Familial Near-Total Intestinal Aganglionosis
Keywords:Hirschsprung’s disease, Total intestinal aganglionosis, Genetic, Familial
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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