at risk of basal ganglia strokes during times of decompensation, particularly
during the first 6 years of life. As with MSUD it is advisable to continue a lysine- and
tryptophan-free amino acid mixture enterally to try and reduce the levels of these amino
acids in the brain. An attempt should be made to use this even if intravenous fluids are
required additionally. Carnitine supplementation is also necessary.
Fatty acid oxidation (FAO) disorders

This group of disorders includes patients with enzyme deficiencies of the carnitine cycle and
of the fatty acid beta-oxidation pathway, such as:
very long-chain acylCoA dehydrogenase
VLCAD deficiency
long-chain hydroxyacylCoA dehydrogenase
LCHAD deficiency
medium-chain acylCoA dehydrogenase
MCAD deficiency
carnitine palmitoyl transferase
CPT1/CPT2 deficiencies
carnitine acylcarnitine translocase.
CACT deficiency
115
These enzyme defects mean that children cannot mobilize fat as a source of energy. As a
result they tend to present to hospital during times of catabolism, when the body is
dependent on fat as a major source of energy. The more severe defects can cause a limited
fasting tolerance, sometimes necessitating overnight tube feeding or cornstarch before
bedtime. However, individuals who are less severely affected may only decompensate
during times of stress.
The emergency treatment for all of these conditions is simply to administer sufficient
carbohydrate to prevent the body from needing to draw on fat stores. This is usually
provided as a glucose polymer drink/feed but if this is not tolerated then IV dextrose
should be given. In these conditions the aim of treatment must always be to intervene
before the blood glucose falls as hypoglycaemia is a late sign of decompensation.
Hyperammonaemia is also seen in some children with acute decompensation of their
FAO disorder.
Glycogen storage diseases

Children with these conditions are unable to release free glucose from glycogen (which
accumulates in an enlarged liver). As a result, they are prone to hypoglycaemia after
fasting. Depending on the condition this may occur as soon as 2 hours after feeding or
only during times of illness. The management of these conditions is to provide sufficient glucose either enterally or, if not tolerated, intravenously to prevent
hypoglycaemia.
Many of the intermediates in the glycogen pathway are metabolized to lactate. As a
result children with these conditions can present with serum lactate levels up to 20 mmol/l.
This will lead to a metabolic acidosis with its ensuing manifestations, e.g. tachypnoea.
Bicarbonate treatment is usually not necessary as the lactic acidosis usually resolves quickly
once glucose is being reliably delivered
Mitochondrial disorders
Children with defects in the components of the mitochondrial respiratory chain, or of
enzymes leading to this such as Krebs-cycle defects and glutaric aciduria type 2 (multiple
acylCoA dehydrogenase deficiency), present with energy failure (particularly affecting
the metabolically active organs), especially during times of stress such as infection. In
severe cases this can be fatal. In less severe cases the acute deterioration may
be reversible with supportive care; however, most children will not recover to their
previous level of functioning. Anaerobic stress manifests as lactic acidosis, which can be
severe and in some cases responds to sodium dichloroacetate. There is much controversy about the use of so-called mitochondrial cocktails of vitamins and co-factors
in these conditions.
116
Table 12.1. Common complications of IMD with the specific diseases that cause them.
System
Organ system
specific problems
Metabolic conditions in which they occur
Cardiovascular
Cardiomyopathy
Fatty acid oxidation disorders (especially long-chain)

Some lysosomal and glycogen storage disorders
(especially Pompes)
Organic acidaemias
Neurological
Encephalopathy
Seizures
Metabolic strokes
Organic acidaemias
Urea cycle disorder
Fatty acid oxidation disorder
Organic acidaemias
MSUD
Glutaric aciduria
Organic acidaemias
Mitochondrial disorders (Leigh syndrome)
Renal
Renal failure
Renal tubular disease
Organic acidaemia
Some lysosomal storage disorders (e.g. Fabry)
Glycogen storage disease
Tyrosinaemia type 1
Blood
Myelosuppression
Organic acidaemia
Glycogen storage disorder (type 1b)
Liver
Liver dysfunction
Galactosaemia
Tyrosinaemia (type 1)
Organic acidaemias
Fatty acid oxidation disorder (especially longer-chain)
Mitochondrial disorders (especially early-onset)
Hereditary fructose intolerance
Some lysosomal storage disorders (e.g. Wolmans,
NiemannPick disease)
Other
problems
Hyperammonaemia
Rhabdomyolysis
Urea cycle disorder

Organic acidaemias
Fatty acid oxidation disorders
Fatty acid oxidation disorders (especially long-chain,
e.g. VLCAD)
117
When to suspect a metabolic condition

Almost all inherited metabolic disorders are inherited in an autosomal recessive manner.
This means that both parents are usually asymptomatic carriers and therefore the suspicion
of a metabolic disease should be raised when there is:
parental consanguinity
a family history of similar disease (including sudden, unexpected death in infancy).
In the newborn period, findings that should raise the suspicion of a metabolic disorder include:
initial symptom-free period during pregnancy accumulation of small molecules will be
masked by placental removal
severe metabolic acidosis
hyperammonaemia in all children an ammonia above 200 mol/l requires investigation;
levels above 100 mol/l may still be significant outside the neonatal period
rapidly progressive encephalopathy, liver disease and/or cardiomyopathy without clear
alternative explanations
characteristic odours such as those in MSUD (burnt sugar) or isovaleric acidaemia
(sweaty feet).
The presentation of metabolic disease, particularly in neonates, can be nonspecific. It is
important to consider inborn errors of metabolism in the differential diagnosis of all sick
neonates, especially those being treated for suspected sepsis.
Targeted treatments for IMD

The emergency management of any sick metabolic patient can be considered under the
following headings:
ensure anabolism
remove toxic precursors
remove toxic metabolites
give supportive treatment to maintain homeostasis
reintroduce appropriate feeds.
118
Ensure anabolism
Decompensation usually occurs during periods of catabolism such as prolonged fasting
or infections. The neonatal period is also a highly catabolic period. The first, and in some
cases only, thing to do is to try and promote anabolism as much as possible. Giving
carbohydrates will:
provide calories for anabolism
prevent the body breaking down muscle protein for fuel
prevent the body breaking down fat for energy
provide free glucose.
Wherever possible this should be given enterally. Most children with IMDs will have
recipes for this provided by their metabolic dietician in an emergency regimen. They
should be encouraged to drink this regularly or be given it continuously through a
nasogastric tube or gastrostomy. Glucose polymer solutions can be considered clear fluids
from an anaesthetic perspective.
If oral solutions are not tolerated intravenous 10% dextrose should be given (lower
concentrations may not provide a sufficient glucose intake to prevent catabolism).
A glucose intake of 58 mg/kg/min should be sufficient in infants to suppress catabolism.
Appropriate electrolyte supplementation should be given if intravenous fluids are to be used
for some time.
If hyperglycaemia develops, add in a background insulin infusion rather than lower the
glucose delivery. This will promote anabolism further. Rates of 0.05 unit/kg/h are usually
sufficient.
An exception to this rule would be for patients with known disorders of mitochondrial
function with lactic acidosis where excess glucose delivery could worsen lactic acidosis. In
such conditions, 5% dextrose concentrations would be acceptable.
Remove toxic precursors

If hyperammonaemia is present, it is advisable in the short term to stop the childs natural
protein-containing feeds during a decompensation in order to minimize ammonia production. The use of the emergency regimen (provided for each child) and intravenous fluids,
if needed, should address this. In MSUD and glutaric aciduria type 1 it is important that
the appropriate amino acid mixture is continued even if protein feeds are discontinued
(see above).
Remove toxic metabolites

Drugs highly specific to children with IMD can be used to detoxify the body of specific
metabolites such as ammonia. It is best to involve your local IMD team before starting
treatment (unless a child has a pre-prepared management plan).
Hyperammonaemia causes stimulation of the respiratory centre, leading to tachypnoea
and a respiratory alkalosis. At high levels, cerebral oedema is inevitable and urgent
management to reduce the ammonia level is needed in order to try to avoid irreversible
neurological damage. Levels greater than 150 mol/l in an older child and 200 mol/l in a
neonate should be treated.
119
The appropriate medication, if started in time, can sometimes reduce ammonia levels
quickly enough to prevent the need for more intensive treatment (see Chapter 36). In severe
cases (e.g. when ammonia levels are over 500 mol/l and/or are increasing despite medical
therapy) haemofiltration or haemodialysis will be used on PICU to reduce ammonia levels
quickly, in order to improve outcome.
Supportive treatment for homeostasis

During metabolic decompensation in many conditions, the pH can reduce dramatically
sometimes to less than 7.0. Remember that treating shock and promoting anabolism are
more important than correcting acidosis the parallels with DKA are useful to consider. In
glycogen storage disease, for example, providing dextrose is sufficient to stop lactate
production and the pH rapidly corrects with no other specific treatment.
Electrolyte imbalance should also be corrected, bearing in mind that many of the
specialist drugs come as sodium salts and therefore have significant sodium content. This
becomes more significant the younger the patient.
Reintroduce appropriate feeds

Children who receive no protein for a length of time can then become catabolic due to
protein deficiency and this can worsen their metabolic condition. It is advisable, therefore,
that some protein-containing feed is restarted within 72 hours at the latest. This will be
taken care of in a specialist centre, after retrieval.
What do once the child is stabilized

Ammonia should be monitored at least every 4 hours until the levels are falling and stable.
It is important to warn your clinical chemistry laboratory about this as ammonia levels
rise if samples are left standing for longer than 30 minutes. It is also useful at this point to
ensure that the complications listed above such as coagulopathy or rhabdomyolysis are
sought and acted on.
For children who do not have a known metabolic diagnosis, but in whom a metabolic
disorder is suspected, it is important that samples for specialist tests are taken at the first
opportunity. These will be advised by the regional IMD team on referral. The value of a first
urine sample cannot be stressed highly enough.
Additional metabolic conditions to consider

There are also some other conditions where acute metabolic decompensation is not
generally seen but attendance at the ED for other reasons can still occur.
Lysosomal storage disorders

These are a group of conditions where lysosomal enzymes are defective, leading to the
accumulation of large molecules such as mucopolysaccharides. These children often have a
number of structural problems affecting multiple organ systems. From an anaesthetic
viewpoint there are several things to be aware of.
120
Table 12.2. Management of IMD.
Phase of management
Assessment
Action
Initial phase
On presentation
of the child
Manage initial phase as

for all conditions (see
Chapter 4)
Give oxygen
Protect the airway
Gain IV access
IO if more than two IV attempts
Give fluid bolus
Repeat as necessary
After initial assessment

Once imminently lifethreatening risks have
been addressed
Ensure anabolism
Start IV 10% dextrose if oral rescue

regime not tolerated (unless known
mitochondrial disease)
Aim to give 58 mg/kg/min of
dextrose
Start insulin infusion if hyperglycaemic
0.05 units/kg/h
Not immediately lifesaving procedures
Remove toxic
precursors
Avoid giving amino acids (especially if

hyperammonaemia)
Except MSUD and glutaric aciduria
Contact IMD team for advice on specific
drugs
Manage the acidosis as in DKA, i.e. will
resolve with management
Look for complications such as
coagulopathy and rhabdomyolysis
Remove toxic
metabolites
Give supportive
treatment to maintain
homeostasis
Intubation may be very difficult due to a short neck, macroglossia, limited mouth
opening and accumulation of storage material in the upper airway. A smaller ET tube is
usually needed than would usually be estimated for the age of the child.
Hypoplasia of the odontoid peg puts some children at risk of atlantoaxial subluxation
especially in Morquio disease (MPS4). Storage material around the cervical spinal canal
can also cause compression. Excessive flexion or extension of the neck during intubation
can lead to permanent cervical cord damage.
Many of these children have enlarged livers and spleens, increasing the risk of aspiration
of gastric contents and making abdominal thrusts for resuscitation potentially dangerous.
Skeletal abnormalities, especially pectus carinatum and kyphoscoliosis, may make
resuscitation techniques difficult.
These children often have cardiomyopathy and/or valvular heart disease.
Intubation should ideally only be attempted by experienced paediatric anaesthetists with
expertise and equipment for a very difficult airway. It is also important that the airway of
these patients is secured before any sedating medication is given.
Neurometabolic disorders
A large number of inborn errors of metabolism affect the brain. These children may
attend the ED with seizures, dystonic spasms or encephalopathy. Although seizures in
121
these conditions may require treatment with specific drugs, SE can usually be treated
according to the standard APLS guidelines.
Summary
Although IMDs are complicated in their aetiology, the management of children with IMDs
is quite straightforward. The most important priority is to get the child back into an
anabolic state by providing substrate, i.e. rescue feeds or a crystalloid infusion that includes
dextrose. For all children, early contact with the local IMD team is essential.
Golden rules
IMD should be considered in any neonate who presents with sepsis
Give dextrose-containing fluids early
If IMD is suspected request a plasma ammonia level
IMDs are multisystem diseases
Seek expert advice early
Further reading
The British Inherited Metabolic Diseases Group has
recently drawn up guidelines for the emergency
management of most metabolic conditions, and
advice for undiagnosed conditions. These
guidelines are freely available to download
from their website: www.bimdg.org.uk.
Hoffman GF, Zschocke J, Nyhan WL (eds.).

Inherited Metabolic Diseases: A Clinical
Approach. Springer, 2009.
Saudubray J-M, van der Berghe G, Walter JH
(eds.). Inborn Metabolic Diseases:
Diagnosis and Treatment, 5th edn. Springer,
2012.
Section 2
Chapter
13
Clinical Conditions
Paediatric toxicology
Marius Holmes
Introduction
Overdose in children is not uncommon. This is reflected in the fact that over a quarter of
the telephone enquiries to TOXBASE in the year 2010/11 concerned children younger than
5 years of age. In many cases ingestion is proven to be benign and a period of observation is
all that is required together with reassurance and home safety advice to parents. However, a
history of ingestion should always be taken seriously.
This chapter will discuss the general management of children who have ingested
poisons. It will also discuss specific drugs and toxins.
Important factors in paediatric overdose

History
Most paediatric poisonings are accidental, due to childrens curiosity and exploration of
their environment. It is important to take the ingestion of more unusual substances
seriously, find out their ingredients (if a commercial product) and reference the product
on databases such as TOXBASE.
There are some factors that need to be explored in detail when taking a history from a
child who may have ingested a poison.
Toxin
Often in paediatric poisonings the agents are known. A clear history of where the child was
and what other medications are available must also be taken. Remember in intentional
overdoses to establish what other prescription drugs and over-the-counter medications
there are in the home.
Dose ingested
Attempt where possible to estimate the maximum potential dose that could have
been ingested. For instance, the parents may know that only one tablet is missing from a
new blister pack. This is less easy with liquids or plant matter as the child is often found
crying with the substance over their clothes and around their mouth. Always estimate
the greatest amount possible, i.e. the dose per kg if a complete pack or bottle had been
ingested.
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Chapter 13: Paediatric toxicology
123
Time of ingestion
The parents should know an approximate time when the ingestion could have taken place.
It may have been witnessed or there could have been a time window when the child was not
supervised. The timing of ingestion is important when calculating periods of observation,
timing of drug levels and even the time to maximum toxicity.
If children are playing and one has taken something, remember to think that they might
have shared it.
Presentation
The presentation of a poisoned child forms a continuum from a well child who needs no
input through to an obtunded patient who is critically ill. Initial management is no different
from any other seriously ill child (see Chapter 4) and is for the most part not dependent on
the ingested agent in question.
Symptoms and signs

As mentioned above the history may not be that clear and you may be presented with an
unconscious child. There are too many individual agents to mention within the scope of
this chapter, but there are several common toxidromes (toxic syndromes) that may help you
to think of different pharmaceutical groups. See Table 13.1.
Table 13.1. Common toxidromes.
Sedatives,
e.g. diazepam
Anti-cholinergics, e.g.
tricyclic antidepressants
Cholinergics, e.g.
organophosphates
Opiates
Sympathomimetics,
e.g. cocaine
Serotonergics,
e.g. SSRI
Consciousness
Reduced
Delirium
Reduced
Reduced
Excitation
Agitation
Pulse
Fast
Fast or slow
Slow
Fast
Fast
Blood
pressure
Reduced
Increased
Increased
Reduced
Increased or
decreased
Increased
Respiratory
rate
Reduced
Increased
Reduced
Increased
Increased
Temperature
Reduced
Increased
Reduced
Increased
Skin
Dry
Sweating
Sweating
Sweating
Pupils
Large
Small
Small
Large
Large
Other features
Ataxia
Urinary retention, broad

QRS, dry mouth, clonus,
delayed gastric emptying
Voiding, defaecation,
vomiting, lacrimation,
drooling
Response to
naloxone
(short lived)
Seizures, tremor and

hyperreflexia
Clonus,
hyperreflexia
125
Specific conditions
Some common substances that cause poisoning are listed below.
Carbon monoxide
Carbon monoxide has an affinity for haemoglobin 250 times greater than oxygen. Once
bound it forms carboxyhaemoglobin, which has a half-life of 4 hours (reduced to 90
minutes in 100% oxygen). Carboxyhaemoglobin cannot transport oxygen to the body.
This condition presents with nonspecific signs and symptoms. When entire families are
subject to this poisoning, children are often the first to show signs. These range from
malaise through to coma. Carbon monoxide poisoning should always be suspected in
nonspecific coma, but especially so during the winter months and when there are reports
of flu-like symptoms in the family. The simplest way to detect carbon monoxide poisoning
is to perform a blood gas test for carboxyhaemoglobin.
Monitoring of oxygen delivery in carbon monoxide poisoning is difficult for the
following reasons:
the absorption pattern of red light by carboxyhaemoglobin will give falsely high
saturation readings on a co-oximetry probe
the PaO2 on a blood gas will only tell you the amount of oxygen dissolved in plasma. It
will not tell you how much is being transported by haemoglobin, i.e. a high PaO2 does
not mean good oxygen delivery.
Therefore, other indicators of oxygenation such as lactate and cardiovascular stability
will guide the need for intubation. Carbon monoxide levels greater than 25% should also
prompt consideration of intubation.
If carbon monoxide poisoning is present remember to assess the rest of the family and
ensure the house boiler or fireplace is serviced. Advice regarding carbon monoxide monitors
should be given.
Paracetamol
Toxic overdoses of paracetamol are usually associated with ingestion of greater than
150 mg/kg. There are very few signs or symptoms in early overdose. Toxicity starts from
about 8 hours. If presentation is after 8 hours and a potentially toxic dose has been ingested
treatment with N-acetylcysteine should be started pending drug level results. Activated
charcoal is only effective if presentation is within 1 hour of overdose.
N-Acetylcysteine treatment takes place over 21 hours and consists of three separate
infusions with different concentrations over different times. It often causes mild symptoms
related to histamine release such as an urticarial rash. Rarely, it can cause anaphylactoid
reactions with airway swelling, bronchospasm and hypotension.
Staggered overdoses are complex and although levels are useful to show that some
paracetamol has been taken any significant overdose should be treated. If the presentation is
delayed there may be a need for early intensive care and specialist hepatology input.
Although many liver specialists would advocate monitoring coagulation as a marker for
severity of liver injury, blood products should be made available for children. They may
need to be given if the child starts haemorrhaging, or if procedures such as central line
insertion need to be performed. There should be a low threshold for intubation in
encephalopathic children who require transfer to a tertiary centre.
126
Iron
Iron supplements are readily available over the counter. The tablets are often an inviting
red colour and can be sugar-coated. Ingestions of 20 mg/kg of elemental iron can be
potentially toxic. Symptoms occurring within the first 6 hours are mostly gastrointestinal,
ranging from nausea to melaena. In large overdoses (>150 mg/kg elemental iron) there
may be:
coma
life-threatening gastrointestinal haemorrhage
cardiovascular collapse
pulmonary oedema
death.
The iron preparation taken should always be worked out as an elemental dose and then
worked out per kilogram body weight of the patient. An abdominal X-ray should be
considered to estimate the number of tablets consumed.
Iron levels should be taken 4 hours after ingestion and levels checked on TOXBASE to
aid further management, including the possible use of desferoxamine, an iron-chelating
agent, as the body has no way of excreting iron. Desferoxamine itself is not without risk as it
can cause pulmonary fibrosis and acute respiratory distress syndrome. Activated charcoal
has no role in iron overdose.
Consideration should be given to haemodialysis and whole bowel irrigation in severe
cases and endoscopy may be considered to remove a tablet concretion from the stomach.
Opiates
Management of acute ingestion of opiates includes airway support, ventilation and
treatment with naloxone. Methadone is often dispensed as a sweet green liquid, which is
quite attractive to young children, so it is worth asking parents about what drugs are
available in the house. It has a long half-life and will require a naloxone infusion to manage
it effectively. If the ingestion has led to a respiratory arrest then basic management should
be as detailed in Chapter 4.
Oral hypoglycaemics
Oral hypoglycaemics are a common prescription medication found in households. Children
are more prone to having hypoglycaemic episodes than adults. Many agents are long-acting
so admission with regular blood sugar measurement and observation is needed.
Tricyclic antidepressants (TCA)

Doses as little as 15 mg/kg should be treated as life-threatening in children. To put that into
perspective, five small 50 mg tablets could kill a 4-year-old.
Early intravenous access and blood gas measurement is important. The child should
have ECG monitoring and the QRS and QT intervals should be formally measured. Any
prolongation of these should be treated with intravenous sodium bicarbonate.
Intravenous alkalinization reduces the active unbound TCA and so can dramatically
narrow the QRS complex, terminate arrhythmias and correct hypotension. Any seizures
should be treated in the usual way. However, phenytoin should be avoided where possible in
127
TCA overdose, due to its possible effects on cardiac conduction. Agitation may be managed
with small aliquots of benzodiazepine. Activated charcoal may be effective if given within
1 hour of ingestion.
Caustic ingestions
With ingestion of caustic chemicals like acids and alkalis the most obvious initial symptoms
are burns to the oropharynx. Symptoms from this include pain, vomiting and drooling.
Because the substances are so unpleasant, usually not much is swallowed. When they are
swallowed, haematemesis and oesophagitis may follow, progressing to oesophageal
perforation.
Swelling around the epiglottis may cause airway obstruction. As with thermal injuries,
early elective intubation may be required in anticipation of worsening oedema. In all of
these children, a difficult intubation should be anticipated (see Chapter 16 for difficult
airways).
If the chemical is in powder form ensure that it is brushed off the patient as much as
possible, as water may activate the powder, causing extensive injuries. No gastric aspiration
or chemical neutralization should be attempted as both of these can worsen the situation.
Initial management
Initial management involves stabilization as detailed in Chapter 4 (see Table 13.2 also). The
targeted treatment for overdoses should then be guided by databases such as TOXBASE and
the patients clinical condition.
Decision to ventilate
The decision to ventilate is unlikely to be driven by respiratory failure in these children.
Instead it will be taken depending on:
need for airway protection, i.e. vomiting or coma
need for invasive intervention, e.g. central line insertion in small children
encephalopathy
carbon monoxide levels (especially if greater than 25%).
If the child seems to be in respiratory distress it is worth performing a blood gas as the
respiratory rate may reflect:
underlying metabolic acidosis
direct stimulation by ammonia or salicylates
aspiration
pulmonary oedema.
Cardiovascular support
Cardiovascular support follows the same principles as in Chapter 4. Fluid boluses, followed by
inotropes, if needed, form the mainstay of treatment. The only differences to consider are:
some drugs lead to arrhythmias, which should be corrected (e.g. TCA)
some drugs have direct myocardial depressant effects which will require inotropes
some children will have taken antihypertensive drugs which need vasopressors.
128
Investigations and other supportive measures

When gaining intravenous access blood should be taken to check:
renal profile
full blood count
clotting screen
specific drug/toxin levels as history/poison dictates
glucose
blood gas (methaemoglobin, carboxyhaemoglobin, anion gap and electrolytes)
the possibility of a cross-match.
Calculate the time when blood needs to be taken for plasma toxin levels, as often this is
separate from the initial time of intravenous access. Others things to consider are investigations specific to the overdose. This may include an electrocardiogram in TCA ingestion,
or an abdominal radiograph in metal overdose, e.g. iron.
Passage of a NG tube will aid in gastric decompression, but will also allow the administration of charcoal if needed or it can be used for whole bowel irrigation (specialist advice
only). It is, however, debatable whether an NG tube should be passed at all in caustic
ingestion. With the situation under control ensure the child is kept warm.
Child protection
Once initial priorities have been addressed it is always important to think of the safety of the
child in the future and also the safety of other children/vulnerable adults in the same home.
Depending on the circumstances a home visit from a health visitor may need to be
organized to ensure that there are no underlying child protection needs or that this event
is not a reflection of lack of appropriate supervision. The involvement of social services and
the police may be required urgently. Any child who has taken an intentional overdose
should have a psychiatric assessment at an appropriate time prior to discharge.

As with other chapters, preparation for transfer will need detailed, written, documentation
of the history, clinical findings and lab results. Timing of ingestions and therapeutic
interventions should also be documented.
129
Table 13.2. Initial management of a poisoned child.
Phase of
Assessment
management
Action
On
presentation
of the child
Airway control
After initial
assessment
Assess ABC
Airway patency
Oxygenation and ventilatory
effort/volume
Cardiovascular status
GCS
Blood sugar
Assisted ventilation with oxygen

IV versus IO access
Give fluid bolus as required
History of event, potential agent and dose Reference toxin any antidote or
suggested therapy
Reassess response to initial treatment
Reassess need to intubate
Ensure no decline in respiratory
function
Concern over high aspiration risk
Need for bowel irrigation
Site NG tube and aspirate prior to

intubation (unless caustic ingestion)
When 40 ml/kg of fluid has been given
prepare inotropes. Consider inotropes
after 60 ml/kg of fluid
Start any antidote or drug treatment

Consider need for more fluid resuscitation needed
and need for inotropic support
Are there child protection concerns?
ECG and careful blood gas interpretation Consider other children in the home
Further
stabilization
Not
immediately
lifesaving
procedures
Close continued monitoring as required Resuscitation room monitoring

Definitive plan
Progression to intubation
Consider invasive monitoring and
Level of continued care needed
central access
on ward
Need for specialist unit transfer
Summary
Most overdoses in children are accidental. It is important to be forthright and direct when
asking about drug availability within the house in order to get a good picture of what the
child may have ingested. Expert advice is advisable in most cases, to ensure the appropriate
management is undertaken.
Golden rules
Find out what other prescribed medicines are in the home
Always check toxins on TOXBASE (some common household substances are surprisingly toxic)
Check the blood sugar
The toxicity is worked out per kilogram weight the child needs to be weighed
Check all the blood gas results, including anion gap, methaemoglobinaemia and carbon
monoxide level
Think of child protection/supervision issues
Further reading
http://www.hpa.org.uk/webc/HPAwebFile/
HPAweb_C/1317130944236.
http://www.toxbase.org/.
Section 2
Chapter
14
Clinical Conditions
The child with anaphylaxis

Nick Sargant
Introduction
Anaphylaxis is defined as a severe, potentially fatal reaction of rapid onset that occurs after
contact with an allergic trigger. It is the most serious manifestation of a continuum of
immunoglobulin E (IgE) mediated allergic disease (type 1 or immediate hypersensitivity).
Anaphylaxis results from degranulation of mast cells and the release of inflammatory
mediators in response to an allergic trigger. Any allergic reaction with the capacity to be
life-threatening should be treated as anaphylaxis, including serious localized reactions such
as angioedema obstructing the upper airway.
The frequency of admissions for anaphylaxis has risen in recent years. In the UK there
are 13 deaths per million people (adults and children) annually. In children, anaphylaxis
appears to be more common in males until the age of 15, after which females are more
commonly affected. Children under 1 year are the most likely group to be admitted.
How anaphylaxis differs in children

Presentation
In up to 20% of cases a trigger allergen may be unknown or unclear. The first signs of an
anaphylactic reaction are often cutaneous. However, up to 18% of childhood cases show no
dermatological features.
Food protiens are the most common cause of anaphylaxis in children. The mode of
delivery of the allergen means that these are more likely to present with respiratory compromise (intravenous drugs or insect bites are more likely to cause cardiovascular problems).
Because of the incidence of food allergy and difficulty in isolating a trigger, anaphylaxis
should be considered in any child presenting with sudden onset of asthma following exposure
to a possible allergen.
Signs to look out for in anaphylaxis are detailed in Table 14.1.
Speed of onset
Reactions to IV drugs and insect stings tend to have a very rapid onset whereas food
anaphylaxis can take hours, especially if the allergenic part of the food is coated in another
foodstuff and only exposed to the immune system during digestion.
130
Chapter 14: The child with anaphylaxis
131
Hypotension tends to occur later in children than in adults. Therefore any child with
tachycardia, respiratory distress, stridor, pallor, drowsiness or uncharacteristic quietness
should be taken very seriously.
Life-threatening reaction
Risk factors for severe or life-threatening reactions are thought to include:
history of asthma
previous or increasingly severe allergic reactions
concurrent treatment with -blockers
allergens including peanut, tree nuts, fish and shell fish.
Table 14.1. Clinical manifestations of anaphylaxis.
System
Mechanism
Effect
Upper
Angioedema of the larynx and upper airways
respiratory tract
Hoarseness
Stridor
Cough
Dyspnoea
Obstruction
Respiratory arrest
Lower
Bronchospasm
respiratory tract Mucosal oedema
Wheeze
Chest tightness
Reduced peak
expiratory flow (PEF)
Tachypnoea
Dyspnoea
Hypoxia
Respiratory arrest
Cardiovascular
Tachycardia
Hypotension
Arrhythmias
Cardiac arrest
Shock results from widespread vasodilatation and loss

of intravascular volume due to capillary leak
Possible direct myocardial suppression by allergic
mediators
Gastrointestinal Mucosal oedema

tract
Nausea
Vomiting
Abdominal cramps
Diarrhoea
Incontinence
Skin
Histamine and other inflammatory mediators cause

extravasation of fluid into the dermis
Urticaria
Flushing
Erythema
Pruritus
Brain
Usually as a consequence of cardiovascular and

respiratory compromise although neurological
symptoms have been reported as presenting
features infrequently
Dizziness
Hypotonia
Syncope
Seizures
132
Management
General principles
The main management priorities after this are early recognition, removal of the allergenic
trigger (where possible) and immediate administration of intramuscular (IM) adrenaline.
Studies have demonstrated increased morbidity and mortality with delay in IM adrenaline
administration. In otherwise healthy children it is better to administer IM adrenaline
(10 g/kg) than risk clinical deterioration by holding off treatment. The adrenaline will
help with respiratory and cardiovascular problems. It is also thought to stabilize mast cell
membranes in order to minimize histamine release.
Intramuscular administration of adrenaline is the preferred route of delivery. Intravenous or intraosseous administration should be reserved for children with life-threatening
features to whom IM adrenaline has been given multiple times or for those in cardiac arrest.
Table 14.2. Initial management of anaphylaxis.
Immediate actions
Rapid assessment
Oxygen
IM adrenaline without delay
Remove allergen if possible
Place patient in a recumbent position and elevate lower limbs if evidence of cardiovascular
compromise
Sit upright if respiratory compromise and cardiovascularly stable
Airway
Partial obstruction:
Repeat IM adrenaline if no response to initial
dose
Nebulized adrenaline
Repeat IM and nebulized adrenaline every 10
minutes as required
Hydrocortisone
Complete obstruction:
Intubation
Surgical airway
Breathing
Wheeze:
dose
Nebulized salbutamol
Hydrocortisone
Consider IV salbutamol or IV aminophylline
Apnoea:
Bagmask ventilation
ET tube
dose of adrenaline
Hydrocortisone
Circulation
Shock:
dose
Crystalloid bolus
IV adrenaline infusion
No pulse:
Advanced life support
Reassess ABC
133
Table 14.3. Drugs used in anaphylaxis.
Drugs in anaphylaxis
<6 months
6 months6
years
IM adrenaline (in-hospital
doses)
10 g/kg
0.1 ml/kg of 1:10000 (infants or young children) OR 0.01 ml/kg of
1:1000 (older children maximum 0.5 ml 1:1000)
Crystalloid (e.g. 0.9% saline)
20 ml/kg IV bolus (can be repeated as required)
400 g/kg
0.4 ml/kg of 1:1000 up to a maximum of 5 ml (dilute to 5 ml with
0.9% saline if necessary)
Hydrocortisone (IV or IM)
25 mg
IV chlorphenamine
(weight 4)
mg
Nebulized salbutamol
2.5 mg
IV adrenaline infusion
Only to be used in a life-threatening anaphylaxis resistant

to IM adrenaline or where multiple IM doses have been
required
Commence at 0.1 g/kg/min and titrate to effect
Vasopressors
Potent vasopressors such as noradrenaline, vasopressin and

metaraminol can be used in cases of anaphylaxis where there is
hypotension refractory to an IV adrenaline infusion and fluid
resuscitation. This should only be done after discussion with the
regional paediatric intensive care unit (PICU) or paediatric
retrieval team
50 mg
2.5 mg
612
years
>12
years
100 mg
200 mg
5 mg
10 mg
5 mg
While waiting for the adrenaline to work, basic management as in Chapter 4 should be
initiated.
Steroids are given at the start of anaphylaxis management. However, their main value is
in minimizing the chance of a biphasic reaction. The mainstay of treatment remains
supportive therapy along with intramuscular adrenaline.
Intubation and initiating intensive care

Intubation
Indications for intubation include:
airway obstruction
severe bronchospasm resulting in hypoxia or apnoea
IV adrenaline infusion requirement (anaphylaxis resistant to IM doses)
reducing consciousness level
cardiac arrest.
Anaphylaxis is one of the indications for using cuffed ET tubes in children, especially if
bronchospasm is a feature, as high ventilation pressures may be required.
Care needs to be taken at induction to use drugs that do not worsen the cardiovascular
state, or cause further histamine release (e.g. atracurium or morphine). Anaesthetic agents
134
with less propensity for histamine release include vecuronium and fentanyl. If an IV
induction is planned, ketamine would be the agent of choice.
In cases where there is upper airway obstruction an experienced ENT surgeon should
also be in attendance and an inhalational induction considered. Inhalational induction
allows for the airway to be assessed at laryngoscopy while spontaneous breathing is
maintained (see Chapter 16). If upper airway swelling is so severe that conventional
intubation is impossible, then it will be necessary to perform tracheostomy or
cricothyroidotomy.
Ventilation
Relative hypovolaemia is a problem in anaphylaxis. It may be worsened after intubation in
children with bronchospasm, as positive-pressure ventilation will result in a fall in venous
return. Air trapping from overly aggressive, rapid ventilation will also have the same effect.
A relatively low respiratory rate should therefore be set on the ventilator and permissive
hypercapnoea practised (as in asthma see Chapter 8).
Cardiovascular
As with all cases fluid should be titrated to clinical need. Any child requiring an adrenaline
infusion and intubation for anaphylaxis will also require central venous access and an
arterial line for invasive blood-pressure monitoring. If this is difficult then an IO needle can
be used for adrenaline infusions.
Acute investigations
Anaphylaxis is a clinical diagnosis and therefore no specific confirmatory investigations are
required. Arterial blood gases and CXR should be performed prior to transfer in order to
guide ventilation, check ET tube position and rule out a pneumothorax.
Serum tryptase
Markers of anaphylaxis can be measured. Unfortunately, tryptase can be an unreliable
marker as it does not reliably increase in all episodes of anaphylaxis. This is particularly true
of food-related anaphylaxis, the most frequent type in children. The 2011 NICE guideline
on anaphylaxis mentions consideration of blood sampling for mast cell tryptase as soon as
possible after initiation of treatment for anaphylaxis and a second sample 12 hours and no
later than 4 hours from the onset of symptoms in children younger than 16 years of age if
the cause is thought to be venom, drug-related or idiopathic.
A rise in serum tryptase levels (over 10 ng/ml) within 15 hours after suspected
anaphylaxis indicates that the reaction was probably anaphylaxis. A persistently elevated
serum tryptase after the cessation of an anaphylactic reaction, or in a well patient, suggests
the possibility of systemic mastocytosis.
Biphasic anaphylaxis
In children who have recovered from anaphylaxis the major concern is the possibility of a
biphasic reaction. Biphasic reactions are defined as a second reaction occurring 172 hours
after the first. The frequency of biphasic reactions remains unclear, with estimates ranging
from 1% to 23% of anaphylaxis cases.
135
Typically, biphasic reactions occur at 8 to 10 hours after the resolution of the acute
phase and for this reason children should be monitored in hospital. The 2011 NICE
guidelines on anaphylaxis recommend admitting children under 16 years who have
required emergency treatment to hospital under the care of a paediatric team.
Admission to hospital should be arranged for any child who has had a particularly
severe reaction and any of the following risk factors:
severe initial phase
delayed or suboptimal doses of adrenaline
laryngeal oedema
hypotension during the initial phase
delayed symptomatic onset after allergen exposure
previous history of biphasic anaphylaxis.
Follow-up
All children with anaphylaxis require follow-up by an allergy specialist even where there is
an obvious cause. In a previously undiagnosed child, the onus to refer to a specialist clinic
lies with the admitting physician. Allergy specialists will then consider the need for further
investigations such as skin prick tests or blood tests for specific IgE (RAST) both to the
likely trigger and to other commonly associated allergens.
The children and families will be given advice on avoidance (often in conjunction with a
dietician) and provided with an anaphylaxis management plan. Where necessary the child
and family will be supplied with an adrenaline auto-injector such as the EpiPen and trained
in its use.
Children are then followed up in allergy clinic and monitored for signs that they have
developed tolerance to an allergen. At this point a supervised in-hospital food challenge
might take place.
Summary
The principles of managing a child with anaphylaxis are similar to those in adults. The
follow-up of a child with anaphylaxis is essential in order to identify an allergen where
possible. Avoidance of triggers and ensuring optimal asthma management, and carrying an
adrenaline auto-injector form the mainstay of ongoing management.
Golden rules
Anaphylaxis can occur without a rash or hypotension
In life-threatening conditions IM adrenaline should be given if there is any doubt about
the cause
Biphasic reactions mean that children should be monitored closely and the 2011 NICE
guideline on anaphylaxis recommends admitting children under 16 who have required
emergency treatment to hospital
Follow-up in an allergy clinic must be arranged by the admitting physician
136
Further reading
Advanced Paediatric Life Support: The
Practical Approach, 5th edn. BMJ
Books, 2011.
Ben-Shoshan M, Clarke AE. Anaphylaxis: past,
present and future. Allergy 2011;66(1):114.
Braganza SC, et al. Paediatric emergency
department anaphylaxis: different patterns
from adults. Arch Dis Child 2006;91(2):
159163.
Du Toit G. Food-dependent exercise-induced
anaphylaxis in childhood. Pediatr Allergy
Immunol 2007;18(5):455463.
Working Group. Ann Allergy Asthma

Immunol 2006;97(5):596602.
National Institute for Health and Clinical
Excellence. Anaphylaxis: assessment to
confirm an anaphylactic episode and the
decision to refer after emergency treatment for
a suspected anaphylactic episode. (Clinical
guideline 134.) 2011.http://guidance.nice.org.
uk/CG134.
Pumphrey RS. Lessons for management of
anaphylaxis from a study of fatal
reactions. Clin Exp Allergy 2000;30
(8):11441150.
Kemp SF. The post-anaphylaxis dilemma: how

long is long enough to observe a patient after
resolution of symptoms? Curr Allergy
Asthma Rep 2008;8(1):4548.
Sampson HA, Muoz-Furlong A, Campbell RL,

et al. Second symposium on the definition
and management of anaphylaxis: summary
report Second National Institute of
Allergy and Infectious Disease/Food Allergy
and Anaphylaxis Network symposium.
J Allergy Clin Immunol 2006;117(2):
391397.
Lieberman P, Camargo CA, Bohlke K, et al.

Epidemiology of anaphylaxis: findings of the
American College of Allergy, Asthma and
Immunology Epidemiology of Anaphylaxis
Sampson HA, Mendelson L, Rosen JP. Fatal

and near-fatal anaphylactic reactions to food
in children and adolescents. N Engl J Med
1992;327(6):380384.
Gupta R, Sheik A, Strachan DP, Anderson HR.

Time trends in allergic disorders in the UK.
Thorax 2007;62(1):9196.
Section 2
Chapter
15
Clinical Conditions
The child with stridor

Ed Carver and Tim Day-Thompson
Introduction
Stridor may be a sign of emergency or non-emergency airway pathology. Its presence,
especially in the acute setting, may herald critical airway obstruction and respiratory
collapse, demanding timely and effective management.
This chapter focusses on assessment, management and preparation for transfer for
definitive care of the acutely stridulous infant or child.
Definition and features

Stridor is a vibratory, often high-pitched sound produced by abnormal airflow through a
partially obstructed airway at the level of the supraglottis, glottis, subglottis or proximal
trachea. It is a pathological sign rather than a diagnosis. It should be differentiated from
breath sounds caused by abnormal airflow elsewhere in the respiratory tree such as stertor
(snoring) or wheeze.
The relationship of stridor to phase of breathing varies with location and severity of the
underlying pathology:
biphasic stridor suggests critical narrowing or fixed obstruction at or below the glottis
inspiratory stridor suggests partial obstruction of the extrathoracic large airways as these
are usually narrowest during inspiration
expiratory stridor (or wheeze with more distal narrowing) suggests partial obstruction
of the intrathoracic upper airways as these are usually narrowest during expiration.
The volume and pitch of stridor correlate poorly with severity of airway obstruction.
How does stridor differ in children?

Important differences in paediatric anatomy and physiology increase susceptibility to
stridor and its effects.
Size
The paediatric airway is small. During laminar airflow resistance is inversely proportional
to the fourth power of its radius. Therefore significant reductions in airflow result from
modest reductions in diameter. When a child is distressed airflow may become turbulent
leading to further increases in flow resistance and increased work of breathing.
137
138
Immaturity
Immature cartilaginous structures support the airway and chest wall. These are relatively
compliant and prone to collapsing inwards with excessive negative airway pressure or
extrinsic compression. This compounds the inefficiency of laboured breathing and may
cause gas trapping.
Infants are more reliant on diaphragmatic breathing due to immaturity of the rib cage
and accessory muscles. Their diaphragm also has fewer fatigue-resistant muscle fibres.
Other factors
Respiratory reserve is further reduced by the high metabolic rate and small functional
residual capacity, particularly in infants.
Causes of acute paediatric stridor

Causes of stridor can be congenital or acquired, with acquired forms further divided into
infective or non-infective causes.
Infective
Viral croup (laryngotracheobronchitis)
This is the most common cause of acute stridor in children. It is most frequently caused by
parainfluenza virus, and commonly occurs between 6 months and 3 years of age.
An upper respiratory tract coryzal prodrome is followed by inflammation and oedema
of the subglottis, resulting in a characteristic barking cough. There may be a low-grade
fever, hoarse cry, stridor and increased respiratory effort. These symptoms are often worse
at night.
Most patients experience a mild, self-limiting illness; however, stridor may prompt an
emergency department visit. Of those patients admitted, only around 1% require intubation
and ventilation, the rest being managed with humidified oxygen and steroids. A high
temperature in any child with the symptoms of croup should prompt consideration of
the pathologies below.
Epiglottitis
This is a medical emergency with potential for rapidly progressive, life-threatening airway
obstruction. It is caused by bacterial infection of the epiglottic/supraglottic tissues. It usually
occurs between 2 and 7 years of age.
Typical clinical presentation involves rapidly progressive high fever, sore throat,
dysphagia, drooling and stridor leading to respiratory failure and shock. Spontaneous
cough is usually absent; the patient appears toxic and prefers sitting forwards if able.
The UK introduction of the Haemophilus influenzae type B (HiB) vaccine (given to
children during the first year of their life) in 1992 dramatically reduced the incidence of
childhood epiglottitis. Despite this, HiB-related epiglottitis has occurred in HiB-immunized
individuals. Epiglottitis may also be caused by other organisms. It therefore remains an
important differential diagnosis in acute stridor. An immunization history should be
sought in all children, as over 4% of the UK population are partially immunized or
unimmunized.
Chapter 15: The child with stridor
139
Most children will require intubation and ventilation to secure the airway and antibiotic
treatment.
Bacterial tracheitis
This is a super-added tracheal bacterial infection following a viral respiratory tract infection. The most common causative organism is Staphylococcus aureus.
Bacterial tracheitis is usually preceded by upper respiratory tract coryza with subsequent
rapid onset of high fever, stridor, respiratory distress and painful cough with copious
mucopurulent tracheal secretions (Table 15.1).
Table 15.1. Differentiating features of viral croup, tracheitis and epiglottitis.
Viral croup
Bacterial tracheitis
Epiglottitis
Commonest
age
6 months3 years
Wide range, average 3 years
27 years
Progression
Gradual
Follows a viral prodrome,

then rapid deterioration
Very rapid
Febrile?
Usually mild fever
High fever
High fever
Swallow
Normal
Normal
Very difficult/drooling
Cough
Frequent, barking
Barking, painful, productive
None /muffled
Toxaemic
appearance?
No
Yes
Yes
Vocalizations
Hoarse
Possibly hoarse
Unable to speak
Other features
Worse at night
Agitated and distressed
Abscess
Metastatic abscess formation in the space between the posterior pharyngeal wall and the
prevertebral fascia (retropharyngeal abscess) usually occurs before 6 years of age. Similar
seeding in the space between the tonsils and the superior constrictor muscles (peritonsillar
abscess) is commoner in older children and adolescents. Staphylococcal and streptococcal
infections are the most common causes.
Typical presentation involves high fever, dysphagia and trismus with swelling and
limited movement of the neck. Stridor and respiratory distress are uncommon.
Non-infective
Foreign-body aspiration
Inhaled small food items are the commonest cause of foreign-body aspiration. Peak incidence is at 1 to 2 years of age. There is often a history of choking or coughing, but this may
be absent. Depending on the location, degree and time course of airway obstruction, there
may be a change in voice, stridor, cough, wheeze, dyspnoea or signs of complete obstruction.
Eighty per cent of inhaled foreign bodies lodge in the lower airways and therefore
stridor is less common as a presenting feature.
140
External airway compression

Neoplasm, enlarged lymph nodes, haematoma and vascular structures may all cause
extrinsic compression of the airway.
Spasmodic croup
This mimics viral croup but is not related to upper respiratory tract infection. It often
occurs with sudden onset at night, can be recurrent and may be related to gastric reflux or
atopic triggers. Clinical management is the same as for viral croup.
Other
Non-infective acute causes of stridor include severe allergic/angioneurotic reaction and
thermal airway injury. Both require careful assessment as airway inflammation may worsen
rapidly. Early intubation is therefore advised.
Chronic causes of stridor

It is important to be aware of a number of structural, often congenital, abnormalities that
can cause chronic stridor. Although they may be incidental and stable, these patients are
susceptible to acute exacerbations and may respond less well to treatment.
Parents may present late with these children, and seem disproportionately calm. It is
useful to get a history of the usual respiratory pattern for these children as they may
normally have a tracheal tug or subcostal recession. The stridor in children with chronic
airway problems may also be a red herring in that it may worsen dramatically with
increased effort of breathing, such as in chest infections.
The main causes of chronic stridor can be divided into either structurally weak airways
(such as laryngomalacia or tracheomalacia) or stenotic airways. The former may present
with acute life-threatening events secondary to airway collapse and difficulty in ventilation,
while the latter may cause difficulty at the time of intubation in finding a small enough ET
tube to pass through the obstruction.
Assessment of a child with stridor

Assessment
Where possible the child should be assessed undisturbed, in their own comfortable position
with parents nearby, as distressing these children can exacerbate stridor.
A timely clinical assessment should be made of the effectiveness and work of
breathing. Any available history will assist diagnosis and thus facilitate targeted treatment.
Further examination involves assessment of the phase of breathing associated with the
stridor. Loudness of stridor correlates poorly with severity and decreases in near complete
obstruction or exhaustion. Cautious chest examination should be possible. The severity of
airway stridor should not distract from other potential problems, e.g. cardiovascular
compromise.
Pulse oximetry should be used where tolerated. Low saturations or a significant supplemental oxygen requirement are a sign that the childs work of breathing has become
excessive.
Croup severity scoring systems can been used to guide management, but these are rarely
used nowadays. More important is recognition of signs of severe croup, including:

141
delayed inspiratory breath sounds

biphasic stridor
suprasternal and intercostal recession
nasal flaring
cyanosis in oxygen of 40% or more.
Intrusive interventions such as cannulation or direct airway examination should be avoided,

unless judged to be low-risk or clinically indicated. This should not delay timely intervention when indicated. Assessment by the most experienced clinicians present and advice
from ENT and PICU/retrieval teams should be sought, if in doubt.
If, after assessment, a decision is taken to watch the child and wait for further help, this
opportunity should be taken to get as many things prepared as possible in anticipation of
the child deteriorating before help arrives.
Signs of a really sick child

Signs of severe airway obstruction and increased work of breathing
These include:
choking
head bobbing and nasal flaring in the infant or seesaw paradoxical chest and
abdominal movements, subcostal, sternal or intercostal recession and tracheal tug;
these signs occur earlier in infants
adopting a specific posture to keep the airway open, e.g. tripod posture in children
or arching in infants
grunting in infants (an attempt to splint the airways open with auto-PEEP)
biphasic stridor
respiratory rate of > 50 in an infant or > 30 in a child.
Signs of respiratory decompensation

Worsening oxygenation, especially with oxygen supplementation.
Hypercarbia this may produce sympathetic overdrive, altered consciousness or
cardiovascular collapse.
Signs of exhaustion and impending respiratory arrest

It is vitally important to recognize these. They may mean that a child is too sick to move to a
theatre to intubate.
Table 15.2. Signs of exhaustion.
Paradoxical reduction in minute ventilation (with/without decreased stridor intensity)

Paradoxical silent chest
Reduced conscious level
Apnoeas
Bradycardia
Cyanosis
142
Beware of severe cardiovascular compromise in addition to respiratory distress in

certain conditions such as sepsis associated with bacterial tracheitis.
Targeted treatments
A variety of treatments to reduce the effects of airway obstruction are available. These may
prevent the need for intubation, or simply buy time to seek help and arrange equipment. As in
other chapters, these treatments follow on from basic medical management (see Chapter 4).
Reduction of mucosal inflammation

These are usually of greater value where potentially reversible mucosal inflammation is
responsible for airway obstruction, e.g. croup or stridor post-intubation/bronchoscopy.
They may have minimal effect in conditions such as bacterial tracheitis and epiglottitis.
These conditions should therefore be considered early if presumed croup fails to respond to
treatment.
For moderate to severe croup, not controlled by steroids, 400 g/kg of adrenaline (0.4 ml/kg
of 1:1000, maximum 5 ml) delivered by a nebulizer may reduce mucosal inflammation
quickly. It can be repeated at 3060-minute intervals. Significant tachycardia and dysrhythmias are rare; nevertheless ECG monitoring should be performed with repeated doses.
Because of the short-term nature of the effects of nebulized adrenaline, the patient must
continue to be closely observed as the stridor will return. The aim of adrenaline nebulizers
is to provide time for the steroids to work. More than two doses of nebulized adrenaline
suggests that the child may need to be monitored on PICU or intubated. Contact your local
PICU retrieval team for advice at this point.
Corticosteroids
Corticosteroids reduce inflammation with a delayed effect of 624 hours. Their effectiveness in croup is well established. They can be given orally, parenterally or by nebulization.
A single dose of dexamethasone (PO (per oral), IV or IM equal efficacy) 0.15 mg/kg
should be administered. Alternatively 2 mg of nebulized budesonide may be used.
Optimization of airway gas flow

Humidification
Where increased airway secretions are present, inspired gases should be humidified to avoid
dried secretions exacerbating airway obstruction.
Heliox
This is a 70:30 helium:oxygen mixture with lower density than air or oxygen. Lack of
availability and limitation of inspired oxygen concentration may limit its utility, but it may
buy time for other targeted treatments to be effective.
Antibiotics
Bacterial infection of the airway requires early antibiotic treatment. First-line antibiotics
should be broad-spectrum and parenteral.
143
A broad-spectrum cephalosporin, e.g. ceftriaxone 80 mg/kg/day (maximum 4 g/day) or

cefotaxime 50 mg/kg 8 hourly (maximum 2 g/dose), would be an appropriate first-line
therapy for most upper airway bacterial infections including epiglottitis and tracheitis. If
MRSA infection is likely, vancomycin should be considered.
Foreign-body removal
An inhaled foreign body preventing cough or vocalization needs immediate basic life
support manoeuvres for the choking child. Pneumothorax from ball-valve effect gas
trapping behind a foreign body must be considered and treated promptly if present.
Severe partial upper airway obstruction may be worsened by attempts to remove the
foreign body in the emergency department. Supplemental oxygen and avoidance of further
distress to the child are required while rapid transfer to theatre for rigid bronchoscopy is
arranged.
The rigid bronchoscopy will require a spontaneously breathing patient, inhalational
induction and maintenance of deep anaesthesia, and topical application of local anaesthetic
to the airway.
Intubating a child with stridor

Intubation considerations
Pre-intubation
The three scenarios that must be anticipated in this situation are a difficult view for
intubation, a narrowed airway (needing a smaller than predicted ET tube) and loss of
airway on induction of anaesthesia.
Unless circumstances mandate a lifesaving attempt elsewhere, intubation should take
place in a safe environment for inhalational induction with all necessary equipment ready
and expertise available. If the child is being transferred to theatre an experienced anaesthetist should accompany them. An appropriately trained ENT surgeon may be required to
perform emergency tracheostomy or bronchoscopy.
The presence of parents with the child up to induction of anaesthesia often helps to keep
the child calm and should be encouraged where possible. The parents can be asked to step
out (with a nurse) once the child is anaesthetized. This should be explained to the parent
before induction.
Equipment that may be required includes:
a selection of small ET tubes (down to a size 2.5mm in babies)
stylet or pre-stiffened refrigerated ET tube
difficult intubation equipment
surgical equipment for bronchoscopy and tracheostomy
fluids and IV access in the severely obtunded child with cardiovascular instability,
early IO access should be obtained where IV access has failed. Syringes (50 ml)
preloaded with fluid boluses for rapid administration are helpful. Where there is relative
cardiovascular stability in a non-obtunded child, IV access can be obtained under
anaesthesia.
Depending on the clinical situation glycopyrrolate pre-treatment should be considered to
reduce airway secretions and obtund vagally mediated bradycardia.
144
Intubation
A challenging and possibly failed intubation should be anticipated, especially in conditions
associated with difficulty visualizing the vocal cords, such as epiglottitis, pharyngeal masses
or craniofacial abnormalities.
Inhalational induction in oxygen with a volatile agent that causes least airway irritation is
the usual technique. This needs to be carefully planned and performed by the most senior
anaesthetist present. Monitoring for induction should include oxygen saturations and endtidal carbon dioxide measurement. ECG monitoring should be attached as a saturation
probe may fail if the child becomes bradycardic.
Gas induction
The position adopted for induction should be the most comfortable for the patient,
usually upright in most patients, irrespective of the pathology.
Alveolar ventilation may be severely compromised by airway obstruction, slowing
the uptake of volatile agents. Patience is crucial it may take up to 10 minutes
before the patient is ready for laryngoscopy and intubation.
Application of CPAP during induction helps to maintain airway patency as muscle
tone diminishes.
Attempting to intubate too early can be disastrous. As a general rule, once the
pupils are small and central wait for a further 30 breaths.
Topical local anaesthetic applied to the airway (e.g. lignocaine spray) will assist in
blunting unwanted responses to intubation in the spontaneously breathing patient.
Laryngoscopic view may be compromised by oedema, especially in epiglottitis. Compression of the chest may produce glottic bubbles to guide intubation. If attempts at intubation
fail, advanced difficult airway rescue techniques, such as emergency cricothyrotomy or
tracheostomy, may be required (see Chapter 16). All of the necessary equipment to
undertake such procedures should be readily available. The ENT team should also be
present when possible.
Post intubation
In the presence of pre-existing hypovolaemia, initiation of ventilation may lead to hypotension. This can usually be rectified by giving fluid boluses.
Muscle relaxation should be continued by infusion to prevent coughing and ET tube
dislodgment. The ET tube position should be confirmed by CXR, and it should be well
secured to minimize accidental extubation. Nasal reintubation for transfer of the child is
not recommended when the intubation has been difficult, or the airway narrowed by
disease or attempts at intubation.
A further reason why controlled ventilation is desirable in this situation is that spontaneous ventilation may be difficult because of the small diameter of the ET tube, lung disease
and sustained hypoxia.
Should the childs ventilation deteriorate acutely it is essential to rule out the following:
obstruction of the small ET tube by secretions or a foreign body
pneumothorax from ball/valve effect of obstructing foreign body or secretions
other underlying lung pathology, e.g. chest infection, pulmonary oedema
inadequate sedation and paralysis.
145
Preparations for transfer

A

record should be made describing:

the state of airway pre-intubation
method of induction of anaesthesia
adjuncts required for airway maintenance and/or intubation
description of view at laryngoscopy and grade of intubation, including manoeuvres
undertaken to achieve it
details of ET tube size, position and fixation
any complications encountered.
Documentation should also include radiological and microbiological investigations, blood
tests and specific treatments given. A CXR should be taken to assess ET tube position and
any chest pathology. If an arterial line is present, blood gas analysis should be undertaken to
determine the effectiveness of ventilation.
The major risk in transferring a child with stridor is accidental extubation. The ET tube
should be fixed well and the child well sedated and paralysed prior to departure.
Summary
Children are particularly susceptible to stridor. They may tire quickly from the effort of
breathing. Therefore, senior, experienced clinicians should be involved early in the management of these children. In most cases planning and ensuring that the right equipment
and team are available will make the process calm and safe.
Golden rules
The volume and pitch of stridor correlate poorly with severity of airway obstruction
Stridor does not usually lead to low oxygen saturations unless airway obstruction is
severe. Alternative reasons should be sought
Timely interventions should not be delayed by efforts to minimize distress to the child
If a child is being monitored at a distance, preparations should be made in anticipation of
rapid deterioration
Expect a difficult airway/intubation
Once the child is intubated, secure ET tube fixation is paramount
Further reading
Handler SD. Stridor. In Fleisher GR, Ludwig S.
(eds.), Textbook of Pediatric Emergency
Medicine. Baltimore: Williams & Wilkins,
1993.
Maloney E, Meakin GH. Acute stridor in
children. Contin Educ Anaesth Crit Care Pain
2007;7(6):183186.
Robinson D. Airway management. In Morton N.

