Up-to-date evolution of small bowel transplantation in children with intestinal failure

Purpose: The aim of the authors was to report an up-to-date review of their experience with 26 intestinal transplantations in children since 1987. Methods: A retrospective study was conducted of 26 patients with a mean age of 5 years (range, 0.3 to 14 years). Three groups were isolated. In group A (1987 to 1990), seven patients received nine isolated intestinal transpla nts for short bowel syndrome. Immunosuppression therapy consisted of cyclos porine, aziathioprine, and corticosteroids. In group B (1994-current), nine patients received nine isolated intestinal transplants for short bowel syn drom (n = 2), intestinal pseudoobstruction (n = 2), neonatal intractable di arrhea (n = 3), and Hirschsprung' disease (n = 1); hepatic biopsy results s howed weak cholestasis or fibrosis. In group C (1994-current), 10 patients received 10 combined liver-small bowel transplants for short bowel syndrome (n = 3), neonatal intractable diarrhea (n = 4), and Hirschsprung' disease (n = 3); hepatic cirrhosis related to total parenteral nutrition (TPN) was shown in all cases. Groups B and C received immunosupressive treatment cons isting of tacrolimus, aziathioprine, and corticosteroids. Posttransplant fo llow-up included intestinal biopsies of the small bowel twice a week and mo re frequently or combined with liver biopsy if rejection was suspected. Results: Overall patient survival (PS) and graft survival (GS) are 61% (16 of 26) and 50% (13 of 26), respectively. In group A, severe intestinal allo graft rejection occurred in six patients leading to graft removal (GS, 11%) . Five patients died of TPN complications after graft removal (PS, 28%). On e survivor is off TPN, and one currently is wailing for a second graft. In group B, six patients survived (PS, 66%). Causes of death include hepatic f ailure (n = 1), renal and liver failure (n = 1), and systemic infection (n = 1). Severe intestinal allograft rejection occurred in five patients, whic h neccessitated aggressive immunosuppression (antilymphocyte serum) leading to an incomplete functional recovery of the graft. Only two patients curre ntly are off TPN. In group C, eight patients survived (PS, 80%) all of whic h are currently off TPN. One patient died during the procedure, and one die d of severe systemic infection. Intestinal graft rejection occurred in six patients; rejection of the liver allograft occurred in five patients, yet a ll rejections were weak and successfully treated by corticosteroids(GS,80%) . Conclusions: Intestinal transplantation is a valid therapeutic option for c hildren with definitive intestinal failure and not only for short bowel syn drome. Tacrolimus improves graft and patient survival (group A v group B). The tower severity of graft rejection in combined liver-small bowel transpl antation improves functional results of intestinal transplantation in child ren without additional mortality or morbidity (group B v group C). Copyrigh t (C) 1999 by W.B. Saunders Company.