Bailey & Love Bailey & Love Bailey & Love

Bailey
PART&11 |Love Bailey & Love Bailey & Love
Abdominal

CH A P T E R

73 Functional disorders of the intestine

Learning objectives
To recognise and understand: • The management of common chronic disorders that
• The spectrum of intestinal disorders resulting from present to surgeons such as chronic constipation and
abnormal neuromuscular functions irritable bowel syndrome
• The management of relatively common acute motility • The existence of several rare neuromuscular diseases that
disturbances may affect the intestine
• The limited role of surgery in the treatment of most of
these disorders

more complicated and still poorly understood with some

APPLIED ANATOMY AND features akin to the MMC but also specific phenomena such
PHYSIOLOGY as retrograde movements (presumed to allow greater resident
The intestine must subserve basic functions of moving contents time and therefore fluid and electrolyte absorption). The main
from proximal to distal in a rhythmical fashion to allow mixing, characteristics of intestinal motility are shown in Table 73.1.
digestion and absorption of contents. The motility of the The intestine, like the heart, is autonomous in generating
intestine has been studied for more than a century and all its own rhythmical electrical, and therefore local motor, activity
readers should know of the seminal experiments of Bayliss and by intrinsic pacemaker activity generated by small fibroblast-
Starling in their 1899 paper ‘The movements and innervation like cells called the interstitial cells of Cajal. These cells, which
of the small intestine’, which led to adoption of the term ‘peri- are mainly resident within the muscularis propria, have several
stalsis’. In the small intestine, fasting motility can be described key functions, including setting the membrane potential of
by the three phases of the migrating motor complex (MMC), smooth muscle cells so that they are primed to contract and
with fed activity resembling phase II. Colonic motility is much connecting smooth muscle cells electrically so that synchronous

TABLE 73.1 Contractile activity of the intestine.

Region Broad category
Small intestine Phase I Quiescence
(40–60% of total time)
Phase II High-frequency contractions allowing mixing and absorption
(20–30% of total time)
Phase III High-amplitude propagated activity
(5–10 minutes)
Large intestine Phasic contractions Low-amplitude propagated pressure waves
High-amplitude propagated pressure wavesa
Retrograde pressure waves
Simultaneous pressure waves
Periodic colonic and rectal motor activity (localised bursts)
Tonic contractions Sustained activity responsible for tone
a
Most akin to phase III of the migrating motor complex and responsible for mass movements of faecal content.

Sir William Bayliss, 1860–1924, physiologist, and Ernest Henry Starling, 1866–1927, University College London, London, UK. Starling’s contributions to
medicine also included Starling’s principle (capillary pressures) and filling of the heart (Frank–Starling law).
Santiago Ramon y Cajal, 1852–1934, Spanish neuroscientist, pathologist and Nobel prize winner (1906) for studies of cellular anatomy of the nervous system.

11_73_B&L28_Pt11_Ch73_4th.indd 1288 01/09/2022 11:55

e PART 11 | ABDOMINAL
Tests of intestinal function 1289

e contraction occurs. Also akin to the heart, this activity is

affected by a hierarchy of external control systems but mainly
connected in a lattice-type network of axons (Figure 73.1).
The ENS has a neurochemical complexity and number of
by the enteric nervous system (ENS) via the myenteric plexus. neurones (five times the number in the spinal cord) that has led
The myenteric plexus is one of the two intramural plexuses of to it being called the ‘little brain’ (Summary box 73.1). Thus,
the ENS (the other being the submucosal plexus). The former although higher control mechanisms including the autonomic
has the major role in motor functions while the latter has roles nervous system (ANS) and brain allow the intestinal motility to
in sensing, mucosal blood flow regulation and secretion. Both respond to wider environmental cues, e.g. waking, exercise, the
are composed of small groups of enteric neurones that smell and taste of food and stress, the intestine can initiate and
congregate with glial cells to form ganglia, these being sustain peristalsis without any external inputs.

Myenteric plexus
Circular muscle

Deep muscular plexus

Outer SMP
Inner SMP
Longitudinal
muscle

Submucosal
artery
Mucosa

Muscularis
mucosae

Figure 73.1 Schematic diagram of the enteric nervous system. SMP, submucosal plexus. (Reproduced by permission
from Springer Nature. Furness JB. The enteric nervous system and neurogastroenterology. Nat Rev Gastroenterol
Hepatol 2012; 9: 286–94. © 2012.)

canal, the rectum is also capable of distinguishing solid, liquid

Summary box 73.1 and gas by the ‘sampling’ reflex. Together the act of defecation
requires complex neuromuscular functions and it is no sur-
Regulation of intestinal contractile activity prise that it goes wrong with sufficient regularity to cause much
Myogenic control mechanisms human misery in the form of constipation and incontinence
● Interstitial cells of Cajal generating slow wave activity
(see Chapter 80).

Neurogenic control mechanisms

● ENS (a variety of cells in myenteric and submucosal ganglia)
TESTS OF INTESTINAL FUNCTION
● ANS (sympathetic and parasympathetic mainly via ENS Subsequent chapters address diagnostic tests specific to the
ganglia) rectum (see Chapter 79) and anus (see Chapter 80). Here the
● Central nervous system (CNS) (brain–gut interactions) focus is on tests that may be relevant to studying the motility of
the small intestine and colon. A general proviso in reading this
Chemical control mechanisms
section is that our current ability to understand the physiology
● Local paracrine (especially from mucosal enteroendocrine cells) of the intestine in humans is limited by both access and under-
● Endocrine standing. In general, we measure what can be measured and
all tests have inherent limitations to interpretation. Summary
box 73.2 provides an overview of all tests, denoting those
The rectum constitutes a final and specialised end to that have general clinical application versus those that are the
the intestine. Its role is mainly for temporary storage of fae- preserve of highly specialised units or research studies.
ces prior to defecation. This role permits both further water Summary box 73.2 makes clear that few tests are in
absorption and the ability of higher mammals to socially defe- general use. Small bowel contrast studies, e.g. barium follow-
cate (an ability shared with small rodents as well as many larger through, although available, have poor sensitivity for detecting
species). To this end, the wall of the rectum is specialised in much other than visceral distension (superseded by axial
terms of compliance and of having nerve endings that provide imaging with computed tomography [CT] or MRI) or grossly
conscious perception of filling. In concert with the upper anal retarded transit. Breath hydrogen testing assesses the presence

11_73_B&L28_Pt11_Ch73_4th.indd 1289 01/09/2022 11:55

 PART 11 | ABDOMINAL
1290 CHAPTER 73 Functional disorders of the intestine

Summary box 73.2

Tests of small intestinal function

Transit
● Small bowel barium contrast studya
● Breath hydrogen small bowel transit tests (lactulose or lactose
13
C-ureide)a
● Wireless motility capsule small bowel transit studyb

Contractile activity
● Antroduodenal manometry (ideally prolonged [24 hours]
ambulatory study)
● Dynamic magnetic resonance imaging (MRI) studies

Tests of colonic function

Transit
● Radio-opaque marker studiesa
● Isotope scintigraphy
● Wireless motility capsule whole-gut transit studyb

Contractile activity
● Colonic manometry
● Dynamic MRI studies
a
Denotes general availability.
b
Adopted by some highly funded health systems.

