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Published on: March 5, 2018
Chronic intestinal pseudoobstruction in young children
Insights
This study investigated chronic intestinal pseudoobstruction in young children, finding severe gastrointestinal and urinary tract dysfunction despite mostly normal histology. Early-onset symptoms like infections and bladder issues are key indicators.
Area of Science:
- Pediatric Gastroenterology
- Urology
- Gastrointestinal Motility Disorders
Background:
- Chronic intestinal pseudoobstruction (CIPO) is a rare, severe motility disorder.
- Early-onset CIPO in children presents diagnostic challenges.
- Associated urinary tract dysfunction is frequently observed.
Observation:
- Eight young children (4 boys, 4 girls) with CIPO were studied.
- Symptoms including recurrent urinary tract infections and dysuria appeared between a few weeks and 5 years of age.
- All patients exhibited marked gastrointestinal tract dilatation and megacystis.
Findings:
- Conventional pathology of gastrointestinal specimens was normal in 5/8 patients; abnormalities included fibrosis and lipofuscin pigment in smooth muscle in 2/8.
- Myenteric plexus examination was normal in the 2 patients tested.
- Urinary bladder biopsies showed collagen separation of smooth muscle cells in 3 patients.
- Intestinal manometry revealed weak, infrequent contractions in 3 patients.
Implications:
- Severe gastrointestinal and urinary tract dysfunction can occur in children with CIPO and relatively normal histologic findings.
- This suggests non-histologic factors or subtle abnormalities may underlie the severe functional deficits.
- Further research into the pathophysiology of pediatric CIPO is warranted to improve diagnosis and management.
Abstract:
We studied 8 young children (4 boys and 4 girls) with chronic intestinal pseudoobstruction. Intestinal pseudoobstruction, recurrent urinary tract infections, and dysuria occurred between the ages of a few weeks to 5 yr old. All had marked dilatation of the entire gastrointestinal tract distal to the esophagus, and megacystis. Conventional pathologic examinations of the full-thickness specimens of the gastrointestinal tract were normal in 5 and abnormal in 2 patients. The abnormalities included increased fibrosis and lipofuscin pigment in the smooth muscle cells. Myenteric plexus examination, using the Smith's method in 2 patients, was normal. Biopsy specimens from urinary bladders examined in 3 patients revealed separation of individual smooth muscle cells by collagen fibers. Intestinal manometric studies performed in 3 patients showed only weak and infrequent contractions during fasting and after feeding. Severe and extensive dysfunction of the gastrointestinal and urinary tracts with relatively normal histologic appearance are typical for these children.
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