Isolated intestinal transplantation for megacystis microcolon intestinal hypoperistalsis syndrome: case report

MMIHS is a rare congenital disease. It is characterized by distended urinary bladder, small colon and intestinal hypoperistalsis, or aperistalsis with normal morphology. There is no specific treatment for MMIHS, and most patients have to be maintained by TPN, which frequently causes TPN-related liver failure, loss of venous access, or catheter-related sepsis. The prognosis of patients with MMIHS is poor, and most patients die early. Multivisceral transplantation including stomach, duodenum, intestine, and liver has been used for the treatment of patients with MMIHS because these patients often have liver failure. We report an eight-yr-old patient with MMIHS who was treated with isolated intestinal transplantation. She had completely oral intake during the four yr of follow-up. The experience in this case suggests isolated intestinal transplantation may be indicated in selected cases with MMIHS.