of carbohydrate malabsorption and is an indirect measure Figure 73.2 Radio-opaque marker transit study in a woman. All 50
markers are retained, indicating slow-transit constipation.
of transit because stagnated content allows some degree of
bacterial overgrowth and fermentation products (hydrogen,
methane and carbon dioxide). Although frequently used
in patients with unexplained chronic abdominal symptoms markers (small pieces of plastic tubing, prepackaged in gelatin
such as irritable bowel syndrome (IBS), its utility in reliably capsules) are ingested and an abdominal radiograph (which
measuring transit or detecting bacterial overgrowth is limited includes the pelvis) taken at an interval. The patient abstains
by issues of reproducibility. from laxatives for the duration of the study. In patients with sig-
The wireless motility capsule measures pH, temperature nificant numbers of retained markers (based on control data),
and pressure as it traverses the whole gastrointestinal tract; slow-transit constipation is diagnosed (Figure 73.2). Other
changes in these variables can be used to determine timings studies of colonic transit, e.g. isotope scintigraphy and direct
as it migrates from stomach to small bowel and large bowel. measurements of colonic contractile activity, are restricted to a
While it offers a number of advantages over and above current very small number of specialist centres worldwide.
techniques, especially with respect to patient tolerability, safety
and standardisation, it is not widely available owing to cost.
Prolonged measurement of small bowel contractile activ- SCOPE OF DISEASE
ity can be performed using multichannel pressure recordings A functional diagnosis is usually made when routine investigations
called manometry that show phases of the MMC. Some find- fail to find an easy explanation (e.g. a structural or biochemical
ings may be indicative of underlying small bowel neuromuscu- cause) for a combination of typical symptoms. For instance, in
lar diseases such as myopathies and neuropathies (see Chronic a patient with lower abdominal pain, constipation and bloating
impairment of intestinal motility with dilatation of the if routine investigation finds a morphological abnormality, e.g.
small intestine: intestinal pseudo-obstruction) but these sigmoid diverticulosis, then the patient will be given a diagnosis
findings have issues of specificity and the technology itself of diverticular disease. However, if all usual tests, including
is only available in a small number of centres worldwide. colonoscopy, yield no findings then the same patient might
Dynamic MRI (long sequences of image acquisition with be described as having IBS – a functional intestinal disorder.
computer analysis) is currently a research tool but may well Like much of medicine, there are however grey areas. Further,
represent the future. understanding is not aided by historic nomenclature where
The radio-opaque marker study is the mainstay of evalua- terms such as pseudo-obstruction describe different entities in
tion of colonic transit. Though variations in technique exist in the small and large intestine (Table 73.2). This chapter consid-
terms of the number of markers, interval to radiograph and ers the main disorders using this classification with a focus on
definition of slow transit, the basic premise is that a number of those most pertinent to the surgical reader.

11_73_B&L28_Pt11_Ch73_4th.indd 1290 01/09/2022 11:55

 PART 11 | ABDOMINAL
Acute adynamic neuromuscular states of the small intestine with dilatation: ileus 1291

TABLE 73.2 Scope of functional intestinal diseases.

History of onset Visceral diameter Region predominantly Nomenclature
affected
Acute Dilated Small intestine Ileus (including postoperative ileus)
Large intestine Acute colonic pseudo-obstruction
Chronic Dilated Small intestine Intestinal pseudo-obstruction
Large intestine Megacolon
Chronic Normal Intestine Constipation and irritable bowel syndrome

ACUTE ADYNAMIC fits nicely with basic ‘fight and flight’ concepts of increased
sympathetic signalling and parasympathetic withdrawal
NEUROMUSCULAR STATES OF during trauma (including surgery), has been superseded by
THE SMALL INTESTINE WITH the concept of a two-phase response. First, an immediate
DILATATION: ILEUS stress response, mediated by spinal reflexes and activation of
the hypothalamic–pituitary–adrenal axis (HPA) axis, leads
Definition to a decrease or abolition of motility. This is then followed
very rapidly by evolution of a more prolonged inflammatory
Ileus can be defined as: response in the bowel wall itself, mediated first by mast cell
a disruption of the normal propulsive ability of activation and thence recruitment and activation of macro-
the intestine due to a malfunction of contractile phages and neutrophils (Figure 73.3). These lead to inhibition
activity in the absence of mechanical obstruction. of enteric neuronal and smooth muscle function as well as
further effects on spinal reflexes.
This definition excepts certain older terms such as ‘meconium
ileus’ and ‘gallstone ileus’ (see Chapters 17 and 78) that persist
in usage, although they are technically misnomers (i.e. there is Clinical features
mechanical obstruction). The term ‘paralytic ileus’, although
descriptive for the student, is outdated and not entirely correct Symptoms include abdominal distension and vomiting akin to
since studies show that motor activity is not abolished but mechanical small bowel obstruction (see Chapter 78); however,
rather dysregulated. colicky pain is less of a feature. On examination, other than
evidence of the cause, e.g. recent surgery, the abdomen will be
distended, tympanic and have reduced or absent bowel sounds.
Causes and risk factors
The risk factors for ileus are listed in Summary box 73.3.
Postoperative ileus (POI) occurs in 10–20% of patients under- Diagnosis
going elective major abdominal surgery and is usually defined CT scanning is frequently required to exclude both mechanical
by a failure to tolerate oral intake or pass stool 72 hours after obstruction and any local driver of ileus in the peritoneum
surgery. such as inflammation or infection (Figure 73.4). In instances of
POI this is required to exclude local complications of surgery.
Summary box 73.3 Blood tests should be used to detect any drivers of ileus such as
metabolic abnormalities (especially hypokalaemia).
Risk factors for ileus
Recent surgery: POI
Management
●

● Local inflammation (peritonitis, severe acute pancreatitis)

● Systemic inflammation by any cause, e.g. sepsis, trauma Ileus may be managed by nasogastric drainage and restriction
● Electrolyte disturbance (especially hypokalaemia and of oral intake until there is evidence of improvement. Support-
hypercalcaemia) ive care such as attention to fluid and electrolyte balance
● Acute endocrine disturbance (hypothyroidism, diabetic and nutrition is also important, especially if ileus persists.
ketoacidosis) Underlying drivers of ileus, e.g. abscess or peritonitis, should
● Medications, e.g. opioids be managed on their merits. Regrettably, despite improved
● Acute CNS disease (especially high spinal transections) knowledge of the pathophysiology, specific drugs aimed at
● Intestinal ischaemia (mesenteric vascular disease) blocking inflammation or stimulating local neuromuscular
function, e.g. prokinetics, have not proved sufficiently effective
yet to be adopted for routine use.
Pathophysiology In patients with POI, if prolonged, CT scanning is the most
Classic teaching points to a reflex inhibition of intestinal effective investigation; it will demonstrate any intra-abdominal
motility caused by deranged ANS inputs. This teaching, which sepsis or mechanical obstruction and therefore guide any

11_73_B&L28_Pt11_Ch73_4th.indd 1291 01/09/2022 11:55

 PART 11 | ABDOMINAL
1292 CHAPTER 73 Functional disorders of the intestine

Inhibitory spinal
(adrenergic)
reflexes Immediate Prolonged
Acute stress decrease or local and
response abolition of distant
HPA axis motility decrease or
activation abolition of
releases motility
catecholamines

Macrophage and
neutrophil migration
and activation

Bowel Mast cell

handling activation
Inhibitory
Prolonged Increased
Afferent spinal
inflammatory mucosal
sensitisation (adrenergic)
response permeability
reflexes

Bacterial
translocation

Figure 73.3 Pathophysiology of postoperative ileus. HPA, hypothalamic–pituitary–adrenal axis.

(a) (b)

Figure 73.4 Computed tomography abdomen scout film (a) and representative coronal image (b) of a 22-year-old woman showing widespread
dilatation of the small intestine (ileus) secondary to a driving inflammatory focus (pelvic collection, arrow) (courtesy of Dr Arman Parsai, Barts
Health NHS Trust, London, UK).

requirement for laparotomy. Otherwise the decision to take a Prevention

patient back to theatre in these circumstances is always difficult. Minimally invasive surgical approaches have reduced risks of
The need for a laparotomy becomes increasingly likely the POI for many operations. The enhanced recovery programme
longer the bowel inactivity persists, particularly if it lasts for (see Chapter 74) seeks to further reduce risk of POI by
more than 7 days or if bowel activity recommences following avoidance of opioid-containing drugs and suppression of the
surgery and then stops again. inflammatory response.

