Living-related small bowel transplantation: the first case in Japan.

Intestinal failure has been managed with total parenteral nutrition (TPN), but occasionally complications such as obliteration of venous access or liver dysfunction occur. To overcome such complications, small bowel transplantation (SBT) was introduced. Since the introduction of tacrolimus in 1990, successful SBT cases have been reported. We performed SBT by using a living donor for a child with short bowel syndrome. The recipient (2.5 years old, male) was born with intestinal necrosis secondary to midgut volvulus. The length of the remaining small bowel was 30 cm. While being managed with TPN, his venous access gradually obliterated. Long-term survival could not be expected because of the difficulty in securing TPN access. The donor was his mother, whose distal ileum (100 cm) was used as a graft. The immunosuppression regimen consisted of tacrolimus, steroids and azathioprine. Three episodes of severe rejection and subsequent episodes of viral (EBV, CMV) infection were managed with steroid pulse therapy and antiviral drugs, respectively. The recipient suffered from anastomotic stenosis, and an operation was performed 13 months after transplantation to resect the stenotic segment. However, the patient died of Pneumocystis carinii pneumonia 16 months after transplantation. We conclude that organ retrieval from a living donor can be performed safely for SBT, but further study of the management of rejection as well as of viral infection is necessary, as it is for non-living-related SBT.