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Infantile hypertrophic pyloric stenosis (IHPS):
it can take away your breath, alertness,
wee and poo
Ramnik V Patel,
1
Rebecca Wockenforth,
2
Irene Milliken,
2
David Marshall
2
1
Department of Paediatric
Urology, University College
London Hospitals NHS
Foundation Trust, London, UK
2
Department of Paediatric
Surgery, Royal Belfast Hospital
for Sick Children, Belfast, UK
Correspondence to
Ramnik V Patel,
ramnik@doctors.org.uk
To cite: Patel RV,
Wockenforth R, Milliken I,
et al.BMJ Case Rep
Published online: [please
include Day Month Year]
doi:10.1136/bcr-2013-
201435
DESCRIPTION
A 5-week old full-term previously healthy first-born
male infant presented with apnoea, desaturations,
increasing lethargy, anuria for 1 day and constipation
for 5 days. This was on a background of persistent
projectile non-bilious vomiting for 10 days, during
which he had been given Carobel for presumed gas-
tritis and gastro-oesophageal reflux. Father’s cousin
had pyloric stenosis. He was lethargic, pale and dehy-
drated with 14% weight loss. Heart rate was 130/
min, shallow respirations of 28/min, intermittent
desaturations to 70–80%. He had epigastric fullness
with visible peristalsis. He was started on 36%
oxygen, given two boluses of normal saline. Urine
dipstick after resuscitation showed pH 7.0, venous
blood gas showed pH 7.53, PCO
2
9.2, PO
2
3.21,
HCO
3
55 and BE 27.8. ECG showed flat T-waves
and ST-segment depression. Babygram ruled out
pulmonary aspiration and showed a dilated stomach
with paucity of distal gas. Ultrasound scan confirmed
infantile hypertrophic pyloric stenosis (IHPS)
(figure 1). He was intubated, ventilated and trans-
ferred to paediatric intensive care unit. After 72 h of
biochemical correction, he underwent supraumbilical
pyloromyotomy uneventfully.
IHPS is a common condition and the severity
and duration of symptoms enhance loss of fluid,
electrolytes and cause severe acid–base imbalance.
This leads to dehydration with oliguria and anuria,
hypokalaemia, hypochloraemia, severely metabolic
alkalosis with compensatory respiratory acidosis.
The mortality associated with IHPS was as high as
14.4% in 1935, but improved to 0.5% in the late
1960s, where it has held eversince.
1
Only rarely
now is apnoea and paradoxical aciduria seen in
patients with pyloric stenosis.
23
Figure 1 (A) Babygram, (B) abdominal ultrasound, (C) urinalysis, (D) capillary blood gas, (E) ECG and (F) serum
electrolytes showing typical changes of advanced infantile hypertrophic pyloric stenosis (IHPS).
Patel RV, et al.BMJ Case Rep 2013. doi:10.1136/bcr-2013-201435 1
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Learning points
▸Gastrointestinal and plasma compensation: Carobel thickens
gastric contents, converting partial obstruction into complete
one and prevents gastrointestinal compensation. Water gets
absorbed from the colon producing constipation in addition
to complete gastric outlet obstruction. Oral rehydration fluid
helps restore balance and reduces vomiting as there are no
milk curds to block the lumen.
▸Renal compensation: The kidney compensates for this shift
in fluid, electrolytes and acid/base imbalance by increasing
the reabsorption of water, producing oliguria, preserves
sodium and allows potassium to be excreted, responds to
the alkalosis by decreasing bicarbonate reabsorption,
producing alkaline urine in an attempt to decrease the
serum bicarbonate level. Later on, when potassium level
falls to dangerously low levels, it starts excreting hydrogen
ions for potassium preservation and paradoxical aciduria is
an advanced renal compensation to preserve life.
▸Respiratory compensation: It promotes hypoventilation
similar to that of hyperventilation in metabolic acidosis and
an increased partial pressure of carbon dioxide and
compensatory respiratory acidosis. The resultant hypoxia as
a result of hypoventilation prevents its progression to overt
respiratory failure. However, hypoventilation can in turn lead
to atelectasis and hypoxaemia and apnoea is considered
secondary to metabolic alkalosis associated with IHPS which
is rare. The management should include continuous
cardiorespiratory monitoring, pulse oximetry, oxygen,
contingency plans for emergent airway stabilisation and
management. Admission to general paediatric units and
infant transport with personnel unprepared for managing
the infant airway is ill-advised and may prove catastrophic.
Acknowledgements The authors are grateful to the paediatric accident and
emergency and paediatric intensive care unit teams of the referring hospital and our
hospital transport and paediatric intensive care unit teams for stabilisation and
transfer safely and effectively.
Contributors All the authors have made substantial contributions to the
conception and design of this manuscript, search of literature, the acquisition,
analysis and interpretation of the data, to drafting the article or revising it critically
for important intellectual content and to the final approval of the version to be
published.
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES
1 Tigges CR, Bigham MT. Hypertrophic pyloric stenosis: it can take your breath away.
Air Med J 2012;31:45–8.
2 Pappano D. Alkalosis-induced respiratory depression from infantile hypertrophic
pyloric stenosis. Pediatr Emerg Care 2011;27:124.
3 McCauley M, Gunawardane M, Cowan MJ. Severe metabolic alkalosis due to pyloric
obstruction: case presentation, evaluation, and management. Am J Med Sci
2006;332:346–50.
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2 Patel RV, et al.BMJ Case Rep 2013. doi:10.1136/bcr-2013-201435
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