Up-to-Date Evolution of Small Bowel Transplantation in Children With Intestinal Failure By Dominique Jan, Jean-Luc Michel, Olivier Goulet, Sabine Sarnacki, Florence Lacaitle, Diane Damotte, Jean-Pierre Cezard, Yves Aigrain, Nicole Brousse, Michel Peuchmaur, Annick Rengeval, Virginie Colomb, Philippe Jouvet, Claude Ricour, and Yann R~villon Paris, France 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 transplants for short bowel syndrome. Immunosuppression therapy consisted of cyclosporine, aziathioprine, and corticosteroids. In group B (1994-current), nine patients received nine isolated intestinal transplants for short bowel syndrom (n = 2), intestinal pseu- doobstruction (n = 2), neonatal intractable diarrhea (n = 3), and Hirschsprung' disease (n = 1); hepatic biopsy results showed 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 intrac- table 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 immunosu- pressive treatment consisting of tacrolimus, aziathioprine, and corticosteroids. Posttransplant follow-up included intes- tinal biopsies of the small bowel twice a week and more 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 allograft rejection occurred in six patients leading to graft removal (GS, 11%). Five patients died of TPN complications after graft removal (PS, 28%). One survivor is off TPN, and one currently is waiting for a second graft. In group B, six patients survived (PS, 66%). Causes of death include hepatic failure (n = 1), renal and liver failure (n = 1), and systemic infection (n = 1). Severe intestinal allograft rejection occurred in five patients, which neccessitated aggres- sive immunosuppression (antilymphocyte serum) leading to an incomplete functional recovery of the graft. Only two patients currently are off TPN. In group C, eight patients survived (PS, 80%) all of which are currently off TPN. One patient died during the procedure, and one died of severe systemic infection. Intestinal graft rejection occurred in six patients; rejection of the liver allograft occurred in five patients, yet all rejections were weak and successfully treated by corticosteroids (GS, 80%). Conclusions: Intestinal transplantation is a valid therapeutic option for children with definitive intestinal failure and not only for short bowel syndrome. Tacrolimus improves graft and patient survival (group A v group B). The lower severity of graft rejection in combined liver-small bowel transplanta- tion improves functional results of intestinal transplantation in children without additional mortality or morbidity (group B vgroup C). J Pediatr Surg 34:841-844. Copyright © 1999 by W.B. Saun- ders Company. INDEX WORDS: Intestinal failure, short gut, intestinal trans- plantation, liver transplantation. T HE LONG-TERM OUTCOME for children who receive parenteral nutrition is not as favorable as it is in adults. Consequently, some patients may benefit from small bowel transplantation. Children dependent on total parenteral nutrition (TPN), more so than adults, are exposed to hepatic complications. Intestinal transplanta- tion was performed in our institution in 1987 using cyclosporine, t With the introduction of tacrolimus we have abruptly changed the clinical application of small bowel transplantation. 2 This is an up-to-date review of our experience with 28 intestinal transplantations in 26 children since 1987. MATERIALS AND METHODS Materials Between January 1987 and June 1998, 26 children received 28 intestinal transplants: nine isolated small bowel (SB) using cyclosporine from 1987 to 1991 (group A). Since 1994, nine isolated small bowel (group B) and 10 combined liver-small bowel (LSB) transplants (group C) using tacrolimus were performed. The liver was included as part of the allograft when parenteral nutrition-induced end-stage liver failure was evident. The recipients consisted of 15 boys and 11 girls, age ranging between 0.5 to 12 years old. All patients were dependant on definitive total parenteral nutrition. Intestinal failure was caused by short gut syndrome (n = 14), intractable diarrhea of infancy (n = 7), From Hopital Necker-Enfants Malades, Paris, France. Presented at the 1998 Annual Meeting of the Section on Surgery of the American Academy of Pediatrics, San Francisco, California, October 16-19, 1998. Address reprint requests to Dominique Jan, MD, Professor of Pediatric Surgery, Hopital Necker-Enfants Malades, 149 rue de S#vres, 75015 Paris, France. Copyright © 1999 by W.B. Saunders Company 0022-3468/99/3405-0035503.00/0 Journal of Pediatric Surgery, Vo134, No 5 (May), 1999: pp 841-844 841