Chronic intestinal pseudoobstruction syndrome - Clinical analysis, outcome, and prognosis in 105 children

Our aim was to collect a large number of cases to characterize clinical pre sentation, outcome, and prognosis of chronic intestinal pseusoobstruction i n children. We conducted a retrospective multicenter study that included ch ildren treated for chronic intestinal pseusoobstruction defined as recurren t episodes of intestinal obstruction with no mechanical obstruction, exclud ing Hirschsprung's disease. In all, 105 children, 57 boys and 48 girls, wer e studied, including five familial forms. Prenatal diagnosis was made in 18 patients. Eighty patients were less than 12 months old at onset; the disea se began at birth for 37 patients. The most frequent signs were abdominal d istension, vomiting, and constipation. Megacystis was noted in myopathies ( 7 cases), neuropathies (10 cases) and unclassified forms (13 cases). For al l but three cases (two patients with CMV infection, one with Munchhausen-by -proxy syndrome), the associated diseases and disorders could not account f or chronic intestinal pseusoobstruction as a secondary disorder. At least o ne full-thickness biopsy from the digestive tract was studied for 99 patien ts. The diagnosis recorded was visceral neuropathy in 58 cases, visceral my opathy in 17 cases, and uncertain or normal biopsy results in 24 cases. Sev enty-eight children were fed intravenously, and only 18 were able to be fed orally throughout their illness. Seventy-one patients underwent surgery du ring their illness, and 217 surgical procedures, a mean of 3 per patient, w ere performed. Ostomy was the most performed procedure. Follow-up continued in 89 patients for 3 months to 16 years (mean 85 months). Forty-two patien ts were still fed by parenteral (39 patients) or enteral nutrition (3 patie nts) at the time of the study. Eleven patients died between the age of 1 mo nth and 14 years 7 months.