Chronic idiopathic intestinal pseudo-obstruction syndrome in children--clinical characteristics and prognosis.

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.