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Chronic intestinal pseudo-obstruction syndrome in pediatric patients
O Goulet1, A Jobert-Giraud, J L Michel
1Département de Pédiatrie, Hôpital Necker-Enfants Malades, Paris, France.
Insights
Chronic intestinal pseudo-obstruction in children often involves the urinary tract and requires significant intervention. Management includes surgery and parenteral nutrition (PN), with a high morbidity rate but potential for survival to adulthood.
Area of Science:
- Pediatric Gastroenterology
- Pediatric Surgery
- Medical Genetics
Background:
- Chronic intestinal pseudo-obstruction (CIPO) is a rare, severe condition affecting children.
- It often presents with gastrointestinal and urinary tract dysfunction.
- Etiology remains largely unknown, with potential myopathic or neuropathic origins.
Purpose of the Study:
- To describe the clinical presentation, histopathological findings, and outcomes of 22 children with CIPO.
- To analyze the management strategies and long-term prognosis for this pediatric population.
Main Methods:
- Retrospective analysis of 22 consecutive pediatric cases of CIPO.
- Clinical data collection, including family history, age at diagnosis, and symptoms.
- Diagnostic investigations: X-ray, small bowel follow-through, duodeno-jejunal manometry, and full-thickness biopsies.
- Assessment of urinary tract involvement and treatment outcomes.
Main Results:
- CIPO presented from birth to adolescence, with 6 cases diagnosed antenatally with megacystis.
- Gastrointestinal symptoms included obstruction and constipation; urinary tract involvement (megacystis, megaureter) was common.
- Histopathology revealed muscle layer or myenteric plexus abnormalities in most cases.
- All patients required parenteral nutrition (PN); 20/22 underwent decompression surgery.
- Mortality was 23% (sepsis or sudden death); 11 patients remained dependent on PN.
Conclusions:
- Pediatric CIPO is a severe condition with high morbidity, often involving both gut and urinary systems.
- Management requires aggressive surgical decompression, treatment of infections, and judicious use of PN.
- Despite challenges, survival into adulthood is possible with comprehensive care.
Abstract:
The aim of this study was to report the presentation and outcome of 22 consecutive children (13 female) who presented with a syndrome of chronic intestinal pseudo-obstruction with or without urinary tract involvement. We analyse the main clinical and histopathological features and discuss therapeutic management. Ten patients had signs of intestinal obstruction at birth, in which 6 presented antenatally with megacystis on ultrasound. Six children presented with constipation and/or obstruction between 1 and 6 months of age and in 6 other patients diagnosis was made between the ages of 1 and 12 years. There was a family history in 4 patients. Investigations showed diffusely dilated gut on x-ray with slow transit on small bowel follow through. Absent or abnormal motor migrating complex with low amplitude contractions were demonstrated on duodeno-jejunal manometry in 12/13. Megacystis occurred in 15/21 and megaureter in 2/21. Full thickness biopsies (n = 22) revealed involvement of muscle layers in 8, and abnormal myenteric plexus on histochemistry in 13. In 1, the biopsies were inconclusive. Recurrent urinary tract infections occurred in all with structural urinary tract abnormality and most had bacterial overgrowth. Severe recurrent episodes of obstruction which required parenteral nutrition (PN) occurred in all patients. Drugs were unhelpful and decompression ileostomies or colostomies were performed in 20/22. Five children died from sepsis (n = 3) or sudden death. Eleven patients remain partially or totally dependent on PN despite decompression ileostomy in 10/11. Six patients underwent colectomy and ileorectal pull-through, 2 of which remain on long-term PN, while the others are totally orally fed. Despite careful histological study pointing to 2 main forms, myopathy and neuropathy, the etiology of primary intestinal pseudoobstruction syndromes remains unknown. It may present antenatally while most of the time the gut and the urinary tract are diffusely involved. The condition has a high morbidity with a percentage requiring long-term PN. Although the mortality rate is high (23%), careful treatment of urinary tract infections and bacterial overgrowth, decompression surgery and judicious use of PN allows survival to adult life.