Nutritional management of allied disorders of Hirschsprung's disease

Nine patients with allied disorders of Hirschsprung's disease were divided into three groups, including 5 patients with immaturity of the ganglia, two with hypogenesis and two with Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). In the immaturity group, enteral feeding was the earliest started among these three groups, and TPN had been continued till the maturation of ganglion cells. In the hypogenesis group, the duration of TPN was prolonged and severe liver dysfunction occurred. In the MMIHS group, enteral feeding could not be admitted and TPN related complications led to death. Intestinal transplantation would be one of the therapeutic routes to follow in the future for this group patients.