(ed.), Paediatric Intensive Care.
Oxford: Oxford University Press,
1997.
Samuels M, Wieteska S. Advanced
Paediatric Life Support: the practical
approach/Advanced Life Support Group,
5th edn. Chichester: Wiley-Blackwell,
2011.
Section 2
Chapter
16
Clinical Conditions
The difficult paediatric airway

Oliver Masters and Alistair Cranston
Introduction
The combination of a critically ill child and a difficult airway might be considered to be one
of the most challenging situations for an anaesthetist, intensivist or ED specialist. Fortunately, the difficult airway in the paediatric population is rare and generally easier to
identify prior to laryngoscopy than in adults.
For those unfamiliar with the paediatric airway, adult skills are transferrable to the
paediatric population. The same basic principles apply. The overall goal is to ensure adequate
oxygenation and ventilation and always to have a back-up plan in the event of difficulty.
There comes a point when one has to weigh up the urgency of the situation and ask
yourself the following questions.
Am I the right person to be managing the airway?
If not, how long will it take for the right person to arrive?
In the meantime, how do I manage the airway?
The aims of this chapter are to provide some of the tools necessary to make this decision, as
well as giving the confidence and knowledge to successfully apply your adult skills to the
paediatric setting.
How do children differ from adults?

It is beyond the scope of this book to go into an extensive list of anatomical and
physiological differences between the paediatric and adult airways this information can
be found in any standard textbook. Instead, we will look at the differences that result in a
need for changes in management between the two.
Head position
The correct head position is the first step in effective airway management. Neck flexion
and head extension (sniffing the morning air) is a position that is suitable for most children
over 2 years of age. In younger children, the head must be kept in a neutral position.
The relatively large head of an infant means that it is sometimes necessary to place a roll
under the shoulders, to prevent over-flexion of the head.
146
Chapter 16: The difficult paediatric airway
147
Bag and mask ventilation

Most of the principles that apply to effective bag and mask ventilation in adult practice can
be applied to children. These are:
select a mask of appropriate size
ensure an airtight seal around the nose and mouth
ensure that the fingers do not compress the area under the chin this can easily cause
airway obstruction
pull the jaw forward by placing a finger behind the angle of the mandible
hold the mouth open with the face mask itself.
Difficulties in bagmask ventilation can be brought about by an obstruction to gas flow at
the laryngeal, supralaryngeal or infralaryngeal levels. Algorithm 16.1 highlights the basic
manoeuvres that should be used to aid ventilation.
Figure 16.1. Basic airway

equipment needed to manage a
paediatric airway.
Airway adjuncts
In the unconscious patient, a Guedel airway may improve airway patency, especially if there
is some degree of functional supralaryngeal obstruction. Nasopharyngeal airways (NPA) are
much better tolerated in semiconscious patients. An endotracheal tube (ET tube) one size
smaller than that for intubation may be used, though care must be taken not to insert it too
far, as this may precipitate laryngospasm. There are no hard and fast rules for ascertaining
the length, but the following guides may be used:
insert the NPA until audible breath sounds and movement of gas is felt at the
external opening
measure from the tip of the nose to the tragus of the ear
148
as a rough guide, in the first year of life insert about 8 0.5 cm and 8.5 0.5 cm in the
second year.
Nasogastric tubes
Children are much more susceptible to a reduction in pulmonary compliance secondary to
insufflation of the stomach during bag-mask ventilation. Fortunately gastric tubes are much
easier to pass blind in children than in adults. Any degree of gastric distension is worth
reducing with a gastric tube.
Intubation
As in adult practice, in the emergency situation pre-oxygenation is mandatory. The
combination of relatively high metabolic rate, closing capacity encroaching on FRC and
underlying disease processes means that desaturation is likely to occur more quickly in
small children.
There are a few anatomical differences in the paediatric airway with which the experienced adult operator may not be familiar. These include:
high larynx (located at C2 in neonates descending to C5/6 by 4 years)
relatively large tongue
small mandible
large, floppy, tubular epiglottis.
A straight-bladed laryngoscope may be preferred in babies. The epiglottis is picked up with
the tip of the laryngoscope. This is more likely to precipitate a bradycardia than placement
in the vallecula, so attempt the conventional approach first and only if the epiglottis is
obscuring the view should the epiglottis be picked up.
It may be necessary to perform a backwards, upwards, rightwards pressure (BURP)
manoeuvre to facilitate proper visualization of the larynx. Although this manoeuvre can be
performed by an experienced assistant in infants, in babies it is often easiest to perform this
with the little finger of the hand holding the laryngoscope.
Cuffed ET tube
Until about 10 years of age the narrowest point of the paediatric airway is the cricoid ring.
A tube that passes with relative ease through the cords may not necessarily be the right size
and care should be taken not to force it through the cricoid ring, as this may precipitate
airway oedema.
Traditionally uncuffed ET tubes have been used in children under the age of 10 years, as
it was thought that a cuffed ET tube was more likely to cause trauma. It is now considered
acceptable to use a cuffed tube in any paediatric airway. In many circumstances, particularly
if the airway is expected to be difficult, it is preferable to use a smaller, cuffed ET tube. This
reduces the likelihood of repeated intubations, in an attempt to gain a snug fit. In certain
circumstances such as the child who has sustained a burn to the airway, it is considered
mandatory to use an uncut, cuffed ET tube to avoid the need for reintubation in the
presence of evolving airway oedema.
149
Length of ET tube
Another significant difference in the paediatric airway is length. It is important to be
meticulous regarding ET tube length to ensure endobronchial intubation does not occur.
Use the following principles:
the formula (age/2)+12 cm is a useful guide
most full-term neonates require the 10 cm mark to be located at the lips
it is essential to listen in both axillae for equal and bilateral breath sounds.
Predicting the difficult paediatric airway

Predicting that an airway is likely to be difficult prior to attempted laryngoscopy gives a
significantly greater chance of being able to deal with ensuing problems. As mentioned, the
difficult paediatric airway is often easier to predict than the adult one. In older children,
characteristics of a difficult adult airway, such as obesity, become more applicable. Predictors applicable to paediatrics are detailed below.
History
The main points to elicit in the history are:
history of snoring or sleep apnoea
previous facial surgery, e.g. cleft lip or palate
presence of any syndrome, particularly involving the face or airway.
Examination
Any degree of facial deformity should be noted. Particular reference should be paid to:
facial asymmetry
small jaw (micrognathia)
low-riding ears
difficulty with mouth opening.
What do I need when faced with a difficult

paediatric airway?
Three things are required once you suspect a difficult paediatric airway:
appropriate equipment
appropriate help
a plan.
Equipment
There is a whole host of equipment and airway adjuncts that are designed to be of assistance
when dealing with a difficult airway. However, the most important equipment is the basic
150
airway equipment you are already familiar with. Some of the most useful basic equipment is
shown in Figure 16.1.
Consider the following things before starting:
make sure you have equipment in a range of sizes rather than just the one that youre
expecting to use
check that the bougie you have will pass through the ET tube you have selected (note
the smaller bougies may be very flimsy and difficult to use)
for very small ET tubes it may be preferable to pre-insert a stylet, so it can be shaped in
a specific manner (often with an anterior bend) to facilitate passage through the larynx.
Ensure the stiff wire does not protrude from the end of the tube, as this may cause
airway trauma.
A significant proportion of airway problems come via the ED. Although this area should be
fully equipped to deal with difficult airways, if the child is stable enough it may be preferable
to transfer the child to the operating theatre. It is a more familiar environment, with a
comprehensive range of airway equipment.
Help
When faced with a potentially difficult airway in an emergency setting, the more help you
have, the more likely the outcome will be successful. Help falls into two main categories:
on-site help readily available in a short time-frame, e.g. ODPs or anaesthetic assistants,
other anaesthetists, paediatricians, neonatologists, critical care and ED personnel
off-site help you may have to wait for this, e.g. specialist paediatric anaesthetist, ENT
surgeon, and retrieval service personnel.
Even in the urgent setting, it is usually possible to wait for the arrival of additional personnel
in the first category. Paediatricians and neonatologists are likely to have experience of
intubating children and neonates, so are worth having around especially for younger
children and babies.
The decision on whether to proceed without help from the second category depends on
a risk:benefit assessment. However, when faced with a potentially difficult paediatric airway,
it would be sensible to concentrate on oxygenation and wait for this level of help to come,
unless immediate intervention is necessary.
Plan
Irrespective of the airway difficulty, it is crucial to have a plan for what to do when things go
wrong. The Association of Paediatric Anaesthetists and the Difficult Airway Society have
produced a guideline for the management of the difficult airway in children between 1 and
8 years (see algorithms). It is split into three sections and deals with:
difficulty in bagmask ventilation
unanticipated difficulty with tracheal intubation
failure to intubate and ventilate.
Although primarily aimed at difficulties encountered during routine induction of anaesthesia, the basic principles can equally be applied to the emergency setting.
Difficult mask ventilation (MV) during routine

induction of anaesthesia in a child aged 1 to 8 years
Difficult MV
Give 100% oxygen
Call for help
Step A Optimise head position
Check equipment
Depth of anaesthesia
Consider:
Adjusting chin lift/jaw thrust
Inserting shoulder roll if <2 years
Neutral head position if >2 years
Adjusting cricoid pressure if used
Ventilating using two person bag mask technique
Consider changing:
Circuit
Mask
Connectors
If equipment failure is suspected, change to self-inflating bag and
isolate from anaesthetic machine promptly
Step B Insert oropharyngeal airway
Call for help again if not arrived
Consider deepening anaesthesia

Use CPAP
Maintain anaesthesia/CPAP
Deepen anaesthesia (Propofol first line)
If relaxant given intubate
If intubation not successful, go to unanticipated
difficult tracheal intubation algorithm
Assess for cause of difficult mask ventilation

Light anaesthesia
Laryngospasm
Gastric distension pass OG/NG tube
Step C Second-line: Insert SAD (e.g. LMATM)

Yes
Insert SAD (e.g. LMATM ) not > 3 attempts
Consider nasopharyngeal airway
Release cricoid pressure
Continue
Consider:
SAD (e.g. LMATM ) malposition/blockage
Equipment malfunction
Bronchospasm
Pneumothorax
SpO2 >80%
SpO2 <80%
Attempt intubation
Consider paralysis
Good airway
No
Wake up patient
Succeed
Proceed
Fail
Go to scenario cannot intubate

cannot ventilate (CICV)
SAD = supraglottic airway device
Algorithm 16.1.
152
Difficulty with bag-mask ventilation

Algorithm 16.1 is self-explanatory and involves working through the issues in a step-wise
fashion. It moves through checking basic technique and equipment, through to insertion of
an oropharyngeal airway, attempted intubation (if muscle relaxant is administered) and use
of a laryngeal mask airway (LMA).
If the LMA provides adequate ventilation and the child has a full stomach, consideration
needs to be given towards intubation. This may be possible via conventional means, or by
using the LMA as a conduit for a fibreoptic scope (see below). If the LMA does not result in
a good airway and the SpO2 is below 80%, then intubation must be attempted. If the SpO2 is
over 80%, then consideration needs to be given towards addressing the reasons for this.
Difficult intubation
The most important aspect of dealing with any difficult intubation scenario, as highlighted
in bold along the top of Algorithm 16.2, is to ensure adequate oxygenation with mask
ventilation.
Unanticipated difficult tracheal intubation during routine

induction of anaesthesia in a child aged 1 to 8 years
Give 100% oxygen and
maintain anaesthesia
Difficult direct laryngoscopy
Step A Initial tracheal intubation plan when mask ventilation is satisfactory

Direct laryngoscopy not > 4 attempts
Check:
Neck flexion and head extension
Laryngoscopy technique
External laryngeal manipulation remove or adjust
Vocal cords open and immobile (adequate paralysis)
If poor view consider bougie, straight blade laryngoscope* and/or smaller ET tube
Call for help
Ensure: Oxygenation, anaesthesia, CPAP, management of gastric distension with OG/NG tube
Verify ET tube position
Succeed
Capnography
Visual if possible
Ausculation
Tracheal intubation
If ET tube too small consider

using throat pack and tie to
ET tube
If in doubt, take ET tube out
Failed intubation with good oxygenation
Step B Secondary tracheal intubation plan

Insert SAD (e.g. LMATM) not > 3 attempts
Oxygenate and ventilate
Consider increasing size of SAD (e.g. LMATM) once if ventilation
inadequate

Succeed
Consider modifying anaesthesia and surgery plan

Assess safety of proceeding with surgery using a
SAD (e.g. LMATM)
Postpone surgery
Wake up patient
Unsafe
Safe
Safe
Failed oxygenation e.g. SpO2 < 90% with FiO2 1.0
Convert to face mask

Optimize head position
Oxygenate and ventilate
Ventilate using two person bag mask technique,
CPAP and oro/nasopharyngeal airway
Manage gastric distension with OG/NG tube
Reverse non-depolarizing relaxant
Following intubation attempts, consider
Algorithm 16.2.
Trauma to the airway
Consider 1 attempt Verify intubation, leave

at FOI via SAD
SAD (e.g. LMATM) in place
(e.g. LMATM)
and proceed with surgery
Proceed with surgery
Succeed
Failed intubation via SAD (e.g. LMATM)
Postpone surgery
Wake up patient
Succeed
Failed ventilation and oxygenation
Extubation in a controlled setting
Go to scenario cannot intubate

cannot ventilate (CICV)
*Consider using indirect larygoscope if experienced in their use
154
Step A applies to the difficult intubation with satisfactory mask ventilation. It addresses
simple manoeuvres that we have discussed earlier to try and improve the chances of
successful intubation. No more than four attempts are recommended. It also highlights
the importance of checking tube position. The old adage of If in doubt take it out is also
emphasized.
Step B moves onto more advanced techniques and what to do when oxygenation starts
to fail. The use of the LMA to facilitate oxygenation is once again emphasized in addition
to its role as a conduit for fibreoptic intubation. This is discussed in more detail below. If
oxygenation is inadequate via the LMA, then bagmask ventilation must be attempted. If
still unsuccessful, move onto the next algorithm, cannot intubate and cannot ventilate.
Cannot intubate and cannot ventilate (CICV) in a

paralysed anaesthetised child aged 1 to 8 years
Failed intubation
inadequate ventilation
Give 100% oxygen
Call for help
Step A Continue to attempt oxygenation and ventilation
FiO2 1.0
Optimise head position and chin lift/jaw thrust
Insert oropharyngeal airway or SAD (e.g. LMATM)
Ventilate using two person bag mask technique
Manage gastric distension with an OG/NG tube
Step B Attempt wake up if maintaining SpO2 >80%

If rocuronium or vecuronium used, consider suggamadex (16mg/kg) for full reversal
Prepare for rescue techniques in case child deteriorates
Step C Airway rescue techniques for CICV (SpO2 <80% and

falling) and/or heart rate decreasing

Consider:
Surgical tracheostomy
Rigid bronchoscopy + ventilate / jet
ventilation (pressure limited)
ENT available
Call for specialist

ENT assistance
Succeed
ENT not available
Percutaneous cannula
cricothyroidotomy /
transtracheal jet ventilation
(pressure limited)
Fail
*Note: Cricothyroidotomy techniques can have serious complications and training is required
only use in life-threatening situations and convert to a definitive airway as soon as possible
Algorithm 16.3.
Continue jet ventilation set to lowest

delivery pressure until wake up or
definitive airway established
Perform surgical cricothyroidotomy /

transtracheal and insertion of ET tube /
tracheostomy tube*
Consider passive O2 insufflation
while preparing
Cannula cricothyroidotomy
Extend the neck (shoulder roll)
Stabilise larynx with non-dominant hand
Access the cricoithyroidotomy membrane
with a dedicated 14/16 gauge cannula
Aim in a caudad direction
Confirm position by air aspiration using
a syringe with saline
Connect to either:
adjustable pressure limiting
device, set to lowest delivery
pressure
or
4Bar O2 source with a flowmeter
(match flow l/min to childs age)
and Y connector
Cautiously increase inflation pressure/flow
rate to achieve adequate chest expansion
Wait for full expiration before next inflation
Maintain uper airway patency to aid
expiration
156
Failure to intubate and failure to adequately ventilate (Algorithm 16.3)

In this situation, continued attempts at oxygenation must be made while a more definitive
solution is sought. The ongoing oxygenation attempts are listed in the Step A section. The
points already discussed are re-emphasized.
If the child is maintaining SpO2 over 80% then it may be possible to attempt to wake the
child up. If an aminosteroid muscle relaxant such as rocuronium has been used, sugammadex in a dose of 16 mg/kg should reverse neuromuscular blockade.
If the child is desaturating below 80% and/or becoming bradycardic, Step C covers
rescue techniques. The first plan is to have an ENT surgeon available to perform either a
surgical tracheostomy or a rigid bronchoscopy. If an ENT surgeon is not immediately
available, then percutaneous cannula cricothyroidotomy should be attempted. It is likely to
be technically challenging in children and reinforces the importance of having planned
properly so that you dont reach this stage.
The algorithm covers the technique for performing cannula cricothyroidotomy. Of note
is the importance of using a pressure-limited transtracheal jet ventilator, if available. If
unavailable, a 4 bar oxygen source connected via a Y connector or a three-way tap with an
occludable orifice should be used.
As a starting point the oxygen flow-rate (in litres/minute) should be matched to patient
age, but increased depending on the degree of chest expansion. Keeping an LMA in place to
help upper airway patency is essential to prevent barotrauma and to facilitate adequate
expiration.
Fibreoptic intubation via an LMA

As mentioned previously, the LMA can provide a useful conduit through which to pass
a fibreoptic scope to facilitate intubation. There are a few problems with this:
most adult fibreoptic scopes have an external diameter of 4 mm; consequently the
smallest size ET tube that can be successfully railroaded over this is a 4.5 mm
the internal diameter of a paediatric LMA may not be large enough to accommodate
a fibreoptic scope and ET tube
the ET tube may not be long enough to protrude far enough from the end of the LMA
to pass through the vocal cords
once in place there is no easy way of removing the LMA.
There are a few ways around these problems. One method is to use the suction port of the
fibrescope to pass a J-shaped guide-wire through the larynx in the following sequence:
pass fibreoptic scope through the LMA, and past the vocal cords
insert J-shaped guide-wire through the suction port of the scope into the trachea
remove fibreoptic scope and LMA, leaving guide-wire in place
pass airway exchange catheter over guide-wire into larynx
remove guide-wire and connect catheter up to breathing circuit, ensuring presence
of end-tidal CO2 and maintaining oxygenation with 100% oxygen
railroad appropriate sized ET tube over catheter and remove catheter.
The process is one that is worth practising prior to being faced with this problem in an
emergency setting.
157
Stabilization prior to transfer

Once the airway is secured there are a few issues to address. The main points are:
ensure the tube position is correct
ensure the tube doesnt come out
ensure that the documentation is complete.
Ensure the tube position is correct

As soon as the tube is in, the end-tidal carbon dioxide (ETCO2) trace should be checked, as
should the presence of equal and bilateral air entry in both axillae. Absence of a CO2 trace
means that tube position must be confirmed visually.
ET tube length (especially in small babies) is crucial too long and endobronchial
intubation will occur, too short and there is a risk of accidental extubation. Prior to
transfer a CXR should be requested and checked.
Neck flexion and extension significantly affect ET tube position in babies, with flexion
pushing the ET tube closer to the carina. Make sure that the CXR is taken with the neck in a
neutral position. If the ET tube length needs to be adjusted, make sure that it is well
documented what the length was when the X-ray was taken and what the length is after
repositioning. This avoids unnecessary confusion at the receiving unit.
Ensure the tube does not come out

The method used to secure the ET tube is not important as long as it is effective. The
difficult airway is best secured using tapes rather than ties.
Ties are often able to work loose and dont hold the tube in as precise a position as wellplaced tapes. It is important to ensure that the tapes are well stuck to the skin. It is
reasonable to use elastoplast initially. If there is difficulty sticking the tapes to the skin a
chlorhexidine wipe can be useful as long as the area is allowed to dry thoroughly. Sleek
may stick better than elastoplast and sometimes zinc oxide tape can be useful when other
tapes do not stick to the skin.
Ensure the documentation is complete

It is important that other medical practitioners know what problems were encountered and
how these were overcome. List the following information:
a clear and concise summary of the indications for intubation
the clinical assessment involved
the names of all the practitioners present
what was actually done.
This provides a useful template and allows others to make an informed decision as to how
things might be done differently in the future. Any investigations relating to the airway
must be commented upon and any actions clearly stated. You must ensure that all
documentation is complete and photocopied for the receiving team prior to transfer.
158
Summary
The difficult paediatric airway is a rare phenomenon. When faced with one the key is to be
well-prepared, seek help and use generic airway skills. Most importantly, as with any other
situation in medicine, do no harm.
Golden rules
Try and maintain oxygenation and ventilation at all times
Have a plan including for when things go wrong
Use all available help
Keep things simple
Dont make things worse be prepared to bail out
Dont forget the LMA
Further reading
Cook TM, Woodall N, Frerk C. Fourth National
Audit Project. Major complications of airway
management in the UK: results of the Fourth
National Audit Project of the Royal College
of Anaesthetists and the Difficult Airway
Society. Part 1: anaesthesia. Br J Anaesth
2011;106:617631.
Cook TM, Woodall N, Harper J, Benger J.
Fourth National Audit Project. Major
complications of airway management in the
UK: results of the Fourth National Audit
Project of the Royal College of Anaesthetists
and the Difficult Airway Society. Part 2:
intensive care and emergency departments.
Br J Anaesth 2011;106:632642.
Cote CJ, Hartnick CJ. Pediatric transtracheal

and cricothyrotomy airway devices for
emergency use: which are appropriate for
infants and children? Pediatr Anesth 2009:
19(Suppl.1):6676.
Walker RWM, Ellwood J. The management
of difficult intubation in children.
Pediatr Anesth 2009;19(Suppl. 1):
7787.
Weiss M, Engelhardt T. Proposal for the
management of the unexpected difficult
pediatric airway. Pediatr Anesth
2010;20:454464.
Section 2
Chapter
17
Clinical Conditions
The surgical abdomen

Suren Arul
Introduction
Children differ from adults in both pathology and presentation when suffering from
abdominal disease. The abdominal pathology seen in sick children admitted to the ICU
can either be the cause of their deterioration or a consequence of the hypotension and sepsis
that results from their main pathology.
This chapter aims to discuss common abdominal pathologies according to age in
children. It will then discuss the management of acutely unwell children with abdominal
pathology, which remains remarkably consistent regardless of cause.
How abdominal pathology differs in children

Pathology
Essentially there are a few basic patterns of primary abdominal emergencies. They include:
bowel obstruction ultimately leading to bowel ischaemia, e.g. intussusception or
incarcerated hernia
intra-abdominal sepsis, e.g. appendicitis
sudden increase in intra-abdominal pressure causing decreased venous return and
splinting of the diaphragm, e.g. massive abdominal tumours
haemorrhage.
The range of possible diagnoses varies with age in children. Table 17.1 gives a brief outline
of common abdominal pathologies according to age. It is useful to bear in mind as it may
help guide investigations and timing of interventions.
159
160
Table 17.1. Differential diagnosis of abdominal pathology in children according to age.
Age
Diagnoses
03 months
Congenital abnormalities of the GI tract, e.g. atresia, stenosis, duplication cysts

Congenital abnormalities of the lower urinary tract (often complicated with
UTI), e.g. pelvic or vesico-ureteric junction obstruction, postero-urethral valve
disorder
Malrotation or volvulus
Incarcerated inguinal hernia
Trauma/non-accidental injury (NAI)
3 months 2
years
Intussusceptions
Trauma/NAI
Obstruction (e.g. Meckels band, bezoar)
Hirschsprungs enterocolitis
25 years
Appendicitis
Trauma/NAI
Obstruction (various causes)
Massive abdominal tumours
Appendicitis
Trauma/NAI
Teenagers
Appendicitis
Inflammatory bowel disease
Pancreatititis
Presentation
The general adage of paediatric surgery is that the younger the child the more non-specific
the presentation. The classic problems seen in paediatric patients are that:
infants and older children may give little verbal history, or make misleading comments
signs in children can be misleading, e.g. many abdominal signs occur in children who
are severely unwell especially if associated with sepsis
children with abdominal pathology will not have the classic history or signs that we
associate with significant abdominal pathology in adults, e.g. pelvic appendicitis.
The key to management is therefore to have a high index of suspicion and to ask advice
from the relevant specialist early. There are, however, certain pointers that act as red flags
for children with abdominal disease (Table 17.2).
Chapter 17: The surgical abdomen
161
Table 17.2. Red flags in abdominal pathology.
History of:
Abdominal pain as the very first symptom to develop
Refusing to even try food/bottle
Bilious green vomiting (rather than yellow)
Increasing lethargy and refusing to move
Symptoms or signs:
Pain on percussion and when child is asleep (often seen as facial grimaces or quiet crying
rather than screaming)
Lying very still
Allowing painful procedures (e.g. cannulation) without moving away
Increasing abdominal distension
Specific conditions
Congenital anomalies
Most congenital anomalies will either be detected antenatally or present acutely at birth.
However, occasionally conditions may be missed because they have developed late in
pregnancy or cause only partial obstruction to the bowel. The cardinal signs of congenital
bowel disease are:
bilious dark green bile
intestinal obstruction
sepsis
ischaemia
shock.
Malrotation
This is a congenital condition in which the small and large bowels are abnormally attached
to the retroperitoneum. The midgut (small bowel and colon) can then twist on the axis of
the superior mesenteric artery, causing a volvulus. This initially leads to pain and vomiting
and eventually midgut ischaemia followed by infarction, circulatory collapse and death. The
majority of children will present within the first month of life while the remaining minority
can potentially develop a volvulus at any age. Once again the red flag sign is bilious green
vomiting; however, if this is missed it can progress to bloody stools and systemic collapse.
In some patients there are few signs prior to the event, so malrotation should be considered
as a diagnosis for any patient with sudden unexplained deterioration.
Hirschsprung disease
This is a rare condition with an incidence of 1 in 3000 in males and much lower incidence
in females. In the classic form the neuro-enteric plexus fails to develop from the rectum to
the sigmoid colon; however, it can extend proximally and potentially affect the entire bowel.
The affected bowel tends to go into spasm, causing a functional intestinal obstruction. The
presentation is by failure to pass meconium in the first 24 hours with increasing evidence of
abdominal distension and bilious green vomiting.
162
Hirschsprungs enterocolitis
This is a life-threatening condition in which functional obstruction rapidly develops into
systemic sepsis. It can occur both before a diagnosis has been made and also after the
operation has been done to remove the aganglionic segment. The signs and symptoms of
this condition include constipation, abdominal distension, evidence of systemic sepsis, and
dilated loops of fluid-filled bowel on the plain X-ray.
Urinary tract pathology

Hydronephrosis secondary to pelvi-ureteric junction obstruction is one of the commonest
antenatal ultrasound findings. It is usually managed conservatively. Other conditions which
can cause hydronephrosis include vesico-ureteric obstruction, posterior-urethral valve
disorder or severe vesico-ureteric reflux. Babies often tolerate significant hydronephrosis
well. However, once an obstructed system becomes infected the baby can develop lifethreatening septic shock within a matter of a few hours.
Acquired intestinal obstruction

Various pathologies can lead to intestinal obstruction, including incarcerated inguinal
hernia, adhesive obstruction, intussusception and internal herniation around a band (such
as a Meckels band). In most cases the presentation is similar:
pain
abdominal distension
bilious vomiting
redcurrent jelly stools
hypotension and sepsis.
Finally the obstructed bowel can perforate, causing a significant deterioration in the
patients condition.
Intussusception
This is the commonest cause of obstructive symptoms in children between 3 months and
2 years of age. The usual pathology is of the ileum invaginating into the ileocaecal valve
(although ileo-ileal intussusception can occur). Recurrent intussusceptions or a presentation outside the classic age group are all suggestive of a pathological lead point such as
inflamed Peyers patches or Meckels diverticulum, which usually needs to be resected
surgically to resolve the problem.
Symptoms are classic in that the child develops the following:
bouts of severe, inconsolable crying
drawing up of knees
intense pallor that lasts approximately 5 to 15 minutes
deep, unrousable sleep
repetition of the cycle.
Non-bilious vomiting may occur in the early stages; however, significant vomiting is not an
early feature. Late signs suggestive of established obstruction and bowel ischaemia include
bilious green vomiting, systemic sepsis and the classic redcurrant jelly stools.
163
Incarcerated inguinal hernia

Indirect inguinal hernia is very common in babies and children. The general rule is that the
earlier the hernia presents the higher the likelihood of incarceration, hence babies and
infants with a reducible inguinal hernia should be referred early for elective repair
(i.e. within days). Reducible, unobstructed hernias do not usually cause significant pain.
Warning signs that a hernia is incarcerated include:
intense pain
inconsolable crying
tender, red fullness in the groin.
Bilious vomiting, systemic sepsis and bloody stools are late signs associated with bowel
ischaemia. All babies or infants admitted with collapse should have their nappy removed
and their groins carefully checked for signs of an inguinal hernia.
Intra-abdominal sepsis
Appendicitis
This is by far the commonest cause of an intra-abdominal catastrophe in children. The peak
age of presentation is at 11 years old, although it can present at any age, including in infants.
Younger children are more likely to have an atypical presentation. In particular pelvic
appendicitis is relatively common in the under 5-year-olds and usually presents late with
perforation or small bowel obstruction.
Although the child will have obvious signs of systemic sepsis the abdominal signs may
still be relatively sparse, especially if the inflamed appendix is walled off within a pelvic
mass. Radiological investigations frequently give false-negative results (especially to radiologists not specializing in paediatrics), therefore there is no substitute for an experienced
clinician repeatedly examining the child.
Peritonitis
Other causes of peritonitis in children include primary peritonitis, perforation of the bowel
from ingested foreign bodies, obstruction and trauma. Sometimes these children present in
extremis with no history of abdominal symptoms, and little to find on examination. Unless
there is another very obvious source, the abdomen should always be considered as a
potential culprit in the list of suspects.
Trauma
Injury to the abdomen is covered in the chapter on trauma. Unexplained collapse can
sometimes be explained by injuries sustained by non-accidental means.
Abdominal signs secondary to other conditions

Pathologies such as meningitis and urinary tract infections can cause vomiting and abdominal pain or distension, the probable cause being the paralytic ileus secondary to systemic
sepsis. It is important not to get overly distracted by the abdominal signs in these patients,
as it is not unheard of for patients to undergo an unnecessary laparotomy.
164
Children with chronic disease or being treated for other conditions

Most children who are treated for chronic or complex congenital bowel conditions, such as
cancer, chronic liver disease or short gut requiring long-term intravenous parenteral
nutrition, will be managed in the community at a considerable distance from their specialist
paediatric centre. If these children become unwell they are likely to present to their local
hospital.
Usually the families of these children will be well versed in their childs condition and
will also have the contact details of the relevant specialist services. It is important to take
advice from the relevant specialist before embarking on major investigation or treatments
in these children, as otherwise significant delays in transfer or unnecessary duplication of
investigations can occur.
Long-term central lines are often a feature of children with chronic or complex disease,
such as short gut syndrome or cancer. Be aware that one of the commonest causes of
collapse in these patients is central line infection.
Initial management
As with other conditions, a systematic approach (as seen in Chapter 4) is important.
Regardless of the particular abdominal pathology, a thorough assessment of the child while
administering oxygen, IV fluids and antibiotics is essential, followed by urgent referral for a
specialist surgical opinion.
In any child where an abdominal pathology is suspected the principles of management
are as follows:
manage the childs physiology (goal-directed therapy)
give broad-spectrum antibiotics
get a prompt surgical opinion
investigate the abdomen radiologically if there is any doubt (and it is safe)
keep other pathologies in mind (and continue to treat them).
165
Table 17.3. Initial management of surgical child.
Phase of management
Assessment
Assess for need to intubate

Initial phase
On presentation of the Poorly responsive
child (see Chapter 4 for Hypoxia refractory to
detailed management)
oxygen
Rising CO2
Moribund
Action
Give oxygen
Gain IV access
Give fluid bolus
Take blood including ABG, clotting
and cross-match
Give antibiotics

Once imminently lifethreatening risks have
been addressed and
abdominal source is
suspected
Reassess need to intubate

Signs of persisting
deterioration despite
treatment
Need for interventions
such as:
CVC/surgery/transfer to PICU
Surgical review
Repeat blood gases to assess
ventilation
Rising CO2
Worsening metabolic acidosis
Give analgesia
Site NG tube and aspirate prior to
intubation
Assess response to fluid bolus
If BP and HR improve continue
with boluses
Consider blood and inotropes
at 60 ml/kg fluid replacement
Not immediately lifesaving procedures


Arterial line if:
Starting inotropes
CVC if:
Starting inotropes
drugs
Need to measure CVP

operate)
Decision to ventilate
Abdominal distension causes a significant challenge to a spontaneously breathing child. The
high closing capacity of infants coupled to a relatively small functional residual capacity can
lead to a large increase in work of breathing and hypoxia. Most children can cope well with
the incursion on lung volume by increasing their respiratory rate. However, as with many
other pathologies, it must be remembered that children can decompensate rapidly after
initially appearing relatively well.
Should a child need intubation it must be remembered that induction of anaesthesia can
lead to rapid desaturation, reflux, vomiting or cardiovascular collapse, especially if dehydration has been underestimated.
166
Cardiovascular support
As with adults it is quite easy to underestimate fluid loss in children with abdominal
pathology. All sick patients with significant abdominal pathology will have a degree of
shock and so early vascular access and appropriate resuscitation with fluid boluses are
essential. The initial management is similar to that of sepsis in that aggressive use of fluid
resuscitation and antibiotics is important. Blood cultures should be taken as soon as
possible (without delaying other interventions).
The volume given in each fluid bolus is to a large degree irrelevant. What is important is
to keep track of how much has been given and to track the childs response (as a reference
value it is useful to remember that the circulating volume of a child is between 70 and
90 ml/kg).
Blood should be cross-matched early, and blood products requested, as these children
may suffer from dilutional coagulopathy or disseminated intravascular coagulation. Once
the child has received 60 ml/kg of fluid consider giving blood products (guided by blood gas
haemoglobin or formal laboratory results).
Other supportive measures

Broad-spectrum antibiotics that include Gram-negative and anaerobic cover are essential
for patients with suspected abdominal sepsis. They should be given early in the management but ideally after blood and urine cultures are taken. A urinary catheter should be
used to gain a sample quickly. If no urine is forthcoming antibiotics need to be given
without delay.
Nasogastric tube
A nasogastric (NG) tube is essential in the management of children with abdominal
pathology (minimum size 10FG (French Gauge)). This can help confirm the diagnosis if
thick green bile is aspirated. It may also decompress the stomach and help alleviate any
impairment to ventilation (especially if there has been over-vigorous bagging of a child
prior to intubation). Although conventional wisdom teaches to remove an NG tube prior to
an RSI in order to minimize reflux, there are many conditions in children where it might be
considered prudent to place one prior to induction. These include conditions such as
congenital diaphragmatic hernia (CDH) or in a child where the X-rays show a large dilated
stomach.
Urinary catheter
Urinary catheter insertion is important in any critically ill child. However, in this situation it
fulfills a dual role. As well as monitoring urine output, it can be used to monitor intraabdominal pressure. The relatively small size of the childs abdomen means that a small
increase in intra-abdominal fluid or organ size can lead to a significant increase in abdominal
pressure. This, in turn, can cause the child to be significantly compromised due to splinting of
the diaphragm or reduced venous return from inferior vena cava obstruction.
167
Table 17.4 details further potential pitfalls in the management of children with abdominal pathology.
Central line/arterial line

Central lines may be very difficult to site in these children. The children are often
hypovolaemic, may be coagulopathic, and their distended abdomen may make femoral
access challenging. It is worth getting as many factors as possible in your favour before
attempting a line, so give plenty of fluid and clotting products.
If the operator feels able, an internal jugular CVC using ultrasound guidance is best for
these children. Remember that during the initial phase an IO cannula can be used for fluids
and inotropes.
Investigations
If there is suspicion of abdominal pathology in a critically unwell child, the following
investigations should be considered:
abdominal X-ray
ultrasound scan
CT scan with double contrast.
If there is a suspicion of perforation or obstruction then a left lateral decubitus abdominal
X-ray should be performed to look for free air or fluid levels.
Other considerations when considering investigations:
do not send for radiological investigations until clinically stable
investigations are dependent on the expertise of the person performing and reporting
them
negative investigations should not prevent further consideration of abdominal
pathology if suspicion is high
there is simply no substitute for repeated examination by an experienced clinician.
Surgery
Immediate surgery is almost never indicated in the management of children with significant
intra-abdominal pathology and that includes trauma. One of the few indications for surgery
in the seriously unwell child would be for the removal of an obviously infected Hickman
line.
Resuscitation, stabilization and appropriate antibiotics are the essential mainstay of
management. Surgery, whilst often needed relatively urgently, is dependent on the appropriate surgical, anaesthetic and intensive care specialists available. Postoperative care in
these children is also very complicated. Hence, it is usually much better to attempt urgent
transfer prior to embarking on surgery if all the appropriate facilities are not available.
168
Table 17.4. Potential pitfalls for general surgical patients.
System
Organ system specific

problems
Consequence of these problems
Respiratory
Lung compression from

Metabolic acidosis
Developing lung injury
Shunting
Tachypnoea to overcome smaller tidal
volume
Increased minute volume due to metabolic
acidosis
May tire from respiratory effort
Rapid desaturation on induction of
anaesthesia
Cardiovascular
Decreased fluid intake

Vomiting
Sepsis
Third space loss
Hypovolaemia
Child may be more hypovolaemic than

anticipated
Hypotension
Poor perfusion of already compromised gut
May crash on induction of anaesthesia
Gastrointestinal
Pain
Vomiting
Ileus
Potential to aspirate is high

Will be hypovolaemic
Will have electrolyte imbalance
Distended abdomen affecting breathing
Pain affecting breathing
Other problems
May be in DIC
Dilutional coagulopathy
Dilutional anaemia
Hidden bleeding
Coagulopathy
Anaemia
Hypovolaemia
Summary
The management of children with abdominal pathology is remarkably consistent regardless
of the exact diagnosis. As with much other pathology in children, early contact with a
specialist centre can help speed up management by reducing the duplication of investigations. The abdomen should always be considered as a cause of illness in a critically ill
child, but should not deter investigation and treatment of other potential disease processes.
Golden rules

Bilious vomiting is a sign of significant pathology

Repeated clinical examination by an experienced clinician is essential
Initial management should include oxygen, fluid resuscitation and antibiotics
Early intubation and ventilation is advisable if significant cardiorespiratory compromise is
present
Immediate surgery is rarely warranted
Section 2
Chapter
18
Clinical Conditions
Paediatric trauma
Karl Thies
Introduction
Trauma is the primary cause of death in children above the age of 1 year. Injury patterns in
children are age-dependent. The majority of injuries are traumatic brain and limb injuries.
Thoracic and abdominal injuries occur less frequently but carry a high mortality, especially
if combined with other injuries.
In the UK, the structure of trauma management has changed towards children being
managed in large tertiary centres. However, all hospitals with the capacity to receive
children should be prepared to manage a sick child who is too unstable to move to another
hospital. Given the low frequency of major trauma in children, trauma teams must receive
regular training and should rehearse repeatedly to enable all team members to fulfil their
roles.
This chapter cannot cover trauma management in detail; instead it aims to build on
existing experience that anaesthetists and ED doctors already have from managing adult
patients. It also aims to highlight the differences between trauma in adults and children.
Paediatric trauma resuscitation

Preparation
When information is received from ambulance control the paediatric trauma team should
be assembled as early as possible. This will allow time to prepare the team and equipment.
Designation of the trauma team leader (TTL) and allocation of roles must happen before
the patient arrives.
When taking a call from ambulance control about an imminent arrival, the agreed
national pre-alert information delivered should consist of:
age
time of injury
mechanism of injury
injuries sustained
signs (AE)
treatment received.
169
170
If a child is being brought to a non-major trauma centre then the pre-hospital system may
have triaged them as too unstable to pass the nearest hospital. Therefore, receipt of a major
trauma alert from the ambulance service should trigger a system that ensures senior doctors
(consultants) are present at the time the child arrives.
Resuscitation and anaesthetic drugs should be prepared according to the age of the
patient and an equipment check should be carried out. If immediate surgical intervention is
likely (penetrating chest injuries, uncontrolled external haemorrhage) theatres should be
informed immediately.
The primary survey

The purpose of the primary survey in trauma is to identify and treat injuries that pose an
immediate threat to life using the established CABCDE system, where the first C stands for
C-spine and catastrophic haemorrhage control.
The team should carry out the primary survey systematically and simultaneously under
the supervision of the TTL. Although X-rays and, where indicated, abdominal sonography
form part of the primary survey, many trauma centres now replace these by performing
full-body CT scans in adults. This should be considered in children.
Chapter 18: Paediatric trauma
Table 18.1. The primary survey timing of interventions.
Within 5 seconds
Initial assessment by TTL to identify
Altered level of consciousness (final common pathway for all vital functions!)
Complete airway obstruction
Massive external haemorrhage
Shock
Cardiac arrest
Within 1 minute
Immediately life-saving interventions (CABC)
Establishing oxygenation and ventilation with basic airway manoeuvres
Compression of massive external haemorrhage
Within 10 minutes
Very urgent interventions
Immobilization of the cervical spine
Chest decompression
Pelvic compression splint
Large-bore vascular access and bloods sent for laboratory examination
Commence monitoring
Analgesia, anaesthesia and definitive airway
Fluid therapy, transfusion of blood products
Sonography
Undressing the patient
Complete primary survey
Chest and pelvic X-ray
Within 30 minutes
Urgent tests and interventions
Urinary catheter
CT scan
Focussed X-rays
Within 3 hours
Completion of diagnostics and therapy
Special investigations and X-rays
Operative care
Secondary survey is carried out after all immediate threats to life are resolved.

A standard trauma panel of blood investigations usually include:

FBC
glucose
group and save with/without cross-match
clotting profile
urea and electrolytes
LFTs including amylase and lipase
a pregnancy test in all female patients of childbearing age.
171
172
Airway management, anaesthesia and ventilation

During handover from the pre-hospital team the person responsible for managing the
airway should:
establish contact with the patient
check airway patency
check conscious level.
A quick check for any overt haemorrhage can be done at the same time. Untreated large
scalp lacerations are notorious for causing significant blood loss in children. All findings
then need to be reported to the TTL.
Initial airway management

All trauma patients should receive high-flow oxygen. If there is any sign of obstruction, the
airway should be cleared with basic manoeuvres:
suction the upper airway carefully with a soft catheter to avoid mucosal lacerations and
reflex vomiting
jaw thrust with/without oropharyngeal airway (Guedel)
cervical collars should be loosened if they impair airway access
if tolerated by the child the stomach should be decompressed with a large-bore
orogastric tube
immobilization of the spine should be maintained at all times until a spinal injury is
ruled out.
In children who arrive with an ET tube in place, the correct position and size need to be
confirmed immediately by auscultation and capnography.
Endotracheal intubation and general anaesthesia

Initial airway control can usually be achieved with basic measures, but the majority of
severely traumatized children require endotracheal intubation (ETI) early on during resuscitation. The decision to intubate is made by the TTL and the anaesthetist but needs to be
communicated to the whole team. Whilst the primary survey is proceeding, the anaesthetic
team can prepare for ETI.
Anaesthetic drugs
The choice and dosage of the drugs used for induction and maintenance depends on the
cardiovascular and neurological condition of the child.
Ketamine is the preferred induction agent in the compromised child because of its more
favourable cardiovascular profile. Ketamine further reliably reduces raised intracranial
pressure (ICP) without decreasing cerebral perfusion pressure (CPP), as long as normoventilation is maintained. Propofol and thiopentone are less suitable because of their
cardiovascular side effects. Etomidate causes long-lasting suppression of the adrenal cortex,
probably affecting the physiological endocrine response to trauma.
Anaesthesia in haemorrhagic shock

Most anaesthetic drugs, analgesics and sedatives attenuate the compensatory vasoconstriction in shock and may cause a dramatic fall in blood pressure in a hypovolaemic child.
173
Ketamine is a useful drug in haemorrhagic shock, because it produces analgesia and

amnesia without affecting compensatory vasoconstriction. For induction of anaesthesia,
ketamine 1 mg/kg can be given repeatedly until the patient becomes unresponsive.
High-dose rocuronium (1.5 mg/kg) provides fast-onset muscle relaxation and has, for
many, replaced suxamethonium as first choice for rapid-sequence induction (RSI). Ensure
sugammadex is available immediately to reverse muscle relaxation if airway control is lost
and ETI is not possible.
A failed ETI protocol needs to be part of the regular paediatric trauma training
(see Chapter 16).
Spinal immobilization
For trauma patients, spinal immobilization before, during and after intubation is essential.
The cervical collar should be removed and manual in-line stabilization of the C-spine
established. The operator should try to intubate with the head in the neutral position,
which inevitably makes laryngoscopy more difficult. A bougie is often useful in this
situation, as it may be easier to pass than the ET tube initially. Intubation of these patients
therefore requires a minimum of four people to manage the process (in-line immobilization, cricoid pressure, intubator, assistant).
Cricoid pressure
There is no evidence that cricoid pressure is beneficial in paediatric RSI. A common-sense
approach to cricoid pressure should be applied. If it impairs the operators view of the
glottis it should be lessened or removed. It should be used with care (or bimanually) if a
C-spine injury is suspected. It should be avoided altogether if there is laryngeal trauma
(swelling, local crepitus, hoarse voice). The endotracheal tube position must be confirmed
by auscultation and capnography immediately after ETI.
Blood in the upper airway

Severe head injury or maxillofacial injury can result in ongoing haemorrhage into the upper
airway, leading to airway obstruction and aspiration. In severe maxillo-facial trauma a soft
nasopharyngeal airway can be life-saving if an oropharyngeal airway is ineffective.
Endotracheal intubation can be difficult or impossible in bleeding patients and constant
oropharyngeal suction should be applied during ETI to obtain an acceptable view. If
intubation is not possible, a supraglottic device (LMA or other device) can be inserted,
which partially protects the lower airway from aspiration of blood. A surgical airway is
required immediately if oxygenation cannot be maintained.
Provisional haemostasis can be achieved with two nasally inserted urinary catheters,
which are inflated in the nasopharynx and fixed under traction. An anterior nasal tampon
can complete haemostasis.
Management of shock
Circulatory shock after major trauma is in most cases due to haemorrhage, although a
tension pneumothorax after ETI decreases venous return and can also cause circulatory
shock.
174
Assessing blood loss

Unfortunately there is no single clinical measurement that can be consistently used for
assessment of blood loss. Blood pressure and heart rate are unreliable because they depend
on too many other factors, e.g. anxiety, pain and concomitant traumatic brain injury (TBI).
They further depend on the type of injury. Penetrating injuries cause less tachycardia than
blunt injuries with extensive tissue injury, for a similar degree of hypovolaemia. Systolic
blood pressure and heart rate can remain normal in the awake patient until more than 30%
of the blood volume is lost.
Other signs when assessing hypovolaemia are capillary refill time (CRT normal
< 2 seconds), skin colour and temperature. Loss of up to 25% blood volume will be
associated with a slight increase in CRT, cool peripheries and mild agitation. As blood loss
increases the peripheries will become cold and mottled and the child increasingly lethargic.
Once more than 40% of the blood volume is lost, peripheral perfusion will be absent, often
with no discernible CRT and cold, waxy-looking skin. Bradycardia in shock usually
precedes cardiac arrest.
A metabolic acidosis can be used to help guide fluid resuscitation. Serum lactate is also
valuable, but has a slower response time than a base deficit.
As with adults, transthoracic echocardiography (TTE) can be used to assess ventricular
filling, and other causes of shock such as pericardial tamponade, myocardial contusion,
valve rupture or haemothorax. TTE can help to optimize cardiac pre- and afterload in shock
but clearly requires an experienced operator.
Fluids
Balanced electrolyte solutions are first-line treatment of hypovolaemia and should be
administered in 10 ml/kg boluses IV or IO. Colloids are second-line treatment for hypovolaemia unless blood products are needed urgently. As with adults, the actual choice of
fluid is a regular point of disagreement amongst physicians. The important factors that
should be remembered are:
the fluid should be warmed
any fluid lost should be replaced.
Blood products, massive haemorrhage and trauma-induced coagulopathy (TIC)

Massive haemorrhage is defined as loss of 50% of the circulating blood volume within
3 hours, or ongoing blood loss of 2 ml/kg/min (150 ml/min in an adult).
In the treatment of uncontrolled haemorrhage, balanced electrolyte solutions and
colloids both aggravate TIC, which increases mortality. Therefore early use of packed red
blood cells (PRBC) in combination with fresh frozen plasma (FFP) is necessary. Longstanding European clinical practice and recent experience from the UK defence service
show that early transfusion of FFP in haemorrhagic shock increases survival. If there is
ongoing haemorrhage, PRBC, FFP and platelets should be transfused in a ratio of 1:1. Even
this regimen cannot reliably prevent TIC.
Every hospital should have a massive haemorrhage guideline or protocol (see Chapter 21),
which should be activated in such cases. Early recognition is important, as it can take
significant time for the necessary blood products to be assembled and delivered to where
they are needed.
175
Damage-control resuscitation
The combination of metabolic acidosis and hypothermia leads to severe coagulopathy and
further blood loss. This in turn triggers a vicious circle of worsening hypothermia, intensifying acidosis, and coagulopathy and bleeding. Damage-control resuscitation aims to break
this circle early, by addressing all three factors simultaneously:
fluid resuscitation and early transfusion of blood products and clotting factors to
re-establish tissue oxygenation and blood clotting
haemorrhage control by hypotensive resuscitation and early surgical intervention,
targeting haemostasis, but not necessarily definitive repair (damage-control surgery)
hypothermia prevention and treatment.
The definitive surgical repair is carried out after retrieval, 1224 hours later, once tissue
oxygenation, blood clotting and normothermia have been re-established.
Hypotensive resuscitation
In adult patients with uncontrolled haemorrhagic shock it is common practice to
maintain the systolic blood pressure at 80 mmHg until haemostasis is achieved. This
approach increases survival by limiting blood loss and maintaining perfusion of the
vital organs at the same time. Evidence in children is limited, but blood pressure
should be maintained in the low-normal range (see Chapter 35), except where there is
TBI, when blood pressure of at least normal levels must be maintained to ensure
adequate cerebral perfusion. Blood pressure targets for isolated head injury are
detailed in Chapter 19.
Endpoints of resuscitation
The endpoints of resuscitation in shock are not well defined. As a general recommendation
in the anaesthetized patient one would aim for:
normal blood pressure once the haemorrhage is under control
haemoglobin of above 100 g/l
platelet count of above 100109/l
CVP of 810 cmH2O
improving base excess and lactate levels
normal clotting
adequate urine output
good ventricular filling and contractility as shown by transoesophageal
echocardiography (TOE) or transthoracic echocardiography (TTE).
Tranexamic acid
The CRASH 2 trial revealed that administration of the antifibrinolytic tranexamic acid
(TXA) in bleeding trauma patients decreases the relative risk of death by 30% if given no
later than 3 hours after the injury. However, TXA should not be started later than 3 hours
after the injury because the relative risk of death is then increased. Children should receive a
loading dose of 20 mg/kg over 10 minutes (up to the adult dose of 1 g) and then 10 mg/kg
over 8 hours.
176
Hypocalcaemia
Hypocalcaemia is a common problem in massive transfusion. It can worsen coagulopathy
and cause ECG changes and myocardial dysfunction. Therefore the plasma calcium concentration should be monitored frequently and corrected as necessary.
Hypothermia
Hypothermia is a common finding in severe trauma. It is associated with a significantly
increased incidence of multiple organ dysfunction in severely injured patients. There is also
evidence that hypothermia is an independent predictor of mortality in major trauma. This
is due to the following factors:
decreased platelet function
decreased clotting factor activity
increased oxygen demand (in awake, hypothermic patients the oxygen demand
can be increased five-fold).
Treatment of hypothermia must start as soon as the patient arrives in hospital, as children
will become cold faster than adults. Forced air-warming devices, overhead heaters, warmed
IV solutions and blood products and high room temperature are used to prevent and treat
hypothermia.
Traumatic brain injury (TBI)

Sixty per cent of all severely injured children suffer isolated TBI. The outcome after TBI
depends on the initial rapid access to definitive care and minimizing secondary injury,
which is aggravated by:
hypoxaemia
hypotension
hypo- or hypercapnia
hypo- or hyperglycaemia.
Anaesthesia for TBI

Patients with major TBI (GCS < 10) require early intubation to secure the airway and to
restore oxygenation of the brain whilst maintaining an adequate cerebral perfusion pressure
(see Chapter 19).
Management of acutely raised intracranial pressure

A decreased GCS, dilated pupils, bradycardia and hypertension despite adequate analgesia
are late signs of increased ICP. Deepening of the sedation and administration of IV
mannitol (0.5 g/kg) or hypertonic saline (5 ml/kg initial dose of 3% saline, followed by
2 ml/kg boluses) can be used to decrease the ICP (see Chapter 19 for the emergency
management of raised ICP).
177
Chest injury
Serious chest injuries in children are rare, but can present without visible external signs.
They are associated with significant morbidity and mortality.
Blunt chest injury

Rib fractures only occur if exceptional force is involved and should raise suspicion
regarding further underlying serious injuries. These patients are at risk of developing a
tension pneumothorax on initiation of mechanical ventilation and consideration should be
given to elective chest drain insertion. Lung contusions can be identified on plain X-ray.
Patients with severe lung contusions require early intubation and mechanical ventilation
(MV), with high PEEP often being required to maintain oxygenation.
If a traumatic broncho-pleural fistula is suspected (pneumothorax, increasing mediastinal size and surgical emphysema) it may be necessary to ventilate a child on one
lung. Older children can be managed as adults; i.e. lung separation with a bronchus
blocker or double-lumen tube can be tried to minimize the air leak. For younger
children, simply advancing the ET tube with manipulation into a bronchus can isolate
a lung. Because the angles at which the bronchi come off the trachea are similar, it is
possible to isolate either lung with this technique. Urgent thoracotomy is then necessary
to repair the fistula.
Aortic rupture in children is seen much less frequently than in adults. A widened
mediastinum on the chest X-ray after deceleration trauma could indicate such an injury.
The diagnosis is confirmed by CT scan or CT angiography.
Penetrating chest injury

Penetrating chest injuries in children are very rare but do occur, mainly as stab wounds in
the adolescent population. These injuries can present as haemopneumothorax, haemopericardium, or transdiaphragmatic injuries. Management is the same as for adults. Some
patients need immediate thoracotomy for haemorrhage control.
Abdominal injury and pelvic fracture

Abdominal injuries are the primary cause of circulatory shock. The abdomen is also the
most common site of initially unrecognized, fatal injury in traumatized children. Focussed
assessment with sonography in trauma (FAST) will identify free fluid in the peritoneal
cavity. CT scanning is necessary to detect solid organ or retroperitoneal injuries. Fortunately, solid organ injuries rarely require laparotomy, current treatment being primarily
conservative or the use of radiological embolization.
Fractures of the elastic immature pelvis are relatively rare, and generally have a good
prognosis. In adolescents, fractures of the pelvic ring can lead to severe life-threatening
retroperitoneal haemorrhage, which requires external splinting in the emergency department. A range of pelvic splints of different sizes should be available in all EDs for children.
Sources of bleeding in pelvic fractures can be arterial, venous or from bones and ligaments.
To control an arterial bleed, immediate radiological embolization should be considered. If
the haemorrhage is primarily venous or if there is no immediate access to interventional
radiology, temporary retroperitoneal packing can be carried out in theatres or the ED.
178
Anaesthesia for emergency laparotomy

Patients requiring emergency laparotomy usually present hypothermic and in circulatory shock. In blunt abdominal trauma the blood loss is limited by the amount
of blood that can be accommodated in the abdominal cavity or retroperitoneal space;
eventually the haemorrhage tamponades itself. When opening the peritoneum, the
tamponading effect is lost, causing severe hypotension and sometimes exsanguination
if the surgeon cannot control the haemorrhage immediately. These patients should be
stabilized by transfusion of blood products before the peritoneum is opened. Good
communication between surgeon and anaesthetist is necessary to prevent this from
happening.
Complex abdominal injuries often require damage-control resuscitation before definitive repair can be carried out safely. The goal is to achieve haemostasis and prevent
contamination of the peritoneal cavity in cases of intestinal perforation.

