A case of cadaveric isolated intestinal transplantation for a megacystis-microcolon-intestinal hypoperistalsis syndrome

Abstract

A 6-year-old girl with megacystis-microcolon-intestinal hypoperistalsis syndrome was referred to our hospital for intestinal rehabilitation. However, oral enteral nutrition did not improve the delayed gastric emptying and intestinal stasis. Furthermore, because intestinal failure-associated liver disease (IFALD) disturbed the increase of parenteral nutrition, growth retardation was further aggravated and renal dysfunction with the primary disease was prolonged. Therefore, cadaveric intestinal transplantation (ITx) was indicated for IFALD and growth retardation at the age of 10 years. The transplanted graft was an isolated intestinal graft with the colon. Systemic drainage was adopted for venous reconstruction. Regarding intestinal reconstruction, native gastric-transplanted intestinal anastomosis was performed. Rabbit antithymocyte globulin was used as the medication for induction treatment. Thereafter, re-colostomy and anastomotic plasty of the native gastric-transplanted intestinal anastomosis were performed at 2.5 months post-transplantation due to stomal prolapse and obstruction of the native gastric-transplanted intestinal anastomosis. Tacrolimus and a steroid were administered as a maintenance immunosuppression therapy post transplantation, and everolimus was added due to the detection of human leukocyte antigen antibodies at 5 months post transplantation. Oral administration for secondary diabetes was started at 6 months post transplantation. At 13 months post transplantation, delayed gastric emptying improved, nourishment management mainly consisted of oral enteral nourishment, and dependence on parenteral nutrition decreased. The growth retardation and vigor improved. No episode of acute rejection occurred after ITx.