11_73_B&L28_Pt11_Ch73_4th.indd 1292 01/09/2022 11:55

 PART 11 | ABDOMINAL
Acute adynamic neuromuscular states of the large intestine with dilatation: acute colonic pseudo-obstruction 1293

ACUTE ADYNAMIC The majority of patients fall into two categories: those that
have a high background risk and a small acute event (e.g. the
NEUROMUSCULAR STATES OF elderly patient with Parkinson’s disease and a urinary tract
THE LARGE INTESTINE WITH infection [UTI]) – the colon has little ‘reserve’ and a small
DILATATION: ACUTE COLONIC insult tips the balance into one of progressive abolition of
motility and tone with consequent gaseous dilatation; and
PSEUDO-OBSTRUCTION those with little background risk and a large acute event, e.g.
major surgery/trauma.
Definition
The term acute colonic pseudo-obstruction (ACPO) is
defined as:
Pathophysiology
This is poorly understood. It can however be appreciated
Acute massive dilatation of the colon with that, like ileus, risk factors reflect both ‘imbalanced’ extrinsic
obstructive symptoms but in the absence of autonomic innervation and an ‘inflammatory’ state. Evidence
mechanical obstruction. to support the former is provided by the response to anticho-
ACPO was first described by Sir William Ogilvie, who in 1948 linesterase pharmacological therapy.
recognised this syndrome in two patients with sudden onset
of abdominal pain, constipation and large bowel dilatation
(hence the eponym Ogilvie’s syndrome). It is one of the
Clinical features
three common diagnoses in patients evaluated for a clinical Symptoms include abdominal distension, absolute constipa-
presentation of large bowel obstruction (see Chapter 78), the tion and, as a later feature, vomiting akin to mechanical large
other two being colorectal cancer and volvulus (remember the bowel obstruction (see Chapter 78); however, colicky pain is
three Ts: tumour, torsion and ‘tired out’). Toxic megacolon (see less of a feature. The history is very important to establish risk
Chapter 75), although conveniently being a fourth ‘T’, should factors, some of which may be modifiable. On abdominal
be considered as a different condition entirely although the end examination, the abdomen is usually grossly distended and
point is also one of acute dilatation. tympanic. In uncomplicated cases, the abdomen should not be
tender. Tenderness and especially any evidence of peritonism
indicate that massive colonic dilatation may have led to isch-
Risk factors aemia with/without perforation – a surgical emergency. Such
In Ogilvie’s original report, the clinical picture was associated complications occur in 3–15% of patients with advanced age
with a retroperitoneal neoplasm infiltrating and destroying and increased caecal diameter, with a delay in decompression
prevertebral ganglia. This is actually a very rare cause. The increasing risk.
main risks are shown in Summary box 73.4. Diagnosis relies upon accurate clinical observation and
plain abdominal radiography showing degrees of colonic dila-
tation, mainly involving the proximal colon. CT is however the
Summary box 73.4 definitive investigation (Figure 73.5) to differentiate mechan-
ical from pseudo-obstruction, to provide a caecal diameter
Risk factors for acute colonic pseudo-obstruction and to show any evidence of complications (e.g. perforation).
● Frailty and senility A CT scan will also differentiate pseudomembranous colitis
● Neurological with toxic dilatation, which is a further differential diagnosis
● Neurodegenerative diseases in hospitalised or institutionalised patients due to Clostridium
● Stroke difficile infection.
● Spinal cord injury

● Retroperitoneum tumour infiltration

● Trauma/surgical
Management
● Major orthopaedic injuries or surgery, e.g. vertebral, pelvic The management of ACPO depends on whether complica-
and femoral tions are evident or considered imminent. In patients with
● Major gynaecological surgery
clinical and radiological features of caecal ischaemia or
● Obstetrics, including caesarean section
perforation, emergency surgery will be required and usually
● Systemic inflammation by any cause, e.g. sepsis, trauma, necessitates a subtotal colectomy and end ileostomy (with high
especially with multiorgan failure
levels of morbidity and mortality). The majority of patients
● Localised infective conditions, e.g. respiratory, urinary
can however follow a more stepwise approach, starting with
● Myocardial infarction
conservative measures (Table 73.3). Clearly the underlying
● Metabolic and electrolyte disturbances
cause where relevant, e.g. UTI, respiratory tract infection or
● Medications, e.g. opioids and any with anticholinergic actions
myocardial infarction, should also be managed in parallel. It
(e.g. psychiatric and Parkinson’s), calcium channel antagonists
is reasonable to wait before progressing from one stage to the

Sir William Heneage Ogilvie, 1887–1973, surgeon, Royal Army Medical Corps (First World War), Oxford, and Guy’s Hospital, London, UK.
James Parkinson, 1755–1824, general practitioner of Shoreditch, London, UK, published ‘An essay on the shaking palsy’ in 1817.

11_73_B&L28_Pt11_Ch73_4th.indd 1293 01/09/2022 11:55

 PART 11 | ABDOMINAL
1294 CHAPTER 73 Functional disorders of the intestine

(a) (b)

(c) (d)

Figure 73.5 Scout film (a) and representative coronal computed tomography
image (b) of a patient with acute colonic pseudo-obstruction. The entire colon
and rectum is variably distended with fluid and gas. (c) Plain abdominal radio-
graph (courtesy of James Hill) and (d) intraoperative photograph of the colon
during surgery for acute colonic pseudo-obstruction (courtesy of James Hill).

TABLE 73.3 Management of acute colonic pseudo-

obstruction.
Reversal of risk Correct fluid and electrolyte imbalances
factors next but caecal diameters of 12 cm or above warrant rapid
Stop or reduce offending drugs, e.g.
opioids, anticholinergics, calcium channel decompression to reduce perforation risk.
blockers (where possible) The decision of whether to use intravenous neostigmine is
Empty the rectum by enemas and/or flatus difficult and is usually reserved for patients in whom supportive
tube measures and colonic decompression have failed. Treatment is
Endoscopic Colonoscopy +/– flatus tube associated with profound autonomic effects (salivation, brady-
decompression cardia, bronchospasm and hypotension) as well as abdominal
Pharmacological Intravenous neostigmine unless cramps, followed often by a massive evacuation of flatus and
decompression contraindicated (risk of arrhythmia and faeces. Cardiac monitoring and a health professional compe-
bronchospasma) tent in the emergency administration of resuscitative drugs
Surgery Subtotal colectomy (usually with (especially atropine) are essential. Contraindications to the use
ileostomy) of neostigmine include renal insufficiency, recent myocardial
Venting stoma, e.g. caecostomy, in very infarct, arrhythmias and asthma.
unfit patients Surgery is associated with high morbidity and mortality
a
Requires high-dependency unit-level monitoring and support on
and should be reserved for those with impending perforation
hand for cardiorespiratory complications. when other treatments have failed or perforation has occurred.

11_73_B&L28_Pt11_Ch73_4th.indd 1294 01/09/2022 11:55

 PART 11 | ABDOMINAL
Chronic impairment of intestinal motility with dilatation of the small intestine: intestinal pseudo-obstruction 1295

Prognosis Diagnosis
ACPO is a life-threatening condition in which prompt diag- IPO presents clinically with the symptoms and signs of small
nosis and appropriate management can limit the occurrence bowel obstruction with pain, distension and vomiting. After
of complications (e.g. ischaemia or perforation). Such clinical evaluation and plain radiology, a degree of suspicion is
complications occur in about 5–10% of patients and require helpful to avoid unnecessary and potentially harmful surgery.
emergency surgery with mortality rates between 30% and Such suspicion is merited when there is no obvious cause for
60%. Recurrence is an issue in some patients with unmodi- mechanical obstruction, i.e. no known bowel disease, previous
fiable risk factors, e.g. senility and neurological disease. Such surgery or hernia, and on the length of history. Here, knowing
patients should have chronic modification of polypharmacy to the list of secondary causes becomes helpful. For instance, in
avoid offending drugs and keep the rectum empty by regular someone who is a smoker with finger clubbing, a small cell
enemas. Prokinetic medications, such as those used for chronic carcinoma of the lung may be the cause of paraneoplastic
constipation, may have a role in such patients, although none pseudo-obstruction; alternatively, the patient may have clinical
are licensed for this indication. signs of scleroderma. Axial imaging is essential to exclude
mechanical obstruction. Adjunctive blood and imaging tests
may help define a cause and these can include MRI of the
CHRONIC IMPAIRMENT OF brain and skeletal muscle biopsy for rare diagnoses such as
INTESTINAL MOTILITY WITH mitochondrial myopathies.
DILATATION OF THE SMALL Primary neuropathies and myopathies can be diagnosed
INTESTINE: INTESTINAL PSEUDO- histologically, but this requires full-thickness tissue and a vari-
ety of special stains (available only in specialist centres). Since
OBSTRUCTION laparotomy and bowel resections are best avoided, a laparo-
scopic or minilaparotomy full-thickness biopsy may be war-
Definition ranted for diagnosis (Figures 73.6 and 73.7).
Intestinal pseudo-obstruction (IPO) is defined as:
A clinical syndrome caused by severe impairment Management
of intestinal motility leading to small intestinal The main lines of management are shown in Summary box
dilatation in the absence of a mechanical cause. 73.6, noting that for most patients there is no cure. Surgery,
with the exception of placing feeding tubes or formation of
The term ‘chronic’ is sometimes added for clarity.
a venting stoma, is impotent for a condition that is a diffuse
neuromuscular disease. Further, surgery worsens the prognosis
Causes by adding the risk of adhesions into the diagnosis and, if
IPO is a rare disease. Approximately half of cases arise shortly resections or complications occur, speeding the patient towards
after birth or in infancy, caused by a number of very rare enteric intestinal failure. Small bowel (or multivisceral) transplantation
neuropathies and myopathies, including genetic and familial, is an option in selected patients.
inflammatory and degenerative forms. Other cases arise later
in life when a secondary aetiology is more common. In some
patients, a cause is not found and these are termed idiopathic.
The full list of causes is given in Summary box 73.5.

Summary box 73.5

Causes of intestinal pseudo-obstruction

Primary
● Several very rare enteric myopathies and neuropathies
● Unknown (termed ‘idiopathic’)

Secondary
● Connective tissue disease, especially scleroderma
● Radiation injury
● Amyloidosis
● Autonomic neuropathies including diabetes and paraneoplasia Figure 73.6 Intestinal pseudo-obstruction in a young male patient. A
full-thickness biopsy was undertaken from the proximal jejunum at
● Infections: Chagas’ disease (South American trypanosomiasis)
minilaparotomy.

Carlos Justiniano Ribeiro Chagas, 1879–1934, Director of the Oswaldo Cruz Institute and Professor of Tropical Medicine, University of Rio de Janeiro,
Brazil.

11_73_B&L28_Pt11_Ch73_4th.indd 1295 01/09/2022 11:55

 PART 11 | ABDOMINAL
1296 CHAPTER 73 Functional disorders of the intestine

drugs. Together these cause a vicious spiral of declining motil-

(a) ity and progression to type II/III intestinal failure with the
need for lifelong parenteral nutrition.

CHRONIC IMPAIRMENT OF
INTESTINAL MOTILITY WITH
DILATATION OF THE LARGE
INTESTINE: MEGACOLON AND
MEGARECTUM
Definition
Chronic dilatation in the absence of a mechanically obstruct-
(b) ing cause can be focused in the colon (megacolon) or rectum
(megarectum), although in practice these commonly overlap
(Figure 73.8). Megacolon may also accompany some forms of
IPO in patients found to have chronic small and large intestinal
dilatation. Toxic megacolon refers to an acute condition in
which acute inflammation leads to a loss of compliance and
rapid dilatation (it has nothing in common other than the
name).

Causes of megacolon and megarectum

Primary and secondary causes (Table 73.4) vary between
megarectum and megacolon. The most common disease to use
the term megacolon is Hirschsprung’s disease (occurring in 1
in 5000 live births) (see Chapter 17). Actually, in this instance,
it can be argued that the so-called ‘congenital megacolon’ does
in fact reflect a degree of distal obstruction from the distal
Figure 73.7 Two examples of myopathy: (a) hollow visceral myopathy contracted aganglionic segment. This leads to the absence of
(note the vacuolation of the smooth muscle, arrows); (b) extra muscle
layer in the muscularis propria (arrows).
passage of meconium at birth and is generally incompatible
with life without urgent surgery. Adult Hirschsprung’s disease
is a very rare disease and leads to a megarectum because the
affected segment is ‘ultrashort’, affecting only the transition
Summary box 73.6 zone of the anus. Histologically, this is very difficult to diagnose
with certainty and some challenge its existence at all.
Management of intestinal pseudo-obstruction
● Nutrition (enteral/parenteral)
● Analgesia (but try to avoid opioids)
Megarectum Megacolon
● Prokinetics (generally disappointing)
● Antibiotics (overgrowth)
● Immunotherapy – specific inflammatory cases (limited data)
● Psychological support, including specific patient support
groups
● Palliative care
● Surgery (very selected cases)

Prognosis
Prognosis is poor – sometimes considered the ‘motor neurone
disease’ of the gut. Infantile forms have a mortality of approx-
imately 50%. This is generally lower in adult forms depending Figure 73.8 Schematic drawing of the distribution of bowel dilatation
in megacolon and megarectum.
on cause and avoiding repeated surgery and overuse of opioid

Harald Hirschsprung, 1830–1916, physician, The Queen Louise Hospital for Children, Copenhagen, Denmark, described congenital megacolon in 1887.

11_73_B&L28_Pt11_Ch73_4th.indd 1296 01/09/2022 11:55

 PART 11 | ABDOMINAL
Chronic impairment of intestinal motility with dilatation of the large intestine: megacolon and megarectum 1297

TABLE 73.4 Causes of megacolon and megarectum.

Megacolon Primary Congenital Classic (rectosigmoid) Hirschsprung’s disease
Rare early-onset (some genetic) myopathies and neuropathies
Acquired Rare late-onset (some genetic mitochondrial) myopathies and neuropathies
Unknown (termed ‘idiopathic’)
Secondary Genetic Muscular dystrophy and other rare genetic muscle diseases
MEN type 2B with ganglioneuromatosis
Rare genetic autonomic neuropathies
Acquired CNS diseases, including senility, Parkinson’s, dementias, amyloid and spinal cord injury
Connective tissue disease, especially scleroderma
Infections: Chagas’ disease (South American trypanosomiasis)
Autonomic neuropathies secondary to diabetes and paraneoplasia
Megarectum Primary Congenital Ultrashort-segment Hirschsprung’s disease (congenital megarectum)
Inadequately resected Hirschsprung’s disease (post reconstruction)
Anorectal malformations (post reconstruction)
Secondary Congenital Severe psychobehavioural + cognitive impairment (+ genetic)
Acquired Later-onset behavioural (autistic spectrum) disorders
Sexual abuse; neglect; parental negativism

CNS, central nervous system; MEN, multiple endocrine neoplasia.

More common causes of megacolon include extreme senil-

ity and CNS neurodegenerative disease, resembling an attenu-
ated form of ACPO. Others are denoted ‘idiopathic’ to reflect
that no cause is established; this group, who are predominantly
female, phenotypically resemble a severe form of slow-transit
constipation (see Constipation). All are rare.
Patients with megarectum are usually divided into two
groups by clinicians. The first are those who have had previ-
ous surgery for Hirschsprung’s disease or anorectal malforma-
tions in whom ongoing problems are common – due perhaps
to surgical reconstruction or an as yet undetermined neuro-
muscular disease. The second, predominantly male, group
are sometimes described as ‘idiopathic’; however, nearly all, if
assessed carefully, will have some form of psychobehavioural
disorder. The pathogenesis is considered to be stool withhold-
ing in infancy or childhood, leading to chronic distension and
loss of compliance.

Diagnosis and management

Megarectum may present with a mass the size of a full-term
baby (Figure 73.9) but diagnosis is mainly radiological. The Figure 73.9 Plain abdominal radiograph of a teenage male with
megarectum.
mainstay of management of both (in brief) requires getting
the rectum empty. In some patients with megarectum this may
require manual disimpaction under anaesthesia. Thereafter, for megarectum. A first step may be an anterograde colonic
high doses of regular osmotic and simulant laxatives orally as enema (ACE) procedure (see Constipation). If this fails, defin-
well as regular enemas (or high-volume transanal irrigation itive surgery includes pull-through procedures, low anterior
(TAI); see Constipation) are required to keep it empty. resection, restorative proctocolectomy and rectum-reducing
Prokinetics may also have a role. Compliance with medication procedures, e.g. vertical reduction rectoplasty. All should be
is often an issue in young patients with psychobehavioural undertaken with covering loop ileostomy and many advocate
problems. Surgery has an important role in patients who fail performing an ileostomy for 6 months to 1 year prior to surgery.
medical management. Colectomy or subtotal colectomy is This allows the rectum to shrink and reduce in vascularity,
generally required for megacolon. A variety of options exist making eventual surgery safer; some patients may also simply

11_73_B&L28_Pt11_Ch73_4th.indd 1297 01/09/2022 11:55

 PART 11 | ABDOMINAL
1298 CHAPTER 73 Functional disorders of the intestine

choose to live with the ileostomy rather than risk pelvic surgery. characterized by infrequent stools, difficult stool passage or both
The hazard of operating on a rectum that occupies the whole for at least 3 months’ – cover most symptoms and introduce
pelvis with serosal veins that sometimes resemble the iliac veins a time criterion to exclude patients with transient symptoms
cannot be underestimated and surgery should be performed in (sometimes called ‘simple constipation’). Stricter definitions of
specialist centres. ‘chronic constipation’ include a measure of resistance to treat-
ment – ‘unsatisfactory defecation characterized by infrequent
stools, difficult stool passage or both for at least 6 months where
CHRONIC IMPAIRMENT OF this has proven unresponsive to lifestyle alterations and basic
INTESTINAL MOTILITY WITHOUT laxative therapy’.
DILATATION Epidemiology
Constipation and IBS are very common conditions and collec- Self-reported constipation is very common, with a worldwide
tively represent about a third of patients presenting to the aver- prevalence of about 10% (making it one of the commonest
age colorectal clinic in the Western world. They are presented ailments in humans). Fortunately, patients with chronic
here as separate entities to reflect the general approach of most constipation (based on 6 months of symptoms and failure of
physicians; however, the reader should be aware that there is at least two laxatives) are much less common (approximately
very considerable overlap, especially between constipation and 0.5%). Most studies report a higher prevalence of self-reported
the constipation-predominant form of IBS (C-IBS). Patients constipation in women than in men with a ratio of 2:1. The
can fulfil the criteria for both diagnoses concurrently or move ratio is much higher for chronic constipation at approximately
between diagnoses over time. 9:1 female to male.

Constipation Risk factors

The vast majority of patients with chronic constipation lack a
Definitions single unifying cause for their problems. The main associated
‘Constipation’ is not a disease but rather a term often used by medical conditions and diseases within the gastrointestinal
patients to describe dissatisfaction with their bowel function tract itself are listed in Table 73.5.
or their ability to defecate. As such it means different things
to different patients (and different doctors) and can describe
symptoms that directly relate to defecation, e.g. straining, or
Diagnosis
those considered consequent in the abdomen, e.g. pain and Clinical history
bloating. More formal definitions such as that of the American A thorough history will determine whether constipation
College of Gastroenterologists – ‘unsatisfactory defecation, represents a new complaint, i.e. one indicative of a change

TABLE 73.5 Risk factors for constipation.

Gastrointestinal causes
Mechanical obstruction Benign and malignant strictures
Functional obstruction Pelvic organ prolapse syndromes (dynamic obstruction at the level of the anorectum)
Megarectum
Anal pain, e.g. chronic fissure
Medical causes
Metabolic disorders Hypercalcaemia, uraemia, hypokalaemia, hypomagnesaemia
Endocrine disorders Hypothyroidism, diabetes, pregnancy
Neurological disorders Degenerative CNS diseases, e.g. multiple sclerosis, Parkinson’s, cerebrovascular disease,
spinal or pelvic nerve lesions, autonomic neuropathies, cognitive impairment
Drugs Opioids
Anticholinergics
Calcium channel blockers
Psychological Severe endogenous depression
Eating disorders
Cognitive behavioural disorders
Other Connective tissue diseases
Joint hypermobility
Causes of immobility, e.g. degenerative joint disease
CNS, central nervous system.

11_73_B&L28_Pt11_Ch73_4th.indd 1298 01/09/2022 11:55

 PART 11 | ABDOMINAL
Chronic impairment of intestinal motility without dilatation 1299

in bowel habit. The patient should be asked specifically about

the frequency and consistency of bowel movements and the Summary box 73.7
progress of such changes over time (as well as other alarm
Symptoms to directly question in patients with
symptoms such as rectal bleeding and weight loss). With
constipation
additional information regarding family history, previous colon
cancer screening and other gastrointestinal investigations, Abdominal symptoms
an informed decision can be made whether intraluminal ● Abdominal pain
investigation of the colon is required. Other organic causes ● Bloating
of constipation may be deduced by appropriate history taking
and biochemical investigation. With the exclusion of treatable Defecatory symptoms
secondary causes, if the history is short and multiple previous ● Frequency of spontaneous or assisted bowel opening
therapies have not already been tried, the patient may be first ● Painful defecation
considered to have ‘simple’ constipation that can be managed ● Stool consistency (can use Bristol stool scale)
with reassurance and lifestyle advice (fibre, fluids and exercise) ● Digitation (vaginal or anal)
with/without simple laxative therapy. ● Straining
In patients with chronic symptoms, after exclusion of a sec- ● Incomplete/unsuccessful evacuation
ondary cause, the focus should shift to the investigation and ● Leakage/incontinence
management of chronic constipation. Many patients may ● Prolapse
attribute the start of symptoms to a major life event. Common
Other pelvic symptoms
among these are hysterectomy and childbirth, other abdomi-
● Vaginal bulging or prolapse
nal surgeries or trauma. Constipation can also be associated
● Urinary incontinence
with previous abuse and it may sometimes be necessary to tact-
fully seek a history of physical or sexual abuse. Other patients
will have no such triggers, having had symptoms from child-
hood and on occasion from infancy. Such patients are impaction and overflow. Some degree of faecal incontinence
overwhelmingly female (>95%) and on investigation are often and chronic constipation coexists in 40% of patients; marked
found to have generalised slow-transit constipation as opposed soiling of the underwear is especially associated with the rarer
to other pathophysiological findings (this group, who represent diagnosis of megarectum. Scarring, e.g. from episiotomy, sen-
5–10% of patients with chronic constipation, are variably tinel pile formation secondary to an underlying anal fissure,
referred to in the literature are ‘idiopathic slow-transit consti- external haemorrhoids or prolapse, may also be present. The
pation’ or ‘colonic inertia’). It is helpful to systematically docu- degree of perineal descent on straining, indicative of pelvic
ment the main symptoms that in the patient’s mind constitute floor weakness, should also be determined visually (>3 cm is
a problem since this has some bearing on treatment decisions usually considered abnormal). A digital rectal examination
and subsequent monitoring of effectiveness. Several questions will diagnose impaction, gain a rough measure of anal tone
form detailed scoring systems to systematically facilitate this in at rest and on squeeze and ascertain obvious sphincter defects.
a research context. However, in routine practice it is sufficient An effort should be made to look for any anterior defect in
to list in the patient’s record the presence or absence of several the rectovaginal septum leading to a rectocele. Anoscopy and
common symptoms (Summary box 73.7). The presence of proctoscopy should be performed if there is any history of
prolapse symptoms reflects the overlap between diagnoses in rectal bleeding and may indicate fissure or internal piles. A
patients with pelvic floor disorders (see Chapter 80). The urogynaecological examination is desirable in all patients with
remaining history should document prescribed and self- suspected pelvic multi-organ prolapse.
administered laxatives (and therapeutic benefit thereof) and
also gain an impression of the quality of diet in respect of fibre Investigations
and fluid intake. While findings from history or physical examination may
indicate a secondary cause of constipation, making further
Clinical examination investigation mandatory, it is also typical practice in patients
Poor nutritional status should prompt a search for a secondary with chronic constipation to exclude certain secondary causes
cause, including occult carcinoma, more widespread intestinal by investigation even though the diagnostic utility of such
motility disorders such as IPO (see Chronic impairment of investigations is acknowledged to be low (the commonest
intestinal motility with dilatation of the small intestine: undiagnosed systemic disease is hypothyroidism). Thus,
intestinal pseudo-obstruction) and eating disorders. An serum electrolyte, creatinine, calcium, glucose, haemoglobin
abdominal examination should be conducted to look for scars, levels and thyroid function tests are usually performed. The
any significant abdominal distension, tenderness or masses. approach taken to structural investigation of the colon when
Bloating is a common and expected finding with chronic patients have no suspected intraluminal pathology varies on
constipation, but significant distension, tenderness or masses the basis of available resource and may include colonoscopy.
should prompt a full investigation. In patients with chronic constipation in whom basic
All patients presenting with constipation should undergo laxatives have failed, further specialist investigative tests
a rectal examination. The perineum and anus should be may be warranted. Colonic transit can be investigated by a
examined for evidence of faecal incontinence that may indicate radio-opaque marker study (Figure 73.2). In addition, rectal

11_73_B&L28_Pt11_Ch73_4th.indd 1299 01/09/2022 11:55

 PART 11 | ABDOMINAL
1300 CHAPTER 73 Functional disorders of the intestine

sensory testing and evacuation proctography will determine Patients with chronic constipation have generally had
if the patient has a functional or dynamic structural cause of symptoms for many years and will have tried a number of rem-
evacuation disorder. Problems such as dyssynergic defecation edies and prescribed laxatives. They will also usually have tried
(functional) and intussusception/rectocele (structural) to address lifestyle modifications. Before resorting to specialist
may occur in isolation or coexist with transit disturbances tests, it is possible to try and rationalise laxative therapy and
(Figure 73.10). provide a programme of nurse-led behavioural interventions.
In regard to laxatives, current advice is to stop current laxatives
Management (unless these are working well) and then titrate an oral osmotic
The treatment of chronic constipation follows a stepwise laxative, e.g. polyethylene glycol (PEG), until the stool form is
progression from lifestyle changes through potentially to major soft or liquid. If this is insufficient then a stimulant laxative
surgery in a small minority of patients. Table 73.6 lists the such as bisacodyl may be added. If symptoms of obstructed
main available approaches, noting where some apply only to defecation predominate then rectal laxatives in the form of
certain diagnoses derived from the results of specialist tests of suppositories or enemas may be tried with or without contin-
colonic and anorectal function. Figure 73.11 provides a basic uation of oral laxatives. The failure of such drugs should then
algorithm to accompany Table 73.6. prompt a trial of one of the newer prokinetic or secretagogue

15% normal

5% STC

45% mixed STC and DDs

40% DDs

Structural Functional

Figure 73.10 Schematic overview of pathophysiology of chronic constipation. DD, defecation disorder; STC, slow-transit constipation.

TABLE 73.6 Treatment options in patients with chronic constipation.

Lifestyle Increase fluid intake
Dietary modification, e.g. increased fibre
Increase exercise
Reduce body mass (pelvic floor prolapse syndromes)
Drugs Oral laxatives (favoured for slow transit)
Rectal laxatives (favoured for rectal evacuation disorders)
Prokinetics, e.g. prucalopride
Secretagogues, e.g. linaclotide
Behavioural therapies Habit training
Habit training with direct visual biofeedback (favoured for dyssynergic defecation)
Pelvic floor muscle training (favoured for pelvic floor prolapse syndromes)
Transanal irrigation High- or low-volume systems available
Surgery See Summary box 73.8

11_73_B&L28_Pt11_Ch73_4th.indd 1300 01/09/2022 11:55

 PART 11 | ABDOMINAL
Chronic impairment of intestinal motility without dilatation 1301

Chronic constipation refractory to lifestyle

modification and basic pharmacological treatment1

Review lifestyle modification (fibre, fluid, exercise)

Rational laxative use (PEG, stimulant laxatives)2 Response
Prokinetics if naive (prucalopride 1–2 mg daily or
linaclotide 290 g or other secretagogues)

No response

Obvious clinical evidence of

Response Habit training
overt pelvic organ prolapse3
No response

Abnormal Anorectal function Normal Colonic/whole gut transit

Dyssynergic Other evacuation
testing (balloon +/– defecography
defecation disorder
expulsion test, +/– adjunctive tests, e.g. urodynamics
No defecography, rectal
response sensory testing and Abnormal Abnormal
Direct visual
anorectal manometry)
biofeedback
MDT meeting to Re-evaluation of
discuss surgical symptom–investigation
options correlation to focus on further
No pharmacology or other untried
Transanal irrigation response interventions
Response
initiated high volume4
Other surgical targets Posterior compartment prolapse
and procedures syndrome with high
grade intussusception +/– retrocele

Consider laparoscopic ventral

rectopexy5 or alternative,
e.g. STARR +/– adjuncts6

Figure 73.11 Algorithm of chronic constipation management. MDT, multidisciplinary team; PEG, polyethylene glycol. 1, alarm features excluded
and secondary causes treated appropriately; 2, in constipation-predominant irritable bowel syndrome, consider antispasmodics or neuromod-
ulators in case constipation improves but abdominal pain persists and is dominant symptom; 3, examples of overt prolapse include anterior
(stage 3 cystocele), middle (stage 3 rectocele, uterovaginal prolapse) and posterior compartments (grade IV/V intussusception); 4, unless patient
preference for low volume or specific contraindications to high volume; 5, may reduce specific symptoms but not have overall effect on quality
of life; 6, common adjuncts include sacrocolpopexy, hysterectomy, transvaginal tape and cystocele repair.

drugs. These drugs are successful in a proportion of patients

but do have some unwanted side effects (that the patient should
be warned about). All drugs should be tried daily for a mini-
mum of 4 weeks before concluding that they are ineffective and
the reactionary use of laxatives, i.e. in response to being con-
stipated, rather than their preventative use, should be strongly
discouraged.
The most common form of behavioural intervention is
often described by the term ‘habit training’. This involves
optimising dietary patterns to maximise gastrocolic response
and the morning clustering of colonic high-amplitude prop-
agated contractions that propel contents towards the rectum
for subsequent evacuation. Dietary advice to optimise intake
of liquid and fibre is given as well as advice about frequency
and length of toilet visits and posture (Figure 73.12). Patients
are also instructed on basic gut anatomy and function and
gain an appreciation of how psychological and social stresses
may influence gut functioning. Simple pelvic floor and balloon
Figure 73.12 Correct posture for defecation.
expulsion exercises are often included. Such appointments also

11_73_B&L28_Pt11_Ch73_4th.indd 1301 01/09/2022 11:55

 PART 11 | ABDOMINAL
1302 CHAPTER 73 Functional disorders of the intestine

offer an opportunity to further rationalise and monitor laxative long-term functional outcomes. On this basis, the following are
therapy. essential requirements before surgery is undertaken:
If this fails, there may be recourse to the specialist tests to
● pathophysiological findings from specialist tests concur
assess colonic transit and also anorectal function (see Chapter
with the symptomatology and findings on clinical exam-
80). Armed with the results of these tests, the patient may have
ination;
a more targeted approach relative to their observed pathophys-
● conservative (non-surgical) treatment options have been
iology. One example of this approach is for patients with a con-
tried;
dition termed ‘dyssynergic defecation’, where there is a failure
● the patient’s case has been reviewed at a multidisciplinary
to relax, or even paradoxical contraction of the pelvic floor
team (MDT) meeting and surgery recommended;
muscles (especially puborectalis) during defecatory efforts. In
● the patient has been consented in the very clear knowledge
such patients, instrument-based biofeedback learning tech-
of the range of possible outcomes;
niques provide direct visual computer-based biofeedback of
● surgery is undertaken in a centre with expertise in manag-
pelvic floor activity. The aim is to retrain the patient to appro-
ing functional conditions.
priately contract abdominal and relax pelvic floor muscles
during defecation with the patient receiving feedback of anal The range of procedures for rectal prolapse are covered in
and pelvic floor muscle activity as recorded by surface electro- detail in Chapter 79. Those primarily targeting the intestine
myographic anal pressure sensors or digital examination by are covered briefly here.
the therapist.
Transanal irrigation (TAI) may be used for any patient with Colectomy
an evacuation disorder when habit training and/or biofeedback Colectomy is a radical and clearly irreversible final solution
have failed. A number of devices are available that administer a for patients with refractory slow-transit constipation. Its use
low (approximately 50–100 mL) or high volume (approximately should be very highly selective, not least because it is not
500 mL) of irrigant fluid into the rectum. The patient sits on actually a solution for many patients even when the surgery
the toilet to evacuate the fluid and faecal material. itself passes without complication. Removal of the whole
Some patients with chronic refractory symptoms may seek colon with ileorectal anastomosis (as performed for inflam-
a surgical solution to their problem. Surgical procedures can matory bowel disease) is best studied; subtotal resections
be broadly divided into those addressing dynamic structural with ileosigmoid or caecorectal anastomosis are alternatives.
problems of the pelvic floor (prolapse procedures), those that Outcomes vary greatly and are often compromised by
seek specifically to address slow-transit constipation and those early problems of ileus and a higher than expected rate of
that may have a role for both (Summary box 73.8). adhesional small bowel obstruction. Later problems include
ongoing constipation and obstructive symptoms, diarrhoea
and urgency, abdominal pain and bloating. Embarking on
this procedure requires very careful MDT review, documen-
Summary box 73.8
tation of generalised slow-transit constipation and exclusion
Surgical options in patients with chronic constipation of a long list of relative contraindications.

Prolapse procedures for dynamic structural causes of ob- Stoma

structed defecation A stoma may be used as a definitive procedure, as a guide to
● Hitching procedures, e.g. rectopexy further treatment or as salvage from a failed or complicated
● Rectal wall excisional procedures, e.g. stapled transanal rectal prior surgical intervention. There are few published data to
resection (STARR) support evidence-based use; however, an ileostomy may be
● Rectovaginal reinforcement procedures, e.g. posterior vaginal employed as a guide to colectomy with subsequent resection
repair, intra-anal Delorme’s procedure avoided if ileostomy output is unsatisfactorily high or symp-
Procedures for slow-transit constipation toms such as pain and bloating are untouched by diversion. As
● Colectomy and ileorectal anastomosis a definitive procedure, there is little evidence in adults to guide
● Other variants of subtotal colectomy the choice of ileostomy or colostomy; however, it is generally
considered that slow-transit constipation is unsatisfactorily
Procedures for refractory chronic constipation in general treated by colostomy.
● Stoma: ileostomy or colostomy
● ACE procedures Anterograde colonic enema procedures
● Neuromodulation The formation of a conduit to introduce irrigant into the colon
is best established in children and in patients with neurological
disease. A variety of methods have been proposed to access
All surgery should be undertaken in the knowledge that the caecum either directly, e.g. with a Chait tube caecostomy,
none of the above-listed operations is perfect. All represent or indirectly via the appendix (appendicostomy). The latter is
a trade-off between benefits and short-term harms and poor almost certainly preferable although only possible when the

Edmond Delorme, 1847–1929, French military surgeon and Professor of Surgery, Val-de-Grace Military Hospital, Paris, France.
Peter Graham Chait, contemporary, radiologist, Toronto, Canada.

11_73_B&L28_Pt11_Ch73_4th.indd 1302 01/09/2022 11:55

 PART 11 | ABDOMINAL
Chronic impairment of intestinal motility without dilatation 1303

native appendix is still present and the patient is not obese.

The appendix can be reversed (Malone anterograde continent
enema technique) or used in its native orientation (much

Constipation
simpler). Outcomes in adults with chronic constipation are
variable but generally this is a good option in patients consid-
ering colectomy or stoma as the only alternative. Abdominal pain

Neuromodulation IBS-C
The attraction of being able to treat chronic constipation
with a minimally invasive and safe approach such as sacral
neuromodulation is supported by research data showing that IBS-M
stimulation improves motility and also some observational
Diarrhoea
data. It is now clear from randomised trials that it has no
role for slow-transit constipation but it may yet have a place
in modifying anorectal function in some patients with severe IBS-U IBS-D
functional syndromes leading to obstructed defecation (as it
does for the bladder).

Irritable bowel syndrome

Figure 73.13 Irritable bowel syndrome (IBS) subtypes according to
Surgery has no role in treating IBS. Nevertheless, patients with the Rome criteria. All patients have abdominal pain but subtypes vary
chronic abdominal pain and a change in their bowels are very according to bowel form at presentation such as to meet criteria for
common in surgical clinics and all surgeons should at least have IBS with constipation (IBS-C), IBS with diarrhoea (IBS-D), mixed-type
a passing familiarity with a disorder that is a source of misery IBS (IBS-M) and unsubtyped (IBS-U).
to millions of people worldwide.

Definitions
IBS is a functional bowel disorder characterised by abdominal implication of the model is that IBS has much overlap with
pain or discomfort, stool irregularities and bloating. The term other medical conditions that have similar or nearly identical
replaced nineteenth century descriptions such as ‘irritable’ or biopsychosocial determinants (Summary box 73.9).
‘spastic’ colon in 1979 to reflect the fact that the colon is not
the only site of the problem. Diagnostic criteria have evolved
to the now used Rome IV Foundation definition:
Summary box 73.9
Recurrent abdominal pain on average at least 1
day/week in the last 3 months, associated with two IBS-associated comorbidities
or more of three criteria: (1). related to defecation;
General
(2). associated with a change in the frequency of
stool and (3). associated with a change in the form ● Fibromyalgia syndrome
(appearance) of stool. These criteria should be ● Chronic fatigue syndrome
fulfilled for the last 3 months with symptom onset ● Chronic pelvic pain, chronic prostatitis and bladder pain
syndromes
at least 6 months prior to diagnosis.
● Chronic back pain
The change in frequency and form of the stool dictates ● Migraines
subdivisions of IBS into constipation-predominant (IBS-C), ● Depression
diarrhoea-predominant (IBS-D) and mixed (IBS-M) subclasses ● Anxiety
(Figure 73.13). ● Somatisation
● Sleep disturbance
Aetiology and risk factors
Gastrointestinal disordersa
Regardless of exact definition, the cardinal symptoms of
chronic abdominal pain and ‘deranged digestion’ favour a ● Eating disorders
‘biopsychosocial model’ (Figure 73.14) that encompasses the ● Dyssynergic defecation
role of stressful life events and brain–gut interactions in symp- ● Levator ani syndrome and proctalgia fugax
tom generation. Several common life events are particularly ● Food intolerances
well documented; these include postinfective IBS, where a a
The overlap of IBS with other Rome-defined functional gastro-
seemingly discrete attack of gastroenteritis (viral, bacterial or intestinal disorders should also be noted, including functional
otherwise) is followed by chronic ongoing symptoms; physical dyspepsia and functional constipation.
and/or sexual abuse (and neglect); surgery; and trauma. One

Patrick Malone, contemporary, surgeon, Southampton, UK.

11_73_B&L28_Pt11_Ch73_4th.indd 1303 01/09/2022 11:55

 PART 11 | ABDOMINAL
1304 CHAPTER 73 Functional disorders of the intestine

Psychosocial factors
Early life • Life stress
• enetics • Psychological state and trait
• pigenetics • oping
• Social support

IBS
Brain-gut axis
• Symptoms
• eha iour

Physiology
Local environmental
• Motility
factors
• Sensation
• Diet
• Permea ility
• Acute infections
• Inflammation
• Surgery
• Altered flora

Figure 73.14 Biopsychosocial model of irritable bowel syndrome (IBS). The scheme is a conceptualisation of the pathogenesis and clinical
expression of IBS showing interrelationships between various risk factors and changes in physiology.

Diagnosis Clinical examination

Clinical history Physical examination helps to reassure patients and also
to exclude another organic cause for symptoms. However,
Besides symptoms required by the diagnostic criteria, other
abdominal examination rarely discloses a specific diagnosis
symptoms may be present. Common associated symptoms
(abdominal tenderness is often present but non-specific); the
include bloating (very common), straining at defecation,
absence of objective findings supports a diagnosis of IBS. A
excessive flatulence and postprandial indigestion. A history of
digital rectal examination may identify patients with dyssyner-
precipitating events (as per the model) and of comorbidities
gic defecation and other causes of constipation.
(Summary box 73.7) should also be sought to support the
diagnosis. The patient may also have a history of multiple
operations, which on reflection may have been directed to Investigations
chronic abdominal pain, e.g. appendicectomy, cholecystectomy There are no valid laboratory biomarkers of IBS. Routine
or hysterectomy. blood panels, including inflammatory markers, are generally

TABLE 73.7 Treatments for irritable bowel syndrome.

Nutrition Increased (constipation) or reduced (bloating) fibre
Gluten-free diet (especially if equivocal diagnosis of coeliac disease)
FODMAP diet
Probiotics
Consider dietary supplements, prebiotics
Drugs Antispasmodics: peppermint oil, hyoscine butylbromide (Buscopan)
Laxatives, e.g. stool softeners, osmotic and stimulant (avoid lactulose because of bloating and pain)
Antidiarrhoeals: loperamide (μ-opioid receptor agonist); 5-HT3 receptor antagonists, such as alosetron, ondansetron
Motility accelerants, e.g. linaclotide (guanylyl cyclase C agonist), prucalopride (5-HT4 receptor agonist)
Low-dose antidepressants: tricyclics and selective serotonin reuptake inhibitors
Manipulation of the microbiota by non-absorbable antibiotics, e.g. rifaximin
Neuromodulators, e.g. gabapentin and pregabalin
Psychotherapy Cognitive–behavioural therapy
Gut-directed hypnosis
Guided self-help interventions
5-HT, 5-hydroxytryptamine; FODMAP, fermentable oligosaccharides, disaccharides, monosaccharides and polyols.

11_73_B&L28_Pt11_Ch73_4th.indd 1304 01/09/2022 11:55

 PART 11 | ABDOMINAL
Further reading 1305

performed to reassure that there are no indicators of organic be tried over the counter by patients, e.g. prebiotics, although
disease, e.g. cancer, inflammatory bowel disease or diverticu- there is no trial evidence.
lar disease. Specific tests include serological tests for coeliac A key point in the management of IBS rests with nota-
disease, faecal calprotectin and stool microbiology in cases of ble exclusions from Table 73.7. Thus the table makes no ref-
diarrhoea predominance. Invasive procedures are generally erence to standard analgesics and surgery. Opioid analgesia
not warranted unless alarm features are present that mandate should be avoided in IBS because the further disturbance to
endoscopy. That noted, it is quite common practice to perform motility worsens the prognosis and in extreme use can lead to
colonoscopy, not least to reassure the patient that their chronic narcotic bowel syndrome (an opioid-induced state of hyper-
symptoms do not have an organic basis. In patients with IBS-D, algesia whose main driver is the activation of glial cells). Sur-
colonoscopy with random biopsies is warranted to exclude gery has a well-documented association with symptom onset
microscopic colitis. Other tests that may be relevant include of IBS (cholecystectomy, appendicectomy, hysterectomy and
(75Se-homocholic acid taurine [75SeHCAT] test or serum back surgery); further surgery leads not only to greater poten-
serum 7-α-hydroxy-4-cholesten-3-one (C4) levels) for bile salt tial visceral sensitisation (via injury) but also serves to confuse
malabsorption, breath testing for carbohydrate malabsorption, subsequent diagnosis, e.g. adhesional versus functional cause
gastrointestinal physiology for constipation and upper gastro- for symptoms. There is also a body of evidence to suggest that
intestinal endoscopy for associated dyspeptic symptoms. surgery perpetuates a search for an ‘organic’ diagnosis that
hinders patient acceptance and adaption to their chronic prob-
Management lem. For the surgeon, the key is to exclude any surgical cause
Only a fraction of patients with IBS-like symptoms seek medi- of pain and then prevent further harm by avoiding surgery.
cal care and most will initially consult primary care physicians
for their symptoms. The factors that drive this consultation are
symptom severity, especially pain, and concerns that symptoms SUMMARY
might indicate an underlying severe disease, e.g. cancer. There- Functional intestinal disorders range from the very common
fore, in many cases, the doctor’s role is to exclude diseases that – constipation and IBS – through to the very rare, e.g. various
can mimic IBS symptoms by relevant investigations such as genetic and familial neuropathies and myopathies causing IPO.
endoscopy. The surgeon will almost certainly encounter acute problems
When a positive diagnosis of IBS has been made, man- such as POI and ACPO. This chapter provides an overview that
agement requires an integrated approach, including educa- can be supplemented by the recommended further reading.
tion, reassurance, dietary alterations, pharmacotherapy and
behavioural or psychological interventions/support. The
initial treatment strategy should be based on predominant FURTHER READING
symptoms and includes antispasmodics for abdominal pain, Bharucha A, Knowles CH. Chronic constipation. In: Sagar PM, Hill
antidiarrhoeals for IBS-D and laxatives for IBS-C, whereas AG, Knowles CH et al. (eds). Keighley & Williams’ surgery of the
nutritional interventions and psychotherapy can be used in all anus, rectum and colon, 4th edn. Boca Raton, FL: Taylor & Francis,
subtypes. Table 73.7 provides a list of potential management 2019: 305–46.
Enck P, Aziz Q , Barbara G et al. Irritable bowel syndrome. Nat Rev Dis
strategies for IBS. This list is not all encompassing, nor does it
Primers 2016; 2: 16014.
provide weighting to one treatment over another in terms of van Bree SHW, Nemethova A, Cailotto C et al. New therapeutic strat-
effectiveness in clinical trials. Some treatments are popular, e.g. egies for postoperative ileus. Nat Rev Gastroenterol Hepatol 2012; 9:
low-dose antidepressants but such use is off-label; others may 675–83.

11_73_B&L28_Pt11_Ch73_4th.indd 1305 01/09/2022 11:55