If a child does need to come to a DGH for management, it is likely to be because they were
too sick to transfer safely to a major trauma centre (MTC). The aim of stopping in the
nearest hospital is to rectify the immediately life-threatening problems before swift transfer
on to the MTC.
The primary survey should be completed swiftly, and any immediate life-threatening
problems rectified. The decision as to whether to operate in a DGH will be decided based on
the childs need, advice from the MTC and the expertise of the surgeons and anaesthetists. It
should also involve discussion with the local PICU retrieval team. Ideally, the child should
be operated on in the MTC wherever possible.
This means that the transfer process often needs to be swift, but safe. Details on
conducting a local team-led transfer are covered in Chapter 24. If the child is to be
transferred, then careful planning needs to take place. The team should not leave without
the following:
written history of what has happened to date including what aspects of the
secondary survey have been performed
drug chart detailing what has been given (including fluids)
blood products
adequate monitoring (this does not necessarily mean a central line discuss
with PICU)
radiological images in a format that can be viewed
a plan of where to take the child (e.g. PICU or theatre at the receiving hospital).
Summary
Current management strategies for major trauma mean that a child should not be managed
outside an MTC unless necessary. However, each department should be ready to treat those
children who are too unstable to reach an MTC safely by ambulance.
179
Golden rules
The principles of the team approach to trauma are the same in children as they are
in adults
Do not underestimate blood loss in children
The patterns of injury are different, but the aims of management are similar to
adults
Aim to avoid the triad of:
Hypothermia
Acidosis
Coagulopathy

Section 2
Chapter
19
Clinical Conditions
The child with raised intracranial

pressure
Phil Hyde
Introduction
Raised intracranial pressure (ICP) is a serious consequence of a variety of pathologies in
children. Without prompt treatment a raised ICP results in morbidity and mortality. The
key components of the management of raised ICP in children are:
recognition of the presence or potential for raised ICP
emergency management of raised ICP
urgent computed tomography of the brain
transfer of the child to a neurosurgical and paediatric intensive care facility.
The aim of this chapter is to cover the management of acutely raised ICP.
Intracranial pressure and cerebral perfusion in children

Pressure
Intracranial pressure is determined by the force exerted on the inner surface of the skull by:
brain
blood volume
cerebrospinal fluid (CSF).
Intracranial pressure may rise if any one of the normal tissues increases in volume or if an
additional mass is present within the skull (Figure 19.1). Normal values for ICP in infants
and children are from 8 to 15 mmHg.
180
Chapter 19: The child with raised intracranial pressure
60
CSF
Venous
CSF
Venous volume
ICP
cmH2O
Figure 19.1. Intracranial

pressure increases with additional
mass within the skull or an
increase in the volume of brain,
CSF or blood.
50
Mass
40
Mass
Arterial
volume
Arterial
volume
Arterial
volume
Brain
Brain
Brain
Normal
ICP
181
Compensated Decompensated
Normal ICP
Increased ICP
30
20
10
0
Perfusion
With normal levels of ICP and normal systemic blood pressure, arterial blood is able to
enter the skull and perfuse the brain. If the ICP is greater than the arterial pressure, blood
will not flow through the brain.
Cerebral perfusion pressure is defined as follows:
cerebral perfusion pressure mean arterial pressure intracranial pressure
As can be seen from this equation, an increase in ICP will cause a decrease in cerebral
perfusion pressure unless a corresponding rise in mean arterial pressure occurs. When
cerebral perfusion pressure falls below a critical value, the corresponding reduction in
cerebral blood flow results in brain ischaemia.
Autoregulation of arterial pressure

In healthy individuals the cerebral blood flow supplying the brain is maintained at a
constant value over a range of blood pressure values. This autoregulation is demonstrated
in Figure 19.2 (using adult values). Autoregulation is lost in the following situations:
at extremes of systemic blood pressure
following traumatic brain injury (perfusion becomes completely pressure-dependent).
182
Cerebral blood
flow (ml/100g/
min)
120
Normal autoregulation
Pressure-dependent blood flow
100
80
60
40
20
200
190
180
170
160
150
140
130
120
110
90
100
80
70
60
50
40
30
20
10
Mean arterial pressure (mmHg)

Figure 19.2. Autoregulation of cerebral blood flow with pressure. Values shown in this graph are adult values.
Corresponding pressure values will be lower in children.
The consequence of the loss of autoregulation, e.g. after head injury, is that maintaining
an adequate blood pressure becomes essential in order to maintain appropriate cerebral
blood flow. It also highlights that surges in blood pressure should also be avoided.
Raised intracranial pressure in children

The age of the child and the rate of change of the volume of intracranial constituents are the
primary determinants of the clinical presentation of raised ICP.
The younger child

The sutures of the skull are able to expand until about 18 months of age. Before this time, a
slowly increasing intracranial volume will result in a rise in ICP and expansion of the
cranial vault with the clinical features of:
widely separated sutures
full fontanelle
enlarged head circumference
setting-sun eyes
vomiting
developmental regression (see Table 19.1 for other signs).
Setting-sun eyes, where the sclera is visible between the upper eyelid and the iris, is an
ophthalmological sign in small children resulting from an upward gaze paresis secondary to
raised ICP.
The compensatory expansion mechanisms seen in young children initially prevent the ICP
from rising to critical values, but have a limited capacity. When a critical volume is reached, or if
the change in volume is rapid, ICP can rise quickly. Signs of raised ICP are seen in Table 19.1.
The older child

Children older than 18 months have a more limited capacity to accommodate rises in ICP
and more rapidly present with the signs in Table 19.1.
183
Table 19.1. Historical features, symptoms and signs of raised intracranial pressure.
Acute change in ICP
Chronic change in ICP
History prompting
consideration of raised ICP
History of trauma
Previous ventricular shunt surgery

Developmental regression
Symptoms of raised ICP
Headache
Vomiting
Diplopia
Headache
Vomiting
Diplopia
Signs of raised ICP

Tense cranial fontanelle
Pupillary asymmetry
Seizures
Widely separated cranial sutures

Tense cranial fontanelle
Enlarged head circumference
Setting-sun eyes
Ataxia
Signs of brain herniation

(preterminal signs)
Pupillary dilatation
Hypertension
Bradycardia
Respiratory depression
Pupillary dilatation
Hypertension
Bradycardia
Respiratory depression
Conditions causing raised intracranial pressure in children

Table 19.2 categorizes the common causes of raised ICP in children.
Traumatic brain injury (TBI) is the commonest cause of raised ICP in children. It
is also the leading cause of death in injured children. In the UK each year there are
2500 brain-injured children admitted to paediatric intensive care units, 500 of whom have
severe injuries.
The three leading causes of brain injury in children over 1 year old are pedestrian versus
car, pedal cyclist versus car and falling from height. In children younger than 1 year old,
non-accidental injury is the leading cause of brain injury and must always be considered
when examining the child (see Chapter 30).
Table 19.2. Causes of raised intracranial pressure in children.
Category
Examples
Traumatic
Intracerebral haemorrhage, diffuse axonal injury
Metabolic
Diabetic ketoacidosis, hepatic encephalopathy, hyperammonaemia, carbon

monoxide poisoning
Hypoxic ischaemic
injury
Drowning, seizures, post-cardiac-arrest
Infection
Meningitis, encephalitis, cerebral abscess
Vascular
Arterio-venous malformation, haemorrhagic or ischaemic stroke, venous

thrombosis, malignant hypertension, eclampsia
Hydrocephalus
Communicating or non-communicating, blocked artificial shunt
Neoplastic
Malignant or non-malignant tumour
184
Treatment of acutely raised ICP in children

Treatment of raised ICP is focussed on:
initial emergency treatment
prevention of secondary brain injury
treatment of the underlying cause.
Determining the nature and/or the extent of the underlying cause requires CT imaging of
the head.
Often the child will need neurosurgical intervention and will need admission to a
paediatric ICU. Therefore, early discussion with the regional paediatric intensive care
retrieval consultant and neurosurgeon is vital.
Emergency management
A primary assessment of airway, breathing, and circulation (as detailed in Chapter 4) must
be completed for all patients presenting with raised ICP. Prompt treatment of the major
causes of secondary brain injury, particularly hypotension and hypoxaemia, must then be
targeted.
Airway
All children with a GCS < 9 must be intubated and ventilated. Once intubated all patients
must be adequately sedated with morphine and midazolam, and muscle relaxation
maintained.
For trauma patients, spinal immobilization before, during and after intubation is
essential. Intubation of these patients therefore requires a minimum of four people to
manage the process (in-line immobilization, cricoid pressure, intubator, assistant). The
choice of anaesthetic agent is up to the individual (e.g. thiopentone, ketamine). Ketamine is
no longer considered contraindicated in head injuries and provides a favourable cardiovascular profile in trauma.
Ventilation
All patients must have end-tidal carbon dioxide (CO2) monitoring. They can then be
ventilated to an end-tidal carbon dioxide level that correlates to a blood carbon dioxide
level (PaCO2) of 4.55 kPa. All patients should be ventilated with a positive end-expiratory
pressure (PEEP) of at least 5 cmH2O and enough oxygen to maintain saturations 98% or
arterial PaO2 > 13 kPa.
Circulation in trauma patients

Treat hypotension aggressively as it is the biggest cause of secondary ischaemic
injury. Every patient should have a minimum of two large-bore peripheral cannulae or
IO needles.
The systolic blood pressure should be maintained above the 95th centile for age, in order
to ensure adequate cerebral perfusion pressure (see Table 19.3), accepting the risk of
increased traumatic bleeding. After a maximum of 40 ml/kg of resuscitation fluid, packed
red cells and fresh frozen plasma should be used for further resuscitation. All patients
should have a urinary catheter placed (after the CT scan if it will delay imaging).
185
Table 19.3. Ninety-fifth centile systolic blood pressures by age.
Age of child
Target systolic blood pressure
< 1 year
> 80 mmHg
15 years
> 90 mmHg
514 years
> 100 mmHg
> 14 years
> 110 mmHg
If an injured child remains cardiovascularly unstable despite fluid resuscitation, it is vital

to reconsider sites of bleeding. These are the same as in adults (external, chest, abdomen,
pelvis or femur). In infants with an open fontanelle, intracranial haemorrhage can be
significant. If there is ongoing bleeding in an injured child, the patient should not be
transferred to CT until the bleeding has been controlled. This might mean that the patient
goes directly to theatre to stop the bleeding, before a CT scan.
Circulation in non-trauma patients

Good intravenous access is still essential in these children in order to administer drugs and
fluids.
In cardiovascularly stable head-injured children additional vasoactive drug support may be
needed to maintain the high target blood pressures. If the patient only has peripheral access, then
use dopamine to maintain their target systolic blood pressure. If the patient has central access,
then use noradrenaline to maintain target systolic blood pressure. In patients with myocardial
dysfunction, an inotrope, e.g. adrenaline, should be used in addition to noradrenaline.
For both sets of patients it is important not to delay computed tomography of the head
for insertion of central and arterial access. Remember that an IO needle can be used for
inotropes if necessary.
Immediate tests
Blood should be taken for:
cross-match
blood sugar
blood gas
urea and electrolytes
full blood count
coagulation
blood culture
liver function tests
ammonia (if metabolic encephalopathy considered).
Carry out a bedside pregnancy test in all post-pubertal girls. Send urine for toxicology in all
possible poisonings.
Neuroprotection
Regardless of the cause of raised ICP, once initial stabilization has occurred, focus moves to
interventions that can minimize the ICP or prevent secondary injury. These are detailed in
Table 19.4.
186
Refractory or progressive rise in ICP

Following optimization of the management described in Table 19.4, the continued presence
of pupillary dilatation, hypertension and bradycardia should stimulate the use of temporary
hyperventilation and a hyperosmolar agent (mannitol or hypertonic saline see Table 19.4
and see Figure 19.3).
Temporary hyperventilation
Reduce the end-tidal carbon dioxide level to correlate with a PaCO2 of 4 to 4.5 kPa. This is
an effective method of reducing ICP acutely. It should be used as a temporizing measure
whilst awaiting the effect of osmotic agents.
Hyperosmolar fluids
Give a dose of either:
2.55.0 ml/kg of 20% mannitol (1.25 ml/kg if cardiovascular instability)
5 ml/kg of 3% saline (23 ml/kg for subsequent doses up to a maximum plasma
sodium of 150 mmol/l).
These therapies act to reduce cerebral oedema by drawing water into the blood. Hypertonic
saline is preferred in cardiovascularly unstable trauma patients, as the diuresis associated
with mannitol is not well tolerated in these patients.
Further management with the regional paediatric intensive care consultant and neurosurgeon should occur while these interventions are ongoing (if not before).
Table 19.4. Neuroprotective manoeuvres.
Minimizing ICP
Ensure adequate analgesia and sedation (often requires large amounts of morphine and
midazolam)
Give muscle relaxants (preferably by infusion)
Ensure the patients head is in the mid-line position
Ensure the bed is tilted to 30 degrees head up
Treat seizures if present
In traumatic head injury, load with phenytoin as seizure prophylaxis (20 mg/kg over 20 min)
Maintain PaCO2 at 4.55.0 kPa (this can be measured on blood gases from venous, capillary or
arterial sources)
Intravenous maintenance fluids should be given at 2/3 maintenance. If the patient weighs more
than 10 kg, use 0.9% saline as maintenance fluid. If the patient weighs less than 10 kg, use 0.9%
saline with 5% dextrose
Keep serum sodium more than 135 mmol/l. Boluses of 3 ml/kg of 3% hypertonic saline are safe
and effective and will raise serum sodium by about 3 mmol/l
Preventing further injury
Ensure blood sugar is >3 mmol/l
Maintain blood pressure targets as already mentioned
Give a broad-spectrum antibiotic (cefotaxime 50 mg/kg) and intravenous acyclovir if infection is
a possible cause of the raised ICP
Maintain good oxygenation (saturations 98% or arterial PaO2 > 13 kPa)
Maintain normothermia (core temperature 3637 C)
187
Cross-sectional imaging
A CT scan of the head is required to assess the nature and extent of an underlying cause
of raised ICP. This should be done in liaison with a consultant radiologist. A plain CT
brain is useful for identifying surgically treatable lesions, to assess the size of the CSF
spaces, including the basal cisterns, to detect herniation and shift and to show the
presence of oedema, haematoma, contusions and fractures. A normal CT brain does not
exclude raised ICP.
CT brain with contrast is advised if a cerebral abscess or venous sinus thrombosis is
part of the differential. In trauma patients cervical spine CT should also be considered.
Other imaging of the chest, abdomen and pelvis should be discussed with a consultant
radiologist.
Lumbar puncture
Lumbar puncture is not required in the acute management of children with decreased
conscious level and suspected or confirmed raised ICP. Relevant information gained from a
lumbar puncture can be obtained after life-threatening physiology has been treated and the
child stabilized.
Specific treatment of the underlying cause

Further management based on an intracranial diagnosis is often dependent on the results of
CT of the brain. In the absence of CT scanning within a hospital (e.g. because of mechanical
breakdown) the child should be treated as if time-critical.
Tumours with mass effect and surrounding oedema and raised ICP
CT scans of intracranial tumours require specialist interpretation from neuroradiologists
and paediatric oncologists. The use of steroids to reduce the mass effect of the tumour
should be discussed with an oncologist. Such advice may be available to you through your
regional paediatric transport services conference call facility.
188
Figure 19.3. Management of acute rise in ICP with

signs of herniation in a ventilated patient.
Check all factors in Table 19.4 are satisfied
Bolus sedation/analgesia
(0.050.1 mg/kg midazolam
0.50.1 mg/kg morphine)
Osmotherapy
3% saline 23 ml/kg (first dose 5 ml/kg) avoid
sodium > 150 mmol/l
or
mannitol 0.5 g/kg (maximum 0.5 g/kg 4 hourly)
Temporary hyperventilation
aim for PaCO2 of 4.04.5 kPa
Discuss furtherer interventions with

PICU/neurosurgeon
Depending on the cause they may consider:
thiopentone
furosemide
cooling
steroids
Blocked ventricular shunts and raised ICP

One group of children with raised ICP in whom immediate treatment is possible is those
with a previously placed ventricular drain with reservoir bulb. Palpation of the ventricular
drain as it passes subcutaneously along the skull and into the neck will enable recognition of
the reservoir bulb. These have a depressible synthetic bulb. Physical depression of the bulb
should result in downward flow of CSF. An inability to depress a rigid bulb can indicate a
malfunction of the shunt system.
189
In a child who has a non-depressible reservoir bulb and signs of raised ICP it is possible
to provide an immediate life-saving intervention prior to CT scan. First clean the skin
overlying the reservoir bulb with 2% chlorhexidine and 70% alcohol solution. Pierce the
childs skin overlying the bulb with an 18-gauge needle attached to a 20 ml syringe. Advance
the needle into the centre of the bulb. Withdraw on the syringe and remove 20 ml of CSF
(Figure 19.4).
Figure 19.4. Ventricular drain
reservoir. In a child with features
of brain herniation from raised
intracranial pressure, a needle and
syringe can be used
percutaneously to withdraw CSF.
Needle
shield
Pressure
dots
Silicone
dome
Silicone
membrane
valve
Plastic valve
base
Reservoir
Radiopaque
marker
Diabetic ketoacidosis and raised ICP

Patients with diabetic ketoacidosis and features of raised ICP need rapid institution of intensive
care and neuroprotection. The most common cause of their deterioration is cerebral oedema.
CT imaging of the head enables confirmation of cerebral oedema and exclusion of venous
thrombosis, infarction or haemorrhage. Prior to being intubated and ventilated the patient will
often have been maintaining their PaCO2 very low with Kussmaul respirations. There are
differences of opinion as to how hard you should try to replicate the low PaCO2 once the patient
is intubated. Some advocate aiming for a low PaCO2 in order to keep the ICP at a minimum.
Others advocate a more lung-friendly, conservative approach to ventilation.
190
Volume resuscitation should be strictly restricted and replacement of estimated fluid

deficit should be stopped and when restarted will be spread over 72 hours (see Chapter 11).
Infectious causes of raised ICP

Suspected infectious causes of raised ICP should be treated with cefotaxime and acyclovir.
A history of foreign travel within malaria zones should trigger appropriate investigations, a
discussion with a consultant microbiologist and treatment with anti-malarial drugs.
Trauma
A pathway for the initial management of severe traumatic brain injury in children (GCS < 9)
can be seen in Figure 19.5 (see also Chapter 18).
Intubate and ventilate with cervical spine control
Figure 19.5. Initial basic management of a head

injured patient with GCS < 9.
Aim for PaO2 >13 kPa

Aim for PaCO2 4.55.0 kPa
Avoid hypotension
Sedation and muscle relaxation
Tilt bed to 30 degree head up
CT head/C-spine within 30 minutes of presentation

Discuss with PICU/retrieval team early
Aim for serum sodium of >135 mmol/l

Consider 5 ml/kg of 3% saline
Load with phenytoin 20 mg/kg
If time-critical injury on CT scan, organize time-critical transfer

of patient to neurosurgical centre with retrieval team
Hypertensive encephalopathy and raised ICP

Children with malignant hypertension should receive the standard neuroprotection
described above. Antihypertensives should only be used with PICU advice. The aim is to
reduce the childs blood pressure slowly with invasive monitoring. Always check a pregnancy test in post-pubertal females. The CT features of posterior subcortical oedema seen in
eclampsia are identical to those observed in malignant hypertension.
Transport of children to the regional care centre

The recognition of a life-threatening abnormality on CT brain amenable to surgical
intervention directs further management towards the safe and rapid transport of the child
by the local team to a neurosurgical centre; this is known as neurosurgical time-critical
191
transfer. A checklist of key interventions to enable time-critical transfer is found in

Chapter 24. Neuroprotective measures, as already described, should be maintained
throughout transfer.
Children without time-critical lesions, whose features of raised intracranial pressure
have resolved, should receive continued neuroprotection and retrieval by the regional
paediatric intensive care transport service. Discussion with the regional paediatric intensive
care transport coordinator is essential in both cases.
Summary
ICP can be raised for many different reasons in children. The aims of management are to
minimize the resulting injuries by maintaining adequate brain perfusion. Careful attention
to detail is essential in order to have every chance of keeping the ICP low.
Golden rules
Early recognition and rapid intervention are critical
Attention to detail for all factors in Table 19.4 is important
Make sure the patient is well sedated
Consider seizures in an intubated child with autonomic disturbance and signs of raised ICP
Further reading
Curry R, Hollingworth W, Ellenbogen R, et al.
Incidence of hypo-hypercarbia in severe
traumatic brain injury before and after 2003
pediatric guidelines. Pediatr Crit Care Med
2008;9:141146.
Kirkham FJ. Non-traumatic coma in children.
Arch Dis Child 2001;85(4):303312.
Kochanek PM, et al. Guidelines for the acute

medical management of severe traumatic
brain injury in infants, children, and
adolescents second edition. Pediatr Crit
Care Med 2012;13 Suppl 1:S182.
Tasker RC, Lutman D, Peters MJ.
Hyperventilation in severe diabetic
ketoacidosis. Pediatr Crit Care Med
2005;6(4):405411.
Section 2
Chapter
20
Clinical Conditions
The child with burns

Sapna Verma and Manu Sundaram
Introduction
Children sustain burns in many different ways and this may be associated with other
injuries. This chapter aims to cover the pertinent management for burns only. Other
co-morbidities such as head or spine injuries should be managed alongside the burns
in order of severity.
How do burns differ in children?

Pathophysiology
Although the assessment and management of a burn may at first glance appear the same for
children and adults, there are significant differences.
Skin thickness
Childrens skin is significantly thinner than adults. The depth of the burn may therefore be
much greater for the equivalent heat source, with burns occurring with temperatures as low
as 40 C.
Airway
Children have narrower airways than adults; oedema can therefore rapidly lead to airway
obstruction.
Surface area to volume ratio

Children will lose proportionately more fluid and rapidly become hypothermic.
Extent of burn
The rule of nines cannot be universally applied to children. It must be modified to take
into account their varying body proportions. See Figure 20.1.
The vast majority of burns occur within the home environment, especially in the kitchen
or bathroom. The aetiology of burns varies with the age of the child; for example, scalds are
more common in children under the age of 4, whereas flame burns are more common in
children over 10 years of age.
192
Chapter 20: The child with burns
193
Cause of burn
Consideration should always be given to the possibility of a non-accidental cause for burn
injuries in children, particularly if the history is inconsistent with the injury and it involves
scalds with a glove and stocking distribution.
Assessment of a child with burns

The child with burns should be managed as any critically ill patient (see Chapter 4).
However, there are additional factors which should be considered for every burns patient.
Initial assessment
Initial assessment should follow the same principles as detailed in Chapter 4. The main aim,
as always, is to treat immediately life-threatening problems and prevent further harm. Hence
a thorough initial approach is essential, being aware of the potential for additional injuries.
Airway and facial burns

Facial and upper airway burns may cause oedema and airway compromise. A very low
threshold for intubation should be set for burns patients. Oedema of the burned airway will
worsen over the first 2448 hours especially following fluid administration. Intubation with
a cuffed and uncut endotracheal tube should be considered early on if there are signs listed
in Table 20.1.
Table 20.1. Signs of potential impending airway compromise in burns.
Stridor
Hoarseness
Circumferential burns to the neck
Carbonaceous sputum
Singeing of the nasal hairs
Facial oedema
A cuffed endotracheal tube should be used (even in prepubescent children) when

available. They are preferred in this situation for the following reasons:
airway security
no need to change tube if size too small
when the oedema starts to improve there is no need to upsize.
Inhalational injury
The products of combustion cause irritation to the lower airways, resulting in bronchospasm and ciliary dysfunction. Similarly, burns to the lower airways or explosions may
cause lung injury, pneumothoraces or pulmonary contusions. These may manifest themselves as respiratory distress, which can be assessed clinically (see Chapters 4 and 7) as well
as with blood gases and radiological imaging.
Once ventilated, high airway pressures or FiO2 may be required, and a lung-protective
strategy of ventilation is recommended. Full-thickness burns or circumferential burns to
194
the chest may hamper ventilation and rarely escharotomies may need to be performed in
the emergency department, if this occurs.
Pulse oximetry must be continuously monitored in burns patients; however, in carbon
monoxide poisoning the oxygen saturations may appear falsely normal. Blood gases should
therefore be obtained and analysed with a co-oximeter to assess gas transfer (using PaO2)
and carboxyhaemoglobin levels. CO levels in excess of 25% should prompt consideration of
ventilation.
If bronchospasm is present administration of 2-agonists may be of benefit. Treatments
such as bicarbonate lavage, nebulized heparin and nebulized N-acetylcysteine should
generally only be undertaken in a tertiary centre.
Depth of burn
Accurate assessment of the burn depth is an essential component of burn management. The
true depth may not be immediately obvious and is rarely uniform.
Superficial burns
These affect the epidermis of the skin only. The skin looks red and is mildly painful. The top
layer of skin may peel a day or so after the burn, but the underlying skin is healthy. It does
not require any treatment.
Partial thickness burns

These cause deeper damage and extend to the dermis. The skin forms blisters and is painful.
As some of the dermis is unharmed these burns usually heal well within 10 days.
Full-thickness burns
These damage all layers of skin, resulting in a white, leathery appearance to the burn. There
may be little or no pain as the nerve endings are destroyed. Skin grafting is usually required.
Deeper burns
These affect underlying structures, e.g. bone and muscle in electrical burns where there may
be minimal damage to the overlying skin.
Exposure and extent of burns

The child must be exposed and examined to enable accurate estimation of both the depth
and extent of the burn. This is essential to calculate the fluid requirements accurately. It also
allows careful removal of wet clothing to minimize temperature loss. Adherent clothing can
be left in place to minimize further damage to underlying tissues. It is important to
document the childs temperature. Active rewarming with a Bair hugger should be used
where appropriate. All jewellery must be removed immediately before tissue swelling
occurs.
Assessing the extent of burns is important as it affects both management and outcome.
The use of the Lund and Browder charts (Figure 20.1), which detail these varying body
proportions in relation to the age of the child, is essential in order to manage a child
appropriately. Alternatively the childs palm with adducted fingers equates to 1% of their
total body surface area. This method is particularly useful in the estimation of smaller
burns.
LUND AND BROWDER CHARTS
IGNORE
SIMPLE ERYTHEMA
195
Figure 20.1. Lund and Browder

chart.
A
1
A
1
13
13
Superficial
Deep
1
1
1
2
REGION
HEAD
NECK
ANT.TRUNK
POST.TRUNK
RIGHT ARM
LEFT ARM
BUTTOCKS
GENITALIA
RIGHT LEG
LEFT LEG
TOTAL BURN
RELATIVE PERCENTAGE OF BODY SURFACE AREA

AFFECTED BY GROWTH
AREA
AGE 0 1
5
10
A = OF HEAD
9
8
6
5
B = OF ONE THIGH 2
3
4
4
C = OF ONE LEG 2
2
2
3
15
4
4
3
ADULT
3
4
3
Treatment of a child with burns

Immediate management should involve removal of smouldering clothing and removal
from the heat source. Cooling of the burn is beneficial when performed within 20
minutes of injury. Thereafter a structured approach to the management of burns should
always be followed to ensure that other associated life-threatening injuries are not
missed.
Securing a definitive airway

There are specific issues relating to intubation of a child with a burn. The combination
of facial swelling, possibility of a cervical spine injury and evolving oedema of the
airway means that early intubation by an experienced anaesthetist with a cuffed uncut
ET tube is recommended. In-line cervical immobilization should always be considered.
When intubating a patient suffering from airway burns, it is important to:
make sure that there is a selection of small ET tubes
use a cuffed ET tube, even in prepubescent children
note the position of the ET tube relative to fixed structures such as teeth or gums
use ET tube ties rather than tapes.
Despite the problems with hyperkalaemia that may occur after 24 hours, suxamethonium
2 mg/kg can be used as a muscle relaxant in the immediate management of the acute
phase.
196
Table 20.2. Initial management of a child with burns.
Phase of management
Assessment
Action
Initial phase
On presentation of the child
Assess need to intubate:

GCS
Hypoxia refractory to
oxygen
Inhalation injury
Airway compromise from
oedema
Carbon monoxide levels
> 40% burns
Need for surgical
intervention
Give oxygen
Remove burnt clothing
Gain IV access preferably
through unburnt skin
IO if more than three IV
attempts, or if difficulty is
anticipated
Give fluid bolus if indicated
10 ml/kg if trauma suspected
Assess cardiovascular system
Take blood including ABG,

clotting and cross-match
Reassess need to intubate in

the presence of:
Stridor
Hoarseness
Circumferential burns
(neck/chest)
Carbonaceous sputum
Singeing of the nasal hairs
Facial oedema
Repeat blood gases to assess

ventilation
Rising CO2
Carbon monoxide levels

Once imminently lifethreatening risks have been
addressed
Assess extent of burn and

calculate fluid deficit
Examine and expose the
whole body
Measure temperature and
start warming
Not immediately life-saving
procedures
If intubating
Contact senior anaesthetist
Anticipate a difficult airway
Use cuffed, uncut ET tube
Give analgesia
Start fluid therapy immediately:
Start point is from time of
burn
Give boluses if indicated
Catheterize bladder
Keep urine output
> 1 ml/kg/h
Continue to reassess clinical Contact retrieval team

Decide on further monitoring operate)
Arterial line if:
Starting inotropes
CVC if:
Starting inotropes
drugs
197
Fluid therapy
The largest amount of fluid loss occurs in the first 24 hours after the burn, while fluid leaks
from the intravascular space into the interstitial space in the first 8 to 12 hours.
In children intravenous fluids must be commenced when the percentage body surface
area affected is 10% or greater to maintain organ perfusion, ensure perfusion to the burnt
area and therefore minimize the burn injury. Erythema of the skin on its own should not
be included in the calculation of per cent body surface area affected.
Fluids are calculated using the Parkland formula as follows:
3 4 ml weight kg % body surface area volume in ml over 24 h
Half of the calculated volume (as Hartmanns) should be infused over the first 8 hours from
the time of the burn and the second half over the next 16 hours.
Although the fluid therapy is replacing lost circulating volume, it is worth crossmatching large burns for blood and fresh frozen plasma early as it may often be needed
prior to transfer.
In addition, the usual maintenance fluids should be given as 0.45% saline/5% dextrose
(see Chapter 32).
Adjusting fluid replacement

Once fluids have been commenced the childs urine output will be used to adjust ongoing
fluid therapy. The optimum urine output in children is 1 ml/kg/h or greater. Early urinary
catheterization in burns to the genitalia is prudent to avoid subsequent problems with
swelling. It is also a requirement in all burns greater than 20% of body surface area.
Shock and fluid boluses

Two reasonable-sized IV cannulae should be inserted peripherally, preferably into unburnt
tissue. If this proves difficult insertion of an IO needle should be attempted and fluid
boluses given.
Fluid boluses
Shock does not usually result directly from a burn unless there is a delay in presentation or
concurrent pathology. If it is present, an alternative reason should be sought. Fluid boluses
should be given when clinically indicated. The fluid boluses will usually not form part of the
formula.
Analgesia
Anyone who has sustained even a very small burn will understand the pain that is associated
with it. Covering a burn with cling film will cover exposed nerve endings and reduce pain.
Analgesia in the form of morphine 0.10.2 mg/kg boluses and a morphine infusion may be
required for severe burns. Alternatively, other opioids such as fentanyl may be administered, depending on individual preference and familiarity.
Dressings
Photographing burns before dressing will often obviate the need for repeated examination
and hence reduce disruption to burnt tissue. Large blisters should be deroofed. Burns
198
affecting more than 20% of the childs body surface area will almost certainly require
debridement in theatre.
The burns should be covered with cling film to protect the underlying skin, reduce
evaporative losses and allow visualization of the affected area if the patient is transferred to
a burns centre. For burns less than 5% of body surface area a non-adherent dressing, for
example silver urgotul, can be applied. Polyfax is the treatment of choice for facial burns.
Additional considerations
Antibiotics are not routinely administered for burns and should only be used where there is
a high index of suspicion for infection. An immunization history should be obtained to
ensure that the child is up to date with tetanus.
Suspicious burns
The incidence of intentional injury is very difficult to quantify, with quoted figures varying
between 1% and 25%. Suspicion should be raised if there are:
immersion burns, i.e. a scald with glove and stocking distribution or sacral sparing
intentional contact burns, e.g. from cigarettes, iron or other hot implements
delays in presentation
burns that cannot be explained by the history provided
unusual history for developmental stage of the child
burns in areas that are not readily accessible, for example buttocks.
It is essential that a detailed history is obtained to determine the following:
Who was present at the time of the burn?
How did the burn occur?
Where did the burn occur?
What was the heat source?
Was it an enclosed space?
What first aid was carried out and for how long?
When did the burn occur?
It is imperative that the circumstances surrounding the burn are clearly documented in the
medical notes (see Chapter 30) and local safeguarding practice is followed.
Signs of a really sick child

Many of the other signs and symptoms used to assess children who are critically unwell,
such as tachycardia, tachypnoea and pain, may be difficult to assess in severe burns patients.
It is important to involve your local PICU/retrieval team early when managing severely
burned children. Problems to look out for are detailed in Table 20.3. The indications for
referral to a burns centre are as follows:
partial thickness burns involving greater than 5% of the total body surface area
full-thickness burns
smoke inhalation
circumferential burns
electrical burns
chemical burns
199
burns to hands, feet, face and genitalia

intentional burn.
Table 20.3. Potential problems for children with burns.
System
Specific problems
Consequence of these problems
Respiratory
Airway compromise
Immediate
Delayed (needs to be
anticipated)
Need for clear management plan as a

straightforward intubation at presentation
may become very difficult with time.
ET tube must be:
Cuffed
Uncut
May develop acute lung injury

from inhalation injury or trauma
May need higher airway pressures than

anticipated
Pulse oximeter probe unreliable
Falsely reassuring reading. Will need

repeated blood gases and carbon
monoxide reading (consider arterial line)
Hypovolaemia
Consider associated injuries as a cause

(rarely the burn)
Massive insensible losses
Will need carefully calculated fluid

replacement
Also likely to need fluid boluses prior to/
during induction
Very difficult IV access
IV access should be attempted through

unburnt skin first
Central venous access may be needed in
large surface burns
Difficult to assess cardiovascular

system as
Pain causes tachycardia
Capillary refill unreliable
The most useful guide to fluid balance

becomes the urine output
Should be more than 1 ml/kg/h
Fluid replacement regime may

seem massive
Care is needed in calculating fluid

replacement.
Gastrointestinal
Gastric stasis
Potential for regurgitation and aspiration is

high
Other
problems
May develop coagulopathy

Dramatic nature of burn may
distract from other lifethreatening injuries
Care must be taken to assess the whole

patient, not just the burn!
Hypothermia may develop

quickly
Make sure that active warming occurs in

hypothermic patients, and that heat loss is
minimized
Rule of nines does not apply
Lund-Bower chart must be used to assess

extent of burn
Cardiovascular
200
Pre-transfer management
As with any transfer, documentation and investigations should be prepared before
departure. For burns patients this must include details of:
extent of burn
circumstances surrounding the injury
fluids given up until the point of departure
details of the airway management (size and length of ET tube)
investigations performed.
If there are safeguarding concerns, for example the burn is thought to be intentional, to
have arisen through significant neglect or there are other injuries that could be nonaccidental in nature, then:
all blood tests should be taken and sent to the laboratories with a chain of evidence form
(see Chapter 30)
clotting samples should be taken
contact with social care services should be documented (including the name of the social
worker, time of the call and a summary of the conversation).
Summary
Burns in children are distressing for all involved. Early intervention and aggressive fluid
resuscitation are essential. Analgesia should not be forgotten. If there is any doubt about the
management of a child with burns, contact the local PICU and burns team for advice.
Golden rules

Start the fluid resuscitation calculation from the time of burn not admission
Early assessment and intervention for airway burns is important
Do not trust the oxygen saturation reading if there is likely to be significant carbon
monoxide inhalation
Children will become hypothermic quickly unless warmed (including fluids)
Always use a cuffed, uncut ET tube where available
Hypovolaemia is usually associated with other injuries
Further reading
Advanced Paediatric Life Support: The Practical
Approach, 5th edn. London: BMJ Books,
2011.
British Burn Association. Emergency
management of severe burns course manual,
UK version.
Fenlon S, Nene S. Burns in children. CEACCP

2007;7(3):7680.
Hettiaratchy S. ABC of Burns. London: BMJ
Publishing, 2004.
Young A. The management of severe burns in
children. Curr Paediatr 2004;14:202207.
Section 2
Chapter
21
Clinical Conditions
Blood product administration

in children
Oliver Bagshaw
Introduction
Critically ill or injured children may need transfusion of blood or other blood products for
a number of different reasons. These include:
disseminated intravascular coagulation, e.g. secondary to systemic sepsis
acute haemorrhage, e.g. trauma or reversal of anticoagulants
acute anaemia secondary to an underlying condition such as haemolytic uraemic
syndrome or sickle-cell disease
plasma exchange for conditions such as thrombotic thrombocytopenic
purpura.
This chapter assumes a degree of understanding of the principles of blood transfusion. The
main considerations and basic rules that apply in children are similar to those in adults, but
with a few key differences. The latter part of the chapter will give guidance for the
management of a massive transfusion.
Considerations in children
Blood or blood products should only be administered if it is deemed essential for the
childs wellbeing. Blood product transfusions can carry significant morbidity (see
below) and this must always be borne in mind when the decision to transfuse is being
made.
Cross-matching products for children

Red cells
Alloantibodies (antibodies produced against antigens from members of the same species)
are very rare in infants younger than 4 months of age, unless the baby has already received
repeated, massive transfusions. Formal cross-matching is therefore usually not necessary.
A maternal sample for antibody screening and a sample from the baby for a direct
antiglobulin test (DAT) are sufficient. If both are negative, blood can be issued according
201
202
to the blood group of the baby. If either is positive, serological investigation and formal
cross-matching must be undertaken.
For older children, if antibodies are absent on screening it is possible to issue typespecific blood electronically for any patient. This makes the process much quicker and
easier in situations where repeated transfusions are necessary as part of the childs acute
management.
Ideally, donor exposure in children should be limited as much as possible. This can be
achieved by avoiding transfusion altogether, or dividing a single unit into smaller satellite
packs (pedipacks). These only have a volume of about 40 ml. They are ideal for top-up
transfusion, but are not suitable if significant, ongoing haemorrhage is present. Local
availability may be a problem.
Fresh frozen plasma (FFP)

FFP administered to children in the UK must be treated with methylene blue and have
come from a donor pool that is free of possible CJD infection. Currently no FFP administered to children is sourced from the UK. An alternative is Octaplas, a pooled donor
product that has some advantages in terms of consistency of clotting factor levels and a
reduced risk of transmitting infection. Small volume packs of 6070 ml are produced, but
availability will depend on local sourcing.
Platelets
In patients who have frequent platelet transfusions, antibodies can become a problem. In
these cases HLA-matched platelets need to be administered, otherwise a rise in platelet
count may not occur.
Special circumstances
Irradiated blood products
In patients with impaired immune function there is a risk of precipitating graft-versus-host
disease (GVHD), when transfused donor T cells (within blood products) proliferate and
engraft in the recipients bone marrow, then attack other organ systems. High-risk patients
are given irradiated blood to try to minimize the risk of T cell transfusion. Examples of
cases in which irradiated blood should be used are:
congenital immune deficiencies
DiGeorge syndrome (common in congenital heart disease)
bone marrow transplantation
lymphoma and leukaemia
chemotherapy patients receiving purine antagonists (e.g. 6-mercaptopurine).
Only red cells and platelets need irradiation, FFP and cryoprecipitate do not. It is important
to note that irradiated blood has a higher potassium concentration than normal blood,
which increases with the age of the unit. Therefore, irradiated blood is not stored beyond 14
days. Rapid transfusion of irradiated blood has led to symptomatic hyperkalaemia in some
patients.
Chapter 21: Blood product administration in children
203
If irradiated products are not immediately available and the clinical urgency of the
situation dictates it, non-irradiated blood products can be administered, as the risks of not
transfusing the patient may outweigh the small risk of GVHD.
CMV-negative blood products

In the UK a recent position statement by the Advisory Committee on the Safety of Blood
Tissues and Organs (SaBTO) has recommended that cytomegalovirus-negative blood
products need only be administered to neonates and pregnant women. Leucodepletion of
blood products in the UK (with the exception of granulocyte) has significantly reduced the
risk of CMV transmission in other patient groups such as immune deficiency or organ
transplantation. If the urgency of the situation demands it then blood products may need to
be administered, irrespective of the CMV status of the product or the patient.
Giving blood products

Packed red cell transfusion
Blood transfusion in stable children can be avoided if the haemoglobin concentration
([Hb]) is above 70 g/l and not dropping. Above a [Hb] of 70 g/l oxygen delivery to the
tissues is likely to be sufficient and further blood administration does not improve outcome.
Different [Hb] targets are aimed for in the following situations:
> 90 g/l patients with symptomatic sickle-cell disease (or [Hb]S < 30%)
> 100 g/l patients with active haemorrhage
> 100 g/l neonates or ex-prems with apnoeic episodes requiring intervention
> 120 g/l patients with cyanotic congenital heart disease.
Calculation of packed red cell volume to be given

The circulating blood volume of the child is greater (per kg) than that of adults and varies
with age. Newborn babies have a circulating blood volume of about 90 ml/kg, whilst in
infants and older children it is closer to 80 ml/kg.
The formula below can be used (if active haemorrhage has ceased) to calculate the
volume of packed red cells needed to achieve a desired increment in [Hb] (in g/l).
Wt kg desired increase in Hb g=l 0:5 ml needed to transfuse
Thus for a 12 kg child with a [Hb] g/l of 70 g/l, in whom you would like to raise the
concentration to 120 g/l, the equation would be
12 120 70 0:5 300 ml of packed red cells
This only gives an approximation, due to the variation in circulating blood volume and
the haematocrit of the blood being transfused. A formal haemoglobin or Hemocue
estimation should always be checked following transfusion. Care should be taken in larger
children, as the calculated volumes may be considerable. As a rule, it should never
be necessary to administer more than one unit of blood (about 275 ml) to raise the
[Hb] by 1 g/dl.
204
FFP
FFP is usually given to children in boluses of 1015 ml/kg. In major trauma it should be
given in a 1:1 ratio with packed red cells. For DIC or other coagulopathies it can be given as
a source of fibrinogen and other clotting factors. This can be titrated to effect (through
repeated boluses).
Platelets
Thresholds for platelet transfusion vary depending on the susceptibility to bleeding
and the nature of the lesion or surgery. Suggested platelet counts that should trigger
a transfusion are detailed below, although discussion with a haematologist is
recommended:

10 109/l in individuals not susceptible to bleeding

20 109/l if bleeding is more likely (infection or pyrexia)
50 109/l for surgical procedures
100 109/l in cases of massive haemorrhage with ongoing bleeding.
If transfusion is necessary, do not worry about making complicated calculations

to determine the desired rise in count. As a rule, children less than 15 kg should
receive 1015 ml/kg, whilst those over 15 kg can receive an adult unit (usually about
200 ml).
Massive haemorrhage
Massive haemorrhage is defined as loss of greater than 40 ml/kg of blood in less than
3 hours, ongoing losses in excess of 2 ml/kg/min or total blood volume loss in 24
hours. It is very rare in children, and is usually a consequence of a traumatic
mechanism of injury or intraoperative bleeding during high-risk surgery. The sort of
injury that causes haemorrhage in adults, e.g. high-speed motor vehicle accidents,
penetrating injuries and abdominal and thoracic trauma, is unusual in children.
However, if a child with a history of traumatic injury is admitted shocked, then active
haemorrhage should be suspected and the cause sought. Concealed haemorrhage may
occur in the:

chest
abdomen
pelvis
long bones
brain and scalp (in babies).
When the blood group of a child who is actively haemorrhaging is not known, it may be
necessary to administer O-negative blood. If active haemorrhage is significant and persists,
a massive haemorrhage protocol (MHP) should be initiated (Figures 21.1 and 21.2). It is
also important that steps to undertake any necessary damage-limitation surgery are
initiated.
The Management of Paediatric Massive Haemorrhage
Take Bloods for:

FBC, Coagulation, Fibrinogen & Blood Gas
Group & Screen (G&S) - minimum 2 ml EDTA
Is Massive Haemorrhage present or likely?

Senior Clinician to Assess
Triggers Massive Haemorrhage Alert
Nominates Co-ordinator to liaise with Blood Bank
Is blood needed immediately for absolute emergency?

Use Group O Negative Red cells warmed through Active Warming Device
Is there is a valid G&S sample with a negative antibody screen?
Blood Bank can issue compatible blood immediately
Blood Bank BCH:

Ext. 9874 (9am-5pm)
On-Call Bleep 55034 (all other times)
Does patient fulfil CODE RED criteria?
TRIGGER CODE RED

Shock Pack Available on Request
RE-ASSESS Is There Ongoing Bleeding?
Results Available
Results NOT Available
Request and replace blood and components based on results:
Request further products based on weight

(Chart A) and continue resuscitation
Regular blood gas analysis

and core temperature
After every 40ml/kg RBC give:

20 ml/kg Fresh Frozen Plasma
10 ml/kg cryoprecipitate
20 ml/kg platelets
Treat:
Hypothermia
Acidosis
Hypocalcaemia
Hyperkalaemia
Hb <100 g/ l give RBCs

If Platelet Count <100x 109/l give platelets
If PT or APTT > 1.5 x normal range give FFP
If Fibrinogen <1g/l give cryoprecipitate
Repeat FBC, PT, APTT and fibrinogen until bleeding stopped
If ongoing bleeding consider recombinant factor VIIa
Discuss with on-call Haematology Consultant
HTC/NB 2011 Review November 2012
Figure 21.1. Management of suspected or likely massive haemorrhage in children. Reproduced with permission of Birmingham Childrens Hospital Transfusion Committee.
Designate a Runner to
Blood Bank and instruct
Notify blood bank if

O negative blood is used
Nominated Co-ordinator
Notify on-call Consultant Haematologist

via switchboard
Liaise with blood bank:

Blood Bank BCH:
ext. 9874 (9am-5pm)
On-Call Bleep 55034 (all other times)
Discuss timing and availability of

blood and clotting products
Activate CODE RED on instruction from Senior Clinician

Call blood bank & state: Code Red
Patient Name, Hospital ID, Age, Weight, Gender
Name of Clinician in Charge of Resuscitation
Contact Duty Consultant Haematologist via Switchboard
Chart A
Blood Products to request by weight
CODE RED Definition

Consider if:
ACTIVE HAEMORRHAGE SUSPECTED AND
>20 ml/kg Red Cells given in 1 hr
>40 ml/kg fluid given in 3 hr
>2 ml/kg/min blood loss
Code red activation enables release of shock pack blood products i.e. red cells
and FFP in 1:1 ratio with platelets and cryoprecipitate if available
Blue light delivery of platelets can be requested if they are unavailable.
In severe trauma red cells, FFP and platelets can be given in 1:1:1 ratio
up to 10 kg 1020 kg
2050 kg
over 50 kg
Packed Cells
One Unit Two Units Three Units
Four Units
FFP
Four Units
Platelets
Four Units
Cryoprecipitate
Five Units Eight Units Twelve Units Fifteen Units
HTC/NB 2011 Review November 2012

Figure 21.2. Management when Code Red triggered. Reproduced with permission of Birmingham Childrens Hospital Transfusion Committee.
207
Table 21.1. Key features of a massive haemorrhage protocol.
1.
2.
3.
4.
5.
6.
7.
8.
When to declare Code Red (see Figure 21.1)

Someone to coordinate with blood bank
Blood bank/consultant haematologist informed at earliest opportunity
Identification of the source of haemorrhage
Haemorrhage control surgical opinion, tourniquets, Celox, tranexamic acid
Rapid availability and administration of blood products shock packs
Ongoing assessment
Damage-limitation surgery
Airway management in massive haemorrhage

Ideally, the child should be resuscitated prior to induction of anaesthesia, but this is not
always possible if haemorrhage is ongoing. Most children are able to mount a very effective
sympathetic response to hypovolaemia which can make it easy to underestimate the degree
of shock. Senior help should be sought urgently if a haemodynamically compromised child
needs intubation.
Rapid-sequence induction is the method of choice. Children are generally much more
robust than adults when it comes to tolerating induction of anaesthesia, but in the presence
of hypovolaemia a significant fall in blood pressure may occur. Ketamine is usually the
induction agent of choice, because of its sympathomimetic properties and greater haemodynamic stability. However, even ketamine is likely to cause a drop in blood pressure if
hypovolaemia is present. It may be necessary to administer blood products and vasopressors to counteract this effect. Concerns about using ketamine in head injury patients are
probably unjustified and the benefits of relative haemodynamic stability outweigh the risks
of increasing ICP.
Circulation management in massive haemorrhage

Assessment of volaemic status is crucial prior to the induction of anaesthesia. The following
clinical features are used:
colour
heart rate
peripheral perfusion
presence or absence of peripheral pulses
respiratory rate
conscious level.
Capillary refill is also used, but too much emphasis has been placed on this sign in the past,
as it can be affected by other factors, such as cold and anxiety.
Two wide-bore peripheral cannulae should be inserted and blood taken for blood
grouping and cross-match. If peripheral access proves difficult, IO access should be sought.
The main aims of circulation management in this situation are:
haemorrhage control
rapid restoration of circulating volume
avoidance of over-transfusion and the risks of clot displacement
208
balancing associated pathology that may influence resuscitation targets, e.g. head injury
avoidance of the deadly triad (see below and Chapter 18).
The deadly triad

The deadly triad within the context of trauma are:
hypothermia
coagulopathy
acidosis.
Efforts to avoid these three factors should be made at all times by ensuring that any blood
given is warmed, in adequate volume and in a 1:1 ratio with FFP (with sufficient platelet
cover). Efforts should be made to warm the child by other methods also.
Tranexamic acid
Currently, no firm recommendations exist in children on the use of tranexamic acid in
major haemorrhage. However, the results of adult studies suggest there may be some benefit
in situations where fibrinolysis is likely to be ongoing, particularly if given early (within
1 hour). The dose in children is 20 mg/kg, although much higher doses have been used in
cardiac surgery (50100 mg/kg). It should be administered under haematological advice.
Imaging and transfer within the hospital

The patient should not be transferred for CT scan until haemodynamic stability has been
achieved, unless it is co-located with the ED and scanning can take place as resuscitation
continues. If haemodynamic stability cannot be achieved, it may be necessary to take the
child straight to the operating theatre for haemorrhage control first.
By establishing a team approach to these patients and utilizing the available expertise, it
should be possible for the initial assessment, resuscitation and stabilization to be successfully undertaken by the admitting hospital, prior to the arrival of a retrieval team or transfer
to definitive care.
What do I need to consider before transfer to another centre?

