Intestinal Transplantation for Total Intestinal Aganglionosis

Intestinal transplantation is a successful treatment for children with irreversible intestinal failure who experience life-threatening complications of parenteral nutrition. Total or near-total intestinal aganglionosis is a rare form of Hirschprung’s disease (HD) with high morbidity and mortality and near-certain dependence on parenteral nutrition. Advances in surgical techniques, immunosuppressive regimens, and posttransplant surveillance and management have led to improvements in patient and graft survival following intestinal transplantation. Intestinal transplantation may provide a life-saving treatment option for children with total or near-total intestinal aganglionosis.