Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Severe gastrointestinal dysmotility (GID ) impairs patients ' quality of life and is almost uniformly fatal after complications of parenteral nutrition . Intestinal and multivisceral transplants have been used as alternative treatment of these disorders . We studied patients with GID treated with transplantation in our center , and reviewed their outcome to determine the therapeutic efficacy of multivisceral transplants .T he transplant database was searched for patients with GID from 1994 to 2001 . We excluded patients with Hirschsprung disease , scleroderma , and diabetic enteropathy . We reviewed explanted organs , histochemistry , and immunohistochemistry and classified cases by etiology .We selected 12 children with GID from 124 patients transplanted . Nine presented before 1 year and 3 started with symptoms between 2 and 8 years . By combined clinical and histopathological features , 6 were classified as megacystis microcolon intestinal hypoperistalsis syndrome , 4 as chronic idiopathic intestinal pseudoobstruction , and 2 as intestinal neuronal dysplasias . Six patients died during the follow-up from 21 to 546 days after transplant . The Kaplan-Meier actuarial survival rates were 66 .7 % at 1 year and 50 % at 3 years .Multivisceral transplantation is a valuable therapeutic alternative for children with severe GID who can not be adequately managed with parenteral nutrition .