If a child is actively haemorrhaging at the time transfer is anticipated, the transfer should be
delayed until haemorrhage control has been established, even if this means undertaking
haemorrhage control surgery at the referring hospital. An ambulance is not a safe environment for a child with ongoing haemorrhage. Transfer of the stable child should then be
undertaken only by an expert retrieval team. If blood products have been commenced prior
to transfer, it is perfectly reasonable to continue these during the journey, with the proviso
that any blood product transfusions are completed within the 4 hour time-frame allowed.
Transfer of blood products may be necessary if the child is still actively bleeding or they
are likely to go for immediate surgery on arrival. Under these circumstances, products
should be issued by the blood bank in the referring hospital. They should liaise with the
blood bank at the receiving hospital; products should be transferred in appropriate, sealed
containers, with the necessary paperwork. The products should then be reissued at the
receiving hospital if the blood bank is satisfied that the transfer has been undertaken
appropriately. Failure to do this is likely to result in the products being wasted and may
compromise the childs safety at the receiving hospital.
209
Problems with blood product administration in children

Blood administration
Always administer blood products manually by syringe or by infusion pump in smaller
children (< 10 kg), due to the risks of over-transfusion if a free-running giving set is used.
Even with massive haemorrhage it is usually possible to keep up with losses in this way.
Care must always be taken to ensure that the three-way tap and giving set are not left open
between boluses.
Complications
The main complications of transfusion are related to either the products being administered
or the process of administration itself. Table 21.2 gives a list of the early and late complications that may occur as a consequence of administration of blood products. The main
life-threatening complications of transfusion are transfusion-related circulatory overload
(TRACO) and transfusion-related acute lung injury (TRALI).
Table 21.2. Early and late complications of blood product transfusion.
Timing
Complications
Early
Febrile reaction
Allergic reaction (urticarial and anaphylactic)
Incompatibility reaction (ABO and non-ABO)
Transfusion-related circulatory overload (TRACO)
Complications related to massive transfusion (hypothermia, hypocalcaemia,
hyperkalaemia, acidbase disturbance)
Bacterial contamination
Late
Transfusion-related acute lung injury (TRALI)

Graft-versus-host disease (GVHD)
Transmission of infection
Post-transfusion purpura
Transfusion-associated circulatory overload (TRACO)

This is a pathological condition related to rapid or massive transfusion, characterized by:
respiratory distress
tachycardia
hypertension
evidence of pulmonary oedema on CXR occurring within a few hours of blood product
administration.
Children most at risk are those under 3 years of age. Management involves stopping the
transfusion, and administration of oxygen and diuretics. In severe cases, positive-pressure
ventilation and inotropic support may be necessary.
Transfusion-related acute lung injury (TRALI)

TRALI produces many of the clinical symptoms of TRACO, but is a distinct entity. The
pathological changes are caused by neutrophil activation within the pulmonary
210
microvasculature. The lung injury leads to high-permeability pulmonary oedema, with

respiratory distress and hypoxaemia, but no evidence of circulatory overload. Treatment
is supportive, with positive airway pressure or mechanical ventilation, depending on the
degree of respiratory compromise.
Summary
Administration of blood products to children is likely in many of the commonly encountered critical illness or injury scenarios, so the attending clinicians should have a good
understanding of the main considerations specific to the child. Good lines of communication need to be established with blood bank and haematology staff early on in the childs
admission.
Golden rules
Blood product administration carries significant morbidity
When indicated blood transfusion needs to be organized and timely
Every hospital should have a massive transfusion protocol to speed up administration of
blood products where needed
Irradiated blood is needed for children with immunodeficiency syndromes
Further reading
Association of Anaesthetists of Great Britain
and Ireland. Blood transfusion and the
anaesthetist: management of massive
haemorrhage. Anaesthesia
2010;65:11531161.
British Committee for Standards in
Haematology. Transfusion guidelines
for neonates and older children. Brit
J Haematol 2004;124:433453 (amended in

2005 and 2007). http://www.bcshguidelines.
com/4_HAEMATOLOGY_GUIDELINES.
html.
McLelland DBL (ed.). Handbook of Transfusion
Medicine, 4th edn. NHS Blood and
Transplant. London: The Stationery Office,
2007. http://www.transfusionguidelines.
org.uk/.
Clinical Conditions
Section 2
Chapter
The sick neonate presenting

with shock
22
Fiona Reynolds
Introduction
The sick neonate may present to the emergency department with a range of symptoms from
poor feeding through to collapse with shock. The differential diagnosis of the sick, shocked
neonate is different from that in other stages of life.
The difficulties in diagnosis should not detract from the basic steps of ensuring oxygen
delivery to tissues. The immediate management of a neonate who presents in shock follows
the principles set out in Chapter 4.
Most of the conditions mentioned below are discussed in greater detail within their
respective chapters. This chapter aims to give a brief overview of the main pathologies that
affect neonates as well as giving advice about management.
Initial management
The management of the airway in neonates follows the same principles as that in older
children. The decision to intubate a neonate is a clinical decision based on:
paediatric GCS score
airway obstruction
respiratory failure
persistent apnoeas.
Glasgow Coma Scale

Although difficult to assess, an attempt should be made to assess the consciousness level of the
neonate using an appropriate Glasgow Coma Scale (GCS) scoring system (see Chapter 35).
Airway obstruction
The airway may be obstructed as a consequence of shock or decreased consciousness or due
to pathologies such as inflammation or infection. Additionally, congenital conditions such
as laryngomalacia may affect the neonate. The child may present with sterterous breathing
or stridor (as discussed in Chapter 15) depending on the condition.
If the airway is compromised to the point of compromising ventilation then intubation
is likely to be indicated. If there is concern about airway calibre, a careful, planned gas
induction is warranted (as in Chapter 15).
211
212
Respiratory failure
It is important to identify respiratory failure rapidly and intervene promptly. Delaying
intubation in severe respiratory failure may turn a difficult procedure into a high-risk one
in many instances.
Apnoeas
Sick neonates suffering from respiratory failure may suffer apnoeas. They should not be
ignored, as they are a sign of severe disease. The apnoeas themselves are often easy to
manage by stimulating the child or by bagging them.
If the child is stable and the apnoeas are short then a child with bronchiolitis may be
observed, or treated with CPAP. However, if a child comes in with signs of shock and
apnoeas, or the apnoeas are increasing in frequency and duration, then intubation is
indicated.
Never forget, apnoeas may be a symptom of hypoglycaemia in the neonate.
Circulation
The management of the paediatric circulation, again, follows the same principle as that in
older children. That is:
give fluid boluses of 1020 ml/kg
watch for a response in heart rate, capillary refill and blood pressure
gain intravenous (IV) access early
move to intraosseous (IO) access if IV access is not possible within 90 seconds
if in doubt about choice of inotropes, start with adrenaline or dopamine.
The main differences in neonates, when managing the circulation, are that:
it is important to correct calcium levels
it doesnt take much fluid loss to become hypovolaemic (circulating volume is only
8090 ml/kg)
neonates should be cross-matched early.
Attention to detail
This is perhaps the area that causes the most problems to doctors who manage children.
Small derangements in physiology can have a disproportionate effect on the child. Factors
that need to be actively managed include:
temperature (hypothermia and hyperthermia)
blood glucose
sodium and calcium levels
ET tube size and position (length).
Conditions that affect neonates

Septic shock (Chapter 5)
Septic shock is the most common reason for cardiovascular collapse in the neonate. The
most common pathogens include:
Chapter 22: The sick neonate presenting with shock

213
Escherichia coli
herpes simplex virus
enterovirus.
The presentation of the septic neonate is often non-specific, with poor feeding, crying and
listlessness, or an unusually quiet baby. Pyrexia or hypothermia may be present. Tachypnoea may reflect pyrexia or an attempt to compensate for metabolic acidosis. Tachycardia
with poor perfusion and cold hands and feet may be present along with hypotension.
The stabilization of the neonate with septic shock follows the principles outlined in
Chapters 4 and 5:
optimize oxygen delivery
fluid balance
electrolytes and glucose correction
temperature control.
Neonates with shock should receive antibiotics early according to local policy, e.g. amoxicillin and cefotaxime. Aciclovir should also be considered. The administration of antibiotics is time-critical as delay increases mortality.
Congenital heart disease (Chapter 6)

Many neonates with congenital heart disease (CHD) are identified by antenatal ultrasound
scans. However, some neonates may present to the ED with cyanosis or shock secondary to
a previously unsuspected CHD. The deterioration usually coincides with the physiological
closure of the ductus arteriosus (DA). Re-opening of the DA with prostaglandin E1 or E2
(PgE) will improve the circulation in most cases (see Chapter 6).
In the shocked state opening of the ductus arteriosus is only one part of the treatment
required. Some thought should be given to what the primary problem associated with the
childs CHD is. The circulatory problem may relate to:
reduced pulmonary blood flow
reduced systemic blood flow
globally impaired ventricular function.
Reduced pulmonary blood flow

The neonate with reduced pulmonary blood flow to the lungs from a right-sided obstruction, e.g. tricuspid atresia, will present with cyanosis, which will increase as the DA closes.
The increasing hypoxaemia may lead to tissue hypoxia, hypoperfusion and shock. It may be
impossible to differentiate from the neonate with septic shock.
The resuscitation should aim to increase oxygen delivery with artificial ventilation,
optimizing preload and cardiac contractility. The induction agent should be chosen to
minimize the effect on cardiac output. Ketamine is often chosen for this reason.
If cardiac disease cannot be confidently excluded PgE should be commenced by
intravenous infusion. The re-opening of the DA allows increased pulmonary blood
flow and improvement in saturations. PgE may cause apnoea if ventilation is not
controlled.
214
Reduced systemic blood flow

Poor femoral pulses may give a clue to an obstructive left-sided heart lesion but may also
reflect poor perfusion in a neonate with a structurally normal heart. A difference in
saturations between the right hand and the lower limbs is also suggestive of congenital
heart disease.
The resuscitation aims are to restore oxygenation and cardiac output. Restoration
of cardiac output is dependent on re-opening of the DA as well as the optimization of
preload, myocardial contractility and afterload.
Globally impaired ventricular function

Impaired ventricular function may occur as a primary condition in:
cardiomyopathy
myocarditis
myocardial ischaemia from congenital abnormalities of coronary arteries.
Secondary impairment of cardiac function may occur in conditions such as critical aortic
stenosis and coarctation of the aorta.
Neonates with impairment of cardiac function may have respiratory distress, hepatomegaly and enlarged heart with pulmonary oedema on CXR. Emergency treatment aims to
optimize oxygen delivery. This may require intubation and ventilation to reduce oxygen
consumption by reducing the work of breathing. As in adults, a high-risk induction of
anaesthesia would need to be undertaken, allowing extra time for circulation of drugs.
Arrhythmia
Abnormalities of heart rhythm may be either slow or fast arrhythmias. These may present
before birth with fetal distress and hydrops or after birth with poor feeding, respiratory
distress or shock.
Supraventricular arrhythmias are often well tolerated. An unremitting supraventricular
arrhythmia or a ventricular arrhythmia will result in impaired ventricular function and
potentially shock. ECG monitoring is sometimes omitted in infants thought to have simple
respiratory distress. This can lead to a delayed diagnosis of a supraventricular tachycardia.
A 12-lead ECG is required if an arrhythmia cannot be excluded on the standard ECG. If
interpretation is difficult it can be faxed to the local paediatric cardiologist.
The management of arrhythmias is dealt with in Chapter 6.
Metabolic disease (Chapter 12)

Metabolic disease in the neonatal period may present with:
encephalopathy
cardiomyopathy
hyperventilation
hypoglycaemia
shock
hyperammonaemia.
215
Hyperventilation may be secondary to metabolic acidosis. It may also be secondary to

hyperammonaemia, which stimulates the respiratory centre and drives a respiratory
alkalosis.
Initially the cardiovascular system tolerates the metabolic derangement but the progressive acidosis will adversely affect myocardial contractility and tissue hypoxia will compound
the primary metabolic derangement.
Metabolic disease should be suspected in any infant presenting with shock. Measurement
of plasma ammonia levels is important in any shocked infant in whom the diagnosis is not
clear. Treatment to reduce ammonia levels is time-critical in order to optimize neurological
outcome. The emergency treatment of hyperammonaemia is described in Chapter 12.
Glucose delivery in intravenous fluids is important to provide substrate and promote
anabolism, which may help reverse the metabolic derangement.
The key steps in management of inherited metabolic disease in a neonate are:
treatment of shock
delivery of glucose
emergency treatment of hyperammonaemia (if present).
Non-accidental injury (Chapter 30)

Non-accidental injury may present with shock. Injury may cause occult bleeding leading to
cardiovascular compromise. The most common form of non-accidental injury is abusive
head trauma. Intracranial blood loss may be of sufficient volume to cause cardiovascular
compromise or anaemia in the small infant.
Endocrine disease
A deficiency of adrenal steroids may present with cardiovascular compromise. Steroid
deficiency is suggested by:
hyponatraemia
hyperkalaemia
hypoglycaemia.
Steroid deficiency in the neonatal period is most commonly secondary to congenital adrenal
hyperplasia, which results in virilization of female infants. This may be evident at birth,
although presentation may be delayed in male infants.
Treatment requires rehydration with saline, glucose infusion and steroid replacement.
Technical details (size of equipment)

Most of the sizes and tips for accomplishing technical procedures are found in Chapter 37.
However, this is a brief summary of the immediate life-saving equipment that needs to be
at hand.

Bagging patients
It is vitally important to use the correct-size facemask for neonates. The appropriate-size
oropharyngeal airway should be used early in order to improve airway management and
216
minimize gastric insufflation. A nasogastric tube should also be inserted early in order to
minimize the impact of air passing into the stomach.
Make sure that the bag attached to the ventilation circuit, such as a T-piece, is the right
size. Too large a bag encourages hyperinflation with each breath.
Intubation
Start with a size 3.5 mm endotracheal tube (ET tube) for a full-term neonate who weighs
about 3 kg. It is important to have smaller and larger ET tube available and ready. A bougie
that passes down a small ET tube should also be readily available. Make sure that the head is
in the neutral position when intubating.
If the ET tube inserted is the wrong size, i.e. the leak is too large, change to a larger ET
tube over a bougie once the child has been preoxygenated.
Circulation
Intravenous access
If a neonate presents in cardiac arrest, the first line for access is the intraosseous route. If
there is time, most of the drugs needed during the resuscitation of a sick neonate can be
given through a 24G cannula. However, if large volumes of fluid are needed, e.g. sepsis or
trauma, a 22G cannula will be needed, usually sited in the long saphenous or femoral vein.
Central venous access

Central venous access should be gained if the operator feels able. Ultrasound-guided
insertion of a 4.5 or 5FG central venous catheter (CVC) is the usual means of access.
Arterial line
A 24G cannula is usually used to site an arterial line in the radial or posterior tibial artery in
neonates. If these are not palpable, then a 22G cannula (or Seldinger arterial line) in the
femoral artery (preferably under ultrasound guidance) will often be more straightforward.
Capillary gases
Capillary gas analyses will be performed by paediatricians as a surrogate for arterial blood
gases. They can provide important information, but are prone to certain errors. For
example:
blood glucose can be unreliable if the child has any sugary substance on their hand/foot
lactate and potassium can be falsely elevated if the sample has been difficult to obtain
it usually does not reflect the arterial oxygen tension
if peripheral perfusion is very poor all figures are likely to be inaccurate.
The information that can be gleaned from a capillary sample includes:
a normal potassium or lactate is probably normal
a high lactate, base deficit or potassium from a good flowing sample is probably high
a high PaCO2 from a good sample is probably high.
As a result all capillary samples should be interpreted in the context of the clinical
assessment of the child. Abnormal results that do not fit the clinical picture should be
rechecked, or confirmed with an arterial sample, if possible.
217
Summary
The management of specific conditions in neonates is detailed in individual chapters within
this book. However, when managing a small baby the main differences are the wider
differential diagnosis, immature physiology and paying scrupulous attention to detail.
Golden rules
Attention to detail is the key to managing neonates
Always consider cardiac and metabolic conditions
If in doubt about whether the pathology is cardiac start PgE
Check all of the blood gas parameters especially glucose and electrolytes
Seek expert advice early
Section 3
Chapter
23
What YouCould Be Expected to Doin a District General Hospital
Ongoing management of the

successful arrest
Barney Scholefield
Introduction
The return of a pulse during paediatric resuscitation is often seen as a triumph for the
resuscitation team, the child and their family. However, for the critical care physician the
hard part is to yet come in managing the post-resuscitation phase.
The four phases of resuscitation (Table 23.1) need to be expertly managed to minimize
further injury to the child and maximize the chance of a permanent return of spontaneous
circulation (ROSC) with successful short- and long-term outcomes.
Table 23.1. The four phases of cardiac arrest.
Phase
Interventions
1. Pre-arrest phase (prevention)
Child safety and injury prevention strategies

Early recognition of patient deterioration through adequate
patient monitoring and early-warning systems
2. No-flow arrest (period of cardiac Minimize delay to basic and advanced life support
arrest prior to starting CPR)
Rapid cardiac arrest team activation
Minimize delay to defibrillation
3. Low-flow resuscitation
(CPR in progress)
Excellent CPR to optimize coronary and cerebral perfusion

Avoidance of over-ventilation whilst maintaining adequate
ventilation and oxygenation
Treat reversible causes of cardiac arrest
4. Post-resuscitation phase
(post-ROSC)
Optimize coronary and cerebral perfusion

Treat arrhythmias
Manage post-cardiac-arrest syndrome
(see Table 23.2)
Rehabilitation
This chapter aims to highlight the epidemiology of paediatric cardiac arrest, emphasizing
the differences between adults and children. It will also highlight the current recommendations for post-resuscitation management, in particular those targeting the post-cardiac-arrest
syndrome during the immediate and early post-arrest stages. Interventions during these
stages, prior to the safe transfer to a tertiary paediatric intensive care unit, may improve the
patients overall outcome.
219
220
Section 3: What You Could Be Expected to Do in a District General Hospital
Why are paediatric arrests different from adult?

The overall incidence of out-of-hospital cardiac arrests in children younger than 16 years
old is low (820/100 000/year). It is much more frequent in infants less than 1 year old,
reaching an incidence comparable to adult levels of 70/100 000/year. The in-hospital cardiac
arrest incidence in children is nearly 100 times higher than out-of-hospital. Unfortunately, the
survival and neurological outcome from cardiac arrest remains poor. Out-of-hospital survival
rates are reported to be as low as 512%, with only 0.34% being neurologically intact.
Paediatric cardiac arrests are usually secondary to severe respiratory compromise or
circulatory shock. These lead to a respiratory arrest with subsequent hypoxia-induced
cardiac arrest, usually presenting as a non-shockable rhythm. This contrasts with the much
higher incidence of ischaemic heart disease in adults, where cardiac arrest is often secondary to a myocardial infarction-induced shockable rhythm.
Nearly 50% of children who suffer a cardiac arrest will also have a chronic underlying
condition such as:
congenital cardiac disorders
chronic respiratory conditions (e.g. asthma)
neurological and developmental impairment.
Awareness of the differences in aetiology is important. Early reversal of the cause of the
respiratory arrest can quickly convert a weakly perfusing or impalpable pulse into an
adequate one. The existence of preceding severe illness with resultant acidosis and hypoxia
can lead to significant neurological and systemic damage before the true cardiac arrest
occurs. However, this cannot be ascertained until later, after stabilization and further
neurological assessment.
The currently recommended compression to ventilation ratio of 15:2 for all infants and
children is a compromise. It aims to provide enough ventilation for oxygenation, and
sufficient cardiac compressions to provide adequate coronary and cerebral perfusion.
In studies of adult patients suffering from ventricular fibrillation the success of
compression-only resuscitation is due to the retained aortic blood oxygen concentration
and pulmonary oxygen reservoir. Only 14% of paediatric cardiac arrests present with a
sudden-onset shockable rhythm, hence there is a need for both ventilation and compressions in the vast majority of cases.
Post-cardiac-arrest syndrome
The post-resuscitation stage after ROSC is the period of highest risk of developing ventricular arrhythmias and reperfusion injuries. The International Liaison Committee on
Resuscitation consensus statement and current paediatric resuscitation guidelines now
emphasize the existence of the post-cardiac-arrest syndrome (PCAS). This syndrome is
the consequence of prolonged whole-body ischaemia and reperfusion affecting the brain
and myocardium. It also has systemic effects similar to those seen in severe sepsis.
PCAS can be divided into four phases with specific treatment goals:
immediate post-arrest (first 20 min)
early post-arrest (20 min to 612 h)
intermediate (612 to 72 h)
recovery phase (3 days onwards).
Chapter 23: Ongoing management of the successful arrest
221
Post-cardiac-arrest therapeutic hypothermia applied during the intermediate phase after

adult ventricular fibrillation has produced positive results in some studies. This has renewed
focus on specific therapies that could further improve survival and neurological outcome
after cardiac arrest (Table 23.2).
Table 23.2. Monitoring and treatment targets for the post-cardiac-arrest syndrome.
Post-cardiac- Monitoring
strategy
arrest
syndrome
Potential
therapy
Physiological
target
Pitfalls
Pyrexia
Core (rectal,
oesophageal and
bladder)
temperature
monitoring
Therapeutic
hypothermia or
strict
normothermia
Initially target
temperature
3337 C
Avoidance of
temperature
<32 C or >38 C
Seizures
Clinical signs
CFAM or EEG
monitoring
Benzodiazepines,
phenytoin,
phenobarbitone
Cessation of
clinical and subclinical seizures
Hypotension, overtreatment
Hyperoxia,
hypoxia
Saturation
monitoring, arterial
PaO2
Normoxia
100% O2 during
arrest
Titrate O2 to
achieve sats of
9496% post
arrest
New diagnosis of
congenital heart
disease
Hypercapnia,
hypocapnia
Arterial PaCO2,
ETCO2
Normoventilation Aim PaCO2 4.55.5

kPa
(normocapnia)
Cerebral
perfusion
Haemodynamic
monitoring
Fluid therapy
Inotropic support,
vasopressors
Head-up
45-degree
position
Physiological
age-specific
normal
parameters for
blood pressure
(see Chapter 19)
Consider need
for neuroradiology
(e.g. cranial CT
scan)
Myocardial
dysfunction
Clinical signs, heart

rate, blood pressure,
liver size, capillary
refill, central venous
pressure
Fluid therapy.
colloid, crystalloid
or blood products
as required
Inotropic support:
dobutamine or
adrenaline
Physiological age
specific normal
parameters
Urine output
>1 ml/kg
Improving lactic
acidosis
Potential acquired
heart disease (e.g.
myocarditis
or
cardiomyopathy)
CFAM, continuous functional amplitude integrated electroencephalography monitoring; EEG,

electroencephalography; sats, saturation; ETCO2, end-tidal carbon dioxide; BP, blood pressure.
The brain
The brain is the most vulnerable organ in the body when subjected to hypoxia and
ischaemia during cardiac arrest. Even the best, closed-chest standard CPR can only achieve
222
50% of normal cerebral blood flow. The cascade of neurologically damaging processes that
occur during cardiac arrest and over the following hours to days leads to neurodegeneration
by a number of mechanisms:
impaired cerebrovascular reactivity
cerebral oedema
cerebral hyperaemia
post-ischaemic biochemical cascades.
The extent of damage is dependent on numerous factors, including the duration of cardiac
arrest and the area of the brain affected. The subsequent brain injury can manifest as:
seizures
cognitive dysfunction
myoclonus
stroke
coma or persistent vegetative state
brain death.
Neurological assessment
Assessment and recording of the patients neurological status, both before cardiac
arrest and after ROSC, can aid prognostication. A short period of assessment prior to
administering intravenous sedation and neuromuscular blockade can help to ascertain the
neurological status. If possible, the following should be assessed:
formal recording of when the patient starts to gasp or make spontaneous respiratory
effort
the components of the GCS
pupillary size and pupillary reactivity.
Timing of sedation or anaesthesia and the use of neuromuscular blockade will depend on the
patients neurological status and haemodynamic stability after ROSC. Rarely, the patient will
wake up, demonstrate intact recovery and be rapidly extubated. Usually, the patient will
remain comatose, and require continued ventilatory and haemodynamic support. Sedation
will need to be adequate to manage the patient on the ventilator and aid additional
interventions, such as central venous access and targeted temperature management.
The heart
The paediatric myocardium is remarkably resilient following cardiac arrest and in cases
where the underlying problem is not related to the heart, such as asphyxial arrests, the heart
can recover function within 1224 hours.
In the immediate and early post-arrest phase, function is impaired due to profound
systemic vasoconstriction and cellular acidosis. Initial support for the myocardium involves
the following:
adequate fluid resuscitation
targeting normal blood pressure for age
monitoring of perfusion and central venous pressure to avoid circulatory overload
inotropic support of the myocardium to achieve these goals.
223
The choice of inotrope follows the same rationale as in adults, and depends on the balance
between inotropy and vasoconstriction. The former favours the use of adrenaline or
dobutamine, whilst the latter is best achieved with noradrenaline.
Specific interventions
Hyperthermia and hypothermia
Hyperthermia after cardiac arrest is common and there is strong evidence linking core
temperature above 38 C with worse neurological outcome. Hyperthermia should trigger
targeted temperature management to reduce the core temperature.
Targeted temperature management to produce mild hypothermia (3234 C) has been
used with success after adult ventricular fibrillation cardiac arrest. It has also been shown to
be safe and effective in neonates suffering hypoxicischaemic encephalopathy soon after
birth. Mild hypothermia after paediatric cardiac arrest is utilized by some paediatric critical
care units. Patients are cooled to a temperature of 3334 C for 24 to 48 hours, followed by
gradual, controlled rewarming. Avoidance of temperatures below 32 C is essential, as this is
associated with the following:
worse survival
arrhythmias
immune suppression
coagulopathy
infections.
Shivering should be prevented by the use of adequate sedation and neuromuscular
blockade.
During the discussion for tertiary transfer of the post-cardiac-arrest patient, a decision
on the target temperature should be made, whilst undertaking accurate and continuous core
temperature monitoring.
Various methods for targeted temperature management are available including:
surface cooling with wet blankets
servo-controlled air or fluid cooling blankets
boluses of cold intravenous fluids (10 ml/kg at 4 C over 10 minutes)
intravenous cooling catheters (unsuitable in smaller children).
Anticonvulsants
The incidence of seizures after paediatric cardiac arrest has been reported to be as high
as 47%, with 35% of patients in refractory SE. Seizures can produce further secondary
neurological damage by increasing metabolic demand. They should be identified quickly
and treated.
In post-cardiac-arrest patients who remain comatose, sedated and receiving neuromuscular blocking drugs, identification of seizures will require a high level of clinical suspicion,
unless continuous neurophysiological monitoring is undertaken. Clues include unexpected
changes in pupillary size and abrupt changes in heart rate or blood pressure.
Treatment of identified or suspected seizures will follow standard guidelines (see
Chapter 10).
224
Oxygen: hypoxia and hyperoxia

Maintaining adequate ventilation and oxygenation is essential during resuscitation and
post-resuscitation care. Hypoxia will cause continued secondary brain injury and must
be avoided. Hyperoxia (defined as PaO2 > 40 kPa) may also be associated with worse
survival. In children, the commonly occurring respiratory compromise preceding
cardiac arrest necessitates high-concentration oxygen therapy during the low-flow resuscitation and in the early post-resuscitation phase. Targeting oxygen saturations between
94 and 96%, with a reduction in FiO2, would appear appropriate whilst avoiding
hypoxia.
If anaemia or carbon monoxide poisoning is suspected, then the highest concentration
of oxygen should be administered.
Cardiac arrest in the infant may be secondary to unidentified congenital cardiac disease.
Initial resuscitation of these infants with a high FiO2 is appropriate; however, introduction
of alprostadil (Prostin) and reduction of FiO2 to 0.5 is necessary in a suspected or
confirmed duct-dependent circulation and should be discussed with a paediatric intensivist
or cardiologist (see Chapter 6).
Ventilation: hypocapnia, hypercapnia

During and after cardiac arrest cerebral autoreactivity is often lost; however, cerebrovasular
reactivity can be preserved. Therefore targeting normocapnia (PaCO2 4.55.5 kPa) through
controlled age-appropriate ventilatory rates is recommended. Hypoventilation may cause
hypoxia and raise intracranial pressure due to hyperaemia. Hyperventilation may cause
further cerebral ischaemia and risks raising intrathoracic pressure and impairing cardiac
venous return to an already stunned myocardium.
Metabolic control
Hypoglycaemia and hyperglycaemia
Post-cardiac-arrest paediatric patients are at high risk of developing hypoglycaemia (plasma
glucose < 3.0 mmol/l). Early glucose monitoring and correction with 2 ml/kg of 10%
dextrose followed by a continuous infusion of a glucose-containing solution is essential to
avoid neurological damage and seizures. Hyperglycaemia (plasma glucose > 13 mmol/l)
can also occur as a physiological stress response and is associated with worse outcome.
However, aggressive glucose control with insulin in the non-diabetic patient has not been
proven to be beneficial in the paediatric population and hyperglycaemia will usually correct
itself within a few hours.
Metabolic acidosis
Profound metabolic acidosis is common during cardiac arrest and after ROSC. However,
the routine use of sodium bicarbonate solution is not recommended. Evidence in other
critically ill states (e.g. adults with septic shock) has failed to show improvements in
haemodynamics when metabolic acidosis is treated with sodium bicarbonate. However,
acidosis may depress the action of catecholamine, so treatment may be considered in the
following situations:

225
acidosis with resistance to catecholamine therapy

cardiac arrest secondary to tricyclic antidepressant overdose
hyperkalaemia
hypermagnesaemia.
Sodium bicarbonate administration will need adequate ventilation, due to the production of
carbon dioxide.
Further investigations and child protection

During the stabilization and management of the post-cardiac-arrest syndrome further
investigations and clinical history will be required to guide ongoing therapy. Table 23.3
outlines, with reasons, useful investigations for use after all cardiac arrests in infants and
children, with suggestions for additional investigation in certain circumstances.
Blood tests
Routine blood sampling for haematological and biochemical blood tests, blood glucose
analysis and arterial blood gas analysis will allow rapid correction of physiological derangement and allow manipulation of the post-cardiac-arrest syndrome treatment strategies.
Additional blood testing is indicated for specific conditions such as:
toxicology for poisoning
carbon monoxide assessment post-burns or fires
metabolic investigations (especially plasma ammonia and lactate where inborn error of
metabolism is suspected).
Other investigations
A chest radiograph will be helpful to assess ET and NG tube placement. If there is any
suspicion of head trauma, an acute intracranial bleed or raised intracranial pressure, urgent
cranial computed tomography scans should be arranged.
Child protection considerations

In all out-of-hospital cardiac arrest situations involving infants and children, consideration
should be given to the initiation of full child protection investigation proceedings (see
Chapter 30). Any infant or child who dies unexpectedly in the UK is subject to a sudden
unexpected death in infancy (SUDI) investigation by a trained team of clinicians, social
workers and police. A near-miss unexpected death, as is the case in most resuscitated
cardiac arrests, should undergo the same level of investigation to ensure non-accidental
injury is not missed as a cause of cardiac arrest. Accurate history-taking and recording
of information during and after resuscitation will be invaluable, not just in the cases of
non-accidental injury, but also in helping families during the aftermath of the cardiac arrest
if the child dies.
226
Table 23.3. Post-resuscitation investigations.
For all
cardiac arrests
Additional
investigations
if indicated
Post-resuscitation investigation
Reason
Full blood count and clotting

screen and group and save for
cross-match
Baseline haematology
Sodium, potassium, urea and

creatinine
Baseline biochemistry
Risk of hyperkalaemia and renal failure
Arterial blood gas analysis
Targeting oxygenation and carbon

dioxide level
Assess acidbase status
Rule out carbon monoxide exposure
(if required)
Glucose
Hypoglycaemia, hyperglycaemia
Chest radiograph (CXR)
Post-intubation tube check,

Assess underlying lung pathology
Electrocardiograph (ECG)
Evidence of myocardial ischaemia/

infarction
Underlying arrhythmias (prolonged QT
interval)
Microbiology cultures: blood and/

or urine cultures
Investigation of sepsis with prompt,

appropriate antibiotic treatment
Computed tomography (CT)
Rule out acute intraventricular bleed or

hydrocephalus. Also consider chest or
abdominal pathology
Child protection investigation
Involving local child protection team,

social services and police should be
considered promptly in ALL out-ofhospital cardiac arrests in children
Further investigations for unknown

cause of cardiac arrest
Toxicology screen (urine and blood)

Inborn error of metabolism screen
Retrieval and transportation considerations

Post-cardiac-arrest patients with sustained ROSC will require transportation to a paediatric
critical care unit for a number of reasons:
continued management of their post-cardiac-arrest syndrome
ongoing investigation and treatment of the underlying cause for the cardiac arrest
sufficient time to allow accurate prognostication of the likely survival outcome.
Stabilization and preparation for transportation of the critically ill child are covered in
greater detail in Chapter 24.
In the post-cardiac-arrest scenario, early discussion with the destination intensive care
unit regarding appropriate physiological targets can be helpful. During transportation the
parameters detailed in Table 23.4 should be maintained:
227
Table 23.4. Targets for physiological parameters.
Ventilation to achieve normocapnia (PaCO2 4.55.5 kPa)

Oxygenation to target arterial saturations of 9496%
Myocardial and circulatory support to maintain age-appropriate normal haemodynamic values
ECG monitoring for arrhythmias
Neurological assessment of seizures and GCS
Continued core temperature monitoring to target temperature range (3337 C)
Summary
The successful resuscitation of an infant or child is just the start of an important phase in
post-cardiac-arrest care. During the period of stabilization prior to transportation to a
paediatric intensive care unit, monitoring and early management of the post-cardiac-arrest
syndrome can potentially limit further damage to the brain and other vital organs. The
historically poor survival and neurological outcome seen after paediatric cardiac arrest can
be improved by applying the increasing body of evidence-based practice outlined in this
chapter.
Golden rules

Monitor temperature continuously

Pay close attention to electrolytes and glucose
Be aware of potential for seizures
Once things have settled, consider further investigations and child protection issues
Contact PICU early and set targets for temperature and ventilation
Further reading
Berg R, Zaristky A, Nadkarni V. Paediatric
cardiopulmonary resuscitation. In
Fuhrman BP, Zimmerman JJ (eds.),
Paediatric Critical Care, 3rd edn. St Louis:
Mosby, 2006.
The Royal College of Pathologists and The

Royal College of Paediatrics and Child
Health. Sudden unexpected death in
infancy. A multiagency protocol for care
and investigation. http://www.rcpch.ac.
uk/sites/default/files/SUDI_report_for_web.
pdf.
Samuels M, Wieteska S (eds.). Advanced

Paediatric Life Support, 5th edn. Oxford:
Blackwell, 2011.
Section 3
Chapter
24
Referring-team-led transfers
Andrew J. Baldock and Gareth D. Jones
Introduction
Transfers conducted by referring teams are usually performed for neurosurgical emergencies. Occasionally clinicians from a referring hospital may also be asked to perform
transfers to PICU for non-neurosurgical patients.
This chapter aims to provide a reference point for those individuals tasked with fulfilling
these obligations. The emphasis is on neurosurgical transfers, but the same principles apply
to other pathologies. Further details on the management of specific conditions can be found
in the relevant chapters.
Planning for transfers

Children who need to be transferred to a tertiary centre must be identified early and
referred to the regional specialists, e.g. neurosurgeons and paediatric intensive care teams,
at the first available opportunity.
The decision to transfer a child must be taken by senior clinicians in each hospital, and
only after appropriate resuscitation and stabilization have taken place. Once the child has
been stabilized, and the decision taken to transfer, management is focussed on the
prevention of secondary injury and on the safety of the child in transit to the definitive
centre.
In anticipation of the above scenario local guidelines should be written for paediatric
transfers. They should contain:
clear contact information for the regional referral teams
defined management goals
named personnel who will undertake the transfer (e.g. senior anaesthetist and trained
assistant)
details on how these staff will be freed from other duties
details of the equipment to be used during transfer for children of all ages.
In the case of neurosurgical emergencies, they should be written as a collaboration between
local hospitals and the regional neurosurgical and critical care services. The local ambulance
service should also contribute to such guidelines, in order to respond appropriately to
requests that the neurosurgical team have defined as time-critical.
228
Chapter 24: Referring-team-led transfers
229
Preparing the patient for transfer

Anticipating problems can make them a lot easier to manage. Table 24.1 addresses common
questions that face the clinician when preparing a patient for transfer.
Table 24.1. Likely management questions when preparing for transfer.
Management questions for transfer
Answers
Should I intubate?
Consider intubation if:

There is an obvious clinical indication at
presentation
The patient is deteriorating despite treatment
There is any chance that the patient will need
intubation during the transfer
It is being used as a treatment modality; e.g. control
of CO2 in head injury or oxygen delivery in carbon
monoxide poisoning
Do I need a nasal ET tube?
No. A well secured oral ET tube will usually suffice

and a nasal tube may be contraindicated
What sedation should I use?
Whatever the transfer team is happy to use, as long

as it is age-appropriate. Morphine and midazolam are
commonly used in paediatrics. Propofol is
controversial in children, but can be used in the short
term
Should I paralyse the patient?
Most retrieval teams will routinely paralyse

patients for transfer (with an infusion of muscle
relaxant)
Do I need an arterial line?
Consider if:
Cardiovascularly unstable
Non-invasive readings are unreliable
Accurate blood pressure readings are essential
Arterial line should not delay a timely transfer
in time-critical conditions, but can be hugely
beneficial
Do I need a central line?
Consider if:
IV access is difficult/inadequate
Inotropes are being administered
CVP monitoring is necessary to guide treatment
Central line should not delay a timely transfer as IO
needles can be used for most access/drugs
How much of everything do I need?
Make certain that you have enough oxygen/infusion

drugs to last at least twice the expected duration
of transfer, and anticipate traffic or ambulance
breakdown
230
Table 24.1. (cont.)
Management questions for transfer
Answers
When should I contact the destination

hospital
Contact:
As soon as the need for tertiary intervention
is recognized
Immediately prior to leaving your hospital
10 minutes before arrival
What problems can occur specific to

transfers?
Hypothermia
Measure temperature throughout
Lack of assistance
Make sure that a mobile phone is at hand throughout
Limited supplies (see above)
Difficult to get to patient
The ambulance should stop where safe if any
intervention is being carried out
Intubation and spinal protection

A controlled intubation pre-departure is likely to be safer than attempting to secure an
airway en route. As a result, any patient with a GCS < 9 or a fall in GCS of more than 3
should be intubated for transfer. Similarly a low threshold should be applied to any patient
whose respiratory or cardiovascular function is likely to deteriorate en route.
Triple immobilization with a hard collar, blocks and tape should be instigated for head
injuries, and maintained throughout the transfer. A NG tube should be considered for all
patients prior to transfer.
Ventilation
All patients requiring ventilation for transfer should be adequately sedated and paralysed.
This is standard practice for many paediatric retrieval teams because of the risk of
accidental extubation. End-tidal CO2 monitoring should be used throughout transfer to
confirm ET tube placement and to monitor the efficacy of ventilation.
The patient should be placed on the transport ventilator for long enough to highlight
any difficulties prior to departure. Before leaving, blood gases should be checked and the
difference between ETCO2 and PaCO2 measured (to guide ventilator management during
the transfer). Although the difference between the two is unlikely to remain constant, it
helps to establish a relationship prior to departure.
During transfer, if gas exchange deteriorates, the management remains similar to that
in hospital:
ventilate the patient manually
ensure that they are adequately sedated and paralysed
check that the ET tube has not moved or kinked
use an appropriately sized catheter to suction the ET tube (see Chapter 3)
the slope on the upward phase of the ETCO2 may be the first obvious sign of airway
obstruction
assess for evolving lung pathology (e.g. pneumothorax).
231
Circulation
The occurrence of hypotension should be anticipated during the course of a transfer and
prepared for. Again the management follows the same principle as in hospital events.
In cases where it is uncertain whether inotropes/vasopressors are needed they should be
made up and attached to the patient prior to departure. Running these drugs at low rates,
e.g. 0.1 ml/h, will help to overcome the dead space in lines and infusion pumps and reduce
the time lag once started.
Access
Where possible at least one cannula should be reserved for fluid boluses and intermittent
drug administration during transfer, e.g. 3% saline to manage intracranial hypertension.
This should be attached to an extension line with a three-way tap at the doctor/nurse end, so
that boluses can be given without having to stop and get out of the ambulance seat.
Central lines may be considered for transfers, but should not be considered essential.
For time-critical transfers it is important that attempts to establish central venous or arterial
access do not slow down the process of transferring the patient for definitive management,
unless the benefits are seen to outweigh the risks. Remember that inotropes and vasopressors may be administered by the intraosseous route.
Adrenaline and inodilators can be administered in weak concentrations peripherally
(e.g. at a quarter of the strength delivered centrally see Chapter 36), although the
access must be good and monitored regularly throughout transfer due to the risk of
extravasation. If there is any doubt about the quality of the cannula, insert an IO needle
or central line for inotrope delivery. The regional paediatric retrieval team can be contacted
for advice.
Indications for an arterial line are the same as for any sick child. Consideration should
be given to the fact that automatic non-invasive blood pressure cuffs can use a lot of energy
(battery power) on portable monitors and can become unreliable on bumpy roads. However, as with central lines, siting an arterial line should not be allowed to slow down a timecritical transfer.
Sedation
Midazolam and morphine infusions are commonly used in paediatrics for all children over
3 months. Younger children are usually managed on morphine as a sole agent. The doses
and infusion are detailed in Chapter 36. As with adult patients, sedation should be titrated
to effect as sedative drugs may contribute to hypotension. This is especially true for
midazolam in small children. Whichever drugs are used, it is helpful to prepare infusions
using the same drugs and concentrations that are used in the regional PICU to facilitate
handover.
Maintenance fluids
Maintenance intravenous fluid should be started if the child is not already receiving too
much fluid (see Chapter 32). The choice of fluid will be dictated by the pathology, e.g. headinjured patients should receive isotonic fluid (0.9% saline is preferred). Patients over 10 kg
are unlikely to require the addition of dextrose to maintenance fluid. Both hyperglycaemia
and hypoglycaemia may worsen the outcome of neurological insults.
232
Temperature control
Hypothermia is another serious issue for children during transfer. They should be well
covered and kept warm throughout (with the obvious exception of therapeutic hypothermia; see Chapter 23). Temperature should be monitored throughout the transfer.
Equipment and monitoring

It is vital that hospitals identify suitable transfer equipment as part of their advanced
planning for critical care transfers. It should be readily available, checked regularly and
fully charged (see Chapter 3). All equipment should be durable, lightweight and able to
operate on battery as well as mains power. Spare batteries should be available.
During transfer, children should be monitored according to the recommendations of
the Association of Anaesthetists of Great Britain and Ireland (AAGBI). This should include
the items listed in Table 24.2.
Table 24.2. Basic monitoring.
ECG
Pulse oximetry
Capnography
Temperature
Non-invasive and invasive blood pressure
Clear illuminated display screen
Audible and visible alarms on all values
Other equipment needed includes:
Self-inflating bag and mask connected to a dedicated oxygen cylinder
Portable suction
Battery-powered infusion pumps.
Fluid boluses pre-drawn in separate syringes
All equipment should be secured below the level of the patient.

The child must be accompanied by a minimum of two suitably trained people, one of
whom will usually be a senior anaesthetist or intensivist capable of securing the airway and
managing likely complications during transfer.
Most transfer vehicles will be provided by the local ambulance service and will take place
in type B (or equivalent) vehicles. These ambulances have 12 V electric sockets and an
oxygen supply. The transfer team should expect to provide all other equipment. Ensure
there is plenty of oxygen and battery life.
Safety
The majority of critically ill children are retrieved without the use of blue lights and sirens.
However, this may be necessary for time-critical transfers. The aim should always be to
complete the transfer swiftly but safely.
Excessive acceleration or deceleration can cause physiological instability and further
rises in ICP. The journey should be as smooth as possible. All personnel should be seated
and wearing seatbelts. Unforeseen medical interventions should be carried out with the
233
vehicle stopped in a safe place. However, undue delays are likely to be to the detriment of
the patient and should be minimized.
What do I need to have organized before departure?

This is best addressed with a checklist, an example of which is given in Table 24.3. A time-out
with the team before departure, to go through the checklist, will not add much time to the
transfer, and may avoid any undue delays.
Table 24.3. Pre-departure checklist.
1. Patient accepted by neurosurgical centre and destination confirmed

(This may be straight to the operating theatre)
2. Referral made to regional paediatric intensive care unit
3. Ambulance service notified of time-critical transfer and confirmed immediate response
4. Appropriate transfer team personnel identified
5. Airway secured, ET tube position confirmed on CXR and C-spine immobilized
6. Adequate IV access: two peripheral cannulae or triple-lumen central line and a dedicated
extension line for drug and fluid boluses
7. Adequate monitoring: ECG, SpO2, ETCO2, temperature, NIBP, IBP
(Do not delay departure if unable to insert arterial line)
8. Physiological targets achieved:
SpO2 > 95%
Age-appropriate CPP
PaCO2 4.55.0 (or ETCO2 4.04.5)
9. Well sedated and muscle-relaxed
10. Drug and fluid infusions and boluses pre-prepared
11. Essential equipment available:
Self-inflating bag and mask with dedicated portable oxygen cylinder
Adequate oxygen
Portable suction unit and suction catheters
Age-appropriate airway bag
Mobile telephone
12. Case notes, X-rays, CT scan, blood results (including clotting studies)
13. Cross-matched blood if available (in a suitable transport container with appropriate
paperwork)
14. Parents fully informed and arrangements made for transport to the neurosurgical centre.
Contact details documented
15. Telephone neurosurgical and PICU teams immediately prior to departure
Constant communication with both the neurosurgical and intensive care team is
essential, and may best be achieved through mobile telephones with the relevant numbers
loaded into their memory.
234
Summary
A swift and safe transfer of a critically ill child takes planning. It should not be carried out in
a rush; instead it should be carried out by senior clinicians in a measured manner. The aim
is to bring a child to definitive care in the safest possible way, with minimal delay. Advice
should always be available from the local PICU retrieval team even if they are not carrying
out the transfer.
Golden rules

Policies, procedures and equipment must be in place before the event

Do not delay transfer of the child by undertaking unnecessary procedures
Suitably skilled personnel must accompany the child
Good communication is essential
If in doubt, secure the airway
Further reading
Association of Anaesthetists of Great
Britain and Ireland. Recommendations
for the Safe Transfer of Patients with
Brain Injury. London: Association of
Anaesthetists of Great Britain and
Ireland, 2006.

and Ireland. Interhospital Transfer. London:
and Ireland, 2009.
Nichols DG (ed.). Rogers Textbook of Paediatric
Intensive Care, 4th edn., Philadelphia:
Lippincott, Williams & Wilkins, 2008.
Section 3
Chapter
25
Anaesthetizing for a surgical

emergency
Andy Tatman and Richard Pierson
Introduction
The majority of sick children requiring surgery are transferred to a tertiary centre.
However, there may be times when this is not possible; the child may be too sick to move,
the surgery may be time-critical or the retrieval team unavailable.
In these situations, high-quality care in the local, non-specialist centre is crucial. Delays
in transfer to a tertiary centre and insufficient confidence of the local team to operate have
been highlighted in the 2011 National Confidential Enquiry into Patient Outcome and
Death (NCEPOD) as contributing factors in paediatric surgical mortality.
The aim of this chapter is to give practical guidance to the non-paediatric anaesthetist
on how to manage a sick child requiring emergency surgery.
How do surgical emergencies differ in children and adults?

Preoperative phase
Presentation
Small children often present late in the disease process. Consequently, by the time a child
presents to the ED, they may be very sick, with hypovolaemia, acidosis and respiratory distress.
They may, for example, have swallowed a great deal of blood from their bleeding tonsillar
bed or have been vomiting for several days, from an appendicitis or intussusception.
Children have the ability to compensate for shock to the point of cardiovascular
collapse. This compensation is achieved through increased heart rate and vasoconstriction.
Hypotension and lethargy are signs of decompensating shock, whereas bradycardia is a sign
of impending cardiorespiratory arrest.
Optimization
The key to optimization is to recognize the sick child (see Table 25.1), give high-flow
oxygen, control the airway and the breathing and then give rapid fluid resuscitation,
followed by prompt surgical correction of the underlying problem.
Children tend not to suffer from degenerative conditions such as atherosclerosis; therefore, adequate resuscitation with oxygen and intravenous fluids usually results in a rapid
improvement, without the need for inotropic support or invasive monitoring. If there is any
doubt about the adequacy of resuscitation, give a further fluid bolus and reassess the child.
Failure to respond to these interventions is a grave sign and should set alarm bells ringing.
235
236
Table 25.1. Recognizing a sick child preoperatively.
System
Preoperative findings
Anaesthetic significance of these problems
Respiratory
Tachypnoea
Low saturations
Grunting
Recession
The child may have these signs as a result of primary

lung pathology, secondary to abdominal pathology,
or as a compensatory response to a metabolic acidosis
Problems may include:
Rapid desaturation on intubation
Difficulty in ventilating
High PEEP requirement
Severe metabolic acidosis
Cardiovascular Tachycardia
Cool peripheries
Mottling of skin
Poor central capillary refill
Hypotension (late)
Bradycardia (terminal)
Intense vasoconstriction and tachycardia can

compensate for marked hypovolaemia in children
Problems in these children include:
Difficult IV access
Decompensation on administration of anaesthetic
agents
Multi-organ failure
Hypothermia
Renal
Oliguria (dry nappies)
Oliguria signals an acute kidney injury

Problems may include:
Acidosis
Electrolyte disturbance
Difficulty in managing fluid balance
Altered drug handling
Neurological
Drowsiness
Lethargy
Lack of response to
painful stimulus, e.g.
cannulation
These signs point to poor brain perfusion, a tiring

child, or a marked metabolic disturbance
Significance of these signs:
Anaesthetic requirement may be reduced
The child may tire and decompensate before theatre
Other
problems
Hypoglycaemia
Hypothermia
Coagulopathy
Thrombocytopenia
These problems will need addressing immediately

before or during surgery
Psychology
Younger children who are ill are usually frightened and their fear can be exacerbated by
parental and staff anxieties. This is best managed by:
staying calm
minimizing the number of people in the room
explaining what you are doing to the child, parents and staff
minimizing the number of interventions (cannulae, blood tests, NG tube) to those that
are absolutely necessary
having experienced staff perform interventions.
Reassurance, distraction techniques and the use of local anaesthetic cream should all be
considered. However, life-saving interventions should not be delayed by fear of causing
transient pain or distress.
Chapter 25: Anaesthetizing for a surgical emergency
237
Consent
When taking consent for an operation on a very ill child, it is important that the consent
process includes a clear, and documented, discussion of the risks, including the risks from
complication of central venous access, blood transfusion, admission to intensive care and
the risk of death.
Intraoperative phase
Intravenous/intraosseous access
IV access should include a smaller cannula, for drugs and maintenance fluid, and a larger
cannula for volume replacement. The size of cannula should be determined by the size of
the child and the underlying condition. In an infant, a 24G cannula is useful for drugs and
can be used to start resuscitation fluids, but an additional cannula, preferably 22G, should
be inserted prior to starting surgery. See Figure 25.1 for suggested sites for insertion.
Figure 25.1. Suggested sites for cannula insertion.

