Chronic Intestinal Pseudoobstruction Syndrome Clinical Analysis, Outcome, and Prognosis in 105 Children

Our aim was to collect a large number of casesto characterize clinical presentation, outcome, andprognosis of chronic intestinal pseusoobstruction inchildren. We conducted a retrospective multicenter study that included children treated forchronic intestinal pseusoobstruction defined asrecurrent episodes of intestinal obstruction with nomechanical obstruction, excluding Hirschsprung'sdisease. In all, 105 children, 57 boys and 48 girls, werestudied, including five familial forms. Prenataldiagnosis was made in 18 patients. Eighty patients wereless than 12 months old at onset; the disease began at birth for 37 patients. The most frequentsigns were abdominal distension, vomiting, andconstipation. Megacystis was noted in myopathies (7cases), neuropathies (10 cases) and unclassified forms(13 cases). For all but three cases (two patientswith CMV infection, one with Munchhausen-by-proxysyndrome), the associated diseases and disorders couldnot account for chronic intestinal pseusoobstruction as a secondary disorder. At least onefull-thickness biopsy from the digestive tract wasstudied for 99 patients. The diagnosis recorded wasvisceral neuropathy in 58 cases, visceral myopathy in 17cases, and uncertain or normal biopsy results in 24cases. Seventy-eight children were fed intravenously,and only 18 were able to be fed orally throughout theirillness. Seventy-one patients underwent surgery during their illness, and 217 surgicalprocedures, a mean of 3 per patient, were performed.Ostomy was the most performed procedure. Follow-upcontinued in 89 patients for 3 months to 16 years (mean85 months). Forty-two patients were still fed byparenteral (39 patients) or enteral nutrition (3patients) at the time of the study. Eleven patients diedbetween the age of 1 month and 14 years 7months.