The First Case of Liver-Intestine En-Bloc Transplantation in Japan.

INTRODUCTION: Simultaneous liver-intestine transplantation is indicated for intestinal failure associated liver disease (IFALD), which can be caused by conditions such as intestinal motility disorders or short bowel syndrome. Currently, the most common grafts for liver-intestine transplantation are multivisceral transplantation (MVT) grafts consisting of the liver, stomach, duodenum, pancreas, small intestine, and colon, or liver-intestine grafts derived from the MVT graft but without the stomach. However, at the time of transplantation in the present case, Japanese regulations did not permit simultaneous pancreas transplantation in a non-diabetic recipient, which is generally required for this type of graft. Therefore, in the 2 previously reported domestic cases, the liver and intestine grafts were transplanted separately as non-composite grafts. Early in the development of MVT, liver-intestine grafts, excluding the stomach, duodenum, and pancreas, were used. We adopted this graft configuration for a 14-year-old female with megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) complicated by IFALD and performed, for the 1st time in Japan, a composite liver-intestine transplantation under existing regulations. CASE PRESENTATION: The patient was a 14-year-old female who was maintained on minimal enteral feeding via an intestinal stoma and parenteral nutrition because of MMIHS. Since approximately year X-7, her IFALD progressed, leading to portal hypertension with gastrointestinal bleeding and hypersplenism, which led to a significant decline in her quality of life. She was listed for deceased donor liver-intestine transplantation and a suitable donor became available in December X. The stomach, duodenum, and pancreas of the donor were resected in situ, and a descending aortic graft was interposed at the caudal side of the superior mesenteric artery; arterial anastomosis was performed on the recipient's infrarenal abdominal aorta. The hepatic vein was anastomosed using the piggyback technique. Although a small amount of parenteral nutrition was still required because of colitis, the patient was gradually progressing toward complete enteral nutrition. No evidence of rejection was observed, and the patient was discharged. CONCLUSIONS: We report successful composite liver-intestine transplantation using a graft that excluded the stomach, duodenum, and pancreas, which led to good clinical outcomes.