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Our aim was to collect a large number of cases to characterize clinical presentation, outcome, and prognosis of chronic intestinal pseusoobstruction in children. We conducted a retrospective multicenter study that included children treated for chronic intestinal pseusoobstruction defined as recurrent episodes of intestinal obstruction with no mechanical obstruction, excluding Hirschsprung's disease. In all, 105 children, 57 boys and 48 girls, were studied, including five familial forms. Prenatal diagnosis was made in 18 patients. Eighty patients were less than 12 months old at onset; the disease began at birth for 37 patients. The most frequent signs were abdominal distension, vomiting, and constipation. Megacystis was noted in myopathies (7 cases), neuropathies (10 cases) and unclassified forms (13 cases). For all but three cases (two patients with CMV infection, one with Munchhausen-by-proxy syndrome), the associated diseases and disorders could not account for chronic intestinal pseusoobstruction as a secondary disorder. At least one full-thickness biopsy from the digestive tract was studied for 99 patients. The diagnosis recorded was visceral neuropathy in 58 cases, visceral myopathy in 17 cases, and uncertain or normal biopsy results in 24 cases. Seventy-eight children were fed intravenously, and only 18 were able to be fed orally throughout their illness. Seventy-one patients underwent surgery during their illness, and 217 surgical procedures, a mean of 3 per patient, were performed. Ostomy was the most performed procedure. Follow-up continued in 89 patients for 3 months to 16 years (mean 85 months). Forty-two patients were still fed by parenteral (39 patients) or enteral nutrition (3 patients) at the time of the study. Eleven patients died between the age of 1 month and 14 years 7 months. [ABSTRACT FROM AUTHOR]

Introduction: L’abaissement trans-anal est une technique récente de traitement de la maladie de Hirschsprung, ses résultats ont été peu étudiés. Le but de cette étude était d’évaluer le devenir fonctionnel des enfants ainsi opérés. Patients et Méthodes: Nous avons colligé 44 dossiers d’enfants opérés depuis 1999 et ayant un recul minimum de 4 ans. Les parents ont été interrogés par téléphone selon les critères du score de Krickenbeck, après avoir reçu un questionnaire. Résultats: Une défécation volontaire et spontanée était présente chez 36% des enfants à 4-5ans, 52% à 6-7 ans et 57% à 8-11ans. Des souillures étaient retrouvées chez 84% des 4-5ans, 88% des 6-7ans et 71% des 8-11ans, dont 66%, 61%, et 52% respectivement avaient des souillures quotidiennes. Une constipation existait chez 32% des enfants de 4-5 ans, 30% des 6-7 ans et 24% des 8-11 ans, dont 50% à 60% nécessitaient un traitement laxatif oral. Conclusion: Notre étude souligne l’existence de troubles fonctionnels fréquents chez ces enfants. Le rôle de la distension peropératoire du sphincter anal dans les troubles de la continence doit être précisé. Il est nécessaire de réaliser une étude à plus grande échelle avec un score adapté à la maladie de Hirschsprung. [ABSTRACT FROM AUTHOR]