238
If IV access is unsuccessful, intraosseous (IO) access should be considered early, even in

a conscious child. It enables early resuscitation and fewer damaged veins, making subsequent IV cannulation easier.
Central venous access is unnecessary in most paediatric surgical emergencies. It should
not be the first line of IV access. It should only be performed on an intubated, sedated and
paralysed child, unless they are old enough to understand and cooperate with the intervention. A large-bore, single-lumen catheter is more effective than a multiple-lumen catheter
for resuscitation.
Intubation and ventilation

The attending physician should have a low threshold for intubation and ventilation in any
critically ill or injured child. The indications are well-recognized and may include:
airway compromise
cardiovascular instability
respiratory failure
reduced consciousness level
need for investigations or interventions
surgery
transfer.
It should be remembered that the presence of a leak around the ET tube is not necessary,
and there is no evidence of an increased incidence of post-extubation stridor when there is
no leak, provided the tracheal tube passed easily. It is also unnecessary to cut the tracheal
tube, as the effect on dead space is minimal.
An example of initial ventilation settings for smaller children is given below:
pressure-control ventilation (PCV) for small children (uncuffed ET tube)
rate of 20 breaths per minute
I:E ratio of 1:2
pressures of 20/5 cmH2O
titrate to a tidal volume of 57 ml/kg (up to 10 ml/kg if adequate chest movement is not
seen in small children).
PCV will compensate for small leaks around the tracheal tube and will ventilate with
appropriately small tidal volumes. Some volume control ventilators may be unable to do this.
Be careful when interpreting the measured tidal volume and ETCO2. If there is a leak
around the tube, it is likely that the tidal volume measured on the ventilator will read less
than what is being delivered. The ETCO2 will also under-read in this situation. Checking a
blood gas will confirm this.
A small infant may require a tidal volume of more than 10 ml/kg to achieve normocapnia (ETCO2 5.0 to 6.0 kPa), as the tidal volume will include the volume change of the
tubing and any dead space in the system. The most important guides to the adequacy of
ventilation are chest wall movement and arterial carbon dioxide tension.
Positioning
Gaining access to a child during an operation can be difficult. Figure 25.2 shows how a child
might be positioned for abdominal surgery for optimum access and warmth. The hand that
239
has a cannula sited on it should be rested by the childs head, as if saluting. This can provide
easy access for assessing capillary refill time and cannula patency.
Capillary
refill time
Hemocue/glucose SpO
2
skin prick
Eyes taped
3-way tap
ET tube
Venous access
Nasopharyngeal
temperature
probe
Oral ET tube
Blood pressure cuff
Diathermy plate
Plastic sheets over

patient to maintain
heat
Venous access
22G in long
saphenous vein
(Volume line)
Warming mattress
Figure 25.2. Positioning a child for abdominal surgery for optimum access and warmth.
Blood from a finger prick to measure blood sugar and haemoglobin can also be taken
easily. A proximal, visible, cannula also reduces the volume of dead space in the IV line
when giving drugs
Intravenous fluids
IV

fluid therapy can be divided into:

correction of hypovolaemia, i.e. circulating volume
correction of fluid deficit, i.e. total body fluid
maintenance fluids.
240
When anaesthetizing a sick child for a surgical emergency, it is often difficult to calculate
the deficit. In the perioperative period, it is simpler to correct hypovolaemia and replace
ongoing losses while ignoring the deficit.
Correction of hypovolaemia
Hypovolaemia should be corrected with 1020 ml/kg boluses of warmed fluids (0.9% saline,
Hartmanns solution or colloid) with attention paid to the response in heart rate, capillary
refill time, peripheral temperature and blood pressure. If there is doubt about the adequacy
of resuscitation, a further bolus of 10 ml/kg should be given, as the vast majority of
problems associated with the adequacy of resuscitation are due to too little volume, rather
than too much.
Blood transfusion
An [Hb] greater than 70 g/l, in an otherwise well child who is not actively bleeding, usually
does not require transfusion. However, intraoperative [Hb] measurement should be performed at regular intervals.
If a transfusion is necessary, 10 ml/kg of packed cells will raise the childs [Hb] by
about 2025 g/l. Once the child has been exposed to a unit of blood, it is desirable to
continue to transfuse from that unit, up to an [Hb] of 120140 g/l, thereby minimizing
the likelihood of requiring a further transfusion, from another donor, later in their hospital
stay.
Care must be taken to avoid an excessive transfusion through an open three-way
tap. One way to avoid this is to keep a three-way tap in the giving set (closed as the default)
and administer the blood aliquots by drawing the correct volume into a syringe before
giving it.
Correction of deficit
Do not try to correct the deficit in the operating theatre. The deficit should be corrected
over 24 to 48 hours, with the volume and choice of fluid being guided by clinical signs and
electrolytes respectively.
Maintenance fluids
Intraoperatively, all IV fluids should be isotonic, with Hartmanns preferred due to its lower
chloride content. For small infants, where hypoglycaemia may be an issue, Hartmanns
with 2.5% dextrose is an alternative. This can be made up by adding 25 ml of 50% dextrose
to 500 ml of Hartmanns solution. See Chapter 32 for calculating fluid maintenance
requirements.
Postoperatively, 0.45% saline with 5% dextrose, prescribed at two-thirds maintenance
requirements, is a suitable fluid. Fluid losses in excess of calculated maintenance volumes
(such as NG and large urinary losses) should be replaced with isotonic crystalloid to
minimize the risk of hyponatraemia.
Temperature control
Having a large surface area with small core mass, small children can get cold very quickly.
Keep them covered whenever possible, but not at the expense of assessing vital parameters,
241
such as chest movement. Clear plastic sheeting, covering the upper body and legs, is useful
in this respect.
Other factors which should be addressed to ensure normothermia are:
the theatre must be warm before bringing the child in
blood products and fluids should be administered through a blood warmer
use external warming devices
core temperature measurement is essential.
Forced warm-air devices will provide adequate warming for most cases. However, these
devices are very powerful, and can result in rapid heating of a small child, with the potential
for serious hyperthermia. Electrically heated mattresses will provide adequate heating for
children, over 1 year of age, lying supine. The mattresses have a lag time to warm the child,
and often the child continues to warm after the mattress has been turned off, so it may be
necessary to turn it off before the child reaches the desired temperature. Remember, if there
is a leak around the ET tube, the nasopharyngeal temperature probe will under-read, due to
cooling, so it is often better to pass it into the upper oesophagus.
Glucose monitoring
Blood glucose should be checked at least once perioperatively in all sick children. Some
anaesthetists routinely use Hartmanns solution with 2.5% glucose in infants. If hypoglycaemia is present, this should be corrected with 2 ml/kg of 10% dextrose, and the maintenance
fluid changed to a non-hypotonic glucose-containing fluid, such as 5% glucose in 0.9% saline.
Inotropes
Inotropes should be considered if hypotension persists, despite apparently adequate resuscitation. The indications for the choice of inotropes are the same as in adults. Adrenaline
should be given via a central line, but if this route is not available it may be given
peripherally in extremis, as a quarter-strength solution and ideally with the maintenance
fluid, to minimize the risk of peripheral ischaemia. It should be changed to a central route
as soon as that route becomes available.
Postoperative care
Following emergency surgery, children should be managed in an appropriate environment.
This is particularly so at night, when a child may be better cared for on an HDU ward or
transferred to a PICU postoperatively.
Examples of surgical emergencies and their management

Postoperative tonsillar bleeding
Postoperative tonsillar bleeding occurs in 12% of elective tonsillectomies. It occurs either
as primary haemorrhage (within 24 hours) or secondary (usually 512 days post surgery).
Common problems with anaesthetizing a child with bleeding tonsils are:
difficulty in quantifying the degree of blood loss
potential for significant hypovolaemia
242
blood in the stomach, which is emetogenic and results in an increased risk of aspiration
intubation may be difficult due to an obscured view or laryngeal oedema
the child and parents may be very anxious
a general anaesthetic earlier in the day may mean that a child is excessively restless or
drowsy.

Table 25.2. Stages in anaesthetizing a patient with a bleeding tonsillar bed.
Phase of management
Actions
Preoperative phase
Resuscitate the child before going to theatre, unless

significant haemorrhage
Give 10 ml/kg fluid boluses and monitor response
Repeat fluid boluses as necessary
Send a full blood count
Cross-match urgently
Consider blood transfusion
Get help from senior anaesthetist
Prepare anaesthetic room with:

Two suction devices
Two ET tubes (same as previous size and a smaller one)
Rapid-sequence inductiona
Postoperative phase
Pass large-bore NG tube and empty the stomach before

extubation
Adequate analgesia (taking into account previous
anaesthetic)
Extubate in left lateral position
Consider high dependency care
Effective preoxygenation may be difficult in an anxious child, so gentle positive-pressure ventilation may be
necessary, prior to intubation.
Traditionally, bleeding tonsils received an inhalational induction. If the child is hypovolaemic, there is rapid equilibration between the inspired concentration of sevoflurane and
the blood, which may result in excessive anaesthetic depth with the potential for severe
decompensation and cardiac arrest. The child should be adequately resuscitated before
induction of anaesthesia. Most anaesthetists would now advocate performing a rapidsequence induction (rather than a gas induction) unless there is a specific indication, i.e.
previous difficult intubation.
Perforated appendix
Only 5% of cases of appendicitis occur in children under 5 years. However, the risk of
perforation is double for this age group, so small children may be very ill when they arrive
for surgery.
243
Table 25.3. Stages in anaesthetizing a child with perforated appendix.
Phase of management
Actions
Preoperative phase
Resuscitate the child before going to theatre

If capillary refill prolonged give fluid boluses
Ensure antibiotics have been started
Rapid-sequence inductiona
Titrate fluids to heart rate and clinical signs
Start maintenance fluid
Give gentamicin if faecal contamination of abdomen (7 mg/kg)
Manage sepsis and hypotension as in previous chapters
Postoperative phase
Adequate analgesia (usually 100 g/kg of morphine followed

by NCA/PCA)
Consider ITU if:
Persistent tachycardia
Tachypnoea
Cool peripheries
Hypotension (despite 3040 ml/kg fluid)
Temperature > 39 C
Intra-abdominal sepsis
If hypovolaemia is suspected, the dose of induction agent should be reduced accordingly.
The child should stay in recovery until fully recovered, with stable cardiorespiratory
parameters. Persistent tachycardia, tachypnoea and cool peripheries are indications that
the child needs further resuscitation. Significant systemic sepsis following a perforated
appendix is uncommon. Remember that a perforated appendix is a surgical emergency,
and delay in getting to the operating theatre can result in a fatal outcome, as was highlighted
in the 2011 NCEPOD report.
Intussusception
This presents most commonly in infants. The majority of intussusceptions can be reduced
by air enema, thereby avoiding the need for surgery. Those that cannot be reduced will
require a laparotomy. Some children, particularly those presenting with a longer history,
may have significant fluid loss and be developing sepsis secondary to the presence of
necrotic bowel. The management is similar to that for children with perforated appendicitis,
with some children requiring 3040 ml/kg of colloid for resuscitation. Blood transfusion
may be necessary, particularly where there is necrotic bowel. These children rarely require
invasive monitoring. Usually, by the end of surgery, the child looks remarkably well.
Tertiary hospital advice and transfer

Anaesthetic advice and support is always available from the tertiary paediatric hospital,
whether it is just to confirm that the anaesthetic plan is reasonable, or for a more detailed
discussion of what to do.
If the child is to be transferred, ensure the patients notes are up to date and all drugs
have been documented, including fluid boluses, inotropes and antibiotics. Document all
244
investigations and results, including radiology and laboratory investigations, and include
any imaging, either as a hard copy or on a disk.
Ensure that the tracheal tube is correctly placed and well secured, with the position
being confirmed by a CXR. If the child is to be retrieved by the tertiary centre, and is stable,
do not feel pressured to put in additional venous access (peripheral or central) prior to the
arrival of the retrieval team, as, if unsuccessful, you may make the retrieval teams job more
difficult.
Summary
Anaesthetizing a child for emergency surgery in a DGH is rare. If it is needed, the same
principles as for an adult anaesthetic apply. The biggest problem is access to a draped child.
Take a moment before allowing the surgeons to start to ensure that you have adequate
access to the child.
Golden rules

Ensure adequate access to the child throughout the operation

Make sure that you can access the cannulae and pay particular attention to temperature
and blood glucose
Make sure that the airway is satisfactory before allowing the drapes to be placed, i.e. not
endobronchial intubation
Pay attention to blood loss, it is easy to underestimate
Further reading
Fleming S, Thompson M, Stevens R, et al.
Normal ranges of heart rate and
respiratory rate in children from birth to
18 years: a systematic review of
observational studies. Lancet
2011;377:10111018.
Nafiu O, Voepel-Lewis T, Morris M, et al. How

do pediatric anesthesiologists define
intraoperative hypotension? Pediatr Anesth
2009;19:10481053.
NCEPOD. Are We There Yet? A Review of
Organisational and Clinical Aspects of
Childrens Surgery. NCEPOD, 2011
Section 3
Chapter
26
Managing children on an adult

critical care unit
Helga Becker
Introduction
Since the publication of the Department of Health Report titled Paediatric Intensive Care:
A Framework for the Future in 1997, a gradual redistribution of surgery for children
towards specialized childrens hospitals has occurred. In many regions, paediatric referral
networks have been developed. These have a lead centre that provides specialist tertiary
services and a 24-hour paediatric retrieval service.
Despite the availability of these services, all hospitals that admit children must provide
the facilities and expertise to:
resuscitate children
deliver high-dependency care
initiate intensive care
stabilize prior to transfer to the tertiary centre.
The advent of paediatric retrieval services has reduced the number of paediatric emergency
admissions to adult critical care units (ACCU). Often the initial management and stabilization of critically ill children are now performed in the emergency department or on
paediatric wards until the arrival of the retrieval team.
Admissions of critically ill children to ACCU do still occur and in selected cases it may
be deemed appropriate that such children are cared for on adult units until their need for
intensive care support has ceased.
This chapter illustrates the issues surrounding the care for critically ill children in this
setting.
Standards for the unit

Any ACCU that agrees to admit critically ill children is well advised to have a basic
infrastructure in place to support this. In 2010 the Paediatric Intensive Care Society
published standards for the provision of paediatric critical care, including critical care units
in district general hospitals (DGH).
In most regions local standards have also been agreed, e.g. Standards for The Care of
Critically Ill and Critically Injured Children in the West Midlands (Version 3). A yearly
self-assessment to ensure ongoing maintenance of good standards of care and up-to-date
facilities should be performed on the critical care unit and within the hospital.
245
246
Multidisciplinary approach
Any ACCU that looks after children should have a consultant with special interest in
paediatric critical care. This is often an intensivist who both has a background in anaesthetics and regularly anaesthetizes children electively, or who has had exposure to paediatric
critical care during general intensive care training.
Good links with the lead clinician for paediatric anaesthesia, the paediatric lead for highdependency care and the resuscitation officer will allow the introduction of protocols and
equipment specific to paediatric anaesthesia and paediatric critical care. These specialists
should be members of a hospital-wide critical care delivery group.
The lead nurse for paediatric critical care needs to work well with the critical care
professional development team to ensure adequate training and to provide a link with
paediatric ward nursing staff.
Environment
The facilities and environment in an adult critical care unit are adapted to the needs of
adults and their relatives rather than children and their care-givers. Facilities are often less
than optimally adapted to the care of small children and suitable equipment may not be
immediately available or very infrequently used.
Equipment
Any ACCU which accommodates critically ill children will need to maintain a basic stock
level of essential equipment such as airway adjuncts and vascular access devices. Simple
devices should not be overlooked, such as suitably small NG tubes with the correct
compatible adapters for enteral feed, small blood-sampling tubes and blood gas capillary
tubes.
Ventilators have to be suitable to ventilate children down to a weight of only a few
kilograms. The knowledge of how to set these up and use them for small children is essential
if a child is to be cared for safely in an ACCU.
Dosing and infusion regimes for commonly encountered drugs such as sedative agents,
muscle relaxants, inotropes, bronchodilators, etc. need to be available. These should be the
same as the regimes used at the lead childrens hospital so that communication between the
two units is simplified.
Despite their design for a different patient group, adult equipment can be surprisingly useful for paediatric care. In the authors unit, we usually nurse children of all
ages in an adult critical care bed as opposed to infant beds with high cot sides. This
improves the accessibility of the child from all sides. All equipment on the ACCU
should be assessed in this way and only be replaced with child-specific equipment if
necessary. This reduces a source of error as staff can continue to work with equipment
they are familiar with.
Specialist paediatric warming devices should be available, although in most cases it is
sufficient to use simple blankets and clothing as long as exposure is reduced to a
minimum.
Most other equipment on the general ICU such as syringe drivers, volumetric pumps,
invasive monitoring transducers, etc. is suitable for patients of all sizes and therefore will
be available.
Chapter 26: Managing children on an adult critical care unit
247
Discharge
Once the acute critical illness has been treated to a degree that critical care support is no
longer needed, adequate step-down arrangements must be in place in the paediatric
department, particularly if the critical care outreach service does not routinely extend to
paediatric wards.
The decision to manage locally

The decision to treat a critically ill child in an ACCU beyond the initial stabilization period
should always be taken in consultation with the local paediatric intensive care unit (PICU)
or retrieval service. The treatment plan and progress should be discussed at least once every
24 hours.
Situations when local management in an ACCU may be appropriate or necessary
include:
the age of the child: children between the ages of 14 and 15 years, particularly if
physically relatively mature, may be managed on an adult unit for conditions which
predictably require only a brief period of ventilation or close observation
the underlying condition: the classic case would be an infant or child with prolonged
febrile convulsions or known epilepsy who required sedation and intubation for
(now resolved) SE and who is expected to need ventilation for no more than 24 hours
the potential for deterioration of the clinical condition, which would require critical care
support: a stable patient requiring close observation, e.g. after smoke inhalation or
accidental ingestion of sedative medication, etc.
lack of capacity of paediatric high-dependency beds in the paediatric department
the lack of availability of PICU beds: despite all efforts to increase PICU capacity
nationwide in recent years, this can pose a problem during the winter months and
during flu pandemics in particular. During such periods, the tertiary centres have to
allocate the available resources carefully, which may lead to a delayed transfer of
older children or children with less complex conditions. Some children may have
their entire critical care episode within an ACCU. The set-up and available medical
expertise in the ACCU in question at the time will also play a role in the decision to
deliver care in a DGH.
The challenges to managing a child on an ACCU

Regardless of the reason for the decision to keep the child in the ACCU beyond the
stabilization period, the care for these critically ill children in the adult setting poses certain
challenges.
Skills
The skills needed are to establish vascular access, sedate, anaesthetize, intubate, ventilate
and give inotropic support at appropriate levels. These also need to be weaned when
indicated.
Initial stabilization skills have much in common with the generic skills of anaesthesia
and are taught on many local and national courses. It is often easier to gain opportunities to
intubate children of all ages during elective theatre lists.
248
The maintenance and weaning of support are skills more specific to paediatric critical
care and require a certain amount of experience to be done well. Nursing staff in particular
may have little exposure to the maintenance and weaning phases of intensive care provision
for a child.
The treatment of some of the conditions for which a child may be cared for in
an ACCU also requires relatively specialist paediatric knowledge. It is paramount that
good links exist between the paediatric medical and nursing teams in the DGH and
critical care.
Exposure
One of the problems a multidisciplinary critical care team in a DGH is faced with when
looking after critically ill children on an ACCU is the acquisition and maintenance of
sufficient skills and experience. Nursing staff, pharmacists and physiotherapists may
encounter children as patients only during a critical illness. This often has a major impact
on their confidence in dealing with the particular issues posed by critically ill children of all
age groups.
A multidisciplinary approach including paediatric medical input and good working
relationships with the paediatric nursing staff is essential to guarantee the best possible
standard of care. For neonates and small infants admitted to the hospital from home, input
from local neonatal intensive care services will be necessary.
Joint training sessions on specialized equipment used on the neonatal and paediatric
high dependency unit such as CPAP and BiPAP machines should be organized.
A professional development plan for nursing and medical staff should be developed
locally, in cooperation with the relevant PICU, to ensure that the necessary knowledge is
acquired, maintained and practised regularly. This should include:
participation in regional and national courses, multidisciplinary simulation training
development of referral pathways taking account of local facilities
secondment of staff to specialist units.
Often, once a core of medical and nursing staff have undergone this training, the distribution of knowledge and skill on the ACCU takes an exponential course, and the successful
management of any critically ill children in this setting enhances the effect greatly.
Parents
Parents are usually allowed access to the bedspace for much longer periods during the day
than the relatives of adult critical care patients and are actively encouraged to take part in
the personal care for their child.
Ongoing development
Constructive feedback from the local lead PICU is extremely important and a system
should be developed with the relevant PICUs or retrieval teams to ensure that this
happens on a regular and timely basis. It is important that all members of the local
multidisciplinary team including the paediatric, general intensive care and nursing teams
are involved. Feedback is essential to reflect on current practice and realize opportunities
for improvement.
Chapter 26: Managing children on an adult critical care unit
249
An ongoing audit of paediatric cases treated on the ACCU is essential to ensure

feedback on all cases. This is an important role for the local nursing or medical lead for
paediatric critical care.
A good working relationship with the respective PICU and/or retrieval team facilitates
communication in the acute situation. It also facilitates establishment of standards and
systems development with:
exchange of knowledge and expertise
continued medical and nursing education
advice on equipment acquisition and use
participation in the development of treatment regimes.
A paediatric critical care network should consist of the local PICUs, the paediatric retrieval
team and all the local acute paediatric care providers including the ACCUs providing care
for children. Within the network, issues may develop where there is a need for education or
the development of guidance or management protocol, which can then be distributed to all
participating units. This was recognized during preparation for the flu pandemic.
Within this network, there should also be an atmosphere of collegial cooperation so that
no clinician feels any inhibition when asking for advice.
Summary
Paediatric critical care services have been centralized in specialist units over the last few
years to focus expertise and achieve best outcomes. This means that ACCU in DGHs with
paediatric inpatient departments may be confronted with severely ill children who require
resuscitation and critical care support against a background of small case numbers.
To maintain good standards of care for such children, multidisciplinary teamworking
and good links between the paediatric, neonatal and critical care specialists are extremely
important. Practical, workable and mutually agreed treatment protocols and referral pathways must be in place. The local set-up must include links with the specialist regional centre
and retrieval service, a robust governance structure and an effective professional development programme for all staff involved. Under these circumstances, critically ill children can
be managed successfully and to a high standard on an ACCU.
Further reading
Edge WE, Kanter RK, Weigle CGM, et al.
Reduction of morbidity in inter-hospital
transport by specialised pediatric staff. Crit
Care Med 1994;22:11861191.
http://www.wmsc.nhs.uk/uploaded_media/CIC
%20standards%20revision%20D9%2029%
207%2009.pdf.
Paediatric Intensive Care Society. Standards for
the Care of the Critically Ill Children, 4th edn.
London: PICS, 2010.
Pollack MM, Alexander SR, Clarke N, et al.

Improved outcomes from tertiary
centre pediatric intensive care.
A statewide comparison of tertiary and non
tertiary care facilities. Crit Care Med
1991;19:150159.
Report from the National Co-ordinating
Group to the Chief Executive of the NHS
Executive. Paediatric Intensive Care:
A Framework for the Future. Department of
Health, July 1997;31.
Section 3
Chapter
27
Pain management in children

Ursula Dickson
Introduction
In critically ill children pain is often taken care of by the use of morphine as a sedative.
However, for any child who is not sedated adequate analgesia is essential in order to:
gain cooperation
unmask clinical signs
minimize the stress response to injury
treat a child humanely.
Recognizing pain in small children can be very difficult. It should never be assumed that
because a child cannot express pain they are comfortable.
As in adults, pain in children has many components which may influence their pain
behaviour. These factors can include:
psychological
social
cultural
previous experiences.
This chapter aims to provide a guide to recognizing and managing pain in children. The
medications recommended should be available in all accident and emergency departments
and paediatric wards.
Assessing pain in children

When asking children about pain, great efforts should be made to avoid using medical
jargon. Take care to explain what you are going to do to help them, and keep to simple
terms. It is often useful to ask parents to help you find words that their child or their family
uses, e.g. hurt rather than sore, and Calpol rather than paracetamol.
Tools used in the assessment of pain need to be quick, reliable and demonstrate low
inter-user variability. Pain scores can be used to fulfil these criteria. Depending on the age of
the patient, the pain scores used can be observer-based or self-reported.
Observer-based behavioural scores

FLACC (face, legs, activity, cry, consolability) for young children
Neonatal Infant Pain Score young infants
250
Chapter 27: Pain management in children
251
The FLACC score

This system has been validated for infants and young children who cannot speak or who
find it difficult to quantify pain and emotions. The system scores from 0 to 10, where
0 equates to no pain and 10 to severe pain. A score greater than 7 suggests severe pain.
The FLACC score is also useful in older children who are unable to communicate
normally, either because of their illness/trauma or because of pre-existing developmental
disorders. The words in italics in Table 27.1 are the terminology developed for children with
cognitive impairment.
Table 27.1. The FLACC score.
Categories
Scoring
0
Face
No particular
expression or
smile
Occasional grimace or frown,

withdrawn or disinterested
appears sad or worried
Frequent to constant
quivering chin, clenched jaw
distressed-looking face,
expression of fright or panic
Legs
Normal position
or relaxed
usual tone and
motion to limbs
Uneasy, restless, tense
Kicking, or legs drawn up
occasional tremors
marked increase in spasticity,

constant tremors or jerking
Lying quietly,
normal position,
moves easily
regular rhythmic
respirations
Squirming, shifting back and

forth, tense
Arched, rigid or jerking
tense or guarded movements,

mildly agitated [e.g. head back
and forth, aggression], shallow,
splinting respirations, intermittent
sighs
severe agitation, head

banging, shivering [not
rigors], breath holding,
gasping or sharp intake of
breaths, severe splinting
Activity
Cry
No cry [awake or Moans or whimpers: occasional

asleep]
complaint
occasional verbal outburst or
grunt
Consolability Content, relaxed Reassured by occasional

touching, hugging or being
talked to, distractable
Crying steadily, screams or

sobs, frequent complaints
repeated outbursts, constant
grunting
Difficult to console or
comfort
pushing away care-giver,
resisting care or comfort
measures
The NIPS score

The Neonatal Infant Pain Score (NIPS; Table 27.2) is considered a better tool for assessing
pain in neonates. The maximum score achievable with this tool is 7. It does have limitations
in that a baby who is severely unwell may score falsely low.
252
Table 27.2. The Neonatal Infant Pain Score (NIPS).
Score
Finding
Details
Facial expression
0
1
Relaxed muscle
Grimace
Restful face, neutral expression

Tight facial muscles, furrowed brow, chin, jaw
Cry
0
1
2
No cry
Whimper
Vigorous cry
Quiet, not crying

Mild moaning, intermittent
Loud scream, rising, shrill, continuous
Breathing patterns
0
1
Relaxed
Changed
Usual pattern for this baby

Indrawing, irregular, faster, gagging
Arms
0
1
Relaxed/restrained
Flexed/extended
No muscular rigidity, occasional movement

Tense, straight arms, rigid, extension/flexion
Legs
0
1
Relaxed/restrained
Flexed/extended
No muscular rigidity, occasional movement

Tense, straight legs, rigid, extension/flexion
State of arousal
0
1
Sleeping/awake
Fussy
Quiet, peaceful, sleeping, or alert and settled

Alert, restless, and thrashing
Self-reporting pain scores

Using self-reported pain scores in young children can be prone to errors. For instance, giving a
small child a colour-coded system may end up with them picking their favourite colour, rather
than one that is representative of their pain score. Children find facial expressions helpful when
quantifying their pain. This can be utilized by giving children a modified Wong Baker faces
score (Figure 27.1). Scores of 26 suggest mild to moderate pain and 8 or more severe pain.
Children may be worried about telling people how much pain they are in because they
fear what will happen if they tell the truth.
0
No Hurt
2
Hurts
Little Bit
4
Hurts
Little More
6
Hurts
Even More
8
Hurts
Whole Lot
10
Hurts
Worst
Figure 27.1. The Wong Baker faces chart. From Wong DL, Hockenberry-Eaton M, Wilson D, Winkelstein ML,
Schwartz P. Wongs Essentials of Pediatric Nursing, 6th edn. St Louis: Mosby, 2001, p. 1301. Copyrighted by Mosby, Inc.
Reprinted by permission.
Which analgesics should you give?

The WHO pain ladder is used in children as it is in adults. Mild to moderate pain can
often be treated with simple analgesia, whereas severe pain will usually require an opiate.
Remember that analgesics that work on different parts of the pain pathway are synergistic.
Paracetamol and non-steroidal anti-inflammatories (NSAIDs) should never be stopped
purely because an opiate is being given.
Child-friendly preparations should be used where possible, i.e. the drug should ideally
be liquid and palatable. Table 27.3 gives dosages based on common practice in childrens
hospitals and follows the guidelines in the BNFc.
Table 27.3. Dosages based on common practice in childrens hospitals and following the guidelines in the BNFc
PO
PR
IV
Neonates
Frequency
Caution
Paracetamol
20 mg/kg
(up to 1 g)
max 90 mg/kg/day
(up to 4 g)
3040 mg/kg
loading dose then
20 mg/kg
max 90 mg/kg/day
(up to 4 g)
15 mg/kg over
10 kg (max
60 mg/kg/day)
PO/PR 15 mg/kg (max

60 mg/kg)
IV term 10 mg/kg (max
30 mg/kg/day)
IV neonates and infants
< 10 kg 7.5 mg/kg
(max 30 mg/kg/day)
46 hourly
Renal impairment
Hepatic
impairment
10 mg/kg
NSAIDS
Diclofenac
Ibuprofen
1 mg/kg
(max 150 mg/day)
510 mg/kg
(max 30 mg/kg/day
max dose 2.4 g/
24 h)
1 mg/kg
n/a
> 2 years 1 mg/kg bd

(max 150 mg/day)
n/a
Not < 6 months of age

Not < 1 month of age
or < 5 kg
8 hourly
68 hourly
Asthma not
absolute
contraindication
Avoid if impaired
renal function
Bleeding
disorders
Hepatic
impairment
Codeine
1 mg/kg
(up to 60 mg)
1 mg/kg
(up to 60 mg)
Never
0.51 mg/kg
46 hourly
Moderate to
severe renal
impairment
reduce dose
Morphine
0.10.5 mg/kg
n/a
Bolus dose
incrementally > 1 year
100200 g/kg
Infants 50100 g/kg
2550 g/kg
Oral 80 g/kg
4 hourly
Moderate to
severe renal
impairment
reduce dose
Hepatic
impairment
reduced
metabolism
254
Table 27.4. Concentration of the formulations available; drug doses should be sensibly rounded off to match.
Suspension Tablets Dispersible Suppositories

tablets
Melts
IV
Paracetamol 120 mg/5 ml 500 mg 500 mg

250 mg/5 ml
30 mg, 60 mg,
120 mg, 240 mg,
500 mg, 1 g
250 mg 10 mg/ml
Ibuprofen
100 mg/5 ml 200 mg n/a
n/a
100 mg n/a
Diclofenac
n/a
25 mg, 50 mg
50 mg
12.5 mg, 25 mg,

50 mg
n/a
75 mg
(see BNFc for
administration
guidance)
Codeine
15 mg/5ml
15 mg, n/a
30 mg,
60 mg
1 mg, 2 mg,
3 mg (requests)
5 mg, 10 mg
n/a
n/a
Morphine
10 mg/5ml
10 mg, n/a
20 mg
n/a
n/a
10 mg/ml
50 mg/50 ml
Paracetamol
This is a centrally acting drug that is a cyclooxygenase inhibitor. It also has a mode of action
via 5HT receptor agonism and possibly a weak cannabinoid effect.
Paracetamol is used as an antipyretic as well as an analgesic in children. It can
be given IV, PO or PR. Absolute contraindications include a history of allergy and liver
failure.
Caution should be exercised in the following cases:
liver disease
reduce dosage as there may be reduced glutathione stores to deactivate toxic
metabolite
renal disease
reduce maximum daily dosages due to reduced excretion of glucuronide metabolites.
Ibuprofen
This is the NSAID drug that has the best safety profile in children. It is a nonspecific
cyclooxygenase inhibitor.
It is recognized that in a few patients NSAIDs can trigger bronchoconstriction. However, in children with well-controlled asthma it can usually be given safely, with no
reporting of wheeze or increased inhaler usage. On testing in adults, only 25% of asthmatic
patients react to skin testing for NSAIDs. It can be given as an oral suspension or as melts.
Ibuprofen should not be given if the patient has any of the following:
impaired renal function
hypovolaemia
acute bleeding

255
brittle asthma
severe sepsis
liver failure
history of gastric bleeding
coagulation abnormality
history of allergic reaction to any NSAID.
Codeine
This is a weak opiate. It is easy to administer orally or rectally and is well tolerated. Codeine
causes less nausea and vomiting than morphine in children.
There is a great deal of genetic variability in peoples ability to metabolize codeine to its
active morphine form via the CYP2D6 part of the cytochrome P450 system. This may
render it of little use in some patients. Also, if a patient is in severe pain it may not be
converted to enough morphine to be effective in everyone. If its administration is evaluated
as ineffective, oramorph or IV morphine should be given instead.
Morphine
Many people may have concerns about the use of morphine in children. Ultimately, it is
very safe as long as you give the right dose, and the patient is adequately monitored.
Incremental IV boluses of morphine allow optimal titration in acutely ill patients.
A morphine bolus will usually last about 24 hours. It can be given PO, IV, PR or
intranasally (as diamorphine).
Respiratory depression is a side effect most feared by doctors prescribing morphine.
However, this is easily reversible with the opiate antagonist naloxone if it becomes problematic. This does not mean that morphine should be given carelessly. Naloxone should be
prescribed (as required) for any child on an opiate infusion who is self-ventilating. If it is
given then the patient should be monitored closely as the half-life of naloxone is shorter
than that of morphine, so it may wear off sooner.
If a child becomes nauseous or starts vomiting then the serotonin antagonist ondansetron should be given as a first-line anti-emetic: 0.1 mg/kg intravenously up to 8 hourly.
Itching is common in children when using opiates. The histamine antagonist chlorphenamine is a good antidote to this (0.1 mg/kg intravenously), although it may exacerbate
drowsiness. Patients experiencing excessive histamine release from morphine may be better
on the synthetic opioid fentanyl.
Patient/nurse-controlled analgesia (PCA/NCA)

If a patient is likely to need a strong opiate for many hours after an IV bolus has been given,
it is best to start either a patient-controlled analgesia or a nurse-controlled analgesia regime
(PCA/NCA) with a background infusion (if your unit has the experience with appropriate
protocols and pumps), or a plain morphine infusion.
To avoid dosing errors when administering morphine infusions, the patients body
weight in kilograms should be converted to milligrams and the equivalent dose of morphine
should be made up to 50 ml using normal saline, i.e. a 45 kg child should have a 50 ml
solution with 45 mg of morphine. Each millilitre of the ensuing solution will then contain
256
20 g/kg. For any patient in severe pain who needs a 100 g/kg loading dose, a 5 ml bolus
from this solution can be given to achieve an adequate plasma concentration of morphine.
Neonates and infants are more sensitive to opiates; therefore it may be wise to manage
their pain with a 2550 g/kg initial bolus, followed by a continuous infusion of 10 g/kg/h
(0.5 ml/h).
Table 27.5. A guide for morphine loading doses and infusion rates according to age.
Patient age group
Bolus loading
dose
Neonate or
ex-premature baby
2550 g/kg
Infusion rate if breathing

spontaneously
Infusion rate if
ventilated
0.10.5 ml/h
02 ml/h
Child 1 month 1 year
50100 g/kg
0.11 ml/h
03 ml/h
Child older than 1 year
100200 g/kg
0.12 ml/h
05 ml/h
Fentanyl
Fentanyl is 100 times more potent than morphine, so the usual dose is 1 g/kg.
When administered as a single bolus, its duration of action is only 2030 minutes. As
with all opiates, incremental boluses are the safest way to administer to a spontaneously
ventilating patient.
Fentanyl can be given by infusion (after a loading dose) in patients with renal failure, as
it has no active metabolites that depend on renal excretion (unlike morphine). However,
this should only be done in centres that are experienced in its use.
Intranasal diamorphine
Over the last few years, many accident and emergency departments have written protocols
for administration of intranasal diamorphine. It provides an easy, quick way of administering potent analgesia to a child who does not have IV access. Diamorphine should be
diluted in a small volume (0.2 ml) of saline and then either sprayed into the nostril via a
mucosal atomization device or dripped onto the nasal mucosa. It is usually effective within
5 minutes.
Most EDs have a chart detailing how to make up the diamorphine solution according to
the childs weight so that they receive a dose of 0.1 mg/kg.
Topical anaesthesia
A topical anaesthetic agent should be applied (and given time to work), if time permits,
prior to cannulation and phlebotomy. Ametop (amethocaine), emla (lignocaine mixed
with prilocaine) and LMX4 (4% lignocaine) are the commonest available formulations for
application to the skin. If there is not enough time to allow these to work then consider using
ethyl chloride spray to numb the desired area.
Entonox
This is a mixture of the gases nitrous oxide and oxygen. It produces analgesia and anxiolysis
for procedures such as plaster application. The patient has to be old enough to
257
self-administer it through a one-way mouthpiece. It is considered safe because if a child

becomes sedated they will stop breathing the gas. However, Entonox may cause nausea,
vomiting and disinhibition.
Entonox should not be used if the child has, or is suspected to have:
a head injury
a pneumothorax
a distended abdomen
maxillo-facial injuries
air embolus.
Nerve blocks
In skilled hands a femoral nerve block can provide effective analgesia for a fractured femur
and, once working, facilitates the application of traction and a Thomas splint. The femoral
nerve lies immediately lateral to the femoral artery. It can be easily visualized with an
ultrasound machine. After negative aspiration for blood, a 0.5 ml/kg slow bolus injection of
0.25% of chirocaine around the nerve should provide analgesia within 1520 minutes and
should last for several hours. However, this procedure should only be performed after
adequate training.
Summary
The principles of pain relief in children are the same as those in adults. A calm, commonsense approach is essential. The two situations commonly seen in children are giving too
little analgesia, or giving too much.
Although the temptation might be to jump straight in to try to alleviate pain in a child
immediately, incremental doses of opiates are the safest way of administration. Remember
the adage that you can always give more, but you cant take away what youve given.
Golden rules
Pain needs to be managed proactively in small children
Morphine can be used safely in children use incremental doses
Use simple language when assessing pain
Explain your management plan clearly and simply to both the child and parents
Section 3
Chapter
28
Children with complex needs

and disability
Kate Skone and Ian Wacogne
Introduction
Children with complex medical problems pose particular challenges to the clinical team.
The family members and carers are often expert in their childs condition, have frequent
visits to hospital and may have very specific expectations.
Despite the fact that these children can have very complicated background medical
problems they are still most likely to present with one of three problems:
respiratory infection or respiratory failure
seizures
systemic infection.
Children with complex needs will be managed along the same principles as detailed in the
other chapters in this book. The aim of this chapter is to highlight two important factors
that should be considered in these children; namely, how to access information about the
child and to consider which other professionals should be involved in the childs care. It will
also discuss how children may deviate from the usual care pathways in some circumstances.
Differences in children
Although the vast majority of the conditions encountered are very rare individually,
children with some form of disability are relatively common. The Office for Disability
Issues reports that approximately 1 in 20 children have a disability. This equates to
approximately 700 000 children across the UK suffering from physical disabilities, learning
difficulties or a combination of both.
Children with complex needs differ from adults with multiple co-morbidities in:
the types of pathology
the challenges of learning difficulties and communication at different developmental
stages
the network of professionals involved in looking after the child.
Often children with a disability may have conditions that cause chronic health problems as
well as disability. In children, long hospital stays and complex medical problems can have
significant effects on growth and development.
258
Chapter 28: Children with complex needs and disability
259
The care package

Primary carers
Children with complex medical problems and disability will have the majority of their care
carried out by their parents and extended family but may have professional carers involved
to provide respite or regular nursing support. The family may be very used to their child
being unwell and have had many hospital stays or visits to intensive care.
Care-givers may be responsible for delivering quite complex medical care for their
children including:
home ventilation
tracheostomy changes
chest physiotherapy
nasogastric feeding
administration of medications, in some cases intravenous.
They will often continue to deliver care while the child is in hospital.
Medical input
Children with disabilities and complex needs will have a lead paediatric consultant who may
be community- or hospital-based. The children may well be known to a number of other
paediatric teams, either locally or specialists in the nearest childrens hospital. They are
often well known to medical and nursing staff on paediatric wards, especially if they are
regularly admitted with medical problems.
Community input
In the community setting, children are often well known to community and school nursing
teams. They are likely to have regular contact with therapists, who may also have information
about a childs usual function.
Respite care
Children may have regular respite care and may have carers outside the family who also
know them well. Children may be brought to hospital by respite carers who understand the
medical issues. However, carers will not have parental responsibility and cannot consent to
treatment. In the absence of parents emergency treatment should be administered working
under the principle of best interests.
It is important to inform and involve the lead clinician or the paediatric team when the
child presents unwell, and to consider other members of the team involved when seeking
information. Many local paediatric departments keep information about children who have
complex health problems readily available, for example in a file on the ward. This can be a
useful source of information, provided the information is up to date.
Communicating with children

It can be difficult to communicate with young children, especially when they are frightened
and unwell. It may be more difficult with children who have underlying disability. It is
essential to discover the childs normal level of function and understanding.
260
Children with disabilities will communicate in a wide range of ways, and use certain
forms of sign language, e.g. Makaton in the UK. They may have hearing impairment and
may wear hearing aids, which should be used even if they are critically unwell. A parent or
carer may need to be involved. This aspect of care should not be neglected.
Particular conditions to consider

Respiratory conditions
The practical management of respiratory problems in children with disability is no different
from that in other children. However, children with underlying disease may get more
unwell more quickly.
Respiratory infections
The management of respiratory infections in children has already been covered in Chapter 7.
Several different mechanisms may make some children prone to recurrent, severe, chest
infections (see Table 28.1).
Table 28.1. Common precipitants of illness in children with complex illnesses.
Mechanism
Examples
Lung disease with colonization or poor reserve
Bronchopulmonary dysplasia
Bronchiectasis
Recurrent aspirations
Tracheostomy (microaspiration)
Chronic neurological disease
Neurodegenerative conditions, e.g.
mitochondrial disease
Epilepsy
Neurological disease with poor respiratory effort
Cerebral palsy
Hypotonia
Scoliosis
Muscular dystrophy
Immunocompromise
Malignancy
Asplenism
Choice of antibiotics
Children with frequent chest infections may be on prophylactic antibiotics during the
winter months or all year round. These children are often colonized with unusual flora. It
is important to look at previous microbiology reports, as this will guide antibiotic choice. If
there is doubt a discussion with their respiratory clinician or microbiologist may be useful.
Prophylactic measures
In children under 1 year the most significant viral illness is usually respiratory syncitial
virus (RSV). The presenting features and management of RSV have been discussed elsewhere (see Chapter 7). Children with complex medical problems may be treated with
palivizumab, a monoclonal RSV antibody that may prevent severe pathology from RSV
261
infection. Passive immunization against RSV requires monthly IM injections. Current UK

guidelines from the Department of Health recommend giving palivizumab:
according to gestation at birth and presence of chronic lung disease at times of high RSV
prevalence
to premature infants with haemodynamically significant acyanotic congenital heart
disease
to children younger than 1 year old who are on long-term ventilation
To children suffering from certain immune deficiency syndromes, e.g. SCID.
Tracheostomy
There are a number of children in the population with long-term tracheostomies. These
may have been inserted to manage:
structural airway problems, such as subglottic stenosis following intubation
congenital airway abnormalities such as Pierre Robin sequence
long-term respiratory disease such as bronchopulmonary dysplasia
children who require home ventilation.
Parents and carers will have been trained in tracheostomy management but they may
present with problems to the emergency department. Tracheostomies may become blocked
or dislodged. It is likely that paediatricians will be less confident with tracheostomy care
than anaesthetists. All children with a tracheostomy are expected to have an appropriatesized replacement and a suction machine with them at all times.
Home oxygen
The commonest reason for children to require home oxygen is prematurity. This is usually
to treat chronic lung disease. Babies are discharged from the neonatal unit with low-flow
oxygen, usually delivered by nasal prongs. In general this is in continuous use and it is
unusual for this to be above 0.5 l/min.
Lung maturation continues, with new alveoli developing, during the first few years of
life. It is unusual for premature babies to need oxygen therapy after the age of 2 years. The
need for oxygen therapy is assessed by regular sleep studies, which are monitored by a
neonatal physician or respiratory team.
The physiology of these children can be compared to adults with chronic obstructive
airways disease in that they often have a compensated respiratory acidosis. It is not
uncommon to see a high PaCO2 of up to 1012 kPa with a compensatory high bicarbonate
level.
Children with limited respiratory function

While some children have limited reserve because of poor gas transfer at an alveolar level,
others may suffer from an inability to mount a respiratory response to infection or acidosis.
These children may have neurological conditions such as cerebral palsy or muscular
dystrophy.
The important thing to note in these children is that they may not look as if they are in
respiratory distress. Signs such as intercostal recession may not be present if they have poor
muscle function. If there is any doubt, a capillary or arterial blood gas analysis should be
performed to evaluate the effectiveness of their ventilation.
262
Neurological problems
Seizures
The standard management of seizures in children has been covered in Chapter 10.
Children with complex epilepsy will often have frequent seizures at home. The manifestation of seizures may be very variable. Some children can present obviously in convulsive status epilepticus with generalized tonic clonic seizures, whereas others may have more
subtle seizures causing facial twitching or eye deviation. To complicate matters, children
with neurological disorders may have other abnormal movements that do not represent
seizures, for example tics or dystonic movements.
In children with severe disabilities it can be difficult to assess how alert the child is and
what is abnormal for them. Parents and carers will be able to describe the various seizures
that their child has and how the current seizures differ. Sometimes the only way to identify
whether repetitive abnormal movements are seizures is to use EEG monitoring.
Clusters of seizures, even partial seizures, may require IV anticonvulsants. Children may
also present in non-convulsive or subclinical status; features that suggest this include:
reduced conscious level
behavioural or mood changes
sleep disturbance
loss of skills or developmental regression.
This is usually confirmed on an EEG. It may require intervention with intravenous
anticonvulsants, most commonly benzodiazapines. This will be managed on the advice of
a paediatrician or neurologist but may necessitate respiratory support on intensive care.
Many families of children with epilepsy will have been taught to administer buccal
midazolam at home. In many cases the parents will have been instructed to present to the
ED whenever they use midazolam. In others, buccal midazolam may be used frequently and
parents will only bring the child into hospital if it has not been effective.
Individualized management plans

Many children with difficult to manage seizures have individualized management plans
written by their lead clinicians. Parents are likely to have a copy of this and it should also be
available in the childs notes. It is usually developed after many previous successes and
failures and may avoid the need for intubation and ventilation. As well as detailing the
drugs that work, the management plan will contain information about which drugs to
avoid; for example, some children have profound respiratory depression with benzodiazepines which often results in admission to ICU.
Many children do end up with frequent ICU admissions when their seizure control
decompensates; however, it is important to make use of the individualized management
plan and if possible discuss the child with the clinicians who know them to try and optimize
further management.
Some children with complex needs may have an advanced care plan regarding escalation
of treatment, e.g. admission to ICU or palliative care (see Chapter 29).
Ventriculoperitoneal shunt
Ventriculoperitoneal (VP) shunt drains fluid from the ventricles of the brain. They may
become blocked, leading to an accumulation of CSF and a rise in ICP. This may or may not
263
be associated with infection. The symptoms and signs of a blocked shunt may be subtle,
especially in non-verbal children. They include:
headaches
increase in frequency of seizures
vomiting
reduced conscious level
full and tense fontanelle
irritability.
If there is any suspicion of a blocked shunt a CT scan should be requested and the child
discussed with their neurosurgical team. Infections of a VP shunt are more likely within the
first 6 months after insertion and may present with meningism.
Infection
Management of sepsis has been covered in Chapter 5. In children with complex medical
problems the signs of sepsis may be subtle. In searching for a source of infection it is
important to remember about indwelling devices as a source.
Children may have long-term vascular access for numerous reasons:
chronic respiratory conditions, e.g. those with cystic fibrosis or bronchiectasis may have
regular courses of IV antibiotics
chronic gastro-intestinal diseases, e.g. those with short gut syndromes or malabsorption
syndromes may need parenteral nutrition and have a central line for this
dialysis lines
malignancy and chemotherapy.
Children on chemotherapy will be immunocompromised and are at risk of being neutropenic. Therefore any child with a malignancy presenting with fever should be treated with
IV antibiotics. The central lines should only be accessed using a sterile technique, and only
if necessary. Ideally the antibiotics should be given within the first hour of presentation as
this has been demonstrated to be associated with reduced morbidity and mortality. If there
is any doubt, consideration should be given to removing the CVC. This should be discussed
with the team responsible for the childs ongoing care.
264
Table 28.2. Potential sources of infection.
Device
Examination
Investigations
Ventriculoperitoneal
shunt
Shunt can normally be felt running

Shunt series to look for
behind the ear and down the back of the fractured shunt (plain X-rays
neck
of shunt tubing, CT head)
Tap of shunt (see Chapter 19)
Central line
Tunnelled line usually palpable on the

chest; examine under the dressing for
infection and along the path of the line
Blood cultures from each

lumen
Swab of line insertion site
If access is possible then send

Implantable long-term Palpate port, usually on chest wall, and
venous access device, examine along the course of the palpable cultures from the port
line
e.g. Portacath
Percutaneous
endoscopic
gastrostomy
Remove dressings over percutaneous

endoscopic gastrostomy and examine
surrounding skin
Swab of site
-ostomies
Examine site
Swab
Send samples to laboratory
Urinary catheter
Contents of urine bag

Abdominal examination
Send urine specimen
Management and sources of information

Often the most complex aspect of management of children with complex needs is communication. It is important to ensure that information is available to the intensive care team
who are involved in looking after the child.
The paediatric team should be leading and coordinating the fact-finding. Sources of
information about the child will include:
the family themselves; they may carry recent letters from appointments with their
community paediatrician that summarize their care
the hospital notes, which should be accessed early, particularly if there is a need for
transfer elsewhere
notes or recent letters, which many paediatric departments will keep available on the
ward for children who present frequently
patient-held notes, which some departments are piloting; which means that the family
may carry some of the medical notes with them.
The childs regular clinician should be involved with any significant decisions about
changes to the long-term treatment plan.
After initial stabilization

The admission of a child to hospital may be an opportunity to review the childs medication. Often children are on many drugs and, on admission, errors in drug doses are
discovered. This is often particularly important for anti-epileptic medication, where the
child may have outgrown their dose.
The admitting team should not change a childs long-term medications without discussion with the primary team; however, taking a detailed history may give an idea as to the
precipitating cause of the problem. This can then help guide the ongoing management.
265
Discussing with parents

Although they may be used to the process of having their child admitted to hospital, parents
of children with complex needs will need a lot of support when their child is critically ill.
The admissions do not stop being scary.
The parents may wish to continue carrying out aspects of their care. They may also have
had previous experiences when their child has been unwell which affect their expectations.
When informing them of the management plan for their child take time to listen to any
worries that they may have. Address each question, and give a clear explanation of what is
going to happen next.
Although many are knowledgeable about their child, the majority of parents who have a
child with a rare condition will not expect you to know everything about their disease. It is
very easy to come unstuck if you are not open with them about the level of your knowledge
of their childs condition.

When transferring a child to another hospital there should be as detailed as possible a
transfer letter so that the team looking after the child understand the medical problems. The
local paediatric team should produce this, especially if they are well known to them. It
should include:
any recent background letters
any information about advanced care plans, which should be clear and discussed with
the family
all investigations performed on this admission (including radiology)
information about who is going to inform the primary teams involved in looking after
the child.
Summary
Managing a child with complex needs can be daunting. However, the majority of management principles are the same as for any other child. If it is possible to get parents on board
early, then the management of the child becomes a lot easier. The main aim of management
is to gather as much information as possible while managing the childs presenting
problem. In reality, the paediatricians present at an emergency will be collecting the information quickly, while the emergency physicians deal with the acute problems.
Golden rules

Gather as much information as possible from the parents

Do not exclude parents from the resuscitation area if they wish to be present
In the absence of any other information treat the child as you would a less complicated
paediatric patient
Be clear about your management plan
You do not need to be an expert in the childs condition most parents will not expect it
Phone the parent team/retrieval team early for advice
Section 3
Chapter
29
When a child dies

Fiona Reynolds
Introduction
A dying child should be afforded the comfort and dignity which good palliative care
provides. ED doctors and anaesthetists are likely to be involved in the care of children at
the end of life either during failed resuscitation attempts or when children on a palliative
care pathway are brought into hospital because of a sudden deterioration.
Children with end of life plans

Unplanned admission
For children with end of life plans hospice admission may be the most appropriate way to
support care-givers and children. Sometimes, however, care-givers may bring their child to
a hospital as they become scared and feel they cannot cope at home with a child at the end
of life. Good planning and palliative care should prevent children who are receiving
palliative care at home from needing to be admitted to hospital.
Setting the environment

Care-givers should be supported and allowed to be present when their child is dying.
If they do not want to be present, they should not be pressured to do so. Most care-givers
choose to be present and ultimately find comfort in being close to their dying child.
Care-givers may need support to be there and may find comfort from the presence of a
senior nurse; other care-givers may want privacy to say goodbye with only family members
present.
Medical and nursing staff who seldom deal with dying children may find this especially
difficult. Staff may experience a mixture of emotions and may find it difficult to perform
their duties. Care-givers and families often remember a variety of acts or words from
professional staff during this time. While it is acceptable for staff to be sad and to look sad,
it is important that professionalism is maintained, as there is a very important job to do
when a child is dying.
Withholding or withdrawal of life-sustaining therapy

Decisions about withdrawal of intensive therapy require a sound knowledge of the condition, treatment and the childs prognosis. They also require a working knowledge of
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Chapter 29: When a child dies
267
ethical and legal aspects of withholding and withdrawing life-sustaining therapy. The
Royal College of Paediatric and Child Health and the General Medical Council in the UK
publish a professional framework for end of life care. It discusses both ethical and legal
aspects of practice.
Medicine relating to withdrawing and withholding life-sustaining therapy

Senior medical and nursing staff should be involved in the medical decisions about the
diagnosis, possible treatments and prognosis for critically ill children. These individuals
should be expert in the appropriate area of medicine and should know the patients clinical
details. Decisions about treatment should involve care-givers: this applies whether it is
continued active treatment or the withholding or withdrawal of life-sustaining therapy.
Decisions about withholding or withdrawal of life-sustaining therapy should be made by
consensus between the senior professionals involved, the care-givers and child if he or she is
able to participate in the decision. Decisions are based on the best interests of the child. If
there is no consensus a second opinion should be requested and may be useful as an
independent review of the childs treatment and prognosis. If no consensus can be reached
ultimately a court may have to decide on whether to continue active treatment or to
withhold or withdraw life-sustaining therapy.
Many children with life-limiting disorders who are expected to die have an advance care
plan. This plan outlines the actions to be followed in the event of acute deterioration. The
treatment plan may range from an attempt at active resuscitation to a plan for comfort care
and to allow natural death to occur. It is important that clinicians are aware of the local
paperwork which supports advance care or Do not attempt cardiopulmonary resuscitation
plans.
Ethics relating to withdrawing and withholding life-sustaining therapy

The duties of a doctor underpin their professional actions. Deontological ethics judges the
morality of an action based on adherence to rules or performance of duties. The duties of a
doctor are considered to be:
autonomy: respect for a person to decide about themselves
beneficence: to do good
nonmaleficence: to do no harm
justice: addresses the question of distribution of scarce healthcare resources.
The primary duties of a doctor are to do good in trying to preserve life but the other duties
of a doctor may challenge the duty of beneficence. A doctor also has a duty to recognize the
dying patient so as not to prolong the process of dying with an unnecessary burden of
treatment, which will not improve the outcome but cause suffering whilst not allowing
natural death to occur.
Law relating to withdrawing and withholding life-sustaining therapy

Allowing natural death or the process of withdrawing or withholding life-sustaining therapy
is both legal and ethical when further treatment is considered futile or not in the best
interests of the patient. Withdrawal or withholding intensive therapy requires a consensus
268
between the medical teams treating the patient and the care-givers. If no consensus can be
reached a second opinion may help the care-givers with an independent objective view.
If a consensus cannot be reached, ultimately a court may need to decide on the issue of
withdrawal of intensive therapy. This is rare; most care-givers initial resistance is part of a
journey of grief, which often starts with denial, but eventual acceptance can allow a
consensus to be built and an agreement to switch to comfort care.
Deaths in the emergency department

Most children who die in ED are admitted with cardiopulmonary resuscitation in progress.
Occasionally a child who is known to have a terminal illness is admitted in extremis and
subsequently dies. Most ED have a system to support care-givers while resuscitation is in
progress.
All sudden or unexpected deaths in children have to be reported to the coroner, who will
establish the cause of death. Most sudden unexpected deaths in childhood are due to natural
causes or the result of accidents. A minority of deaths are the result of non-accidental
injury.
When the cause of death is not clear, there are often local arrangements for the ED to
take blood and small biopsy samples with the coroners permission to look for infection or
inherited diseases which may be the cause of death. These samples are often best taken as
soon as possible after death.
If the coroner is to hold a post mortem or an inquest, he or she will be responsible for
issuing the death certificate rather than the hospital.
Witnessed resuscitation
In the UK it is now common practice to allow care-givers to be present during attempts
at resuscitation if they wish. This may be in the ED, on the childrens ward or in the
PICU. A senior member of staff should support the care-givers if they wish to be present
during resuscitation. The member of staff should explain what is going on and answer any
questions that the care-givers have.
Although it may be distressing at the time, there is evidence that parental presence
during resuscitation attempts reassures care-givers in the long term that everything possible
was done for their child.
Talking to care-givers after the death of a child

This can be particularly stressful for a doctor who is not used to dealing with this situation.
Care-givers often do not remember much of what is said but will remember kindnesses and
people who take the time to talk to them and show empathy after the death of a child. Being
honest particularly about the things that are not understood, e.g. the cause of death or why
an accident happened, is particularly important.
Siblings
There are no rules about what to say to siblings, or whether they should visit or see a dying
sibling. Many children will have created an impression of what is happening to a loved
sibling. Visiting their sick brother or sister and seeing the reality of what is happening may
bring comfort as the childs imagination may have created a more distressing image.
Chapter 29: When a child dies
269
Bereavement counselling and follow-up

Bereavement counselling can be accessed through either hospital or community services.
Care-givers may find this useful immediately after the death of a child or in the months or
years afterwards. Counselling can be accessed through voluntary organizations or the
family doctor.
Care-givers frequently have unanswered questions after the death of a child. An
individual who can act as a point of contact at the hospital to signpost care-givers to an
individual who can answer questions is important. Most hospitals offer care-givers
an opportunity to return after a few weeks so that the results of any tests or post mortem
examination can be shared with them and to discuss any questions they may have.
Unanswered questions can make grieving more difficult and where answers are available
they should be given to care-givers so that they understand as much as possible about what
happened to their child.
Organ donation
Children may donate organs after death. This usually occurs when patients die in PICU
following brainstem death after neurological injury. Organ donation is done with the
consent of the care-givers.
Organ donation is also possible after cardiac death when the patient has a nonsurvivable condition or injury and withdrawal of intensive care results in cardiac arrest.
Kidney, liver, lung, corneas, heart valves and small bowel may be donated. However, in
these circumstances heart transplantation is not possible.
In the UK organ donation does not usually occur in children under 6 months of age. In
the USA there is no lower age limit.
Further reading
Beauchamp TL, Childress JF. Principles of
Biomedical Ethics. Oxford University Press,
2008.
Royal College of Paediatrics and Child Health.

