Treatment of Congenital Hypertrophic Pyloric Stenosis (CHPS)
Surgical pyloromyotomy is the definitive treatment for infants with CHPS, with the Fredet-Ramstedt procedure being the gold standard approach that provides immediate and permanent resolution of gastric outlet obstruction. 1, 2
Preoperative Management
Before any surgical intervention, meticulous correction of fluid and electrolyte abnormalities is absolutely essential:
- Assess and correct dehydration and metabolic alkalosis (commonly present due to persistent vomiting) before proceeding to surgery 1, 2
- Infants typically present with hypochloremic, hypokalemic metabolic alkalosis that must be normalized 1
- Surgery should never be considered emergent; adequate time must be taken to optimize the infant's metabolic status 1
Surgical Treatment Options
Standard Surgical Approach (First-Line)
The Fredet-Ramstedt pyloromyotomy remains the treatment of choice, performed either as:
This procedure involves splitting the hypertrophied pyloric muscle longitudinally without entering the mucosa, allowing the mucosa to bulge through and relieve the obstruction 1
Emerging Minimally Invasive Option
Gastric peroral endoscopic pyloromyotomy (G-POEM) has shown promising results as a novel minimally invasive alternative:
- Successfully performed in 21 infants across three Chinese tertiary centers with 100% technical success 3
- Median operative duration of 49 minutes 3
- Submucosal tunnel created along the greater curvature (or lesser curvature if technically difficult) 3
- Myotomy depth of 2-3 mm for pyloric wall thickness of 4-6 mm, or 3-4 mm when wall exceeds 6 mm 3, 4
- Feeding can be initiated rapidly, typically 2-10 hours post-procedure 3, 4
- No major adverse events reported; minor complications included inconsequential mucosal injury and unsatisfactory mucosal closure 3
- Median follow-up of 25.5 months showed no recurrent symptoms 3
Medical Management (Alternative in Select Cases)
Atropine sulfate therapy may be considered as a non-surgical option, though it is not standard first-line treatment:
- Initially given intravenously until vomiting is controlled, then orally at double the effective IV dose for 3 weeks 2
- Side effects include tachycardia, elevated liver enzymes, and hyperthermia 2
- This approach is described as effective, cheap, and safe, but requires prolonged treatment compared to immediate surgical resolution 2
Postoperative Management
Feeding Protocol
- Upper gastrointestinal contrast studies should demonstrate smooth passage through the pylorus by postoperative day 3 3
- Vomiting typically stops within 1 week in most cases, though occasional patients may take up to 1 month 4
- Growth curves should normalize within 3 months post-procedure 3
Expected Outcomes
- After successful treatment, infants are healthy and normal with zero mortality when performed correctly 1
- Attention to surgical detail is necessary to achieve minimal morbidity 1
- Recurrence is rare but has been reported; one case required repeat endoscopic pyloromyotomy with successful outcome 4
Important Clinical Caveats
- CHPS typically presents at 3-6 weeks of life, though rare neonatal cases exist that may represent congenital rather than acquired disease 1, 5
- Classic presentation includes non-bilious projectile vomiting with a palpable "olive" mass in the epigastrium 1
- Diagnosis is confirmed by transabdominal ultrasound showing pyloric wall thickening and gastroenterography demonstrating gastric outlet obstruction 4
- Untreated CHPS carries high mortality, making timely intervention critical 1
- The etiology involves polygenic inheritance as a predisposing factor, though specific precipitating factors remain unclear 1