Chronic idiopathic intestinal pseudo-obstruction syndrome in children--clinical characteristics and prognosis
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Chronic idiopathic intestinal pseudo-obstruction (CIIPS) in children presents with varied symptoms and requires radiographic diagnosis. This condition often leads to poor long-term prognosis, necessitating supportive care.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Motility Disorders
Background:
- Chronic idiopathic intestinal pseudo-obstruction (CIIPS) is a rare condition affecting children.
- It presents with symptoms mimicking mechanical bowel obstruction without an apparent physical blockage.
Purpose of the Study:
- To describe the clinical presentation, diagnostic methods, and outcomes of CIIPS in a pediatric cohort.
- To highlight the role of radiographic studies in diagnosing CIIPS.
Main Methods:
- Retrospective review of eleven children diagnosed with CIIPS.
- Analysis of clinical data, radiographic findings (upper GI series, barium enema), manometric studies, and biopsies.
- Evaluation of treatment strategies and long-term prognosis.
Main Results:
- Four asymptomatic siblings were diagnosed radiographically.
- Common symptoms included abdominal distention, vomiting, pain, and malnutrition.
- Radiography revealed abnormal motility, delayed gastric emptying, and disorganized transit; half had abnormal barium enema findings.
- Esophageal manometry was abnormal in 7/10 children; biopsies confirmed ganglion cells.
- Four patients required total parenteral nutrition due to persistent symptoms.
Conclusions:
- CIIPS in children has a variable clinical course, with some diagnosed incidentally.
- Radiographic studies are crucial for diagnosis, revealing characteristic motility abnormalities.
- The condition carries a poor long-term prognosis in pediatric patients.
Abstract:
Eleven children with the diagnosis of chronic idiopathic intestinal pseudo-obstruction are presented. Four children, all siblings of a symptomatic patient, were asymptomatic and were diagnosed radiographically. The clinical course was characterized by intermittent episodes of abdominal distention, vomiting, abdominal pain, diarrhea, constipation, and malnutrition. Radiographic studies were most helpful in making the diagnosis. Findings on upper gastrointestinal series included abnormal esophageal motility, delayed gastric emptying, dilated loops of small bowel, and disorganized transit of barium. Half of the patients had abnormal evacuation patterns on barium enema. Manometric studies of esophageal motility were abnormal in seven of ten children. In those patients studied, small bowel and rectal biopsies contained ganglion cells. Treatment was directed at relieving symptoms, which in four patients became persistent and required total parenteral nutrition. CIIPS carries a poor long-term prognosis in children.