Withholding or Withdrawing Life Sustaining
Therapy. A Framework for Practice, 2nd edn.
2004.
General Medical Council. Treatment and care

towards the end of life. Good practice in
decision making. July 2010.
Section 3
Chapter
30
What You Could Be Expected to Do in a District General Hospital
Child protection
Kate Skone and Geoff Debelle
Introduction
It is estimated that around 1% of injuries seen in EDs are not accidental. Children of any age
are at risk of assault and non-accidental injury (NAI). However, NAI is commonest under
1 year of age. A Welsh study in 2002 estimated that approximately 1 in 880 babies are
abused in the first year of life. The mode of injury, particularly NAI, should be considered
in all critically unwell children.
The majority of sudden unexpected deaths in infancy (SUDIs) are due to natural causes; only
a small minority are homicides. The sudden unexpected death of a child or infant will automatically initiate an investigation into the cause of death. This will include looking for evidence of NAI
by the investigating team. The priorities in the management of child protection issues include:
involving the sudden unexpected death in infancy (SUDI) team as soon as concerns are raised
ensuring documentation is detailed
taking appropriate samples and using the correct procedures when handling samples.
The management issues in a child where NAI is suspected and the protocols associated with
the death of a child will be described in this chapter.
Documentation standards
The medical notes for a child who has died may well form evidence in court. However,
documentation standards for child protection purposes are no different from standards for
all clinical notes (see Table 30.1).
Table 30.1. Minimum standards for documentation.
Name, designation, GMC number on every page

Name, hospital number of patient, date of birth of patient on every page
Date and time of writing notes (24-hour clock)
Full names of others present clinical staff and family members
Detailed description of clinical events
Any clinical or family history obtained
Any direct quotes from family that are relevant in full
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Chapter 30: Child protection
271
The paediatric team will complete a detailed assessment, usually on a pre-printed

proforma, that includes a detailed history, past medical history, social history and family
history. In addition they will carry out a full and detailed examination that is recorded on a
body map diagram. If it is possible, consent for this assessment should be taken from a
person with parental responsibility.
There will be many other team members involved in the care of a critically ill child. In
cases of NAI, the responsible consultant paediatrician will usually write the medical reports
that are sent to the police. However, they may not be present when the child arrives
in hospital. The police, therefore, often request reports from all of the clinicians involved
in the care of the child. All reports should be reviewed by the consultant paediatrician or, in
the case of a death, the consultant leading the SUDI team, before submission.
A key responsibility of every team member is therefore accurate and contemporaneous
documentation of their observations and the resuscitation.
It is possible that, following investigation, a case may go to court and the court may
contact all clinical staff involved in the case. However, the responsibility for attending court
and providing medical information usually falls to the lead consultant paediatrician.
Management of laboratory samples the chain of evidence

All samples should be taken to the laboratories using a chain of evidence. A chain of evidence
is a legal concept that requires the names of all persons handling a sample, and the places and
conditions of storage, to be documented. The form accompanying each sample will include:
the person handling the samples signature
the time and date
place of storage or transfer.
This is to ensure that samples are not tampered with. If a chain of evidence is not started
when a sample is taken, it is invalid. Chain of evidence proformas should be available in all
EDs and PICU.
Critically ill children where NAI is suspected

It is important to consider NAI when looking after a seriously ill or injured child. The
clinical scenario will dictate how high on the list of differential diagnoses this may be
considered. An inconsistent history or worrying patterns of injury should alert staff to the
possibility of harm being inflicted on a child.
History
After the initial stabilization of a sick child an experienced clinician, probably a paediatrician,
will need to take a history of events. There may be information from other healthcare
professionals, such as paramedics, which may indicate that NAI should be considered.
When the history is taken the following factors may increase suspicion of NAI:
history that does not seem to fit with the pattern of injuries
inconsistent history between family members
injuries that are attributed to a child or sibling and are not commensurate with their
developmental stage
delay in presentation to healthcare.
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Examination
If a child presents with any of the injuries in Table 30.2 it is important to consider whether
they are inflicted injuries and take a careful history (see above).
Table 30.2. Injuries that should prompt consideration of NAI as a cause.
Complex skull fractures (bilateral, stellate, crossing suture lines)

Abdominal trauma without any history of abdominal injury
Multiple clusters of bruises
Bruises away from bony prominences such as ears, face, chin, buttocks, trunk and posterior thigh
Bruises in the shape of objects
Drowning (in infants)
Human bite marks
Poisoning, such as methadone ingestion, that may present as non-traumatic coma
Burns forced-immersion scalds, contact burns, burns in unusual areas and burns from house fires
Fractures
Long bone fractures in infants < 18 months
Multiple fractures
Fractures in unusual sites (scapula, sternum)
Occult fractures such as rib or metaphyseal
Torn labial or lingual frenulum
Abusive head injury is the commonest inflicted life-threatening injury. Features that
would raise concerns of NAI in a head injury include:
subdural haemorrhages in children under 1 year old, particularly multiple subdurals
associated hypoxic ischaemic injury and cerebral oedema
no evidence of impact injury
co-existing acute encephalopathy (with apnoea, seizures and collapse)
other injuries to the head and neck or long bone fractures
multiple retinal haemorrhages through all layers of the retina, may be unilateral.
Management
Non-clinical
The principal differences in the care of a child when NAIs are suspected are the additional
medical investigations and social enquiries that are carried out. It is important to inform the
parents of each step in this process. This process will include referral to childrens social
services. Parents should also be informed about this, unless this action will put the child at
increased risk.
Any members of the team who have concerns about child protection must make sure
that these are communicated to the team leader and the child safeguarding teams so that
273
they can be investigated appropriately. The child must not be discharged home without a
clear decision being made and documented.
Clinical
When managing a child with suspected NAI it is important to remember that the child may
have additional unseen injuries. They should therefore be managed as a trauma patient. The
important sites to consider are:
abdominal injury: there may be no signs of external injury but visceral trauma may
result in the patient becoming cardiovascularly unstable
intracranial injury: children with abusive head injury may have multiple subdurals,
parenchymal contusions and generalized cerebral hypoxic ischaemia with brain swelling
limb injuries: children who have been assaulted may also have new or old fractures that
will be revealed on a skeletal survey.
Additional investigations
Alongside the important clinical management, additional investigations will be necessary to
assess for further injuries or look for medical causes that explain the illness or injury (see
below).
Ongoing child protection issues

Once the child has been stabilized ensure that the child is in a place of safety and that they
are kept free from further harm. A critically ill child is likely to go to intensive care. Police
and social services have powers at their disposal to keep children in a place of safety and
limit or supervise visitors.
Once a referral has been made to social services and the police are involved in ongoing
investigations a range of court orders can be used to ensure the child is kept safe. These can
be initiated immediately, and are then continued by the family courts. It is the responsibility
of social services to set these up but the team looking after the child need to know if the
child is under a court order, who is allowed to visit, and if the visits need supervising.
Police and social services need to be involved early as there may be other children who
need to be in a place of safety.
Sudden unexpected death in children and infants (SUDI)

The investigation of deaths in children is always a difficult and sensitive issue. Over 300
babies a year die suddenly and unexpectedly in the UK. Following several recent highprofile cases it is an area that is under a great deal of scrutiny. Government guidance has led
to the structure and organization of child protection services being tightly legislated.
Children may have a sudden and unexpected death either in hospital or as an out-ofhospital cardiac arrest. Every ED and paediatric department will have a local protocol
for the assessment and management of the sudden, unexpected deaths. The SUDI team
and the on-call paediatric consultant should be involved in all of these cases. This team is
also involved in investigating traffic accidents, suicides and children who have a pre-existing
life-limiting condition who are deemed to have died unexpectedly.
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When a child is found dead resuscitation is usually attempted and is often ongoing on
arrival at hospital. The police are informed by the ambulance crew and are often present
when the child is brought into hospital. The police are responsible for leading the investigation, along with childrens services.
The role of the health professionals is to provide appropriate medical care. If the child
dies they must carry out the relevant investigations into the cause of death and produce a
medical report. This medical report will be written by the paediatric consultant involved in
the case or by the named doctor for child protection. As part of the investigation the SUDI
team will carry out a rapid response investigation that may include a home visit by a senior
clinician. These home visits can provide a wealth of information for the subsequent
investigations. This is always carried out when a child dies and may also be appropriate
following an out-of-hospital arrest.
Medical investigations to consider in a critically ill child or

following a SUDI
The investigations carried out in each case of critical illness or death will be dictated by the
clinical situation. The results may form part of a criminal investigation, and should
therefore be managed with a chain of evidence (see above).
If a critical illness or death is unexpected the purpose of investigations is to look for a
medical cause, including rare causes, or to provide evidence of inflicted injury. This usually
includes detailed imaging and extensive investigations for infection, toxicology, metabolic
disease and inherited conditions. The majority of these additional investigations can be
carried out after the child has been stabilized.
A medical investigation into the cause of an unexpected death will involve a post mortem.
Any samples taken during resuscitation may also be important in determining the cause of
death. There are also investigations that need to be sent off immediately after death.
The RCPCH Child Protection Companion is a valuable source of suggested investigations in children who present with particular patterns of injuries; an example of
investigations for abusive head trauma can be seen in Table 30.3.
Table 30.3. Investigations in abusive head trauma.
Formal indirect opthalmoscopy through dilated pupils for retinal haemorrhages using RETCAM
Neuroimaging:
CT scan of head as soon as stable on presentation
If CT head abnormal then MRI head and spinal cord on day 35
Following MRI if abnormalities at 36 months after injury
Skeletal survey when stable and repeat survey or chest film in 14 days
Coagulation studies full blood count and film, PT, APTT (not INR), thrombin time, serum
fibrinogen, factor VIIIc, VWF antigen and activity, platelet glycoproteins Ib, IIb/IIIa or PFA closure
time with epinephrine and ADP, factor XIII screen (if under 3 months)
Blood for carnitine and acyl carnitine profile
Blood cultures to exclude sepsis
Urine for toxicology and metabolic screen
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Some children who present seriously unwell to the ED may go on to die on PICU.
Samples taken at the time of presentation can be vital in investigating the cause of death; for
example, it is important that coagulation studies are done before administering blood
products where possible, or toxicology is taken immediately at the time of presentation.
Every region will have a list of recommended investigations that will be carried out
following a SUDI; an example of these is detailed below from the West Midlands SUDI
protocol (Table 30.4).
Table 30.4. Example of investigations undertaken following a SUDI (from West Midlands SUDI protocol).
Blood should be taken ideally within 30 minutes of death and not more than 4 hours after death.
All the sites of sampling should be documented and a cardiac stab may be necessary. The
investigations include tests for infection, toxicology, metabolic causes and genetic causes of death
Blood cultures
Full blood count (consider carboxyhaemoglobin)
Electrolytes, renal and liver function
Serum for toxicology
Blood for a Guthrie card (if available) for inherited metabolic diseases
Cytogenetics
CSF for culture and microscopy
Nasopharyngeal aspirate for microbiology and virology and immunofluorescence
Swabs from any identifiable lesions for microbiology
Urine for culture, toxicology and inherited diseases
Throat swab for culture and microscopy
Skin biopsy for fibroblast culture
Skeletal survey; it is important that parents do not have unsupervised access to the body prior to
this happening
Organ donation
The process of investigation does not exclude the possibility of organ donation. However,
coroners may differ in their response. Some may allow donation of organs if the paediatric
and forensic pathologist is present in the operating theatre to ensure that no evidence is lost
in the process. If organ donation is a possibility then the regional donor transplant
coordinator should be involved as soon as possible.
What do I need to have organized before departure to PICU?

In suspected NAI, the clinical management of a critically ill child takes priority. In order to
ensure that any necessary investigations are carried out as smoothly as possible the most
important thing to do is to ensure that the contact information for the local paediatrician
who is going to lead on the child protection investigation is available. The local paediatric
276
team will be involved in any child protection investigations and they will liaise with social
services, the police and the ICU as child protection investigations continue.
It is important to keep parents and carers informed of all steps in the child protection
process and investigation.
Summary
Child protection is the responsibility of the local paediatric team but careful documentation
and management of samples is crucial. This chapter has detailed the child protection
management that will occur alongside the stabilization of a sick child.
Golden rules
Documentation must be scrupulous
Samples must be transported with a chain of evidence
Samples should be taken for coagulation studies before any blood products are given
Treat a child who has been assaulted as a trauma patient and remember to look for
additional injuries
Families must be handled sensitively
Further reading
Craft A. Child Protection Companion. RCPCH,
2006. (Revised edition to be published 13
March 2013.)
RCoA child protection guidelines.

http://www.rcoa.ac.uk/document-store/
child-protection-and-the-anaesthetistsafeguarding-children-the-operatingtheatre.
http://www.core-info.cardiff.ac.uk.
Section 4
Chapter
31
The Childrens Hospital Setting
Ventilation
J. Nick Pratap
Introduction
Critically ill children often require ventilatory support as part of their intensive care
management. Indications include:
prevention of airway soiling
reversal of failing gas exchange
overcoming airway obstruction or protecting airway structures
reduction in the work of breathing, e.g. advanced circulatory failure
access for suction of secretions
initiation of therapies, such as bronchial lavage in burns, or surfactant administration in
prematurity.
Considerations when ventilating children

Physiology
A respiratory membrane capable of gas exchange develops by 2223 weeks gestation, but
true alveoli only develop after 30 weeks. Very premature birth, especially before 26 weeks,
disrupts lung development. This can be compounded by factors such as pneumonia, oxygen
toxicity and the mechanical effects of artificial ventilation, leading to chronic lung disease
(CLD) of prematurity.
Smaller children
There are a number of physiological differences between infants and older children/adults,
including:
at term there are only a third to half the adult number of alveoli
they have a high oxygen requirement per unit body weight and low relative functional
residual capacity (leading to rapid desaturation)
respiratory muscles of young children have a low proportion of high-endurance muscle
fibres, so they tire more readily than adults
apnoeas are often accompanied by bradycardia and desaturation.
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Section 4: The Childrens Hospital Setting
For premature babies, the difference is even greater:

surfactant is not secreted in adequate quantity until approximately 30 weeks (may be
stimulated earlier by exogenous corticosteroids if given to the mother prenatally)
control of breathing is not fully matured in preterm infants central apnoeas occur in
response to a wide range of insults.
The work of breathing is higher in spontaneously breathing young children, consequently
establishing mechanical ventilation can substantially reduce these demands.
Ventilators and associated equipment in PICUs

Ventilators
Children requiring ventilation on PICU can vary from below 500 g to more than 100 kg in
weight. No single ventilator is ideal for the whole range. NICU ventilators often work well
for infants up to 510 kg. Above this adult ventilators are often suitable, although paediatric
software upgrades may be needed.
Humidification
For PICU ventilators active humidification is standard, often composed of a hot water bath
with heating wires in the inspiratory limb to reduce condensation. For transport purposes
heat and moisture exchange (HME) filters are suitable.
End-tidal capnography
This may reduce the need for blood gas analysis, but is less accurate with small tidal volumes
and fast respiratory rates. Main-stream analysers are less affected than side-stream, so may
be better in small children. Care needs to be taken to ensure the weight of the sensor does not
kink the endotracheal tube (ET tube).
Suction
ET tube blockage is a problem, especially with the smaller sizes necessary in newborns;
urgent re-intubation may be necessary. An inability to pass an endotracheal suction catheter
(in French gauge, twice the internal diameter of the ET tube) may be the first sign, but the
problem should also be considered in the face of increasing ventilator pressure requirements or an obstructive ETCO2 trace.
When suctioning the trachea in children, many prefer to limit passage of the catheter to
the end of the ET tube, to avoid damaging the fragile mucosa.
Ventilator therapies
Non-invasive ventilatory support (NIVS)
In this instance NIVS is used to cover both CPAP and non-invasive ventilation (NIV).
Ventilation support that avoids intubating the trachea may reduce both the risk of respiratory infection and the need for sedation. Problems when using NIVS in children include:
lack of cooperation careful use of sedation can help
skin trauma may develop rapidly, but can be ameliorated by application of colloid
dressings
Chapter 31: Ventilation
279
gastric distension and diaphragmatic splinting inserting a gastric tube (via the oral
route if using nasal NIVS) helps overcome this
difficulty clearing lower respiratory tract secretions can be overcome with effective
physiotherapy
it may not be possible in cases of orofacial trauma or congenital anomaly, such as
choanal atresia.
In neonatal practice NIVS is commonly chosen as a first-line support, mainly in the form of
nasal CPAP, as it is useful for apnoeas related to both prematurity and respiratory infection.
Infants with very immature lungs are often extubated early to CPAP after intratracheal
administration of surfactant, to protect the lungs from iatrogenic damage.
Some of the indications for NIVS in children are given in Table 31.1. However, it tends
to fail in patients who meet ARDS criteria or have multi-organ dysfunction, due to severity
of underlying disease.
Table 31.1. Indications for NIVS in children.
Condition
Comments
Neuromuscular disease Useful with intercurrent pneumonia avoids risks and complications of
intubation, including ventilator dependence
Obstructive sleep
apnoea
Usually nocturnal support only
Immunocompromise
Intubation associated with poor outcome
Sickle-cell acute chest

syndrome
Use in combination with good analgesia
Cardiomyopathy/
myocarditis
Supports myocardium through cardiopulmonary interactions and

avoids cardiovascular system decompensation at intubation
Laryngomalacia
Helps overcome upper airway obstruction
Post-extubation
May avoid need for reintubation if ongoing CVS or RS compromise, such

as impaired cardiac function or post-surgical diaphragmatic paresis
Continuous positive airway pressure (CPAP)

A face mask, covering both nose and mouth, is often used to deliver CPAP, but nose-only
masks and helmets have a role too. For infants the nasal route is preferred, with short,
binasal prongs shown to be more effective than a single nasopharyngeal prong.
Bi-level ventilation
Non-invasive bi-level ventilation can be used instead of CPAP, particularly if CO2 clearance
is a problem. Generally a flow trigger is used, as this is most sensitive to small respiratory
efforts. A backup rate may also be provided. Typical set pressures are 1020 cmH2O and
510 cmH2O for inspiratory and expiratory phases, respectively.
Invasive positive-pressure ventilation

For the majority of children with normal lungs, such as postoperative surgical cases,
conventional pressure-controlled ventilation is ideal. This is because it compensates better
280
than volume-control for a leak around an uncuffed ET tube. Typical ventilator parameters
are set as follows:
peak inspiratory pressure (PIP) of 1418 cmH2O
positive end expiratory pressure (PEEP) 5 cmH2O
I:E ratio around 1:1.52
respiratory rate in the low normal range for spontaneous breathing in that age group
(see Chapter 35).
When adjusting the ventilator to ideal settings, change the PIP to achieve a tidal volume of
57 ml/kg. If the child should start breathing, it may be more comfortable for the child to
set a SIMV or bi-level version of the mode.
Displayed tidal volumes may be inaccurate in small children as ventilator tubing distends
with increased pressure, giving a falsely high reading. A visual check for adequate chest
movement along with assessment of end-tidal and arterial CO2 should always be performed.
In order to achieve adequate chest movement, tidal volumes up to 10 ml/kg may be used.
In synchronized (spontaneous) modes flow rather than pressure triggers are preferred, as
less respiratory effort is required. Although there may be concern about the work of breathing
through a narrow ET tube, studies have shown this to be negligible, even for small infants.
A pressure-generated volume guarantee mode (such as pressure-regulated volume
control, PRVC) is also suitable, especially where it is desirable to maintain a stable PaCO2
or where compliance and resistance may change rapidly.
As with adults, permissive hypercapnia may be an appropriate strategy, if not contraindicated. Following the influential ARDSnet study of lung-protective ventilation, respiratory acidosis is tolerated as long as the pH remains above 7.15.
High-frequency oscillatory ventilation (HFOV)

HFOV is frequently used in PICU, with the aim of avoiding ventilator-induced lung injury
and improving oxygenation. The ventilator generates compressions and rarefactions in
the inspired gases, similar to sound waves but below the threshold of human hearing. As the
tidal volumes generated are smaller than the anatomical dead space, gas transport to the
respiratory membrane depends on non-physiological mechanisms.
The main adjustable parameters used are:
mean airway pressure (MAP)
frequency (f ) of oscillation
amplitude or power (P) of oscillation
To improve oxygenation MAP (and FiO2) can be altered whereas P and f govern CO2
clearance.
HFOV is most frequently used following failure of adequate oxygenation or CO2
clearance on conventional ventilation. It may also be used if high inspiratory pressures
and FiO2 are required to maintain oxygenation with conventional ventilation, or the patient
has developed an air leak.
Disadvantages of HFOV include:
elevation of intrathoracic pressure avoid in intracranial hypertension
drop in BP when HFOV initiated, by reducing venous return fluid bolus often needed
ET tube suction leads to lung derecruitment
no clinical information from auscultation and ETCO2 measurement.
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Initiating HFOV should be learned in a practical session and follow local guidelines. The
following is provided as a guide.
Ensure a relatively straight path from the ventilator to the patient. Avoid even minor
kinks of the ET tube and ventilator tube. Do not use an angle connector.
Frequency should be set at 1012 Hz for a preterm neonate, 810 Hz for an infant, and
5 Hz for a larger child.
MAP should be set 24 cmH2O higher than the MAP on conventional ventilation.
Set the P at 10, then increase whilst directly observing the chest of the patient until
adequate bounce is seen.
Perform an arterial blood gas within 20 minutes as a precipitous drop in PaCO2 is
common.
Perform a CXR within the first few hours. Over-distension of the lung may occur. This
is associated with suboptimal gas exchange and barotrauma. The diaphragm should be
at the level of the eighth rib posteriorly.
MAP should remain unchanged until the FiO2 has been weaned to 0.5 or less.
CO2 levels can be adjusted through the P and the frequency (reducing f increases CO2
clearance).
Increasing the MAP to improve oxygenation only works until the lung is over-distended. If
increasing the MAP worsens oxygenation then reduce the pressure and reassess. Over time
the lung compliance will change on the oscillator; this means that the original MAP may
become an over-distending pressure over time. Repeated CXRs and clinical monitoring of
the patient should avoid this.
HFOV works better in diffuse lung disease and less well in unilateral disease, due to the
risks of over-distending normal lung.
Aids to ventilation
Inhaled nitric oxide (iNO)
iNO dilates the blood vessels associated with ventilated alveoli. This may be useful in both
pulmonary hypertension and parenchymal lung disease where there is substantial ventilation/perfusion mismatch. There is minimal systemic vasodilatation as nitric oxide (NO) is
readily inactivated by binding to haemoglobin in the pulmonary circulation.
iNO concentrations greater than 1020 parts per million (ppm) have no additional
benefit. Intensivists tend to start at 20 ppm and reduce according to response. Because
habituation occurs within a few hours, NO must be weaned slowly, even if there is no
clinical benefit. There is a risk of methaemoglobinaemia with prolonged use, so arterial
blood co-oximetry should be performed at least daily to identify this. Nitrogen dioxide is
inevitably delivered with iNO due to reaction with oxygen. The concentration should be
monitored and not allowed to rise above 2 ppm due to the potential for pulmonary toxicity.
Specific ventilatory strategies for different conditions

Preterm neonates
Extremely preterm infant lungs are deficient in surfactant. This increases the work done
with each breath to re-open collapsed, poorly compliant alveoli. When combined with
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minimal airway resistance, this means the time constant () of the respiratory system
(compliance resistance) is low. Neonatology practice reflects this, with a preference for
a short inspiratory time (0.40.5 s). Meta-analyses confirm that this is associated with lower
incidence of air leak syndromes and even a decrease in mortality.
Pressure-controlled ventilation is traditionally used, but volume-targeted modes can
result in earlier extubation, and a reduction in both pneumothorax and severe intraventricular haemorrhage rates. Synchronized ventilatory modes show benefit in terms of air
leak and duration of ventilation. From large meta-analyses it is clear that choosing conventional ventilation or HFOV does not affect long-term respiratory or neurological outcome.
In severe necrotizing enterocolitis (NEC) patients suffer overwhelming Gram-negative
sepsis and develop respiratory failure similar to ARDS. They may also have a tense
abdomen, causing considerable mechanical compromise of ventilation. In these patients a
longer inspiratory time is often necessary to maintain oxygenation.
The target for oxygenation in premature neonates is lower than in older children in
order to reduce the risks of oxygen toxicity, including CLD and retinopathy of prematurity
(ROP). However, there are dangers of insufficient oxygenation in this group too, notably
patent ductus arteriosus, developmental delay and even increased mortality. The precise
at-risk group is not yet defined, but it seems prudent to exercise caution with infants born
below 32 weeks or birthweight less than 1.5 kg. An oxygen saturation target of 9294% is
appropriate below 32 weeks of age, thereafter a higher target of 9499%.
Current practice supports extubation of premature infants from a low ventilator rate
without a trial of CPAP. However, electively putting infants on nasal CPAP reduces
extubation failure risk.
There has been considerable research into pharmacological strategies to reduce length of
ventilatory support and to decrease the incidence of CLD. Diuretics do not appear helpful in
this regard. Postnatal steroids have benefits in terms of CLD reduction, but may be
associated with worse neurobehavioural outcomes. Antenatal steroid administration to
the mother and early postnatal surfactant do improve outcomes.
Chronic lung disease of prematurity

Premature infants requiring aggressive ventilation, or with a genetic predisposition, may
suffer scarring and overinflation of the lungs, particularly involving the bronchioles and
alveolar septi. This bronchopulmonary dysplasia may be so severe that the infant remains
ventilator-dependent for months or even years.
Some children receive long-term ventilatory support in hospital, whilst others are
discharged home, often with a tracheostomy. Exacerbations of lung disease may be triggered by common respiratory viruses and can be life-threatening, so PICU admission is
common. Aspiration pneumonia is frequent in this population, as long periods of intubation tend to disrupt the usual protective laryngeal mechanisms.
Asthma
Ventilation of children with asthma follows similar principles to that in adults. Slow rates are
preferred to allow adequate time for expiration. PEEP should be matched to the patients
intrinsic PEEP, which may vary considerably over time (see Chapter 8 for more details).
Barotrauma and air leaks are very real risks, so pressure-control modes are advisable.
A higher than normal maximum peak inspiratory pressure can be tolerated (up to
283
35 cmH2O), as this is not transmitted to the alveoli, due to increased airway resistance.
Hypercapnia is usually well tolerated.
Bronchiolitis
The pathophysiology of viral bronchiolitis is narrowing of the small airways. It is commoner
in young children than asthma, but the principles of mechanical ventilation are broadly
similar as it is an acute obstructive lung disease.
Many of the worst affected infants have pre-existing cardiac or lung disease, particularly
chronic lung disease of prematurity. HFOV is sometimes used, if conventional ventilation
fails.
Tracheobronchomalacia (TBM)
Floppiness of tracheal and/or bronchial wall cartilage results in a tendency for the airway to
collapse during active expiration. The key role of the intensivist in TBM is to consider this
diagnosis in the infant who is failing a respiratory wean, particularly if they suffer cyanotic
spells when agitated.
The diagnosis is best made by bronchography or flexible bronchoscopy. The condition
may result from a developmental abnormality of the airway cartilage, from prolonged
ventilation (particularly in premature infants) or secondary to external compression by a
cardiovascular malformation. High CPAP levels following extubation act as a pneumatic
splint for the airways. Surgical treatment or stenting is sometimes required.
Congenital diaphragmatic hernia (CDH)

In this condition the fetus develops with abdominal viscera within the thoracic cavity.
Repair of CDH has become a relatively straightforward surgical procedure. However, the
increasing success of surgical intervention has highlighted the major cause of morbidity and
mortality in these children as pulmonary hypoplasia and pulmonary hypertension. In fact,
the pulmonary hypoplasia may be a primary event during the formation of a CDH and not
just a consequence. Management following delivery includes:
avoiding positive-pressure ventilation by face mask
immediate intubation and ventilation with paralysis to prevent air swallowing and
bowel distension
decompression of the stomach with an NG tube (may be seen to loop back up into the
chest on CXR)
high PaCO2, which is often tolerated and may be necessary to avoid ventilator-induced
lung injury (even in the presence of pulmonary hypertension).
Decisions regarding therapy, particularly consideration for extracorporeal membrane
oxygenation (ECMO), are complicated by prognostic uncertainty, as the quantity of healthy
lung tissue may be inadequate for a good long-term outcome.
Surgical repair of the CDH is usually delayed until pulmonary hypertension is under
control and the long-term outlook is clearer.
Congenital heart disease

While standard principles of ventilation apply to most children with congenital heart
disease, there are important considerations in certain pathologies.
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When there is unrestricted flow between the pulmonary and systemic circulations the
proportion of blood flow that passes to each depends on the balance of the pulmonary
vascular resistance (PVR) and the systemic vascular resistance (SVR). Connections that can
allow unrestricted flow include:
VSD
ductus arteriosus
BlalockTaussig shunt/aortopulmonary window.
In

these patients the oxygenation of arterial blood is dependent on the following factors:
gas exchange in the lung
the ratio of pulmonary to systemic blood flow
degree of mixing of arterial and venous blood.
There are two corollaries of vital importance.

The usual target of oxygen saturations in the high 90s may be dangerous, as it would
require such high pulmonary blood flow that systemic perfusion would be compromised.
Should the childs oxygen saturations change, it is necessary to consider both the lungs
and cardiovascular system as possible causative factors.
Balancing circulations
In patients with univentricular physiology, maintaining good lung function is vital. It is
desirable to prevent atelectasis. Low levels of CPAP or PEEP should be used routinely.
Excessive intrathoracic pressure impedes pulmonary blood flow, so high PEEP is best
avoided. To prevent a precipitous drop in PVR and therefore systemic perfusion, FiO2 is
kept low, targeting oxygen saturations of 7585%. Indeed it is common to ventilate in air.
For the same reason, PaCO2 is often kept towards the upper end of the normal range (see
Chapter 6).
Children with surgical shunts

As children with surgically shunted physiology grow, their shunts do not. The ratio of
pulmonary to systemic blood flow progressively reduces. When pulmonary blood flow is
insufficient, further intervention is required.
Failing right heart

Restrictive right heart failure
Acute right heart failure is sometimes seen postoperatively. A classic example occurs after
surgical correction of tetralogy of Fallot. This restrictive physiology is exquisitely sensitive
to loading conditions, including intrathoracic pressure.
Univentricular systems
For most children with univentricular hearts the Fontan circulation (total cavopulmonary
connection) represents their final physiology. To achieve this, following a cavopulmonary
shunt, the inferior vena cava is anastomosed to the pulmonary arteries. In these children, all
systemic venous blood bypasses the heart and passes directly to the lungs. The absence of a
right ventricle can be thought of as an extreme form of right heart failure.
285
Both the above groups of patients are kept on low PEEP (usually 5 cmH2O) when intubated.
Spontaneous modes and early extubation are preferred where possible, since the negative
intrathoracic pressure generated by spontaneous ventilation promotes better pulmonary blood
flow. Patients are very dependent on adequate circulating blood volume. Hypovolaemia must
be considered should either blood pressure or systemic oxygen saturations drop.
The child who is difficult to ventilate

When difficulties are encountered in achieving adequate gas exchange the following initial
steps should be undertaken:
muscle relaxation (with adequate sedation) metabolic oxygen requirements are
minimized and unhelpful respiratory efforts abolished
review the CXR for correct ET tube position, lung expansion, areas of collapse and
evidence of fluid overload
optimize cardiac output with fluids and inotropes
consider changing ET tube, especially in infants, as partial ET tube blockage is hard to detect
correct anaemia (or increase Hb if cyanotic CHD)
review the target for oxygenation and PaCO2 depending on underlying conditions
(e.g. CLD and CHD) and evidence of tissue oxygenation (e.g. lactate and mixed venous
oxygen saturation)
prone positioning may be beneficial and is usually easy in young children, though care
must be taken with lines and tubes.
If the child remains difficult to ventilate, a therapeutic trial of HFOV may be performed.
iNO can be tried if severe hypoxaemia remains. Exogenous surfactant administration has
been shown to reduce mortality in ventilated children with acute respiratory failure.
Bronchoscopy may elucidate uncertain diagnoses in ventilated children. It also facilitates instillation of the mucolytic agent deoxyribonuclease (DNase), with reports of success
in conditions such as lobar collapse, asthma and acute sickle-cell chest crisis.
Refractory respiratory failure

Extracorporeal life support (ECLS) is the subject of renewed interest following trial evidence
of good outcome in adults in whom ventilatory strategies fail to achieve adequate gas
exchange. Extracorporeal membrane oxygenation (ECMO) is the most common modality.
The principle is similar to cardiopulmonary bypass, but the circuit is optimized for
longevity. Percutaneous cannulae are used and generally inserted by surgeons. Two types
of treatment are available:
veno-venous ECMO blood is both drained and returned to the right atrium
veno-arterial ECMO blood is drained from the right atrium and returned to the aorta.
The latter does not depend on native myocardial function, so is ideal for cases of cardiogenic shock or where high inotrope doses are needed.
Weaning and extubation

No single mode of ventilation or weaning protocol has been shown to facilitate ventilatory
weaning in paediatric critical care. Many PICU patients can be extubated successfully
286
without protracted weaning, although no clinical test adequately predicts readiness for
extubation.
Upper airway obstruction accounts for around one-third of PICU extubation failures.
Unfortunately, there is no reliable way of predicting this. Dexamethasone, started at least
6 hours before planned extubation, reduces the need for re-intubation, but is usually
reserved for those most likely to benefit, such as prolonged intubations, difficult airways
or patients with trisomy 21.
Weaning difficulty
Achieving successful extubation can be a challenge in paediatric practice, for a number of
reasons:
neuromuscular weakness may occur in very ill children with a prolonged PICU stay
undiagnosed congenital conditions may be present in newborns
residual shunts, valvular lesions or myocardial failure occur following cardiac surgery
airway compression by abnormal vascular structures may cause airway obstruction and
tracheobronchomalacia
phrenic or laryngeal nerve lesions may occur after thoracic surgery.
Flexible bronchoscopy may reveal unanticipated airways problems. As in adults a tracheostomy may facilitate weaning, but this is less frequently employed, as it needs to be
performed surgically. A good way to consider the causes of failure to wean is to think of
the six Fs:
failure of organ systems
fluid balance
feeding
fever
fear, pain and anxiety
farmacology (sic).
Summary
The need for ventilation is common in critically ill children. By fully understanding their
different physiological and pathological considerations, it is possible to undertake this safely
and effectively.
Golden rules
Always ventilate children when indicated dont delay as they can decompensate very
rapidly
The majority of children can be managed with similar ventilatory strategies to adults
Avoid interventions that may cause harm to the patient or their lungs
Further reading
Essouri S, Chevret L, Durand P, et al.
Noninvasive positive pressure ventilation:
five years of experience in a pediatric
intensive care unit. Pediatr Crit Care Med
2006;7:329334.
Newth CJL, Venkataraman S, Willson DF, et al.
Weaning and extubation readiness in
pediatric patients. Pediatr Crit Care Med
2009;10:111.
287
Saugstad OD, Aune D. In search of the optimal

oxygen saturation for extremely low birth
weight infants: a systematic review and metaanalysis. Neonatology 2010;100:18.
Stocks J. The respiratory system. In Bingham R,
Lloyd-Thomas A, Sury M (eds.), Hatch &
Sumners Textbook of Paediatric Anaesthesia.
London: Hodder Arnold, 2008.
Section 4
Chapter
Fluid therapy
32
Adrian Plunkett
Introduction
Acutely ill children often present with derangements in water and electrolyte homeostasis.
Although these presentations frequently fall into recognized patterns, an individualized
approach to fluid therapy is required to correct deficits safely and establish appropriate
maintenance therapy.
A clinician who does not encounter paediatric patients in his or her daily practice may
feel daunted when faced with a requirement to prescribe fluid therapy for a sick child.
However, an understanding of basic physiological principles and knowledge of some
important potential pitfalls are sufficient to inform safe fluid therapy prescribing.
The main aim of this chapter is to describe a structured and simple approach to
paediatric fluid therapy, using physiological principles. Prior knowledge of the concepts
of osmolarity and tonicity and an understanding of the composition of common IV fluid
preparations are assumed (see Tables 32.1 and 32.2 for a summary). There is also a small
section on feeding, which is less relevant to the acutely ill child, but important to consider if
the child subsequently requires intensive care.
Table 32.1. Osmolarity and tonicity.
Concept
Definition
Osmolarity
The number of osmoles (particles exerting an osmolar effect) per litre of solution
Tonicity
The sum of the concentrations of the solutes which exert an osmotic effect across a
cell membrane, i.e. the effective osmolarity. The in vivo tonicity of 5% dextrose is
zero, because the dextrose is rapidly metabolized after infusion
288
Chapter 32: Fluid therapy
289
Table 32.2. Osmolarity and tonicity of common intravenous fluid preparations.
Fluid
Sodium ion
(mmol/l)
5% dextrose
Osmolarity
(mOsm/l)
Tonicity, in
vivo (mOsm/l)
278
0.18% sodium chloride and 4% dextrose
30
300
60
0.45% sodium chloride
75
154
154
75
432
154
0.9% sodium chloride
154
308
308
154
586
308
How does fluid therapy differ in children?

The principles of fluid therapy are consistent across all ages. However, it is important to
recognize certain physiological differences between adults and children when prescribing
fluid therapy. These differences are more pronounced in younger age groups, so particular
caution is required when prescribing fluid therapy for infants and newborns. Some important physiological differences between children and adults are listed below.
Energy expenditure (metabolic rate)

The relationship between metabolic rate and body mass is not linear; at smaller mass the
metabolic rate is considerably higher. Maintenance water and electrolyte requirements
are directly related to energy expenditure as maintenance requirements are replacement for the
physiological losses of metabolism. For this reason children require higher rates of maintenance
fluid therapy relative to their body size, compared with adults. Quantitative estimates of water
and electrolyte maintenance therapy are based on estimates of metabolic rate (see Table 32.4).
Urinary concentrating ability

Newborns typically pass isotonic urine but are only able to concentrate the urine to levels of
approximately 500700 mOsm/l (approximately 50% of maximal adult urine concentration). Along with the greater metabolic demands, this renders newborns and small infants
susceptible to dehydration. The urinary concentrating ability typically reaches adult levels
by 1 year of age.
Distribution of body water

The proportion of body mass made of water decreases with age until adulthood. Newborn
body mass is approximately 7580% water, compared with adult values of approximately
60%. The extracellular fluid compartment is particularly large in newborns, and undergoes
significant contraction in the first few days of life, resulting in physiological weight loss of
up to 10% of birth weight. Thus, fluid therapy in the first few days of life should be
restricted to avoid fluid overload.
Energy stores
Small children and infants have minimal energy reserves. Maintenance fluid therapy should
therefore include adequate glucose to prevent the onset of hypoglycaemia and catabolism.
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Non-osmotic release of antidiuretic hormone (ADH)

This is common in sick children. Under normal conditions, ADH is secreted in response to
increased serum osmolality, hypotension and hypovolaemia. However, it is also secreted in
response to a variety of non-osmotic stimuli, including illnesses such as pneumonia and
meningitis. ADH reduces the ability of the kidneys to excrete electrolyte-free water. In the
presence of non-osmotic ADH secretion, administration of hypotonic maintenance fluid
and failure to restrict water intake will result in hyponatraemia.
Surface area to volume ratio

Because of their high surface area to volume ratio, infants and small children are more
susceptible to dehydration secondary to increased evaporative losses of water.
Estimations of fluid and electrolyte therapy how much to give,

and how fast to give it
Acutely ill children often have deficits of water and electrolytes due to their presenting
illness. Fluid therapy is composed of three main elements:
restoring deficits treatment of shock, or correction of dehydration
maintenance therapy providing adequate water, electrolytes and energy to replace the
normal losses resulting from metabolism
replacement of ongoing, non-physiological losses replacement of excess loss from a
pathological process such as diarrhoea.
A fourth category would be replacement of circulatory volume, as in active haemorrhage.
Ideally, each of these three components of fluid therapy should be calculated separately
and combined to make a fluid prescription. It is important to remember that these
calculations will necessarily be based on gross estimates. Regular assessment of clinical
signs, with appropriate alterations to the fluid therapy, is therefore necessary to optimize
therapy.
Restoring deficits
Expansion of circulating volume in shock
Some patients present in shock and require rapid resuscitation. The goal of this stage of
therapy is to rapidly expand the circulating volume. Initial therapy should consist of
isotonic crystalloid or colloid, given in aliquots of 20 ml/kg as a rapid bolus. Repeat this
until haemodynamic stability is achieved.
If shock is still present after 60 ml/kg, further haemodynamic support is required, and
the patient is likely to require mechanical ventilation, central line insertion and inotropic
support.
Choice of fluid for volume expansion

Use an isotonic fluid. Current evidence does not support a preference regarding crystalloid
or colloid. Most clinicians favour using crystalloid over colloid as the initial therapy,
although colloid may be preferred in septic shock. Resuscitation fluid should not contain
potassium or glucose.
291
Correction of dehydration
The goal of this stage of therapy is to replace water and electrolyte deficits more slowly, over
2448 hours. Fluid deficit is normally presented as a percentage of body weight, as any
short-term reduction in weight is due to fluid loss alone. Estimate of severity of dehydration
and calculation of replacement therapy can be done in two ways:
clinical signs can be used to estimate the severity of dehydration as a proportion of body
weight (see Table 32.3)
a recent, reliable body weight (e.g. from an outpatient appointment) makes a more
accurate estimation of dehydration possible.
The rate of fluid deficit replacements and the type of fluid used depend on the plasma sodium
level but, in general, the fluid deficit should be replaced slowly (usually 2448 hours), avoiding
rapid changes in plasma sodium levels. Regular electrolyte monitoring is therefore essential.
Remember to include maintenance fluid and replacement of ongoing non-physiological
losses in addition to this fluid regimen.
Table 32.3. Clinical signs and symptoms of dehydration.
Degree of
dehydration
Mild
Moderate
Severe
Lethargic or irritable; postural

Clinical signs and Restless, thirsty;
symptoms
no clinical signs of hypotension; tachycardia;
dehydration
sunken eyes and fontanelle;
dry mucous membranes;
decreased urine output
Lethargic or comatose;
shocked; hypotensive;
grossly sunken eyes and
fontanelle; anuria
Deficit as % of
body weight
35%
69%
10%
Deficit in ml/kg
3050
6090
100
Fluid choice to correct dehydration according to sodium level

Isonatraemic dehydration (plasma sodium between 130 and 150 mmol/l)
This is the commonest type of dehydration, usually due to gastroenteritis. There has been
net loss of isotonic fluid, so replacement therapy should comprise isotonic, or near isotonic,
electrolyte solution (e.g. 0.9% saline or Hartmanns solution).
Hypernatraemic dehydration (plasma sodium >150 mmol/l)
In this situation water loss has exceeded sodium loss, so it would seem logical to replace
fluid losses with hypotonic fluid. However, there is a danger of reducing the plasma sodium
too rapidly, resulting in rapid influx of water to the intracellular compartment, potentially
causing fatal cerebral oedema. Infants and small children are particularly susceptible to this
event, due to the relatively high ratio of cerebral intracellular volume to skull-vault volume.
A safer approach is to commence replacement fluid therapy with isotonic fluid, and
consider reducing the tonicity (e.g. 0.45% saline 5% dextrose) if the plasma sodium level
is not falling after 6 hours.
The rate of decline in plasma sodium should be carefully monitored (12 hourly
sampling initially) aiming for a fall of no greater than 1 mmol/l every 2 hours.
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Worked example of fluid deficit calculation in dehydration:

a case of a 10 kg child with 10% dehydration; plasma sodium is in the normal
range; isonatraemic dehydration diagnosed.
In this situation, there has been net loss of isotonic fluid
Isotonic fluid should be used for deficit replacement
Aim to replace the deficit over 24 hours, with 50% in the first 8 hours
The deficit is 1000 ml (10% of 10 kg, or 1 kg fluid). Replace 500 ml over the
first 8 hours, and the remaining 500 ml over the next 16 hours
Maintenance fluid and replacement of ongoing non-physiological losses
should be added to this prescription
Hyponatraemic dehydration (plasma sodium < 130 mmol/l)

Here, sodium loss has exceeded water loss. Neurological symptoms due to cerebral intracellular water influx may occur if the hyponatraemia is severe (< 120 mmol/l) or the fall in
sodium is rapid.
If this is the case, urgent correction of hyponatraemia is required with hypertonic fluid
(e.g. 13 ml/kg 3% saline over 30 minutes). This is an emergency and the patient should be
managed in PICU. Consider intubation and ventilation if neurological symptoms are
present.
Chronic hyponatraemia may be tolerated better and can be corrected more slowly.
In less severe hyponatraemic dehydration the deficit may be adequately replaced with
isotonic fluid.
The ongoing, non-physiological losses of sodium (e.g. diarrhoea) will also require
replacement as part of the overall fluid prescription see Replacement of ongoing nonphysiological losses below.
Maintenance fluid therapy

This is the replacement of physiological losses of fluid and electrolytes incurred as a result of
metabolism. It includes both sensible losses (e.g. urine) and insensible losses (e.g. sweat).
Electrolytes are always lost with fluid, so both must be replaced.
Fluid replacement
Metabolic rate is influenced by multiple factors, including underlying disease processes.
Therefore, estimation of the patients metabolic rate, and hence the maintenance fluid
requirement, is imprecise. The most widely used method for estimation of metabolic rate
in hospitalized children is the Holliday and Segar method (Table 32.4):
it is a simplified formula using body weight, based on the calorie requirements of the
average hospital patient
the method separates patients into convenient groups according to body weight, and
assumes 1 ml of water is required for every 1 kcal expended
it is applicable to all age ranges, including adults
whilst the method is convenient and simple, it provides only a gross estimate of energy
expenditure and thus fluid requirements
this method gives rise to the 421 rule of hourly fluid requirement (as long as one
assumes a 25-hour day).
293
It is essential to adjust the maintenance fluid according to individual factors that are likely
to influence energy expenditure or water and electrolyte requirements. For example, nonosmotic ADH may significantly reduce the ability to excrete water. The requirement for water
per kcal expended will then be less than 1 ml, so restriction of water intake is required. Other
factors influencing energy expenditure and water requirement are shown in Table 32.5.
Table 32.4. Holliday and Segar simplified method for calculating maintenance fluid
requirements, based on estimated energy expenditure.
Body weight (kg)
Maintenance fluid requirement
Up to 10 kg
100 ml/kg/day
1020 kg
1000 ml plus 50 ml/kg/day for each kg above 10 kg
Over 20 kg
1500 ml plus 20 ml/kg/day for each kg above 20 kg
Table 32.5. Approximate adjustments to resting energy expenditure (and thus fluid requirement)
required for certain factors.
Factor
Resting energy expenditure adjustment (approximate)
Fever
12% per C above 37 C
Hypothermia
12% per C below 37 C
Pharmacological paralysis
30%
Humidified ventilation gases
25%
Non-osmotic ADH
3050%
Prematurity
20%
Normal activity
50%
Electrolyte replacement
Electrolyte maintenance replacement for a child under normal conditions is 3 mmol of sodium
and 2 mmol of potassium per kcal of energy expended. Thus, a solution of 0.18% saline with
20 mmol/l of potassium chloride should provide adequate maintenance of electrolytes.
Until recently, this was the moot common IV fluid therapy in hospitalized children.
However, if the energy expenditure and water requirement are overestimated, there is a
risk of iatrogenic hyponatraemia, as the solution is hypotonic. Consequently, it is now
rarely used for this purpose. Current recommendations for paediatric maintenance IV
fluid therapy are to use either 0.45% saline or 0.9% saline (or other near-isotonic fluid,
such as Hartmanns solution).
Use of these higher-tonicity fluids reduces the risk of hyponatraemia, at the expense of
exposing the patient to a larger sodium load. In most situations, the renal function is
capable of excreting the excess sodium, so the risk of hypernatraemia is low.
Dextrose replacement
Glucose requirement is related to body weight. For small children and infants a solution
containing 5% dextrose will usually provide adequate carbohydrate to avoid hypoglycaemia
and catabolism.
294
For neonates, a higher dextrose solution is usually required (e.g. 10% dextrose).
Although IV dextrose will provide some caloric input, catabolism will ultimately ensue if
enteral feeding is not established. Therefore, if a patient requires IV fluid therapy for more
than 48 hours and enteral feeding is not possible, consideration should be given to
commencing parenteral nutrition.
Replacement of ongoing non-physiological losses

Acutely ill children may have ongoing non-physiological fluid losses, necessitating further
adjustment to their individualized fluid regimen. A thorough clinical assessment will allow
anticipation of these losses. Knowledge of the composition of various body fluid compartments will aid in the prescription of replacement fluid; aim to replace like for like. See
Table 32.6 for electrolyte composition of various body fluids. Examples of clinical
scenarios with ongoing fluid losses are:
diarrhoea
osmotic diuresis (e.g. glucose)
gastric losses (e.g. pyloric stenosis)
sweat and increased evaporative losses in fever
burns.
Table 32.6. Examples of body-fluid electrolyte composition.
Body fluid
Sodium (mmol/l)
Potassium (mmol/l)
Chloride (mmol/l)
Gastric
2080
520
100150
Ileosotomy
45135
315
20115
Diarrhoea
1090
1080
10110
Sweat
1030
310
Burn
140
1035
110
Monitoring, warning signs and pitfalls

Estimation of fluid and electrolyte requirements is a blunt tool, so the clinician should
carefully monitor the response to therapy. Regular assessments of clinical and biochemical
endpoints will inform ongoing therapy.
Clinical monitoring in shock

During urgent treatment of shock with volume expansion, improving clinical signs such as
capillary refill time, heart rate, blood pressure and urine output are the goals against which to
judge initial therapy. Failure to respond to volume expansion signifies the need to escalate
therapy or change the treatment strategy (e.g. provide inotropic or vasopressor support).
Continuous clinical assessment by an experienced clinician is mandatory in these
patients.
Biochemical markers
Biochemical markers form useful goals against which to judge maintenance and replacement fluid therapy, outside the setting of urgent treatment of shock.
295
A normal plasma sodium level should be targeted, and maintained. Care should be given
to avoiding rapid changes in plasma sodium concentration, particularly in the case of
hypernatraemic dehydration. Compartment shifts of water should be anticipated when
giving fluid therapy for a hyperosmolar state. Regular monitoring of plasma sodium and
plasma osmolality will help identify rapid changes, but the clinician should also be vigilant
for clinical signs of cerebral oedema (e.g. irritability, drowsiness and seizures).
See Tables 32.7 and 32.8 for a list of causes of hypernatraemia and hyponatraemia and
important implications for fluid therapy.
Table 32.7. Hypernatraemia: causes and clinical implications for fluid therapy.
Cause
Implications for fluid therapy
Relative water deficit:

Diarrhoea
Treat dehydration, but recognize ongoing nonphysiological loss of water and electrolytes in the
stool
Burns
In addition to the burn losses, patients often lose

insensible water due to hyperthermia
Osmotic diuresis (e.g. DKA)
Monitor and replace ongoing losses
Diabetes insipidus
May respond to ADH analogue therapy (e.g.

desmopressin), depending on the type of
diabetes insipidus. Seek expert advice from
endocrinologist
Inadequate feed intake (e.g. newborn infant

with inadequate breast-feeding)
History may provide clues to this diagnosis
Excess sodium administration:

Salt poisoning
Rare. Usually affects infants on formula feeds.

