Combined living-related segmental liver and bowel transplantation for megacystis-microcolon-intestinal hypoperistalsis syndrome.

[megacystis-microcolon-intestinal hypoperistalsis syndrome]

Megacystis-microcolon -intestinal hypoperistalsis syndrome (MMIHS ) is the most severe form of functional intestinal obstruction in the newborn . To date , multivisceral transplantation has been the only accepted treatment modality for these patients , and the results have met with marginal success . We report the first case of a patient affected by MMIHS and cholestatic liver failure treated by a combined living-related liver and intestinal transplant (CLRLITx ).The patient was a 1 -year -old Hispanic girl born with MMIHS and maintained on total parenteral nutrition since birth . Once liver failure developed , she was referred for evaluation for possible CLRLITx . The patient 's mother volunteered as the donor . The left lateral segment was used for the liver transplant . The intestinal graft consisted of the terminal 180 cm of the ileum with a single vascular pedicle . Initially , the patient continued to have severe gastroparesis ; however , by 8 months posttransplant , stomach function had returned to normal . Currently , at 2 years posttransplant , she is tolerating an oral diet with gastric tube supplementation . Results of absorption studies are within normal , and she has shown catch-up growth .A CLRLITx can be a viable alternative for infants diagnosed with MMIHS . This procedure can help avoid the 25 % wait-list mortality for children who are in need of a combined transplant .