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

BACKGROUND: 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). CASE REPORT: 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.
CONCLUSION: 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.