Patients are unwell but not dehydrated. High
urinary fractional excretion of sodium and water.
Seek expert opinion (paediatric nephrology or
endocrinology)
Iatrogenic administration (e.g. hypertonic

saline or hypertonic sodium bicarbonate
infusions)
Consider reducing sodium administration in

maintenance fluid
296
Table 32.8. Hyponatraemia: causes and implications for fluid therapy.
Cause
Implications for fluid therapy
Iatrogenic (e.g. administration of

hypotonic fluid in excess of water
requirement)
Avoid very hypotonic fluids (0.18% saline), and

regularly reassess effect of fluid regimen on sodium
concentration. Can be managed by fluid restriction
alone
Non-osmotic ADH
Restrict fluid therapy to 5075% of calculated

maintenance and avoid very hypotonic fluid, if risk
factors for non-osmotic ADH (e.g. pneumonia,
meningitis, bronchiolitis, sepsis)
Gastrointestinal losses (e.g.

hyponatraemic dehydration due to
diarrhoea)
Adjust replacement of losses to provide more

sodium
Adrenal failure (rare) e.g. congenital

adrenal hyperplasia, Addison disease
Other signs of adrenal failure may be present (e.g.

hyperkalaemia, shock, hypoglycaemia). Will require
steroid therapy and referral to paediatric
endocrinology
Other hyperosmolar states

Be aware that hyperosmolar states can exist without a raised plasma sodium level, e.g.
diabetic ketoacidosis (DKA see Chapter 11), where the hyperglycaemia exerts a significant
effect on osmolality. Other examples are uraemia and hyperlipidaemia.
Slow correction of the hyperosmolar state should follow the same principles as treatment of hypernatraemic dehydration. It can be useful to calculate the corrected sodium
level to monitor these cases, although sodium normally self-corrects once the underlying
cause has been treated.
Specialist advice should be sought for management of these cases (e.g. paediatric
intensivist).
Hypoglycaemia
Hypoglycaemia (blood glucose < 2.6 mmol/l) is common in small infants after only short
periods of starvation. Hypoglycaemia in newborns may manifest as drowsiness, lethargy,
apnoeas or seizures, therefore delays in administering IV fluid therapy should be avoided in
sick neonates and regular monitoring of plasma glucose is required.
If the plasma glucose levels are below 3 mmol/l then the dextrose delivery should be
increased. Symptomatic (or ventilated) babies should receive a bolus of 2 ml/kg of 10%
dextrose in addition to the increase in background rate of dextrose. The background rate of
dextrose can be increased by increasing the rate of maintenance administration or by
increasing the concentration of dextrose in the fluid.
Some newborns exhibit hyperinsulinism, e.g. secondary to maternal diabetes mellitus.
These children require significantly greater amounts of glucose to avoid hypoglycaemia,
sometimes necessitating infusions of fluid containing very high dextrose concentration
(e.g. 1520%) via a central venous catheter.
297
Feeding on the PICU

Patients requiring intensive care often become catabolic due to increased metabolic
demands and inadequate calorie delivery. Every effort should be made to satisfy the
patients nutritional requirements at the earliest opportunity. The options available are
enteral feeding, parenteral feeding or a combination of both.
Enteral feeding
This is the preferred option in most patients. It is technically easier, has fewer side effects
and is cheaper than parenteral nutrition. Contraindications may include:
necrotizing enterocolitis
short-gut syndrome
low cardiac output states (especially with cyanotic congenital heart disease)
chylothorax unresponsive to medium chain triglycerides (MCT) feed
feed intolerance (vomiting and diarrhoea)
high-output enteral fistula.
Different standard feed formulae are available. The choice depends on the availability of
breast milk as an alternative, the age and calorie requirements of the patient. Dietetic advice
should be sought to identify the best feed for the patient.
Most feeds are delivered continuously, as this is better tolerated by patients with
respiratory compromise. It also allows assessment of feed absorption, without risking large
gastric aspirates and potential regurgitation or vomiting. Feed intolerance is common and
related to a number of factors including:
gastric stasis
drug effects, e.g. opioids or anticholinergics
bowel obstruction
paralytic ileus.
This can be overcome by reducing feed volumes, adding in drugs to increase gastric
emptying (e.g. domperidone), administering other prokinetics (e.g. erythromycin) or passing a nasojejunal tube.
In certain situations special feeds are required. These include thickened feeds (for gastric
reflux), modular feeds (e.g. MCT feeds in chylothorax), lactose-free feeds, pre-digested feeds
or calorie-loaded feeds when volumes are limited or growth poor.
Tolerance of feeds should be ascertained on a regular basis by aspiration of the NG tube.
It is common to aspirate some feed, but this can be replaced if the volume is not too great
(< 5 ml/kg). Indications that feed is not being tolerated include:
persistent, large NG aspirates
nausea and vomiting
abdominal pain
diarrhoea.
Once respiratory function has improved, problems such as hunger and hypoglycaemia can be
addressed by introducing bolus feeds. At first these are 2 hourly, but the interval can be increased
to 34 hourly, as long as the patient is absorbing the feed and can tolerate the increased volumes.
298
Parenteral feeding
Patients who do not tolerate enteral feeding or in whom it is contraindicated need to receive
total parenteral nutrition (TPN). Ideally, this involves feeding through a central venous line
(central or peripheral long line). It is possible to administer TPN through a peripheral
cannula. However, this rarely provides sufficient calories, as the glucose concentration is
limited to 12.5%. It should, therefore, only be used for short-term nutritional support.
The main components of TPN are:
glucose
amino acids (as Vamin)
fat (as Intralipid)
electrolytes, trace elements and vitamins.
Most TPN is bespoke and manufactured for the patient on a daily basis. However,
standard bags are available for times when it is not possible to manufacture TPN. The
complicated process of prescribing TPN is increasingly being devolved to specialist nutritional support teams or pharmacy departments. The general principles are:
calorie requirements differ with age growing children require more, e.g. 90120 kcal/kg/day
in neonates compared with 3060 kcal/kg/day in a sedentary adolescent
most daily calories are provided by glucose (6070%)
fat is also a valuable source of calories and requirements range from 34 g/kg/day
(infants) to 0.51 g/kg/day (adolescents)
protein requirements are for growth and healing, not calories again requirements vary
with age from 33.5 g/kg/day in infancy to 11.5 g/kg/day in older children.
In patients with hypercapnoea, it may be possible to reduce CO2 production by reducing
the glucose concentration in the TPN and replacing it with fat or Vamin.
The lipid component of TPN is administered separately and interruption of the infusion
for 4 hours per day is commonly done to reduce some of the pathophysiological effects on
the liver. Regular monitoring of the patient on TPN is vital and should include:
blood sugar
electrolytes
urea and creatinine
liver function tests
phosphate
triglycerides
trace elements.
TPN should be replaced with enteral nutrition at the earliest opportunity, but increasingly we
are seeing patients on the PICU who are TPN-dependent, so a good understanding of the
principles and problems involved with administering it are essential to those working there.
Summary
Fluid therapy should be an individualized prescription based on an assessment of the
patients deficits, maintenance requirements and ongoing non-physiological losses. Fluid
prescriptions are based on gross estimates, and therefore regular monitoring of clinical and
299
biochemical end-points should take place to inform alterations in fluid therapy. The goal is
to restore and maintain homeostasis.
No single IV fluid solution is perfect for every paediatric patient, across all phases of
illness. However, avoidance of very hypotonic fluids will reduce the risk of hyponatraemia.
Golden rules
Fluid therapy does not conform to a one size fits all protocol
Ongoing assessment is essential
Normal blood glucose levels should be maintained at all times
Electrolytes should be checked on a regular basis
Further reading
Behrman RE, Kliegman RM, Jenson HB.
Nelsons Textbook of Pediatrics, 16th edn. WB
Saunders, 2000.
Coulthard MG. Will changing maintenance
intravenous fluid from 0.18% to 0.45% saline
do more harm than good? Arch Dis Child
2008;93:335340.
Holliday MA, Segar WE. The maintenance need

for water in parenteral fluid therapy.
Pediatrics 1957;19:823832.
NPSA. Patient safety alert 22: reducing the risk of
hyponatraemia when administering infusions to
children. National Patient Safety Agency, 2007.
Taylor D, Durward A. Pouring salt on troubled
waters. Arch Dis Child 2004;89:414418.
Section 4
Chapter
33
Pharmacology in children
Rhian Isaac
Introduction
Drug therapies in children require individualization, because of their size and immature
metabolic pathways. This is usually done by calculating doses in direct relation to body size
(weight or body surface area) or by using a surrogate for normal size (e.g. age). The
critically ill child will also show variation in pharmacokinetics and pharmacodynamics as
a result of their disease process.
Although the dosing of drugs can seem daunting, especially in time-critical circumstances, it is helped by some general rules; for example, it is rare to require a dose above the
adult maximum dosage recommendations. This is particularly important to avoid overdosing the adolescent age group or the obese child. In these populations a dose for age banding
is usually more appropriate.
This chapter aims to provide a brief synopsis of how drugs commonly used in paediatric
intensive care affect critically ill children. It also serves as a brief reminder of how paediatric
pharmacology differs from that of adults.
Pharmacokinetic and pharmacodynamic differences

throughout childhood
Pharmacokinetics relates to how the body alters the absorption, distribution, metabolism
and elimination of a drug. The differences in the pharmacokinetics seen in small children
are summarized in Table 33.1.
Absorption
Oral route
Oral absorption of drugs is mainly affected by pH-dependent passive diffusion and/or
gastric emptying. Usually a drug needs to be in the un-ionized (lipophyllic) form to cross
membranes. Gastric pH is relatively elevated in the neonatal period, reaching adult values
by 2 years of age. This increased gastric pH (i.e. more alkaline environment) will increase
the absorption of weak bases and decrease that of acidic drugs (see Figure 33.1). Also, the
slower gastric emptying time seen in neonates will increase the time a drug takes to reach its
peak levels. Gastric emptying times will shorten by 68 months of age.
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Chapter 33: Pharmacology in children
301
Intestinal transit time is prolonged in neonates due to decreased motility and peristalsis,
whereas motility in the infant may exceed adult values. In neonates the dosing of drugs that
have a high intestinal absorption needs to take account of:
slower gut motility increasing absorption for some drugs that are actively absorbed via
specific transporters
increased permeability of immature intestinal mucosa
decreased intestinal enzyme activity.
Enterohepatic recirculation (or reabsorption) is a process whereby a drug or its conjugate
metabolites are excreted via the biliary tree and then reabsorbed in the lower intestine.
Critical illness may reduce drug recirculation in children because of decreased bile salt
production or reduced bacterial colonization of the gastrointestinal tract (GIT) (required to
cleave the conjugate for reabsorption). These bacteria are absent during the first few days of
life and following administration of broad-spectrum antibiotics.
Many medicines, such as newer anticonvulsants, are only available in oral form. Where
there is dubious absorption from the gut due to decreased blood flow or oedematous gut,
regular oral medication will require changing to the parenteral form.
Table 33.1. The physiological parameter changes during childhood that affect pharmacokinetic and
pharmacodynamic processes compared with the adult.
Physiological parameter
Neonate
Child
Post-pubertal
adolescent
PK/PD
affected
Gastric pH
Gastric emptying
Skin absorption
Total body water
Intracellular fluid
Extracellular fluid
Adipose tissue
Albumin concentration
DE
Albumin binding/affinity
DE
Enzyme capacity
ME
Renal function
Glomerular filtration rate (GFR)
Tubular secretion
Receptor sensitivity
PD
Bloodbrain barrier (BBB) permeability
PD
PK, pharmacokinetic; PD, pharmacodynamic; A, absorption; D, distribution; M, metabolism; E, elimination.
302
Low pH
Acid
Acid-H
Figure 33.1. Effect of pH on ionization status

of drugs.
High pH
(e.g. COO )
(e.g. COOH)
High pH
Base-H+
Base
Low pH
(e.g. NH3+)
(e.g. NH2)
Ionized
watersoluble form
Un-ionized
lipidsoluble form
Rectal route
Rectal absorption is variable in neonates due to reduced GI motility. For all ages, the
depth of insertion of rectal drugs will alter bioavailability. Drugs placed in the upper
rectum will undergo first-pass metabolism, whereas those placed in the middle and lower
sections may bypass the liver, as the inferior rectal veins drain into the vena cava, rather
than the portal vein.
The drugs most commonly administered rectally in critically ill children are paracetamol, chloral hydrate and diazepam. Lack of availability of suitable-sized suppositories may
make the rectal route less practical. Cutting suppositories is not advisable, as the drug may
not be evenly distributed throughout the excipient.
Distribution
For a drug to have an effect, it must reach its target by being distributed to the appropriate
compartment. Distribution is dependent on both drug and patient factors (see Table 33.2).
Smaller molecules and lipophilic drugs tend to distribute widely, i.e. they have a higher
volume of distribution.
Table 33.2. Factors affecting distribution of a drug.
Drug factors
Patient factors
Protein bound (%)
Total body water (%)
Lipophilicity
Ratio of extracellular to intracellular water
Molecular size
Adipose tissue
Plasma protein levels
Affinity of plasma proteins to bind with drug
Uraemia/bilirubinaemia
Acidbase status
Distribution can be affected by:

blood loss
fluid accumulation
changes in plasma protein levels.
303
The increase in extracellular water seen in critically ill children can lead to a higher
proportion of hydrophilic compounds being limited to the extracellular compartment. This
results in lower concentrations of a drug in the interstitial tissues with standard doses (e.g.
-lactams and aminoglycosides). This can be overcome by an adequate loading dose
followed by standard maintenance dosing. It should be remembered that steady state will
not be achieved for 4 to 5 half-lives of the drug unless an adequate loading dose is given.
Protein binding
Only the unbound drug is free to act at the receptor site. Albumin is the plasma protein that
binds mainly acidic drugs, while -1-acid glycoprotein tends to bind basic drugs.
Critically ill and very young children may have a lower than usual concentration of
albumin in their blood. As a result, the albumin binding sites can become saturated with a
given drug. Other plasma proteins have a lower affinity for binding drugs and endogenous
circulating agents such as bilirubin may displace them from their binding sites. This is
compounded in burns, stress, trauma, and liver and renal impairment, where there is a
further decrease in plasma proteins.
If protein binding decreases, the free fraction of a drug available to act at receptor sites
increases. This increases the free fraction of a drug and amplifies the pharmacological
response to a given dose. It also increases the risk of adverse drug reactions. Conversely, in
sepsis, there is an increase in -1-acid glycoprotein which can lead to a decrease in the free
fraction of basic drugs.
Clearance
If a drug is cleared at a high rate then there is less time for the drug to distribute to the target
compartment. Many interventions for the critically ill can increase the clearance of a drug,
such as renal replacement therapy and forced diuresis.
Bloodbrain barrier
The volume of the CNS is relatively large in children, reaching adult values by 46 years.
It also receives a higher amount of the cardiac output. The bloodbrain barrier
(BBB) is thought to be more permeable in the younger infant and may allow passage of
non-lipid-soluble drugs. Whilst this can be beneficial, it can also increase the risk of CNS side
effects (e.g. cerebral irritation), such as when giving higher doses of the penicillins for CNS
infections. Sepsis, hypoxia and acidosis also increase the BBB permeability. Acidosis in the
critically ill child will also increase the permeability of the BBB to acidic drugs due to an increase
in the un-ionized fraction.
Metabolism
Metabolism is the process by which the body breaks down or converts drugs into alternative, sometimes active, products. Each individual isoform of the enzymes involved in phase
I and II metabolism has its own developmental profile; reaching and in some cases
exceeding adult values throughout childhood.
Depending on the profile of the enzymes involved, drug metabolism may be limited by
either the capacity of the enzymes (saturation-limited) or delivery of the drug to the relevant
organ (perfusion-limited). There is a decrease in cytochrome-oxidase-mediated drug
metabolism in sepsis and during an inflammatory response which may cause a drugs
304
metabolism to be saturation-limited. Perfusion-limited metabolism may occur if the liver

blood flow is decreased, such as in:
hypotension
hypothermia
shunting of blood, e.g. through a patent ductus arteriosus in neonates can impair blood
flow to both the liver and kidneys.
An increase in metabolic activity occurs with age and can lead to an improved clearance of a
drug. For example, morphine clearance increases six-fold from birth to 6 months due to
increased biotransformation to its glucuronide metabolites.
Elimination
Drugs are usually eliminated via the kidneys or the biliary system. Critically ill children, as
with adults, commonly suffer from impairment of these organs. Drug elimination may,
therefore, be affected. Hypoxic episodes in neonates will decrease GFR and tubular function; therefore doses should be modified accordingly or levels ascertained before repeat
dosing.
Drugs that are haemodynamically active, such as inotropes, may improve renal blood
flow and as a result can increase the clearance of certain drugs. This is only usually
significant in drugs that have a high unchanged moiety when cleared by the kidneys.
Considerations with parenteral routes of administration

in children
Intravenous administration
Most medicines are not licensed for use in children and therefore the technical information
provided with the medicine does not offer paediatric parameters, increasing the risk of
unsafe administration. The age and weight of a child must be taken into account when
determining the volume for dilution, diluents and rate of administration. For example:
to maintain the higher glucose intake for a neonate, continuous infusions may require
dilution in 10% glucose
the volume and sodium load of the drugs and flushes can add up to a considerable
amount in the smaller child
in adults the maximum rate of phenytoin administration is 50 mg/min, which would
have serious consequences if applied to a small child, where the maximum rate for
treatment of SE is 1 mg/kg/min
in neonates, many antibiotics have a longer infusion time depending on the dose, such
as high-dose benzylpenicillin, which should be given as an infusion to minimize cerebral
irritation.
Intraosseous (IO) route

When IV access is difficult to obtain the IO route may be used in the resuscitation situation.
The same dose as the IV route should be used. Bolus administration or small-volume doses
can result in a depot-type effect, due to the drug remaining in the medullary cavity, leading
305
to lower serum peak concentrations and a longer time for drug distribution. This can be
overcome by giving a flush of 510 ml after the dose.
Common drugs used in critically ill children that differ from

adults in pharmacokinetics
Paracetamol
Paracetamol is metabolized principally via the glucuronidation pathway in children and
adults; approximately a third undergoes sulphonation. Small amounts are oxidized by the
CYP450 enzymes to a toxic metabolite, which causes necrosis to liver cells unless neutralized or scavenged by glutathione. With higher doses of paracetamol, more drug is oxidized
and the glutathione stores become depleted, leading to hepatocyte damage. In infants less
than 3 months old the sulphonation pathway is dominant and the oxidation pathway less
developed. As a result production of the toxic metabolite is less likely. In addition, neonates
synthesize more glutathione than adults; hence hepatic damage from overdose is less likely.
Paracetamol has dosing differences for each age group, indication and route, thus the
prescriber should refer to a reputable formulary, such as the BNFc.
Morphine
Morphine is metabolized via the glucuronidation pathway to active metabolites morphine3-glucuronide (an antanalgesic) and morphine-6-glucuronide (1020 times more potent
than morphine). In the first 2 months of life glucuronidation by the UGT2B7 enzyme is
immature. There is also a decrease in hepatic and renal clearance. This accounts for the
higher respiratory depression seen in the younger infant. Clearance reaches approximately
80% of adult values by 6 months of age and 100% by 1 year.
Whilst morphine is not considered lipid-soluble, the increased permeability of the BBB
in the younger infant allows greater CNS penetration and thus increased sensitivity. When
giving morphine to children, lower doses and closer monitoring and observations should be
applied to those under 1 year of age. This should not, however, discourage the use of
morphine as an analgesic or sedative when appropriate in these children (see Chapter 27).
Midazolam
Midazolam is water-soluble as a commercial formulation, but becomes lipid-soluble at
physiological pH. In the latter state it is able to cross the bloodbrain barrier. It is
extensively metabolized by CYP3A4/5 liver enzymes to an active and equipotent metabolite
(1-hydroxymethylmidazolam). This metabolite is then conjugated to a minimally active
glucuronide for renal clearance. The dose of midazolam should be reduced if renal function
is impaired.
The half-life of midazolam in children is approximately four times longer than in adults,
and is extended further in critically ill children, due to their decreased ability to metabolize
the drug. Common drugs used in the critically ill child that will increase midazolam levels
include fluconazole and omeprazole.
Hypotension as a result of midazolam administration is commonly seen in neonates and
infants; the risk is increased further with rapid IV bolus administration. Because of this, and
306
the variable metabolism and clearance seen in this age group, midazolam is rarely used as a
sedative in the young infant population (< 3 months).
Drugs used commonly in the critically ill child that you may
not be familiar with
Prostaglandin E1 and E2
These are potent vasodilators used to maintain the patency of the ductus arteriosus in the
neonate awaiting surgery for correction of a duct-dependent cardiac abnormality. In the
fetal circulation the duct is kept open by the effects of low PO2 and the high concentration
of prostaglandins (particularly prostaglandin E2). After birth, the smooth muscle constricts
as arterial oxygenation increases and placental concentrations of prostaglandin decrease.
This can be counteracted by giving continuous infusions of prostaglandin.
Alprostadil (E1) and dinoprostone (E2) are interchangeable (alprostadil is the licensed
version). Initial higher doses may be required (50100 ng/kg/min) to open the duct, but the
lowest dose should be used where possible, due to the risk of apnoeas and hypotension. A dose
of 510 ng/kg/min is usually sufficient for most patients. Prostaglandin E1 and E2 have halflives of minutes and therefore interrupting the continuous infusion should be avoided.
Milrinone
Milrinone is a phosphodiesterase III inhibitor which produces a positive inotropic effect and
vasodilatation. Inhibition of phosphodiesterase III leads to an accumulation of cyclic
adenosine monophosphate cAMP in the cardiac cell and in the vascular muscle. The effects
are dose-dependent (continuous infusion of 0.250.75 g/kg/min). The main advantage of
milrinone in children, when compared to other inotropic agents, includes increasing cardiac
output without effects such as:
excessive increases in heart rate (more of a problem in adults)
increased myocardial oxygen consumption
down-regulation of -adrenoreceptors
increased systemic vascular resistance.
In infants and children milrinone has a higher volume of distribution and increased renal
clearance. However, the half-life appears to be longer in infants compared with children and
adults. As with the catecholamines, it does carry a risk of arrhythmias and may exacerbate
hypotension if given too fast.
Whilst many texts suggest a loading dose, this should be avoided or reduced in patients
at risk of hypotension. Milrinone is cleared by the renal route, so the dose should be
decreased in renal impairment.
Chloral hydrate
This is a non-barbiturate hypnotic metabolized by the liver to active trichloroethanol. It is
used as an oral or rectal sedative in PICU. It has minimal effects on the respiratory system
and in lower doses (30 mg/kg) is haemodynamically stable. There is an increased risk of
myocardial depression, apnoeas and arrhythmias in children younger than 3 months old.
307
Drugs that should be avoided in children

Propofol
Prolonged use of propofol for sedation in children under 16 years old is contraindicated due
to the increased risk of the potentially fatal propofol infusion syndrome. This is characterized by:
arrhythmias
rhabdomyolysis
hepatomegaly
lipaemia
myocardial failure
hyperkalaemia.
Multiple drug therapy used in the critically ill child can alter propofol metabolism. During
stress children are more dependent on fatty acid metabolism for energy; propofol is
associated with mitochondrial respiration failure and defects in fatty acid oxidation.
It can be used for short procedures such as dressing changes, but due to its lack of
analgesic properties an opioid is usually required.
Drug dosing in renal impairment

Use of the GFR or creatinine clearance has limitations in critically ill children and therefore
makes dosing of renally cleared drugs challenging; where possible drug levels should be
used to guide therapy. Baseline creatinine, and subsequent increases, along with decreased
urine output, should be used to highlight when drug therapy and dosing require scrutiny.
Drug dosing in renal replacement therapy

Literature regarding drug dosing in paediatrics is scarce. Renal dosing books do not often
differentiate between high and low ultrafiltration rates which can lead to under- or
overdosing of medicines. As a result, the prescriber may be required to estimate the dose
reduction or whether supplemental doses are required. Table 33.3 highlights some of the
factors affecting dosing.
308
Table 33.3. Factors affecting drug removal in CVVH.
Drug factors favouring removal by CVVH
High % renal eliminated

Low protein binding
Low volume of distribution
Molecular weight of 10005000
Anions
CVVH factors favouring drug removal
Synthetic membranes
Large membrane surface area
High ultrafiltration rate
Post-dilution ultrafiltration
Patient factors that may affect drug removal
Liver impairment
Low albumin, increased free drug
Fluid overload, potentially larger
volume distribution
Deranged acidbase status
If the indication for continuous veno-venous haemofiltration (CVVH) is acute renal

impairment, further dose adjustments may be required when stopping CVVH depending
on the degree of renal impairment. Consideration should be given if there is a lack of
clinical response, especially with antimicrobials, to whether the patient is being underdosed. In these circumstances the adverse effects in accumulation versus the benefits of
therapy should be discussed. The pharmacists should be consulted to assist with drug
dosing in continuous renal replacement therapy (CRRT).
Summary
Prescribing drugs to children can be a complex and difficult task. Consideration needs to be
given to contraindications, the patients underlying illness, co-morbidity, physiology, and
patient maturity and size. The dose of many drugs needs to be modified in children,
particularly in the infant age group. There are a number of very helpful reference formularies that can be used to aid the process but, as always, if in doubt, seek expert advice from
an intensivist or pharmacist.
Golden rules
Never exceed the adult dose of a drug, unless there is a specific indication
Always consider co-morbidity when prescribing in critically ill children
Always double-check doses in children, particularly if it is a drug you are not used to
prescribing
Give a loading dose if a desired drug level needs to be achieved quickly
Further reading
Kearns G, Alander S, Leeder J. Developmental
pharmacology drug disposition, action, and
therapy in infants and children. N Engl J Med
2003;349:11571167.
Krishnan V, Murray P. Pharmacological issues

in the critically ill. Clin Chest Med
2003;24:671688.
Pea F, Viale P, Furlanut M. Antimicrobial
therapy in critically ill patients. Clin
Pharmacokinet 2005;44(10):10091034.
Section 4
Chapter
34
Neonates
Richard Skone
Introduction
A neonate is a term that describes any child from birth up to 28 days old. Paediatric
intensive care unit interactions with neonates tend to be limited to those with surgical
problems, e.g. congenital diaphragmatic hernias, or congenital heart disease. However,
units will also look after babies who have been discharged from neonatal units and
subsequently need re-admission.
This chapter will address the common principles in managing neonates on PICUs
although cardiac problems have been mentioned in an earlier chapter. Greater attention
will be paid to conditions seen on PICU, rather than on neonatal intensive care units
(NICU). The first part will focus on the day-to-day practicalities of intensive care
management. The latter part will describe common conditions seen on PICU.
Managing a neonate on PICU

When compared to looking after adults on intensive care units the approach to neonates is
one of having a light touch. For much of the time in PICU a great deal of effort is made in
not disturbing the child. However, vigilance is needed as the signs of significant pathology
can be very subtle. Sepsis, for instance, may only be heralded by brief episodes of desaturation and bradycardias.
Calculating the age of a neonate

As babies may be born significantly preterm, their corrected gestational age (CGA) is
important. It is unfair to compare two children at 1 month old if one was born at 24 weeks
while the other was born at term.
Until a prematurely born child reaches the date on which they were predicted to be born
their age is usually referred to as their gestational age, i.e. 32 weeks post menstrual age. After
the child crosses their due date, the age is often corrected, counting their due date
as day 1; e.g. a child aged 3 months but born at 32 weeks would be referred to as being
an ex-32 week neonate currently 4 weeks corrected gestational age.
The more premature a child, the greater the significance of the correction as a baby born
at 24 weeks when 4 months old chronologically will only (to a certain extent) be the size and
maturity of a normal newborn.
309
310
Intubation
Size of ET tube
There are no reliable equations for calculating the size of ET tube needed for children this
small. It is therefore useful to remember that a full-term (3 kg) neonate will usually be
intubated with a size 3.5 mm ET tube. The size of the tube should be adjusted according to
the size of the baby. It is important in babies to have a variety of ET tube sizes available at
the time of intubation as some of the neonates will have been ventilated on NICU
previously and may suffer from sub-glottic stenosis.
Although conventional teaching has been against cuffed ET tube in neonates due to the
risk of subglottic stenosis, the development of high-volume, low-pressure cuffs has meant
that they are now used for specific conditions. The indications are rare in neonatal practice
and include burns or trauma.
Length of ET tube
The distance from vocal cords to the carina in a term neonate may be as little as 5 cm
(shorter in premature babies). Endobronchial intubation is, therefore, a common problem. The most useful guide to the appropriate length of an ET tube is the intubation depth
mark present on many ET tubes. ET tubes are often secured by elastoplast or tape
strapping in an attempt to minimize movement of the ET tube once the appropriate
distance is decided upon.
Because of the short length of ET tube distal to the vocal cords unplanned extubation
can occur in neonates if great care is not exercised. It is possible to have a child who is
saturating adequately but difficult to bag who has managed to expel the ET tube into the
pharynx.
Nasal ET tube
Children are often intubated nasally on PICU (after an initial oral intubation) as nasal ET
tubes are better tolerated than oral ones. The sinuses are not fully developed in young
children; hence sinusitis does not occur as it does in adults.
ET tube blockage
One of the problems associated with the use of small ET tubes is the risk of blockage. This
may manifest as:
increased airway pressures
a changing or unstable ETCO2 dioxide level
a baby who is fighting the ventilator
desaturation.
This situation should be treated urgently as desaturation can be rapid and precipitous. Most
problems can be treated by changing to a T-piece to ventilate the patient manually, and
passing a suction catheter down the ET tube. If this does not work, and chest expansion or
breath sounds do not appear convincing, then a decision about whether to change the ET
tube should be made promptly. As with adults, if you are doubtful about the ET tube (and
the child has been intubated in a straightforward manner previously) then remove the ET
tube and resort to mask ventilation.
Chapter 34: Neonates
311
Ventilation
Setting the ventilator
Inspiratory (I) time
Children on neonatal units are often ventilated with an inspiratory time (I-time) of
0.3 seconds. This is based on the neonates having an alveolar time constant of 0.1 seconds.
There is also some evidence that long I-times in neonates can adversely affect outcome.
However, a significant number of young babies are admitted to PICU with conditions such
as bronchiolitis or RDS from sepsis. It is therefore reasonable to assume that the time
constant for their alveoli is prolonged (although not necessarily homogeneously in some
conditions). The I-time on PICU is therefore more often set to 0.5 seconds, or longer in
larger babies. This should be altered for the very low birthweight neonates.
Pressure
As with adults, barotrauma can cause long-term problems in children. Although a healthy
neonate has a more compliant chest than an adult, this may be altered by disease states.
Ultimately ventilator pressures should be set similar to those seen in adults.
Positive end-expiratory pressure is also used to the same effect, although it may cause
more cardiovascular instability if excessive. If lung disease is mild or moderate without
oxygenation difficulties a PEEP of 46 is generally used. In more severe lung disease the
lower point of inflection of the compliance curve should be estimated and the PEEP level
set above it.
Volume
Volutrauma also causes long-term problems in neonates. Tidal volumes should aim to
deliver between 5 and 7 ml/kg. This can be difficult to measure in small babies. The values
displayed on many ventilators reflect the volume change of the ventilator circuit, not
the actual volume delivered to a child. Some ventilators will compensate to an extent for
the volume of compression within the ventilator circuit when ventilating small infants. The
baby needs to be assessed clinically for adequate chest movement. Volume-controlled
ventilation with pressure limitation is often used to ventilate neonates on PICU. If this is
ineffective or problematic then pressure-controlled ventilation can be used.
Dead space
This is often a major preoccupation for many anaesthetists. In practice the length of an
ET tube does not often compromise ventilation. It is important, however, that appropriately
sized HME filters and ETCO2 monitors are used. The most important factor in managing
ventilation in neonates is to be aware of the potential for re-breathing. If the arterial PaCO2
continues to rise in spite of what appears to be adequate ventilation, then attempts should
be made to minimize the dead space within a circuit.
End-tidal carbon dioxide monitoring
This can prove very difficult to monitor in neonates due to the small tidal volumes
generated; however, ETCO2 monitoring is still the least invasive and best way of helping
to confirm correct ET tube placement.
In the absence of an arterial line, capillary gases can be used as an indicator of arterial
CO2 tension.
312
Circulation
Blood pressure
As a rule of thumb, for premature babies (up to 40 weeks) it is often useful to aim for a
mean arterial blood pressure (in mmHg) which is equal to the babys gestational age in weeks.
This gives a rough idea of an adequate blood pressure. As with adults, adequate pressure
does not always equate to adequate flow so it should only be used in conjunction with other
clinical observations.
Fluid boluses
Received wisdom is that blood pressure in neonates is largely heart-rate dependent. This is
not always reflected in clinical situations. Hypotension is still best treated with fluid boluses,
which are usually administered as 510 ml/kg fluid boluses, with clinical response monitored. Similarly sepsis and vasodilatation will also require adequate fluid resuscitation and
vasoconstrictors, much as in adults. The choice of fluid is still controversial, and the colloid
vs. crystalloid debate continues with the same vigour in paediatrics as in adults.
Remember that a 5 ml flush through a neonatal cannula will constitute a fluid bolus!
Inotropes
The choice of inotrope is one area which can confuse adult physicians. There are arguments
for and against the use of dopamine in neonates. What is certain is dopamine may be an
adequate initial inotrope which can be administered peripherally. Most PICUs will be
happy to use more familiar inotropes and vasoconstrictors on very sick babies. Adrenaline
and noradrenaline are used for the same indications as in adults. Correcting ionized
calcium in neonates is very important. This is because of the lower stores of intracellular
calcium within the sarcoplasmic reticulum of the myocardium.
Renal function
The kidneys, like other neonatal organs, are immature. They have decreased urineconcentrating ability which renders neonates susceptible to fluid overload or dehydration
if not managed carefully. Fluid overload in neonates can compromise ventilation in severe
cases as the oedema can compromise chest wall compliance.
Because of the poor urine-concentrating ability of neonates, it is usual to aim for an
hourly urine output of 1 ml/kg/h. Neonates have a high calcium output in their urine which
is exacerbated by the use of calciuric drugs such as furosemide. This can lead to nephrocalcinosis if care is not taken. Fully developed function of the kidneys does not occur until
approximately 1 year old.
When taken in combination with immature liver function, great care must be taken in
prescribing drugs to neonates. A neonatal pharmacist should be consulted when prescribing
for premature babies.
Access
Cannulae
Cannulae of 24 gauge are often used as a means of gaining access in neonates. The size and
fragility of veins does, however, mean that care is required in order to avoid extravasation
injuries even in well-sited cannulae.
313
Long lines
Longer-term central access is often acquired using long lines. These are narrow, peripheral
lines that are sited under aseptic technique and are fed through until the tip sits in the great
veins. The diameter of these lines can be as little as 28 gauge (1 French). They can be used
for TPN or inotropes, or for giving high-concentration infusions. Traditional, triple-lumen
central venous catheters are also used, but the large diameter of the catheters means that
they are often associated with complications such as venous obstruction and clot formation.
Arterial lines
For arterial lines, as with adults, peripheral lines are preferred at sites where good collateral
flow ensures adequate perfusion. Typically 24G cannulae are used in the radial or posterior
tibial arteries. Brachial artery cannulae are associated with a high level of morbidity in
premature babies and are therefore avoided where possible. Palpating and fixing an artery
in a neonate can be difficult. One technique used in neonates is to transillumintae the arm
or foot with a cold light. These lights minimize the risk of thermal injury while showing
the arteries or veins as black threads which can be targeted.
Capillary gases
In the absence of an arterial line, capillary gas samples are used as a way of monitoring the
status of a child. There are important caveats to interpreting capillary gas samples. When
they have been difficult to sample, it is possible to get spuriously raised:
potassium
lactate
hydrogen ion levels (lower pH).
The PaO2 on a capillary gas does not necessarily reflect the arterial PaO2. Unusual results,
which do not correlate with the state of the patient, should be interpreted with care.
Umbilical lines
Some babies may arrive on PICU from neonatal units with umbilical artery (UAC) or
venous (UVC) catheters in situ. These can act as a method of invasive arterial monitoring or
central venous access respectively. Care should be taken in their management in terms of
positioning and infection-control measures.
Sedation
Morphine is used as a sole agent for sedation in neonates. It is usually diluted in 5% or 10%
dextrose and started at a dose of 40 g/kg/h (titrated to effect). Morphine metabolism is
underdeveloped in neonates and differs slightly from that of adults (see Chapter 33).
Midazolam is rarely used in children younger than 3 months old (CGA) as its cardiovascular depressant activities are significant. There are also concerns that it may worsen
neurological outcomes on NICU.
As with adults, environment plays a large part in successful sedation of neonates.
Incubators have been shown to amplify sounds of any tapping or knocking on their surface.
This noise can agitate neonates, causing them to experience apnoeas or bradycardias or to
desaturate. Opening and closing incubator doors can also cause a significant level of
distress.
314
Feeds/maintenance fluids
Enteral feeding
An approximation to the usual feeding regime aims to give neonates a daily calorie intake of
100 kcal/kg. Formula and breast milk contain approximately two-thirds of a kcal per ml.
Therefore babies are usually allowed 150 ml/kg/day if enteral feeds are used.
Breast milk provides the gold standard form of nutrition for neonates due to the
additional benefits of maternal enzyme and antibody transfer (passive immunity). Many
neonates will be too ill to manage breast-feeding directly, so most PICU and NICU will
have facilities for breast milk expression by mothers. This can then be administered via an
NG tube.
High-risk feeding groups

Certain groups of children are kept nil by mouth for fear of precipitating necrotizing
enterocolitis (NEC) (see below). These include children with cardiac defects where bowel
perfusion may be compromised, e.g. coarctation of the aorta. A combination of highly
concentrated (hyperosmolar) feeds and bowel ischaemia can precipitate NEC.
Fluid maintenance
A neonate who is nil by mouth, receiving IV crystalloid maintenance fluid, needs to be
monitored very closely. A child receiving 100 ml/kg/day of IV fluid may get overloaded over
a period of days, and iatrogenic electrolyte imbalances are easy to manufacture. When
giving the fluid it should be remembered that the requirements are:
sodium 13 mmol/kg/day
potassium 12 mmol/kg/day
glucose 46 mmol/kg/min.
One of the reasons that many drugs are made in high-concentration dextrose fluids is that,
using the above fluid regime, a 2 kg neonate will be allowed 8.3 ml/h of maintenance fluid.
This can be easily exceeded if a child is on sedation, multiple inotropes, and drugs which
need to be diluted in large volumes, e.g. antibiotics. In paediatric practice drug infusions are
included in the total hourly fluid allowance. In order to meet the childs glucose requirement and restrict fluid appropriately it may be necessary to:
increase the concentration of drugs in infusions
increase the concentration of glucose in the infusions
keep to essential drugs.
Hypoglycaemia
The lack of carbohydrate reserve and the catabolic nature of critically ill neonates make
them prone to hypoglycaemia. Regular checking of blood sugars is therefore essential with
blood sugars of less than 2.6 mmol/l (or higher if symptomatic), prompting intervention;
i.e. give 2 ml/kg of 10% dextrose and increase basal glucose delivery. As can be seen, lower
blood glucose is tolerated in stable neonates compared with the other children mentioned in
this book.
Glucose requirements in neonates may rise as high as 12 mg/kg/min in some neonates.
If the requirement is greater than this then a hypoglycaemia screen should be performed
while the child is hypoglycaemic (if there is no need for urgent intervention). The purpose
315
of this screen is to look for conditions such as inborn errors of metabolism or hyperinsulinism, e.g. BeckwithWiedeman syndrome (see Chapter 9 for a list of tests). Do not forget to
include the drug infusion fluids when calculating the amount of dextrose that a neonate is
receiving.
TPN
For neonates where enteral feeding is not possible or advisable most PICUs have a low
threshold for starting parenteral nutrition in order to sustain growth and development.
They will be aiming for a growth rate of 10 g/kg per day.
Temperature
Thermoregulation is a significant problem for premature neonates compared to term
babies. This is due to:
large surface area to volume ratio
fewer subcutaneous fat deposits
less brown fat
immature development of neurological response to cold.
Great effort has to be made therefore to maintain an appropriate temperature at all times.
This can be accomplished by:
not taking the baby out of the incubator unless essential
placing the neonate on a warming device if they have to be taken out of the incubator
keeping the baby well wrapped
avoiding long procedures
temperature monitoring.
Surgical pathologies
There are a large number of surgical conditions that can affect neonates. The most common
ones are detailed below.
Necrotizing enterocolitis (NEC)

NEC is the most common surgical problem affecting neonates. Risk factors for NEC
include:
prematurity
very low birthweight
congenital heart disease/patent ductus arteriosus
poor umbilical blood flow in utero
perinatal asphyxia.
Its exact cause is uncertain and it is likely to be multifactorial. The idiopathic nature of the
condition has led to speculation that it is a perfusion/reperfusion problem; it may also be
due to dysregulation of the pro-inflammatory cascade, while some epidemiological patterns
point to a possible infective cause.
As the name suggests NEC affects the GI tract. It typically presents with feed intolerance,
abdominal distension and bilious aspirates from the NG tube, although signs can be much
316
more subtle. The disease can range in its severity from feed intolerance through to multiorgan failure. The condition can be managed conservatively in the majority of cases. This
involves keeping the child nil by mouth for 710 days (gut rest) and giving appropriate
antibiotics. However, surgical review should be sought, especially if there are signs of
perforation or systemic sepsis.
Findings on the abdominal X-ray can include:
fixed dilated loops
bowel wall thickening
intramural gas
perforation.
Blood results may show:
metabolic acidosis
thrombocytopenia
white cell count may be raised or worryingly low
raised lactate.
Supportive therapy may be necessary for multi-organ failure and bacterial translocation.
TPN will also be necessary to try to maintain an anabolic state in these very small babies, in
an attempt to encourage growth.
Surgical intervention often involves a defunctioning colostomy/ileostomy and/or resection of diseased bowel. This can be extensive. Postoperatively it is important to take note of
the extent of gut resection (< 40 cm of remaining small intestine is associated with an
increased chance of short gut syndrome, although shorter can be tolerated if the ileo-caecal
valve remains in place). Premature neonates deemed too sick for surgery may have intraabdominal drains sited on the unit instead of surgery. The mortality from NEC can be up to
30% depending on weight and gestation.
Patent ductus arteriosus (PDA)

Persistence of the ductus arteriosus (DA) after day 10 of life is termed a PDA. The duct
closes in two stages. Usually the duct undergoes functional closure at about 15 hours, by
smooth muscle contraction, followed by true anatomical closure, which can take a couple of
weeks. Persistence of the duct is suggested by:
a characteristic continuous machine like murmur
a low diastolic blood pressure
pulmonary overcirculation.
The impact of a PDA depends on the size of the lesion. The signs can be subtle, and so a
high degree of suspicion is essential to avoid complications such as:
heart failure
pulmonary hypertension
NEC.
Management can be conservative. Indomethacin is given in an attempt to block production
of prostaglandins which maintain patency of the duct. This is, however, associated with an
increased risk of GI bleeds, perforation and NEC. If conservative management fails then
317
children may be referred for a surgical ligation of the duct. This is performed without the
need for cardiac bypass, through a left-sided thoracotomy incision.
Congenital diaphragmatic hernia (CDH)

As the name suggests, this condition involves development of a fetus with abdominal
viscera within the thoracic cavity. Eighty-five per cent of CDH are left-sided; the remainder
are either right-sided or bilateral. Over time the repair of CDH has become a relatively
straightforward surgical procedure. However, increasing success of surgical intervention
has highlighted that the major cause of morbidity and mortality in these children is
pulmonary hypoplasia and pulmonary hypertension. In fact the pulmonary hypoplasia is
thought to be a primary event during the formation of a CDH (not just a consequence). Up
to half of children with CDH will have it as part of another syndrome; therefore a thorough
plan of investigation is needed before surgery.
As with other neonatal surgical conditions the timing of surgery is important. Children
may initially need HFOV, inhaled nitric oxide and even ECMO. Where possible these will
need to be weaned prior to surgery. Predicting outcome in CDH preoperatively can be
attempted using the oxygenation index as a means of assessing pulmonary function (being
mindful of the site of ABG sampling due to the likely presence of a DA).
Intracranial haemorrhage (ICH)

ICHs are relevant to PICU because children will either develop them during the course of
their illness or present for surgery to decompress obstructed CSF drainage by creating a
shunt.
Premature neonates are at increased risk of developing intracranial bleeds. This is due to
the immature nature of the germinal matrix. The germinal matrix is a highly vascular
region of the brain, from which cells migrate outwards during development. Extravasation
of blood from the matrix leads to periventricular or intraventricular haemorrhage (PVH/
IVH). Other types of intracranial haemorrhages can occur in neonates, e.g. subdural
haematomas, but are now less common as obstetric practice improves.
The majority of neonates with ICH are managed conservatively, with correction of
coagulopathy and supportive treatment for any consequence of the bleed. Outcome in these
children largely depends on the severity of the bleed but is difficult to predict in individuals.
IVH and parenchymal bleeding can be diagnosed on ultrasound due to the window
provided by the anterior fontanelle.
An intracranial bleed should be suspected in any neonate who:
shows signs of cerebral irritation
appears floppy or lethargic
has a change in neurology associated with a significant drop in haemoglobin
shows any signs of focal neurological changes.
Although the above gives a list of when to suspect a PVH/IVH, essentially it is prudent to
suspect it in any premature neonate who is seriously unwell. It is also wise to remember that
any ex-prem child who presents with neurological problems, e.g. meningitis, should be
examined carefully for a VP shunt, as this may be driving a CNS infection. Hence a detailed
birth history can be of use even in older children.
318
Prenantal diagnosis of surgical conditions

Prenatal diagnosis of conditions such as gastroschisis, exomphalos and oesophageal atresia
has advanced the management of many neonatal surgical conditions. It enables neonatologists to plan the care of neonates, prior to delivery, in a way that minimizes harm and
facilitates prompt management. It also allows time to search for associated pathologies; for
instance children with conditions such as exomphalos are likely to suffer from other
congenital abnormalities (such as trisomies 13, 18 and 21), while oesophageal atresia can
be seen in the VACTERL association.
Conditions associated with prematurity

Respiratory distress of the newborn (RDS)
RDS increases in incidence the more premature the neonate. Diagnosis is based on clinical
observation, blood gases and a CXR that shows diffuse ground glass type changes. RDS has
similarities to adult RDS in terms of being exacerbated by overenthusiastic ventilation and
benefiting from early recruitment of collapsed airways. There are, however, major differences. RDS in neonates is a disease of lung immaturity and surfactant deficiency. As such,
two interventions that help to decrease the incidence of RDS in newborns are antenatal
steroid administration and the early administration of surfactant to at risk neonates.
Complications of RDS include:
pulmonary and intracranial haemorrhage
PDA
progression to chronic lung disease.
Once it has developed, management is as mentioned above coupled with supportive care
and protective lung ventilation strategies.
Chronic lung disease (CLD)

CLD in neonates is defined as supplemental oxygen dependence at 36-week post menstrual
age or 28-day postnatal age in conjunction with persistent clinical respiratory symptoms and
compatible abnormalities on CXRs. Although management of this condition is improving,
the increased survival of younger preterm babies means that this condition still forms a large
burden. Many children with chronic lung disease will have clinically undetectable long-term
consequences from CLD, although others will demonstrate a rise in pulmonary vascular
resistance, arterial carbon dioxide tension and respiratory rate as well as a lower arterial
oxygen tension.
The definition of CLD is also changing as many more babies survive being born at 23
and 24 weeks gestation. This is because these babies will often be oxygen-dependent 28 days
after delivery, when their corrected gestational age is still only 27 or 28 weeks. However,
they may not require oxygen by the time they are discharged.
Other terminology used in describing lung pathology includes:
bronchopulmonary dysplasia (BPD) a term which has had various definitions in the
past. It refers to children with chronic lung disease who have been ventilated with IPPV
pulmonary interstitial emphysema (PIE) a condition in which air tracks out into the
peribronchial space causing obstruction to airflow, pneumothoraces and
pneumomediastinum.
319
Retinopathy of prematurity (ROP)

ROP is a condition that affects premature neonates. Abnormal blood vessels grow in the
retina, causing scarring and blindness. Normal retinal vascular growth in the fetus is
complete by 36 weeks; however, prematurity, periods of hypoxia and the use of supplemental oxygen before completion of development of the vessels can lead to abnormal growth.
Laser therapy can improve outcome and reduces the risk of visual impairment in ROP. It
can be performed on very small and sick neonates on PICU.
ROP is one of the reasons that neonatologists previously targeted oxygen saturations of
8892% on their units as a routine. However, recent evidence points to higher saturations
(9195%) improving outcomes in premature neonates.
Seizures
The causes of seizures in neonates are legion. Searching for a cause requires significant
neonatal experience. However, basic correctable causes and life-threatening treatable causes
need to be diagnosed quickly. As with adults the basic management of a seizure is straightforward (see Chapter 10). Many of the metabolic causes of seizure activity in a neonate can be
detected on a blood gas analysis. They include blood sugar, ionized calcium or plasma sodium
concentrations. The first two can be corrected quickly, while the latter needs acute management of the seizure followed by a gradual correction of the sodium (see Chapter 32).
Intracranial causes of seizures in neonates include infection and haemorrhage.
A ventilated child may demonstrate seizure activity through autonomic changes.
A sharp jump in heart rate and blood pressure coupled with dilated pupils may be the
main presentation. Conversely sudden bradycardias in the absence of hypoxia or vagal
stimulation may also herald a seizure.
Infection
Although sepsis is often managed on NICU there are occasions when a neonate with sepsis
may be admitted to a PICU. Primarily these are when:
the child has been discharged home prior to acquiring the infection
when the infection leads to a need for surgical intervention, e.g. congenital HSV causing
fulminant liver failure
the sepsis is associated with a surgical pathology, e.g. volvulus or NEC.
Neonates are more susceptible to infections than older children due to the relative immaturity of their immune system. Premature neonates are at an even higher risk. The commonest
organisms affecting neonates can be thought of according to timing of presentation. Earlyonset (within 672 hours of birth) infections tend to be caused by organisms acquired
intrapartum. These include:
Gram-negative enteric bacilli
Haemophilus influenzae.
The pathogens causing later-onset sepsis in a neonate are more likely to have been acquired
from the environment. The management of sepsis in neonates follows the same principles
as for other children (see Chapter 5). Remember that care should be taken with neonates to
pay particular attention to blood sugar and temperature.
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Summary
It is impossible to cover the whole of neonatology in one chapter. While there are some
similarities in the approach to ITU in neonates to that in adults, the differences become
most marked as the child becomes younger. There are fewer hard signs than in older
children and treatment often feels as if it is blind. Probably the hardest thing to remember
in neonates is that being aggressive in your day-to-day management is not always for the
best. A quiet, warm incubator with as few lines as possible, while ensuring adequate caloric
intake, is often the best approach. The caveat to this is for time-critical conditions such as
sepsis or bleeding.
Golden rules
Signs of a deteriorating neonate can be very subtle
ET tubes can block easily due to their narrow lumen
Flushing cannulae can be the equivalent of a fluid bolus if care is not taken
Do not take a neonate out of an incubator for practical procedures without some way of
keeping them warm
Despite all of the above, minimal interference and a delicate touch are needed in order to
avoid apnoeas and bradycardias in premature neonates
Further reading
Lissauer T, Fanaroff AA. Neonatology at a
Glance, 2nd edn. Wiley-Blackwell, 2011.
Golden Rules
Section 5
Chapter
Quick reference for emergencies
35
Kasyap Jamalapuram
Normal physiological paramaters

Age
Respiratory rate range

(breaths per minute)
Heart rate range

(beats per minute)
Systolic blood
pressure range (mmHg)
< 1 year
3060
110150
7090
15 years
2040
90120
80100
512 years
1530
80120
90110
> 12 years
1525
60100
100130
Common equations in emergencies

Weight
(Age 4) 2 weight in kg
Adrenaline dose in an arrested patient

0.1 ml/kg of 1:10 000 concentration
Defibrillator
4 weight joules (ventricular fibrillation)
Endotracheal tube
Age/4 4 mm (internal diameter)
321
322
Section 5: Golden Rules
Paediatric Glasgow Coma Scale (child/infant)

Eye opening (E)
4
Spontaneous
To verbal stimuli
To painful stimuli
No response
Eyes closed due to swelling/bandage
Verbal
Grimace
Alert, babbles, coos,

words or to normal ability
Spontaneous
normalfacial/oro-motor activity
Less than usual ability or

spontaneous/irritable cry
Less than usual spontaneous

ability or only response to
touch stimulus
Cries inappropriately
Vigorous grimace to pain
Occasionally whimpers
and/or moans
Mild grimace to pain
No response
No response
Intubated
Motor
6
Normal spontaneous movements
Withdraws to touch
Withdraws to painful stimuli
Abnormal flexion to pain
Abnormal extension to pain
No response to pain
Golden Rules
Section 5
Chapter
Drug infusions
36
Kasyap Jamalapuram
Table 36.1. Commonly used drugs for critically ill children.
Drug
Dose to
draw up
Dilute to 50 ml total
volume with:
Rate/dose
equivalents
Dose
range
Adrenaline (central)
0.3 mg/kg
5% Dex/10% Dex/NS
1ml/h 0.1 g/kg/

min
0.011.0
g/kg/min
Adrenaline (peripheral
discuss with PICU)
of above
strength
(0.07 5 mg/kg)
5% Dex/10% Dex/NS
1ml/h 0.025 g/
kg/min
0.011.0
g/kg/min
Noradrenaline (central)
0.3 mg/kg
5% Dex/10% Dex/NS
1ml/h 0.1 g/
kg/min
0.011.0
g/kg/min
Dobutamine (central)
15 mg/kg
5% Dex/10% Dex/NS
1ml/h 5 g/
kg/min
1.020 g/
kg/min
Morphine
1 mg/kg
5% Dex/10% Dex/NS
1ml/h 20 g/kg/h
1040 g/
kg/h
Midazolam (> 33 kg)
100 mg
5% Dex/10% Dex/NS
(wt 0.03) ml/h

1 g/kg/min
0.53.0
g/kg/min
Midazolam (< 33 kg)
3 mg/kg
5% Dex/10% Dex/NS
1 ml/h 1 g/
kg/min
0.53.0
g/kg/min
Prostaglandin E1/E2
50 g
5% Dex/10% Dex/NS
(wt 0.3) ml/h

5 ng/kg/min
5.020 ng/
kg/min
Salbutamol
(peripheral)
10 mg
5% Dex/NS
(wt 0.3) ml/h

1 g/kg/min
1.02.0
g/kg/min
Aminophyline (> 50 kg)
1250 mg
5% Dex/10% Dex/NS
(wt/50) ml/h
0.5 mg/kg/h
0.51.0
g/kg/h
Aminophyline (< 50 kg)
25 mg/kg
5% Dex/10% Dex/NS
1 ml/h 0.5 mg/

kg/h
0.51.0
g/kg/h
Insulin for DKA (> 20 kg)
50 units
5% Dex/10% Dex/NS
(wt/10) ml/h
0.1 units/kg/h
0050.1
units/kg/h
Insulin for DKA (< 20 kg)
2.5 units/kg
5% Dex/10% Dex/NS
2 ml/h
0.1 units/kg/h
0050.1
units/kg/h
Peripheral-strength adrenaline needs to be used with great caution. Only in emergency, via a good IV cannula
(to avoid extravasation). Consider IO administration instead.
323
324
Table 36.2. Drugs used to lower plasma ammonia in IMD.
Drug
Mode of action
Conditions
Dose
Notes
Sodium
benzoate
Scavenges
ammonia by
converting to
hippuric acid,
which is excreted
in urine

Organic acidurias with
hyperammonaemia,
e.g. PA, MMA, IVA
when carglumic acid
250 mg/kg PO/NG is
not available
250 mg/kg
loading
dose over
90 min then
250 mg/
kg/day
Maximum
peripheral
concentration
50 mg/ml
Sodium
phenylbutyrate
Scavenges
ammonia by
converting to
phenylacetic acid,
which is excreted
in urine
250 mg/kg
loading
dose over
90 min then
250 mg/kg/
day
Maximum
peripheral
concentration
50 mg/ml
L-Arginine
Replenishes urea
cycle for
continued
function despite
enzyme block
150 mg/kg
loading
dose over
90 min then
150 mg/kg/
day
Double dose can

be used in
citrullinaemia
and
argininosuccinic
aciduria
References
KIDS. West Midlands regional retrieval service
website: http://kids.bch.nhs.uk/wp-content/
uploads/2011/10/KIDS-Infusion-guideV1.pdf.
KIDS drug calculator: http://kids.bch.nhs.uk/

wp-content/uploads/2011/10/Drug_Calculator_
V1.pdf.
Section 5
Chapter
37
Golden Rules
Top tips for practical procedures

