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Familial Near-Total Intestinal Aganglionosis
Hidouri Saida1,2, Zitouni Hayet1, Chahed Jamila1,2
1Department of Pediatric Surgery, Medical School Hospital of Monastir, Tunisia.
Abstract:
Near total aganglionosis represents the most extreme and rare form of Hirschsprung's disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a jejunostomy was performed. Outcome was dismal.
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