- It is not truly a congenital disorder
- Often called infantile hypertrophic pyloric stenosis
- Results in hypertrophy and hyperplasia of pyloric sphincter in neonatal period
- Mainly affects circular muscle fibres of pylorus
- Pylorus becomes elongated and thickened
- ? Due to failure of nitric oxide synthesis
- Results in gastric outflow obstruction, vomiting and dehydration
- Affects 3 per 1000 live births
- Male : female 4:1
- Most common in first born males
- Multifactorial inheritance
- Strong genetic factor
- Risk to son if affected mother = 20%
- Risk to daughter if affected mother = 7%
- Risk to son if affected father = 5%
- Risk to daughter if affected father = 2%
Clinical features
- Usually presents between 3 and 6 weeks of age
- Late presentation up to 6 months can occur
- Rapidly progressive projectile vomiting without bile
- Child hungry and often feeds immediately after vomiting
- Dehydration and alkalosis is a prominent clinical feature
- Palpable 'tumour' in right upper quadrant best felt from left during test feed
- Visible peristalsis often seen
- Diagnosis can be confirmed by abdominal ultrasound
- Biochemically a hypochloraemic alkalosis exists
Picture provided by Fahid Abu-Zant, Neblus Speciality Hospital, Neblus, Palestine
Treatment
- Correct dehydration over a 24 - 72 hour period
- Nasogastric tube often required
- Ramstedt's pyloromyotomy first described in 1911
- Transverse right upper quadrant or circumumbilical incision
- Longitudinal incision in pylorus down to mucosa
- Incision extend from duodenum onto the gastric antrum
- Need to try and avoid mucosal perforation
- Feeding re-established within 12-24 hours of surgery
- Recurrence does not occur
Bibliography
Huddart
S,
Bianchi A,
Kumar V,
Gough D C S.
Ramstedt's pyloromyotomy: circumumbilical versus transverse approach.
Pediatr Surg Int 1993;
8:
395-396
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