AASLD Abstract (pp. 162A–266A)
Authors: Benjamin Shneider, Ezequiel Neimark, Frederick Suchy, