Case report Open Access

Generalized tetanus in a 4-year old boy presenting with

dysphagia and trismus: a case report
Petrus Rudolf de Jong
1
, Thea de Heer-Groen
2
, Cornelis Hendrik Schrder
2
and Nicolaas Johannes Georgius Jansen
1
*
Address:
1
Department of Pediatric Intensive Care, Wilhelmina Childrens Hospital, University Medical Center Utrecht,
Home mailbox KG.01.319.0, PO Box 85090, 3508 AB Utrecht, The Netherlands and
2
Department of Pediatrics, Lukas Hospital Center,
Apeldoorn, The Netherlands
Email: PRJ - [email protected]; THG - [email protected]; CHS - [email protected]; NJGJ* - [email protected]
* Corresponding author
Published: 29 April 2009 Received: 18 July 2008
Accepted: 19 March 2009
Cases Journal 2009, 2:7003 doi: 10.1186/1757-1626-2-7003
This article is available from: http://casesjournal.com/casesjournal/article/view/2/4/7003
2009 de Jong et al; licensee Cases Network Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),
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Abstract
Introduction: The low incidence of tetanus in developed countries has resulted in a decreased
vigilance of this disease. This raises concern, as the prodromal stadium of a generalized tetanus
infection may lack the characteristic paroxysmal muscle spasms. Tetanus can rapidly progress into
life-threatening muscle spasms accompanied by respiratory insufficiency and/or autonomic
dysfunction. This emphasizes the need for early diagnosis and treatment.
Case presentation: A 4-year-old Caucasian boy presented with a one-week history of general
malaise, mild fever, indolence and anorexia. He subsequently developed dysphagia, sialorrhoea,
difficulties opening the mouth and eventually dehydration. Due to parental concerns about the boys
refusal of fluids, a pediatrician was consulted. At that time of presentation he showed signs of trismus
and muscle rigidity. Together with the lack of immunization and a toe nail infection, this lead to the
suspicion of a generalized tetanus infection. After sedation, endotracheal intubation and ventilation,
passive immunization and initiation of antimicrobial treatment, he was immediately transferred to a
pediatric intensive care unit (PICU) for further treatment. The frequency and severity of paroxysmal
muscle spasms increased progressively during his PICU stay, despite high doses of sedatives. Not
before two weeks after admittance, extubation and careful weaning off sedatives was achieved.
Conclusion: Tetanus infection remains a rare but potentially lethal disease in developed countries.
As the full scope of classical symptoms may be absent at first presentation, tetanus should always be
considered in non-immunized patients with an acute onset of dysphagia and trismus.
Introduction
Tetanus is a neurotoxin-mediated disease characterized by
a progressive spastic paralysis of multiple muscle groups.
The neurotoxin (tetanospasmin) disrupts neurotransmit-
ter release in inhibitory neurons, leading to peripheral
muscle rigidity and spasms. Tetanospasmin is produced by
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the obligate anaerobic species Clostridium tetani, of which
its spores are ubiquitously distributed in our environment.
This causes the inevitable increased risk of tetanus
infection after wound contamination. Although tetanus
has become rare in developed countries due to the
successful implementation of primary immunization
series, infants that are not immunized on religious or
philosophical grounds are still at risk [1].
Muscle rigidity and spasms constitute the typical clinical
hallmarks of generalized tetanus e.g. trismus (lockjaw) and
opisthotonus [2]. Importantly, the onset of a generalized
tetanus infection is not always associated with the clinical
signs described above. Tetanus presenting with solely
oropharyngeal symptoms can be misdiagnosed as a more
common oropharyngeal infection (i.e. peritonsillar
abscess). However, unrecognized tetanus may rapidly
progress into a critical condition with severe muscle
spasms, autonomic dysfunction and/or respiratory failure
[3]. Patients with a clinical suspicion of tetanus must
therefore receive local wound care, tetanus immuno-
globulins plus antimicrobials and be transferred to a
specialized intensive care unit without any delay. We
report a child who presented with general malaise,
anorexia, dysphagia, trismus and dehydration, which
rapidly developed into severe generalized tetanus.
Case presentation
A 4-year-old Caucasian boy presented to a regional
hospital with a one-week history of general malaise,
indolence, mild fever and progressive anorexia. Three days
prior to presentation at the hospital he had started to
refuse all food and fluids, accompanied by a progressive
dysphagia, sore throat and sialorrhoea. An otorhinolar-
yngologist had been consulted two days before presenta-
tion, who had considered a peritonsillar abscess. However,
his examination at that time did not provide any clues for
an oropharyngeal infection. Subsequently, the boy
demonstrated increased difficulties with opening his
mouth and experienced a progressive dehydration. Due
to the parents concern about the refusal of fluids and
dehydration, a pediatrician was consulted.
