Experiences of Living Donor Small Bowel Transplantation for Hypoganglionosis.

Abstract

PURPOSE: We recently performed living donor small bowel transplant (LDSBT) in three patients with hypoganglionosis. We report here the peri- and post-operative management and discuss several issues about LDSBT. PATIENTS: The patients were 14 (Case1), 11(Case2) and 15(Case 3) years old boy. All of them were with total parenteral nutrition associated with hypoganglionosis. Transplantation Procedure: One third of the donor bowel was harvested. The graft vessels were connected to the recipient's infra renal aorta and inferior vena cava (Case 1), or SMA and SMV (Case 2,3) Postoperative course:The immunosuppressive regimen consisted of daclizumab, tacrolimus, and steroids. The first patient developed liver dysfunction on POD 7, subsided spontaneously on POD 12, requiring no additional therapy. Two months after transplantation, he was weaned off TPN, tolerating oral intake with a fully functioning graft. The second patient was weaned off TPN on 3 months after transplantation with a functioning graft. He developed mild acute cellular rejection on day 111, which was successfully treated with bolus injections of steroid. The third patient encountered mild rejection at 1 year after transplantation. Subsequently intestinal perforation of graft occurred maybe due to adverse effect of steroid therapy. This was fortunately successfully managed. However, graft function deteriorated due to chronic rejection. We had to remove the graft. Re-transplantation is currently under consideration. CONCLUSION: LDSBTs were performed to the three patients with hypoganglionosis successfully. Long-term well functioning grafts were obtained in two of three cases. It is advisable that LDSBT would be performed before getting more serious liver dysfunction or blood access route problem. It would lead to physical and psychological improvement of quality of life.