Congenital Hypertrophic Pyloric Stenosis (CHPS) : Definition, Etiopathology, Risk Factors, Signs, Symptoms, Examination, Diagnosis, Radiology, Complications, Management, Surgery, PPT
Synonym: Idiopathic Hypertrophic Pyloric Stenosis (IHPS), Infantile Hypertrophic Pyloric Stenosis
Definition
- Congenital Hypertrophic Pyloric Stenosis is the hypertrophy of musculature of pyloric antrum, particularly circular muscle fibres, causing the primary failure of pylorus to relax.
- Age of onset: 2 week to 3 months (∼ 75-80% cases). Rarely, present in first 2 week.
- Incidence⇒ 3:1000
- Familial
- Male : Female :: 4 : 1 (Common in first born male child).
- White > Black race
Pathology
Progressive hypertrophy of circular smooth muscle of pylorus & rarely, antrum
⇓
Gastric outlet obstruction
Risk Factors
- Male child
- First born child
- Blood group B & O
- Syndromes: Edward, Klinefelter, Turner
- Use of erythromycin or azithromycin in neonatal period
Clinical Features
1. Vomiting
- Projectile, forcible, post feed non-bilious.
- Occurs 3-8 weeks after birth, the time taken by hypertrophied muscle to cause complete obstruction.
Gastric outlet obstruction
⇓
Vomiting
2. Visible gastric peristalsis (VGP)
3. Olive shaped palpable lump of hypertrophied pylorus
4. Constipation
5. Dehydration
6. Electrolyte imbalance
7. Weight loss
In premature infants
- Visible gastric peristalsis (VGP) & palpable lump is better seen & felt.
- Vomiting: Regurgitant
- Anorexia is common
What are not observed?
- No succussion splash
- No polyhydramnios
- PEM not found
What are observed?
- Child hungry
- Stomach empty
Associations
- Constipation
- Jaundice
Physical examination
- Visible peristalsis
- Palpate olive shaped epigastric mass during or just post feed.
Investigations
1. Ultrasound abdomen (USG abdomen)
- Investigation of choice
- > 95% accurate
- Carried out on empty stomach
Pylorus
- Pyloric length ≥ 16mm
- Pyloric width or thickness ≥ 4 mm
Signs on USG
- Target sign
- Doughnut sign
- Cervix sign
- Antral nipple sign
2. X-Ray
- Single bubble sign
3. Ba Meal or Fluoroscopy
Signs on contrast study
- String sign
- Mushroom sign
- Caterpillar sign
- Railroad track sign
- Double track sign
Differential Diagnosis
- Duodenal atresia
- High intestinal obstruction
- Intracranial haemorrhage
Complications
Electrolyte imbalance
⇓
Hypochloremic hypokalemic metabolic alkalosis with paradoxical aciduria
(Activation of Na+/H+ antiport in distal tubules of kidney ⇒ paradoxical aciduria)
Treatment
1. Correction of electrolyte & fluid imbalance (1st step)
0.45 % NS + 2.5 % Dextrose + KCl
or
Ringer lactate RL (130 meq)
2. Surgery (2nd step)
- Ramstedt’s pyloromyotomy
- Laparoscopic pyloromyotomy
Complications
- Postoperative pyrexia
- Gastroenteritis
- Electrolyte imbalance
3. Medical (Not recommended)
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