Steven Cray
Airway management

If the airway is compromised for non-infective reasons, always consider a nasopharyngeal

airway or placing the child in the prone position
Paediatric colleagues and the local retrieval service are a resource of useful information,
e.g. whether the child is syndromic and a potentially difficult intubation
Difficult laryngoscopy is rare in children and usually predictable if in doubt keep them
breathing
Dont overextend the head when managing the airway and intubating infants or children
Placing a NG tube before induction in a neonate and infant facilitates stomach decompression
Preoxygenation may be ineffective in children dont be afraid to gently ventilate following
induction, even if an RSI
A roll under the shoulders of an infant may aid laryngoscopy
Laryngeal pressure is a useful means of improving laryngoscopic view in young children
Incorrectly applied cricoid pressure may make laryngoscopy more difficult dont be afraid
to remove it
The paediatric larynx is funnel-shaped; a circular motion of the tip of an ET tube will help its
passage
The tongue is your biggest enemy when intubating children make sure you get it out
of the way during laryngoscopy
The epiglottis is long and floppy in younger children consider placing the laryngoscope
blade posterior to the epiglottis when attempting intubation
Always have a bougie handy to intubate a child with suspected C-spine injury
Children can desaturate very quickly during laryngoscopy attempts do not persist too
hard or too long to intubate
The commonest error in ET tube placement is a tube which is too small and too long
Failed endotracheal intubation is not a disaster the laryngeal mask airway is a perfectly
reasonable, temporary, alternative
A neutral head position is required for a CXR in order to evaluate ET tube position
325
326
Vascular access and circulation

Long sapheonous and scalp veins are often easier to access than veins in small arms
and hands
IO needles should be used if IV access cannot be obtained within 90 seconds in a critically
ill child
IO access is the first choice access in a paediatric arrest situation (if no cannula in situ)
Paediatric veins can be transfixed and successfully cannulated always remove needle and
slowly withdraw cannula following an attempt, even if there was no flashback initially
Always use isotonic fluids for resuscitation in children
Dont be afraid to administer volume resuscitation quickly in sick children they tolerate it
extremely well
An increase in blood pressure with gentle pressure on the liver may be an indication of
hypovolaemia
Central line

Ultrasound should be used to identify the vein and during line placement
Children who have complex past medical histories may have central veins that have
been occluded following previous lines. It is important to establish vein patency before
attempting insertion
The internal jugular and femoral veins are most commonly used for central venous access
in children. There is no difference in the incidence of infection between the two sites in
children
The internal jugular vein is generally very easily identified with ultrasound. The femoral
vein is a smaller vessel and may be more difficult to distinguish from other structures
For infants a 4.5 or 5FG triple-lumen line is appropriate for internal jugular placement. For
femoral access in babies a 4.5FG line is more suitable because of the smaller size of the
vein
A femoral line may be transduced for a central venous pressure measurement; this is
generally a good reflection of right atrial pressure
For femoral venous insertion aim to have the catheter tip at L3 level (below the level of
the renal veins). The optimal insertion length (cm) 0.45 body weight (kg) + 8.13.
This gives an insertion length of 10 cm in a 4 kg baby, for example
Reverse Trendelenburg position, hepatic pressure and Valsalva manoeuvre can all increase
the diameter of the femoral vein
Give at least one fluid bolus before attempting central line insertion
Chaptert 37: Top tips for practical procedures
Arterial line

The patient should be positioned carefully. For example for radial artery access the wrist
can be extended over a roll of gauze and immobilized with tape. A pad under the bottom
will help with femoral access in infants
A transfixition technique is generally necessary when cannulating an artery in a small child
Use arteries where you can feel a pulse dont waste time trying peripherally if the arteries
are impalpable. Use the femoral artery (or axillary artery if necessary)
A Babywire (0.01200 ) should always be available when attempting arterial cannulation in
small children
The brachial artery should not be cannulated in premature neonates
22G is suitable for most infants and children, although 24G is used in premature neonates
and small babies
Seldinger-type cannulae are well suited to the femoral artery as standard cannulae
may be too short
An aseptic technique should be used especially for femoral arterial lines
The use of ultrasound should be considered for femoral and axillary sites
Urethral catheters
Age
Weight (kg)
French gauge
06 months
3.57
1 year
10
68
2 years
12
3 years
14
810
5 years
18
10
6 years
21
12
8 years
27
12
12 years
varies
1214
NG tubes
Age
Weight (kg)
NG tube (French gauge)
06 months
3.57
1 year
10
10
2 years
12
10
3 years
14
1012
5 years
18
12
6 years
21
12
8 years
27
14
12 years
varies
1416
810
327
328
Chest drains
Age
Newborn
Chest drain size (French gauge)

812Fr
Infant
1216Fr
Child
1624Fr
Adolescent
2032Fr
Section 5
Chapter
38
Golden Rules
UK vaccination schedule
Richard Skone
Age
Vaccinations given
Indication
Birth
BCG
Regional variations usually

given if family members from
areas where TB is endemic
Hepatitis B (with/without
immunoglobulin within 12 hours
of birth)
If mother is a carrier
Boosters at 1 month, 2 months
and 12 months
2 months
DTaP/IPV/Hib: diphtheria, tetanus,

pertussis, polio, Haemophilus influenzae
type b
Pneumococcus
Routine vaccinations
3 months
DTaP/IPV/Hib
Meningitis C
4 months
DTaP/IPV/Hib:
Pneumococcus
Meningitis C
12 months
Hib/MenC: Haemophilus influenzae

type b, Meningitis C
MMR: measles, mumps, rubella
Pneumococcus
Pre-school boosters
(3.55yrs)
DTaP/IPV: diphtheria, tetanus,

pertussis, polio
MMR
Girls 1213yrs
Human papillomavirus vaccine (three

injections at 0, 2 and 6 months)
1318 yrs
Td/IPV:
Tetanus, diphtheria
Polio
The BCG and MMR vaccinations are live vaccinations and therefore cannot be used in immunocompromised
children.
329
Section 5
Chapter
39
Golden Rules
Common syndromes in the

critically ill child
Oliver Bagshaw
Introduction
There are nearly 7000 described syndromes and diseases that are known to occur in
children. Syndromes may be caused by:
chromosome abnormalities
single gene defects
familial association
the environment
no apparent reason.
It is impossible for even an experienced paediatrician to have knowledge of all possible
syndromes. However, every doctor should have an understanding of the common syndromes that have implications in the critically ill child.
A syndrome is defined as a group of symptoms and signs that are characteristic of a
particular disorder. Many conditions in children that have implications for the anaesthetist
do not have the term syndrome associated with them. However, for the sake of completeness, we will assume that all of the conditions mentioned in this chapter have the characteristics of a syndrome.
In keeping with the fact that most of these children will be encountered in a resuscitation scenario, the information on each condition is presented in an ABC format, so that
the treating clinician can quickly refer to the specific, relevant abnormalities in a systematic
manner.
Common syndromes
Down syndrome
Trisomy 21 (Down syndrome) is one of the commonest, best-known and most easily
recognized chromosome abnormality syndromes in children. It occurs in about 1.5 per
1000 live births, the incidence increasing with increasing maternal age.
Airway
Trisomy 21 causes the following difficulties when managing the airway:
330
Chapter 39: Common syndromes in the critically ill child
331
a large tongue, which is more likely to protrude from the mouth; this may make
attempts at intubation more difficult, as the tongue may have a tendency to herniate
over the laryngoscope blade and obscure the view of the larynx
a narrower than normal larynx, which in some cases may be related to subglottic
stenosis; an endotracheal tube 0.51 mm ID smaller than predicted should be used, to
avoid causing laryngeal oedema and post-extubation stridor.
Breathing
There is an increased incidence of respiratory tract complications due to a number of
different factors. These include:
airway abnormalities
hypotonia
relative obesity
cardiovascular anomalies
immune deficiency
gastroesophageal reflux
obstructive sleep apnoea.
Sedative and narcotic agents are likely to make this worse and should be used with caution
in the non-intubated patient.
Circulation
Cardiovascular anomalies occur in up to 50% of children with trisomy 21. The commonest
anomalies are (see Chapter 6 for details):
ventriculoseptal defect (VSD); depending on the size this may cause significant left to
right shunting
endocardial cushion defect, which most often presents with atrioventricular septal defect
and left to right shunting of blood
persistent ductus arteriosus like endocardial cushion defects, this can lead to increased
pulmonary blood flow and heart failure
tetralogy of Fallot; this leads to reduced pulmonary flow and hypoxia.
Another feature of these children is increased atropine sensitivity. This should be taken into
account if administering atropine for vagolytic effects. However, they are also characterized
by reduced sympathetic activity, which may counteract this effect.
Disability
Neurological problems include:
significant learning disabilities, which may compromise understanding of any intended
interventions; parental presence may help to calm any fears and anxieties
atlantoaxial instability, which may be found in up to 20% of children with trisomy 21; it
should be assumed to be present unless radiological investigations have proved
otherwise; frank atlantoaxial subluxation with spinal cord compression is much rarer
(2%) and is nearly always associated with neurological symptoms, including:
neck pain
decreased mobility
332
abnormal gait
torticollis
sensory deficits.
The head should be maintained in the neutral position when intubating these patients and
when managing them post-intubation.
Other
Hypothyroidism.
Potential for haematological malignancies, such as acute lymphoblastic leukaemia, with
anaemia, thrombocytopenia and increased infection risk.
Increased sensitivity to volatile anaesthetic agents.
Cystic fibrosis
Cystic fibrosis (CF) is an inherited disorder of the chloride channels in various epithelial
surfaces, resulting in the production of abnormally viscid secretions, which block organ
passages. The frequency of occurrence depends on ethnic group, with Caucasian people
having the highest incidence (1 in 3000 live births).
Airway
There are no airway abnormalities attributable to this condition. However, patients with CF
are prone to gastroesophageal reflux, with an incidence of approximately 20% in children.
Breathing
One of the most prominent features of CF is the pulmonary manifestation of the disease.
Mucus plugging and pulmonary infiltrates lead to recurrent chest infections, which may in
turn lead to:
chronic cough
pulmonary obstructive disease
bronchiectasis
haemoptysis
hypoxia
cor pulmonale.
Intubation may be straightforward, but ventilation can be problematic, with copious
secretions, bronchospasm, atelectasis, hypoxaemia, gas trapping and barotrauma all occurring in anaesthetized patients.
Active infection, particularly in the lungs, should always be considered in these patients
Pseudomonas aeruginosa and Staphylococcus aureus are the most common infecting
organisms.
Circulation
Advanced lung disease may have secondary effects on the CVS, with the development of
pulmonary hypertension, right ventricular failure and cor pulmonale. Intubating patients
with known pulmonary hypertension is a high-risk procedure. Expert advice should be
sought first, where possible.
333
Disability
Children with CF do not usually suffer from intellectual impairment.
Other
Patients with CF may be malnourished and underweight, due to impaired pancreatic
exocrine function, although this is proving less of a problem with modern enzyme
therapy.
The endocrine function of the pancreas may be affected as well, leading to diabetes
mellitus. Hypo- or hyperglycaemia should always be considered in a patient with CF
who has a depressed conscious level.
Hepatic involvement may lead to impaired function, with cholestasis and reduced
production of clotting factors.
Muscular dystrophy
Several types of muscular dystrophy (MD) exist; some exhibit X-linked inheritance
(Duchenne, Becker) while others demonstrate autosomal dominant inheritance (limbgirdle, facioscapulohumeral). All patients are characterized by an abnormality of the
dystrophin proteins in skeletal muscle, which leads to impaired function. The muscle
dysfunction varies in both severity and the muscles affected, depending on the type of
dystrophy. Weakness is usually progressive and leads to significant disability. Duchenne
(DMD) is the commonest and most severe form of the disease.
Airway
There are no specific airway problems for patients with muscular dystrophy, although
patients have been reported with a larger than normal tongue. Other problems include:
swallowing difficulties, which may lead to recurrent aspiration
gastric hypomotility, which may also increase the aspiration risk.
Breathing
Progressive respiratory and abdominal muscle weakness is characteristic. Combined
with aspiration episodes, this makes recurrent chest infections common.
Diaphragmatic function is initially preserved, but will eventually deteriorate.
Respiratory failure is the main mode of death.
Scoliosis is often present and may lead to restrictive lung problems.
Circulation
As a muscle, the heart is often affected in patients with muscular dystrophy.
Patients may develop cardiomyopathy. They may be relatively asymptomatic, due to
limited activity. Heart failure often appears in adolescence.
Arrhythmias are rare, but can occur with fibrosis of the conducting system, and may
include heart block. The ECG is usually abnormal, with sinus tachycardia, tall R waves
in the right chest leads and deep Q waves in the left chest leads.
Hyperkalaemia and cardiac arrest may occur following administration of
suxamethonium or volatile anaesthetic agents. Suxamethonium is contraindicated in
MD and volatile anaesthetic agents should be used with caution.
334
Disability
Developmental delay occurs in some, but not all patients.
Difficulty with verbal communication may be related to respiratory failure.
Other
Patients may be obese, due to replacement of muscle with fat, and inactivity.
The association between MD and malignant hyperthermia is unproven and probably
does not exist.
Congenital adrenal hypoplasia may also be present, necessitating steroid-replacement
therapy.
CHARGE association
CHARGE is an autosomal dominant disorder characterized by a number of different but
characteristic abnormalities. Not all are present in patients, although several of the major
and minor features need to be present for the diagnosis to be made. CHARGE is an
acronym of several of the common features (Coloboma, Heart defects, Atresia of choanae,
Retarded growth, Genital abnormalities, Ear abnormalities).
Airway
Choanal atresia or stenosis may be present, resulting in complete airway obstruction or
respiratory distress. This will usually have been treated in older children, but re-stenosis
can occur and the nasal passages may be small for age.
Cleft lip and/or palate.
Cranial nerve involvement can lead to swallowing problems.
Profuse oral secretion production is characteristic of the condition.
Laryngomalacia may be present, causing stridor.
Breathing
Recurrent aspiration may occur in relation to profuse secretions, swallowing difficulties
or the presence of a trachea-oesophageal fistula.
Vertebral anomalies such as hemivertebrae and scoliosis may give rise to restrictive lung
disease.
Facial abnormalities may make facemask ventilation difficult.
Circulation
Congenital heart defects such as septal defects, conotruncal abnormalities and vessel
stenosis are very common.
Disability
Common findings in this association are:
developmental delay
hearing loss
cranial nerve anomalies
seizure disorder.
335
Other
Renal abnormalities may occur which can adversely affect renal function.
Truncal hypotonia.
VATER/VACTERL association
This condition is also a collection of congenital abnormalities, characterized by the main
features of Vertebral anomalies, Anal atresia, (Cardiac defects), Tracheo-Esophageal fistula,
Renal and Limb abnormalities. Many other abnormal features have been described.
Airway
Choanal atresia may lead to airway obstruction.
Micrognathia may cause airway obstruction and difficulties with intubation.
Tracheo-oesophageal fistula may cause recurrent aspiration and difficulty in bagging as
the stomach distends.
Breathing

Aspiration and pneumonia from trachea-oesophageal fistula.

Hypoplastic lungs.
Restrictive lung disease from vertebral anomalies and scoliosis.
Increased pulmonary blood flow secondary to congenital heart disease.
Circulation
The cardiac defects seen in VACTERL association include:
VSD
PDA
tetralogy of Fallot
transposition of the great arteries.
Disability
The condition can be associated with hydrocephalus and other CNS abnormalities such as
absent corpus callosum.
Other
Renal anomalies are very common, including urethral atresia, renal agenesis and
hydronephrosis.
Bowel obstruction secondary to imperforate anus or duodenal atresia may occur.
Limb abnormalities may make IV and arterial access difficult.
Inborn errors of metabolism

The inborn errors of metabolism (IEM) constitute a constellation of conditions characterized by deficiencies of certain enzymes. Under certain conditions this can lead to an
accumulation of toxic metabolites (see Chapter 12).
More severe symptoms usually occur in the neonate and infant, when a diagnosis has
often not yet been made. Decompensation nearly always occurs as a result of metabolic
336
stress, due to intercurrent illness, change in feed or a period of starvation. An IEM should
always be considered in the differential diagnosis of the collapsed infant.
Airway
Encephalopathy and coma are a common presenting feature and may lead to loss of the
airway.
Seizure may also occur and lead to airway compromise.
Vomiting may lead to aspiration.
Breathing
Apnoea is a common presenting feature in encephalopathic infants.
Respiratory distress and increased work of breathing may be present as a result of LRTI,
acidosis or ammonia-induced central hyperventilation.
Circulation

Cardiomyopathy may develop in older children (see Chapter 12).

Circulatory collapse may be due to the accumulation of cardiotoxic metabolites.
Poor feeding and vomiting may have led to significant dehydration.
Primary or secondary lactic acidosis may be present, with a profound anion gap.
Disability
Neurological signs are often the most significant presenting feature of the infant with IEM.
Causes include:
encephalopathy and coma (usually secondary to hyperammonaemia)
seizures
hypoglycaemia
raised intracranial pressure
shock and poor cerebral perfusion
profound metabolic derangement.
Dont forget possible underlying CNS infection as a cause of neurological abnormality
Older children may already have developed significant developmental delay
Other
Hepatic disturbances are common in the acute stages of illness.
Key management actions include administration of glucose-containing IV solutions,
urgent PICU referral if significant hyperammonaemia is present (poor outcome if
treatment is delayed), administration of carnitine, benzoate and phenylacetate and
urgent consultation with an IMD specialist.
Mucopolysaccharidoses
The name includes several conditions characterized by deficiency of the enzymes responsible for mucopolysaccharide degradation in tissues. Accumulation of mucopolysaccharides
in various tissues leads to progressive cellular damage and destruction.
337
Airway
These children provide a particular challenge for anyone trying to manage their airway.
Even hand ventilation may be difficult. This is due to:
the presence of a large, thick tongue, short neck and immobile cervical spine, which can
make these patients very difficult to intubate; the problem tends to get worse as the child
gets older
obstructive sleep apnoea, which is commonly present
copious secretions necessitating antisialogogue treatment
MPS deposition in the airway, which may mean that a smaller than anticipated ET Tube
needs to be used.
Breathing
Skeletal deformities and chest wall involvement may lead to restrictive lung disease.
Thick secretions may increase the risk of respiratory complications, including recurrent
chest infections.
Circulation
Cardiac involvement presents as:
valvular disease such as mitral and aortic valve thickening
left ventricular hypertrophy
pulmonary hypertension.
Disability
In addition to developmental delay these children may have physical problems such as:
odontoid hypoplasia, creating instability of the cervical spine
progressive narrowing of the spinal canal, which may cause myelopathy, which can be
worsened by recurrent flexion and extension of the cervical spine
hydrocephalus.
Other
Prolonged bleeding times can occur in some conditions.
Joint contractures.
Coarse, dry skin making IV access more difficult.
Specialist paediatric metabolic units dealing with MPS patients are a good source of advice
and in the UK are situated in Manchester Childrens Hospital, Birmingham Childrens
Hospital and Great Ormond Street Hospital, London.
Summary
Due to the large and varied number of conditions that may be encountered in critically ill
children, it is best for the attending physicians to use their combined knowledge and
experience to manage the child appropriately. Table 39.1 is a brief summary of some of
the problems that may be encountered in syndromic children and the specific syndromes to
which they relate.
Table 39.1. Pathological conditions that may be encountered during critical care management and some of the syndromes in which they are most common.a
Airway
Breathing
Circulation
Disability
Other
Problem
Syndromes
Obstructed upper airway
Choanal atresia, craniosynostoses, Beckwith syndrome, cystic hygroma
Difficult intubation
PierreRobin syndrome, Goldenhar syndrome, TreacherCollins syndrome, hemifacial microsomia, MPS, osteopetrosis, epidermolysis bullosa,
KlippelFeil syndrome, cystic hygroma
Impaired bulbar function
Myasthenia gravis, leukodystrophy
Hypoventilation
Spinal muscular atrophy, muscular dystrophy, central hypoventilation syndrome, myasthenia gravis, Leigh disease, Jeune syndrome
Diaphragmatic weakness
Myasthenia gravis, SMA, muscular dystrophy, mitochondrial disease
Increased risk of chest infection
Cystic fibrosis, cerebral palsy, myopathies, sickle-cell disease, MPS, pulmonary alveolar proteinosis, prune belly, immune deficiency syndromes
Restrictive lung disease
PraderWilli syndrome, achondroplasia, Jeune syndrome, MPS, arthrogryposis, osteopetrosis
Congenital cardiac abnormalities
Down syndrome, VACTERL, CHARGE, DiGeorge syndrome, velocardiofacial syndrome, Noonan syndrome, Marfan syndrome, Turner syndrome,
Shone complex, Alagille syndrome, Cornelia de Lange syndrome
Cardiomyopathy
Muscular dystrophy, Pompe disease, IEM, MPS, haemochromatosis, mitochondrial disease, Friedrich ataxia, Kawasaki disease, arthrogryposis, SLE
Arrhythmias
WolfParkinsonWhite syndrome, PraderWilli syndrome, congenital lupus, LownGanongLevine syndrome, long QT syndrome, Rett syndrome
Raised lactate
Mitochondrial disease, Leigh disease, pyruvate dehydrogenate deficiency
Raised ICP
Achondroplasia, craniosynostoses, DandyWalker malformation, ArnoldChiari malformation, spina bifida
Unstable cervical spine
MPS, achondroplasia, KlippelFeil, EhlersDanlos, arthrogryposis
Hypoglycaemia
MCADD, Prader-Willi syndrome, glycogen storage disease, Beckwith syndrome, several IEM
Seizures
Cerebral palsy, several IEM (particularly with hyperammonaemia), leukodystrophy, LennoxGastaut syndrome, DandyWalker malformation, absent corpus
callosum, Cornelia de Lange syndrome, West syndrome, tuberous sclerosis, SturgeWeber syndrome, Rett syndrome
Hepatic insufficiency
Alagille syndrome, haemochromatosis, glycogen storage disease, mitochondrial disease, galactosamia, BuddChiari syndrome,
1-antitrypsin deficiency, Wilson disease, tyrosinaemia
Renal insufficiency
Scoliosis
Nephrotic syndrome, haemolyticuraemic syndrome, Goodpasture syndrome, polycystic kidney disease, SLE
Muscular dystrophy, achondroplasia, osteogenesis imperfecta, CHARGE, cerebral palsy, spinal muscular atrophy, Prader-Willi syndrome,
neurofibromatosis, Marfan syndrome, KlippelFeil syndrome, arthrogryposis
Malignant hyperpyrexia risk
Central core disease, multiminicore disease, KingDenborough syndrome, Brody myopathy
Anaemia
Sickle-cell disease, thalassaemia, haemoglobin C disease, G-6-PD deficiency, haemolytic uraemic syndrome, hereditary spherocytosis, Fanconi
anaemia, DiamondBlackfan anaemia
Muscular dystrophies are not thought to be associated with malignant hyperpyrexia, although triggering agents such as halothane and suxamethonium can precipitate rhabdomyolysis
and hyperkalaemia. Suxamethonium should also be avoided in any other myopathic or neuropathic disorder that causes significant muscular weakness, due to
the risks of massive potassium release and life-threatening arrhythmias.
IEM, inborn errors of metabolism; MCADD, medium chain acyl-coA dehydrogenase deficiency; MPS, mucopolysaccharidosis; SLE, systemic lupus erythematosus; G-6-PD,
glucose-6-phosphate dehydrogenase.
Further reading
Baum VC, OFlaherty JE. Anesthesia for Genetic,
Metabolic, and Dysmorphic Syndromes of
Childhood, 2nd edn. Lippincott, Williams
and Wilkins, 2007.
339
Bissonnette B, Dalens B. Syndromes: Rapid

Recognition and Perioperative Implications.
McGraw-Hill Professional, 2006.
http://www.orpha.net.
http://www.rarediseases.org.
Index
abdominal pathology, 159,
See also specific
conditions
acquired intestinal
obstruction, 162163
children versus adults,
159160
chronic conditions, 164
congenital anomalies,
161162
differential diagnosis, 160
intra-abdominal sepsis,
163
investigations, 167
management, 164167
cardiovascular support, 166
nasogastric tubes, 166
pitfalls, 168
surgery, 160, 167168
urinary catheter, 166167
vascular access, 167
ventilation, 166
presentation, 160
red flags, 161
signs secondary to other
conditions, 164
trauma, 163, 177
abscess, stridor, 139
acidaemias, 114
adrenaline, 30, 321
anaphylaxis management,
132133
stridor treatment, 142
adult critical care unit (ACCU),
245
challenges, 247249
decision to manage locally,
247
discharge, 247
equipment, 246247
multidisciplinary approach,
246, 248
professional development, 248
standards, 245247
airway
assessment, 24
burns patients, 193
signs of obstruction, 24, 141
blood in, 173

146149
head position, 147
difficult airway management,
146, 149157
equipment, 149150
help, 150
plan, 150156
predicting the difficult
airway, 149
stabilization prior to
transfer, 157
equipment, 1415
endotracheal tube
(ET tube), 14
suction systems, 15
tracheostomy, 15
external compression, 140
management, 2728, 325
See also intubation;
ventilation
burns patients, 195
massive haemorrhage
and, 207
neonatal shock, 211212
raised intracranial
pressure, 184
trauma resuscitation,
172173
obstruction, 211212
alprostadil, 306
ambulance, 232
aminophylline, 83
anaesthesia
asthma, 83
emergency laparotomy, 178
haemorrhagic shock,
172173
topical, 256
trauma resuscitation, 172
traumatic brain injury, 176
analgesia, 250, 252257
See also pain
burns patients, 197
codeine, 255
entonox, 256257
fentanyl, 256
formulations, 254
ibuprofen, 254255
intranasal diamorphine, 256

morphine, 255256
nerve blocks, 257
paracetamol, 254
patient/nurse-controlled
analgesia, 256
topical anaesthesia, 256
anaphylaxis, 130
biphasic, 134135
follow-up, 135
investigations, 134
life-threatening reaction, 131
management, 132134
presentation, 130
signs, 131
speed of onset, 130131
antibiotic therapy
bronchiolitis, 68
diabetic ketoacidosis, 109
meningitis, 37
pleural effusion/empyema,
7172
pneumonia, 70
respiratory infections, 260
stridor, 143
anticonvulsants, 223224
antidiuretic hormone
(ADH), 290
apnoeas, 67, 212
appendicitis, 163
appendix, perforated, 243
arrhythmias, 55
decreased consciousness, 93
management, 5960
bradycardia, 60
heart block, 60
spelling with tetralogy
of Fallot, 60
tachycardia, 5960
neonates, 214
arterial lines, 17, 313, 327
See also vascular
access
neonates, 216
assessment, 2427,
See also specific
conditions
airway, 24
approach to child, 24
340
Index
blood gas sampling

devices, 18
blood sugar, 26
breathing, 25
cardiovascular, 2526
electrolytes, 26
pain, 250252
temperature, 2627
asthma, 76
assessment, 7677
children versus adults, 76
investigations, 7274
management, 7983
aminophylline, 83
beta-2 agonists, 82
intravenous salbutamol, 82
intubation, 8385
life-threatening asthma,
8283
magnesium sulphate, 83
oxygen, 82
steroids, 82
ventilation, 8385, 283
pathophysiology, 76
presentation of acute
exacerbations,
7778
risk factors for exacerbations,
78
severity assessment, 79
transfer preparation, 86
auto-positive end expiratory
pressure (auto-PEEP),
84
bagmask ventilation, 27
difficulty, 152
neonates, 216
bereavement counselling, 269
beta-2 agonists
asthma treatment, 82
intravenous salbutamol, 82
preschool wheeze
management, 69
bi-level ventilation, 279
biochemical markers, 295
bloodbrain barrier, 303
blood gas sampling, 26
congenital heart disease, 49
devices, 18
neonates, 216
respiratory pathology, 73
asthma, 78
blood loss assessment, 174
blood pressure, neonates, 312
blood products, 203204,

See also blood
transfusion
CMV-negative, 203
complications, 209
fresh frozen plasma (FFP),
204
haemorrhage treatment, 174
irradiated, 202203
packed red cell transfusion,
203
platelets, 204
problems with
administration in
children, 209210
sepsis management,
42, 46
blood tests
after cardiac arrest, 225
blood sugar assessment, 26,
See also glucose
during surgical
emergencies, 241
raised intracranial pressure,
185
sepsis, 35
shortness of breath,
7273
toxin ingestion, 128
blood transfusion, 201,
See also blood products
complications, 209
transfusion-associated
circulatory overload
(TRACO), 209
transfusion-related
acute lung injury
(TRALI), 210
considerations in children,
201203
CMV-negative blood
products, 203
202
irradiated blood products,
202203
platelets, 202
red cells, 201202
in surgical emergencies, 240
bradycardia, 60
brain injury, 176177
post-cardiac-arrest
syndrome, 221222
raised intracranial pressure, 183
management, 176
341
brain tumours, 187

breathing assessment, 25
breathing circuits for
ventilators, 15
bronchiolitis, 16, 6668
high-risk patients, 67
identification of sicker
patients, 66
signs of deterioration, 67
treatment, 6768
antibiotics, 68
fluids, 67
oxygen, 67
pitfalls, 68
bronchopulmonary dysplasia
(BPD), 318
burns, 192
assessment, 193194
airway, 193
depth of burns, 194
exposure/extent of burns,
194
inhalation injury,
193194
child protection issues, 193,
198
pathophysiology in children,
192193
potential problems, 199
signs that child is really sick,
198199
treatment, 195198
airway management, 195
analgesia, 197
dressings, 198
fluid therapy, 197
intubation, 193, 195
shock, 197
cannulae, 312
capillary gases, 313
capillary refill time (CRT), 26,
174
carbon monoxide poisoning, 125
cardiac arrest, 220,
See also post-cardiacarrest syndrome
(PCAS)
child protection issues, 225
phases of, 219
post-arrest interventions,
223225
anticonvulsants, 223224
342
Index
cardiac arrest, (cont.)

metabolic control,
224225
oxygen management, 224
temperature management,
223
ventilation, 224
transfer after, 226227
cardiovascular assessment,
2526
signs that child is really
ill, 36
cardiovascular disease.
See congenital heart
disease (CHD)
care. See also end of life care
community input, 259
infections, 263
medical input, 259
neurological problems,
262263
primary carers, 259
respiratory pathology,
260261
respite care, 259
care-givers, 31, See also parents
communication with
following death of child,
268269
primary carers, 259
caustic ingestion, 127
central lines, 17, 30, 326
neonates, 216
cerebral oedema, 104
causes, 105
cerebral perfusion, 181
autoregulation, 181182
cervical hard collars, 19
chain of evidence, 271
CHARGE association,
334335
chest drains, 16, 328
pleural effusion
management, 7172
chest infection. See respiratory
pathology
chest injury, 177
chest X-ray
asthma, 78
congenital heart disease, 49
shortness of breath, 73
child protection, 270,
See also non-accidental
injury (NAI); sudden
unexpected death in
infancy (SUDI)
burns, 193, 198

cardiac arrest, 225
documentation standards,
270271
laboratory samples and chain
of evidence, 271
neonates, 215
ongoing issues, 273
chloral hydrate, 307
chronic lung disease of
prematurity, 282,
318319
CMV-negative blood products,
203
coarctation of the aorta, 51
codeine, 255
cold shock response, 26,
3536
collars, 19
communication, 264265
following death of child,
268269
with children, 260
with parents, 265
complex needs, 258
care, 259
communication issues,
264265
infection sources, 264
precipitants of illness, 260
congenital diaphragmatic
hernia (CDH),
283, 317
congenital heart disease
(CHD). See also specific
conditions
complications, 6062
conditions presenting in
infants/older children,
5355
confirmation as cause of
collapse, 49
management, 5561
aims, 5758
arrhythmias, 5960
balancing circulations,
5758, 284
inotropes, 57, 59
invasive monitoring, 57
neonates, 5557, 63
older children, 5861, 63
prostaglandin E (PgE),
5657
surgical shunts, 284

ventilation, 283284
neonates, 4849, 5153,
213214
arrhythmias, 214
reduced pulmonary blood
flow, 213214
reduced systemic blood
flow, 214
ventricular function
impairment, 214
worsening cyanosis, 62
consciousness
asthma assessment, 77
decreased, 87
assessment, 88
causes, 88
general management,
9092
management of specific
conditions, 9293
transfer preparation,
9394
consent, in surgical
emergencies, 237
continuous insulin infusion,
107108
continuous positive airway
pressure (CPAP), 279,
See also ventilation
devices, 16
convulsions. See seizures
corticosteroid therapy.
See also steroid
therapy
stridor, 142
cricoid pressure, 173
croup. See also stridor
assessment, 140141
spasmodic, 140
viral, 138139
CT scan
diabetic ketoacidosis
management, 109
187
cyanosis, 62
cystic fibrosis (CF), 332
death of child. See also end of
life care
bereavement counselling,
269
Index
communication with
care-givers, 268269
in the ED, 268
organ donation, 269
sudden unexpected death in
infancy (SUDI),
273274
defibrillators, 18, 321
external pacing, 18
dehydration correction,
291292, See also
fluids
dextrose replacement, 294
diabetic ketoacidosis (DKA),
104
cerebral oedema causes, 105
post-intubation care,
109110
timing of observations, 106
treatments, 106109
antibiotics, 109
electrolytes and
osmolality, 108
fluids, 107
heparin, 109
insulin, 107108
intubation, 108
raised intracranial
pressure, 109, 189190
warning signs that child is
really ill, 105106
diamorphine, intranasal, 256
diaphragmatic hernia,
congenital (CDH),
283, 317
dinoprostone, 306
District General Hospital
(DGH)
changing practice, 23
organization for critical
care, 34
equipment, 35
hospitals with no acute
paediatric unit, 4
planning for paediatric
emergencies, 6
relationship with tertiary
centre, 7
Down syndrome, 330332
dressings, burns, 198
drug therapy. See pharmacology;
specific drugs and
conditions
ductus arteriosus (DA), 5051

conditions with ductdependent mixed flow,
5253
conditions with ductdependent pulmonary
flow, 51
conditions with ductdependent systemic
flow, 5152
patent (PDA), 316317
persistence of, 50
echocardiography, 49
transthoracic (TTE), 174
electrolyte assessment, 26
electrolyte management.
See also fluids
replacement therapy, 293
emergencies. See paediatric
emergencies
empyema, 7172
end-of-life care, 266
withholding/withdrawing
intensive therapy,
266268
ethical issues, 267
legal issues, 268
medical issues, 267
endocarditis, 62
endotracheal tube (ET tube),
14, 147, 321
blockage, 278, 310
cuffed, 14, 149
length of, 149, 310
nasal, 310
neonates, 216, 310
suction catheters, 15
end-tidal capnography, 278
enteral feeding, 297
neonates, 314
entonox, 256257
epiglottitis, 138139
epilepsy, 9798,
See also seizures
equipment, 35
adult critical care unit, 246247
airway, 1415, 149150
blood gas sampling devices, 18
cervical hard collars, 19
defibrillators, 18
external pacing, 18
for transfer, 232233
monitoring, 18
343
nasogastric tubes, 1819

ventilation, 1516
errors, reasons for, 1112
Escherichia coli, 37
exhaustion signs, 141
extracorporeal membrane
oxygenation (ECMO),
285
extubation, 285286
fatty acid oxidation (FAO)
disorders, 114115
febrile seizures, 97
feeding, 297298
enteral feeding, 297, 314
neonates, 314315
parenteral feeding, 298
fentanyl, 256
fibreoptic intubation, 156
FLACC score, 251
fluids, 2930, 288
abdominal pathology, 166
body fluid electrolyte
compositions, 294
bronchiolitis management, 67
burns patients, 197
289290
ADH release, 290
body water distribution, 289
urinary concentrating
ability, 289
management, 2930,
106107
calculating fluid
replacement, 107
shock, 107
slow rehydration, 107
estimations, 290292
dehydration correction,
291292
volume expansion, 290
in surgical emergencies,
239240
maintenance therapy,
292294
during transfer, 231
electrolyte replacement, 293
fluid replacement,
292293
neonates, 314
ongoing non-physiological
losses, 294
344
Index
fluids (cont.)
monitoring, 294296
neonates, 312, 314
management, 186
sepsis, 30, 4142
shock management, 174
foreign body aspiration, 140
removal, 143
202, 204
gas trapping, 84
Glasgow Coma Scale, 322
glucose, 31
blood sugar assessment, 26
during surgical
emergencies, 241
hypoglycaemia, 31, 296
decreased consciousness,
92
neonates, 314315
glutaric aciduria type 1, 114
glycogen storage disorders, 115
group B streptococcus (GBS), 37
Haemophilus influenzae type
B (Hib), 38
epiglottitis, 138
haemorrhage. See also blood
transfusion
intracranial (ICH),
317318
massive, 174, 204208
airway management, 207
circulation management,
207208
imaging, 208
protocol, 207
transfer issues, 208209
postoperative tonsillar
bleeding, 241242
treatment, 174
tranexamic acid (TXA),
176
heart block, 60
heart disease. See congenital
heart disease (CHD)
heart failure, 5354, 62, 72
ventilation issues, 284285
heart rate, 77
heatmoisture exchanger
(HME), 16
heliox, 142
heparin, diabetic ketoacidosis

management, 109
hepatomegaly, congenital heart
disease, 49
herpes simplex, 38
high-frequency oscillatory
ventilation (HFOV),
280281
Hirschsprungs disease, 161
Hirschsprungs enterocolitis,
162
humidification, 16, 142, 278
hydronephrosis, 162
hyperammonaemia, 114115,
119
hypercapnoea, in asthma
management, 85
hyperglycaemia after cardiac
arrest, 224
hypernatraemia, 295
hyperoxia, after cardiac
arrest, 224
hypertensive encephalopathy,
93, 190
hyperthermia after cardiac
arrest, 223
hypertrophic ventricles, 55
hyperventilation, 186
management, 109
hypocalcaemia, 176
hypocapnoea, 105
hypoglycaemia, 31, 296
decreased consciousness
and, 92
glycogen storage disorders,
115
neonates, 314315
hypoglycaemics, 126
hyponatraemia, 296
hypoplastic left heart syndrome
(HLHS), 5152
hypothermia, 176
prevention during transfer,
232
hypoventilation, after cardiac
arrest, 224
hypovolaemia, 29
hypoxia
in asthma treatment, 85
ibuprofen, 254255
immune function, 34
inborn errors of metabolism,
336, See also inherited
metabolic disorders
(IMD)
infections, 33, See also sepsis;
specific infections
care issues, 263
9293
potential sources, 264
preterm infants, 319
190
respiratory, 260261
seizures, 97
stridor causes, 138139
susceptibility to, 3435
infective endocarditis, 62
information sources, 264265
inguinal hernia incarceration,
163
inhalation injury, 193194
inhaled bronchodilators.
See beta-2 agonists
inhaled nitric oxide (iNO), 281
inherited metabolic disorders
(IMD), 112,
See also specific
disorders
effects on child, 112113
complications, 116
management following
stabilization, 119
neonates, 214215
recognition of, 117
treatments, 117120
drugs to lower plasma
ammonia, 324
ensuring anabolism,
118119
reintroduction of
appropriate feeds, 119
removing toxic
metabolites, 119
removing toxic precursors,
118
supportive treatment for
homeostasis, 119
inotropes, 30
congenital heart disease
management, 57, 59
neonates, 312
sepsis management, 42
Index
insulin therapy
cerebral oedema, 105
diabetic ketoacidosis,
107108
intracranial haemorrhage
(ICH), 317318
intracranial pressure (ICP),
180, See also raised
intracranial pressure
autoregulation, 181182
intraosseous access, 17, 30
intravenous access. See vascular
access
intubation, 2728
133134
asthma management, 8385
burns patients, 193, 195
9091
management, 108
difficulty, 152154
failure, 156
fibreoptic, 156
indications, 25
neonates, 216, 310
post-intubation
management, 74
seizure management, 101
sepsis and, 4243
stridor management,
143145
trauma resuscitation, 172
intussusception, 162, 243
invasive positive-pressure
ventilation, 279280
iron overdose, 126
isovaleric acidaemia (IVA), 113
laparotomy, emergency, 178
laryngeal mask airway (LMA),
152
fibreoptic intubation via, 156
laryngotracheobronchitis, 138
left to right shunts, 54
listeria, 37
long lines, 313
lumbar puncture, 187
Lund and Browder charts, 194
lysosomal storage disorders,
119120
magnesium sulphate, 83
malaria, 190
malrotation, 161
maple syrup urine disease
(MSUD), 114
massive haemorrhage.
See haemorrhage
meningitis, 3738
meningococcal, 3839
pneumococcal, 38
seizures, 97
treatment, 37
meningoencephalitis, 38
metabolic acidosis, 72
glycogen storage diseases, 115
metabolic rate, 289
methadone ingestion, 126
methicillin-resistant
Staphylococcus aureus
(MRSA), 39
methylmalonic acidaemia
(MMA), 113
midazolam, 306
milrinone, 306
mitochondrial disorders, 115
monitoring
during transfer, 232
equipment, 18
morphine, 255256
pharmacology, 305
mucopolysaccharidoses (MPS),
336337
muscle relaxants, 30
muscular dystrophy (MD), 334
Mycoplasma pneumoniae, 38
nasogastric tubes, 1819, 148,
327
abdominal pathology
management, 166
nasopharyngeal airway (NPA),
147
nasopharyngeal aspirate
(NPA), 74
(NEC), 282, 315316
Neisseria meningitides, 38
Neonatal Infant Pain Score
(NIPS), 251252
neonates. See also preterm
infants
age calculation, 309
circulation management, 312
conditions affecting
neonates, 212215
345
hernia (CDH), 317
congenital heart disease,
4849, 5153, 213214
arrhythmias, 214
reduced pulmonary blood
flow, 213214
reduced systemic blood
flow, 214
ventricular function
impairment, 214
feeding, 314
hypoglycaemia, 314315
intracranial haemorrhage
(ICH), 317318
intubation, 216, 310
ET tube blockage, 310
ET tube length, 310
ET tube size, 310
nasal ET tube, 310
maintenance fluids, 314
metabolic disorders,
214215
(NEC), 315316
non-accidental injury, 215
pain assessment, 251252
patent ductus arteriosus
(PDA), 316317
renal function, 312
sedation, 313
seizures, 9697
sepsis, 34
shock, 211212
septic shock, 212213
technical issues, 215217
temperature management,
315
vascular access, 216, 312313
preterm neonates, 281282
nerve blocks, 257
networking, 7
neurological assessment, 92
neurometabolic disorders, 121
neutropenic sepsis, 39
non-accidental injury (NAI),
215, 270272
See also child protection
examination, 272
history-taking, 271272
injuries to arouse suspicion,
272
investigations, 274
management, 272273
346
Index
non-accidental injury (NAI)

(cont.)
non-clinical, 272273
275276
non-invasive ventilatory support
(NIVS), 278279
notification of disease, 46
opiate ingestion, 126
organ donation, 269, 275
osmolarity, 288289
overdose. See toxin ingestion
oxygen therapy
asthma, 82
bronchiolitis, 67
heliox, 142
home oxygen, 261
pacing, external, 18
packed red cell transfusion, 203
paediatric emergencies.
See also surgical
emergencies
differences in, 10
planning for, 6
pain, 250, See also analgesia
assessment, 250252
FLACC score, 251
NIPS score, 251252
self-reporting scores, 252
palliative care. See end-of-life
care
palpitations, 62
paracetamol, 254
overdose, 125
pharmacology, 305
parenteral feeding, 298
parents, 31, 248 See also
care-givers
communication with, 265
patent ductus arteriosus (PDA),
316317
pelvic fracture, 177
perforated appendix, 243
peripheral lines, 17
peritonitis, 163
pharmacokinetics, 300304
See also pharmacology
absorption, 300302
oral route, 300301
rectal route, 302
distribution, 302303
bloodbrain barrier, 303
clearance, 303
protein binding, 303
elimination, 304
metabolism, 303304
pharmacology, 300,
See also pharmacokinetics;
specific drugs and
conditions
drug dosing in renal
replacement therapy,
307308
drug infusions, 323
parenteral administration
routes, 304305
intraosseous (IO)
administration, 305
intravenous
administration, 304
physiological parameters, 321
physiotherapy, pneumonia, 70
platelet transfusions, 202, 204
pleural effusion, 7172
pneumococcal meningitis, 38
pneumonia, 6971
causes, 70
clinical features, 70
treatment, 7071
poisoning. See toxin ingestion
positive end expiratory
pressure (PEEP), 28
asthma management, 85
auto-PEEP, 84
post-cardiac-arrest syndrome
(PCAS), 220223
brain, 221222
heart, 223
bleeding, 241242
prenatal diagnosis, 318
pressure-control ventilation, 28
preterm infants, 318320
chronic lung disease (CLD),
282, 318319
infections, 319
respiratory distress of the
newborn (RDS), 318
retinopathy of prematurity
(ROP), 319
seizures, 319
ventilation, 281282
professional development, 248
propionic acidaemia (PA), 113
propofol, 307
prostaglandin E (PgE), 5657,
306
pulmonary atresia, 51
pulmonary interstitial
emphysema (PIE), 318
pulmonary oedema, 43, 49

pulmonary stenosis, 51
pulmonary vascular resistance
(PVR), 58
pyrexia, 2627, 31
(ICP), 180, 182183
conditions causing, 183
in diabetic ketoacidosis, 106,
109, 189190
in traumatic brain injury,
176, 183
190191
treatment, 184187
emergency management,
184, 186
refractory or progressive
rise in ICP, 186187
underlying cause, 187190
red blood cells, 201202
packed red cell transfusion,
203
rehydration. See fluids
renal function, neonates, 312
renal replacement therapy,
307308
respiratory distress, 36,
See also shortness of
breath
of the newborn (RDS), 318
respiratory pathology.
See also airway; specific
conditions
care issues, 260261
infections, 260261
antibiotic treatment, 260
prophylaxis, 261
post-intubation
management, 74
signs, 25
really ill, 65
respiratory rate, 77
respiratory syncytial virus
(RSV), 69, 260
immunization, 261
respite care, 259
resuscitation, 2
damage-control
resuscitation, 175
endpoints, 175
Index
hypotensive, 175
phases of, 219
sepsis treatment, 4142
teamwork, 6
training, 6
trauma, 169
witnessed, 268
retinopathy of prematurity
(ROP), 319
retrieval services, 7
salbutamol, intravenous, 82,
See also beta-2 agonists
sedation, 30
asthma, 84
neonates, 313
seizures, 101102
transfer, 231
seizures, 93, 95
care issues, 262
causes, 9598
epilepsy, 9798
febrile seizures, 97
infection, 97
neonates, 9697
poisoning, 97
management, 98103
drug treatment, 99101
examination, 98
extubation, 102103
history, 98
intubation, 101
sedation, 101102
premature infants, 319
refractory, 98
status epilepticus, 95
sepsis, 3334, See also specific
infections
care issues, 263
cold shock, 3536
vasoconstriction
monitoring, 36
intra-abdominal, 163
neonatal septic shock,
212213
neutropenic, 39
notification of disease, 46
pathogens, 34
presentation, 35
susceptibility to infection,
3435
treatment, 3943
blood products, 42
fluid resuscitation, 30,

4142
goal-directed therapy, 41
intubation and ventilation,
4243
pitfalls, 46
post-intubation, 4344
steroids, 43
vasoactive drugs, 42
really ill, 3637
serum tryptase, 134
shock
anaesthesia, 172173
cold shock response, 26, 36
airway, 211212
burns patients, 197
damage control
resuscitation, 175
fluids, 174, 212, 290
hypotensive resuscitation,
175
resuscitation endpoints,
175
monitoring, 294
neonatal. See neonates
septic, 212213
toxic shock syndrome, 39
shortness of breath, 64,
See also respiratory
pathology; specific
conditions
non-pulmonary causes, 72
respiratory distress, 36
sodium bicarbonate, 105
sodium management, diabetic
ketoacidosis, 108
spelling with tetralogy of Fallot,
60
spinal immobilization, 173
before transfer, 230
stabilization, 2
Staphylococcus aureus, 39
methicillin-resistant
(MRSA), 39
status epilepticus (SE), 95
steroid deficiency, 215
steroid therapy
anaphylaxis, 133
asthma, 82
preschool wheeze, 69
sepsis, 43
stridor, 142
347
Streptococcus, 39
stridor, 137
assessment, 140141
biphasic, 137
causes, 138140
chronic, 140
infective, 138139
non-infective, 139140
137138
expiratory, 137
inspiratory, 137
intubation, 143145
gas induction, 144
signs that child is really sick,
141142
treatments, 117119
antibiotics, 143
corticosteroids, 142
foreign-body removal, 143
heliox, 142
humidification, 142
nebulized adrenaline, 142
stroke volume, 29
suction systems, 15, 278
sudden unexpected death in
infancy (SUDI),
273274
investigations, 274, 275
organ donation, 275
support services, 4
surgical emergencies.
See also paediatric
emergencies
235241
consent, 237
fluids, 239240
glucose monitoring, 241
inotropes, 241
intubation and ventilation,
238
optimization, 235
patient positioning, 238239
perforated appendix, 243
postoperative care, 241
bleeding, 242
presentation, 235
psychological management,
236237
recognition of, 236
temperature control,
240241
transfer, 243244
348
Index
surgical emergencies. (cont.)

syncope, 62
syndromes, 330, 338
See also specific
syndromes
systemic inflammatory
response syndrome
(SIRS), 33
criteria for in children, 33
systemic vascular resistance
(SVR), 58
tachycardia, 5960
Tanner report (2006), 2
teamwork, 6, 9
before the child arrives, 12
differences of opinion, 12
raising concerns, 12
team assembly, 1213
team composition, 4
team integration, 13
team leader, 11
team management, 10
team members, 11
temperature
assessment, 2627
management, 31
neonates, 315
really ill, 36
tetralogy of Fallot (ToF), 54
management of spelling, 60
tonicity, 288289
tonsillar bleeding,
postoperative, 241242
total anomalous pulmonary
venous connection
(TAPVC), 53
total parenteral nutrition
(TPN), 298
neonates, 315
toxic shock syndrome, 39
toxin ingestion, 122,
See also specific toxins
child protection issues, 128
history-taking, 122123
dose ingested, 123
time of ingestion, 123
toxin, 122
management, 127129
cardiovascular support,
127
ventilation, 127
presentation, 123
seizures, 97
symptoms and signs,
123124
tracheitis, bacterial, 139
tracheobronchomalacia, 283
tracheostomy, 15, 261
training, 6, 248
tranexamic acid (TXA), 176,
208
transfer, 2, 228
after cardiac arrest,
226227
asthma patients, 86
burns patients, 200
checklist, 233
children with complex needs,
265
9394
difficult airway and, 157
massive haemorrhage,
208209
monitoring, 232
non-accidental injury,
275276
preparation, 228229, 234
patient preparation,
229233
190191
respiratory pathology, 74
safety issues, 233
seizures, 103
sepsis, 4446
stridor, 145
surgical emergencies,
243244
trauma and, 178
transfusion-associated
circulatory overload
(TRACO), 209
transfusion-related acute lung
injury (TRALI), 210
transposition of the great
arteries (TGA), 5253
transthoracic echocardiography
(TTE), 174
trauma, 169, See also nonaccidental injury (NAI)
abdominal injury, 163, 177
brain injury. See traumatic
brain injury (TBI)
chest injury, 177

pelvic fracture, 177
primary survey, 170171
resuscitation, 169
airway management,
172173
anaesthesia, 172
hypothermia, 176
preparation, 169170
shock management,
173176
trauma-induced coagulopathy
(TIC), 174
traumatic brain injury (TBI),
176177
anaesthesia, 176
183
tricuspid atresia, 51
tricyclic antidepressant (TCA)
ingestion, 127
ultrasound evaluation
umbilical lines, 313
urea cycle disorders, 113
urinary catheter, 166167, 327
urinary concentrating ability,
289
urinary tract pathology, 162
vaccination schedule, 329
vascular access, 1718,
30, 326
arterial lines, 17
central lines, 17, 30
in surgical emergencies,
237238
neonates, 216, 312313
peripheral lines, 17
vasoactive drugs, 30
sepsis management, 42
VATER/VACTERL
association, 335
ventilation, 29
aids, 281
134
asthma management,
83, 283
complications, 84
Index
problems, 85
ventilator setting, 8485
bagmask, 27, 216
difficulty, 152
bronchiolitis management,
68, 283
hernia management,
283
congenital heart disease and,
283284
difficulty ventilating, 2829,
74, 152, 285
failure, 156
heart failure, 284285
high-frequency oscillatory
ventilation (HFOV),
280281
indications, 24, 277
invasive positive-pressure
ventilation, 279280
neonates, 216, 311312
non-invasive ventilatory
support (NIVS),
278279
physiological considerations,
277278
pneumonia management,
7071
pressure control, 28
preterm neonates, 281282
184
refractory respiratory failure,
285
sepsis, 4243
tracheobronchomalacia
management, 283
weaning, 285286
ventilation equipment, 1516,
278
breathing circuits for
ventilators, 15
chest drains, 16
349
continuous positive airway

pressure (CPAP)
devices, 16
end-tidal capnography,
278
humidification, 16, 278
mechanical ventilators, 15
non-invasive ventilation, 16
suction, 278
ventricular hypertrophy, 55
ventricular shunts, 262263
blockage, 189
viral croup, 138
wheeze
asthma, 77
preschool children, 6869
withholding/withdrawing
intensive therapy,
266268
ethical issues, 267
legal issues, 268
medical issues, 267
witnessed resuscitation, 268

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Managing the Critically Ill Child

A Guide for Anaesthetists and Emergency

Managing the Critically

CAMBRIDGE UNIVERSITY PRESS

Cambridge, New York, Melbourne, Madrid, Cape Town,

Section 1 The District General

10 The child with seizures

Setting up a department for

11 The child with diabetic

Team approach and organization

Stabilization of the critically

The child with sepsis 33

The child with cardiac disease 48

The child with shortness of

14 The child with anaphylaxis

15 The child with stridor 137

19 The child with raised intracranial

22 The sick neonate presenting

Section 4 The Childrens

Section 3 What You Could Be

25 Anaesthetizing for a surgical

32 Fluid therapy 288

23 Ongoing management of the

Section 5 Golden Rules

39 Common syndromes in the

30 Child protection 270

The District General Hospital Setting

Setting up a department for

Central venous access

First inotropic agent

Section 1: The District General Hospital Setting

Changing practice in district general hospitals (DGH)

The Tanner report

Chapter 1: Setting up a department for managing a sick child

Organizing a DGH to manage a critically ill child

Section 1: The District General Hospital Setting

Hospitals with no acute paediatric units

Chapter 1: Setting up a department for managing a sick child

Suggested equipment needed

Airway and breathing

Oxygen mask with reservoir bag

Intravenous cannulas (24G upwards)

Locally agreed emergency drugs should be kept on the trolley,

Blood glucose monitor

Section 1: The District General Hospital Setting

Teamwork and training

Actions needed in preparing for a sick child

Regular scenario training

Prompts, e.g. wall chart with age-appropriate GCS

Age-specific early warning scores to identify sick inpatients

Chapter 1: Setting up a department for managing a sick child

Relationship with the tertiary centre

Preparation is key to managing a sick child

Section 1: The District General Hospital Setting

for Children and Young People in

The District General Hospital Setting

Team approach and organization

Differences in paediatric emergencies

Section 1: The District General Hospital Setting