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Familial Near-Total Intestinal Aganglionosis. 2017
Hidouri Saida, and
Zitouni Hayet, and
Chahed Jamila, and
Mosbahi Sana, and
Belhassen Samia, and
Ksiaa Amine, and
Hmida Badii, and
Krichene Imed, and
Sahnoun Lassad, and
Mekki Mongi, and
Belguith Mohsen, and
Nouri Abdellatif
Department of Pediatric Surgery, Medical School Hospital of Monastir, Tunisia.
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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