The history revealed that the boy had recently injured his
left hallux. This had resulted in a small local hematoma
and loose toenail. There were no recorded insect or animal
bites. Based on religious grounds, the boy had not received
immunization according to the Dutch National Immuni-
zation Program. The other children, including his identical
twin, were healthy.
On physical examination in the regional hospital we saw
an afebrile, irritable and anxious boy gently playing at the
table, with trismus and mild dehydration. After being
asked to walk, he showed muscle spasms of the back and
thighs evidently worsening during examination. There was
no cervical lymphadenopathy and the ear and nose
examination was unremarkable. Inspection of the oro-
pharynx was not possible due to trismus. Tendon reflexes
were normal, there was no meningeal irritation. The loose
toenail did not show clear signs of inflammation. The
heart rate was slightly increased, the blood pressure was
normal and further clinical examination was unremark-
able. The initial differential diagnosis included orophar-
yngeal infections (e.g. tonsillitis, peritonsillar abscess),
botulism, rabies, strychnine poisoning, hypocalcemia,
psychogenic causes and tetanus. Based on normal com-
plete blood cell count and chemistry profiles, immuniza-
tion status and the presence of generalized muscle spasms
and a possible portal of entry, the working diagnosis
generalized tetanus was established.
Treatment was initiated immediately with the administra-
tion of anti-tetanus immunoglobulins (3000 IU i.m.) and
amoxicillin (100 mg/kg i.v.). In order to prevent respira-
tory failure, the boy was intubated and mechanical
ventilation was started. Thereafter the boy was transferred
to the pediatric intensive care unit (PICU) Figure 1A)
for further treatment. The antibiotic regimen was then
converted to metronidazole (30 mg/kg/day i.v.) for
10 days in accordance with local guidelines. On the
second day of admittance, surgical debridement of the left
hallux toenail was performed (Figure 1B). In addition, the
second intramuscular dosage of anti-tetanus immuno-
globulins was administered. Active immunization against
diphteria, tetanus and polio (DTP) was started after one
week. Despite evident clinical signs of tetanus, repeated
blood and wound cultures were negative for C. tetani.
During the entire stay at the PICU repeated cultures of
blood, urine and tracheal aspirates remained negative and
white blood cell counts remained unremarkable. In
contrast, CRP levels increased to a maximum of 63 mg/L
at day 6 of PICU stay (Figure 2).
In the second week, the frequency and severity of muscle
spasms and trismus significantly worsened. Dosages of
midazolam and morphine (i.v.) were increased and
clonidine and lorazepam were added to the regimen. In
this period, creatine kinase levels were maximal on day 12
at 945 U/mL [reference value: 15175 U/mL] (Figure 2).
Due to anxiety, haloperidol was started. The frequency of
muscle spasms and anxiety decreased in the third week,
after which gradual weaning off sedatives was started.
Uneventful extubation was performed at day 16. Two
short periods with increased muscle spasms occurred
thereafter, which were successfully treated with diazepam.
On day 21 he was transferred back to the referring hospital
where he was discharged after 9 days. During regular
follow-up visits to the outpatient clinic, no adverse long-
term effects were registered.
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Discussion
In developed countries, national immunization programs
have succesfully decimated the number of tetanus infec-
tions during the previous four decades [4]. In particular,
generalized tetanus infections within the first month after
birth (tetanus neonatorum) have become extremely rare in
the Western world [1]. However, the widespread distribu-
tion of C. tetani spores in our environment in combination
with the lack of herd immunity still leads to incidental
tetanus infections in non-immunized individuals. This
case report urges on considering generalized tetanus
infection, also in developed countries, when confronted
with non-immunized children who present with unex-
plained oropharyngeal symptoms.
In our patient, dysphagia, sore throat and difficulty opening
the mouth (with the suspicion of a peritonsillar abscess)
were the main complaints in the prodromal stadium of
severe generalized tetanus. These and other complaints i.e.
neck stiffness are common in general practices and are rarely
regarded as early signs of tetanus. Set against this clinical
picture in the prodromal stadium, patients in developing
countries are more likely to present with progressed and
unambiguous symptoms i.e. severe spasms of the facial
musculature (risus sardonicus) and opisthotonus [2].
In literature, only two adult case reports demonstrate the
diagnostic challenge with oropharyngeal symptoms in
generalized tetanus in developed countries [5,6]. In both
cases, the patients presented with isolated symptoms of
dysphagia and trismus, and generalized tetanus infection
was not recognized at first. The initial diagnostic confusion
was soon followed by rapid clinical deterioration with
either respiratory failure [5] or autonomic dysfunction [6],
necessitating prolonged intensive care. These reports
emphasize the challenge of diagnosing generalized tetanus
infection in the mere presence of dysphagia and trismus,
which is accompanied by an undiminished risk of rapid
clinical deterioration.
Another complicating matter is the distinction with
localized tetanus, in which the distribution of tetanus
toxin and muscle spasms is limited to specific body areas.
Although these cases are usually associated with good
Figure 2.
C-reactive protein (CRP; filled squares) levels reached a
maximum at the end of the first week of PICU stay. In
contrast, creatine kinase (CK; open triangles) levels only
started to increase in the second week of admittance. Maximal
creatine kinase levels were measured at the end of the second
week and were accompanied by increases in the severity of
muscle spasms and trismus. PICU: Pediatric Intensive Care
Unit; TIG: anti-tetanus immunoglobulins.
Figure 1.
(A) A 4-year-old caucasian boy with generalized tetanus at the
time of admittance to the pediatric intensive care unit where
mechanical ventilation, deep sedation and extensive
cardiorespiratory monitoring were performed. (B) More
detailed photograph of the left hallux toenail, in this case the
most likely portal of entry, after surgical debridement.
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outcome, rare cases with involvement of the cranial nerves
(cephalic tetanus) have a high risk of progressing to
generalized tetanus with a high mortality [2]. In tetanus
neonatorum, the early symptoms include suckling and
feeding problems, vomiting and seizures. These often
rapidly progress into generalized spasms, opisthotonus or
even septic complications [1,2].
Appropriate treatment of generalized tetanus consists of
neutralization of free circulating tetanus toxin, surgical
debridement, eradication of the bacterial load and
advanced supportive care. Human tetanus-specific immu-
noglobulin (TIG) is available for neutralization of
tetanospasmin [7], although there are contradictory
reports about the optimal dosage and route of adminis-
tration [8]. A recent meta-analysis showed that a combina-
tion of intramuscular and intrathecal TIG administration
is superior to intramuscular treatment alone with regard to
mortality fromtetanus [9]. Pharmacological eradication of
C. tetani bacilli can be achieved by either penicillin or
metronidazole based regimens. A randomized clinical trial
that compared these antibiotics for the treatment of
generalized tetanus showed no differences with regard to
in-hospital mortality or autonomic dysfunction [10]. In
fact, the most contributing factor to reduce mortality from
generalized tetanus is treatment within modern (pediatric)
intensive care units (ICU) with aggressive sedation
protocols and advanced ventilatory support. Benzodiaze-
pine derivatives are the mainstay for sedation in the ICU
during the course of generalized tetanus [11]. Autonomic
dysfunction remains the major clinical challenge, as
hypotension, arrhythmia and cardiac arrest are important
predictors of fatality [12]. Multidrug regimens are impera-
tive for the optimal management of autonomic dysfunc-
tion, although magnesium sulphate may reduce the need
for other pharmacological agents [13].
Conclusion
The diagnosis of generalized tetanus in children remains a
diagnostic challenge in developed countries, as the
classical symptoms may be absent at presentation. Early
recognition and immediate initiation of advanced critical
care are necessary to prevent rapid clinical deterioration.
Therefore, the differential diagnosis of non-immunized
children with an acute onset of dysphagia and trismus
should always include generalized tetanus.
Consent
Written informed consent was obtained from the parents
of the patient for publication of this case report and
accompanying images. A copy of the written consent is
available for review by the Editor-in-Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors contributions
PRJ wrote the manuscript and was involved in the clinical
care. TAHG and NJGJ provided clinical care to the patient
and revised the manuscript. CHS was involved in the
design and preparation of the manuscript. All authors read
and approved the final manuscript.
Acknowledgements
We would like to thank TFW Wolfs from the Department
of Pediatric Infectious Diseases and SO Algra from the
Department of Pediatric Intensive Care, Wilhelmina
Childrens Hospital, Utrecht for their help in drafting the
manuscript. We also thank AWL Schadenberg for con-
ducting a thorough review of the manuscript.
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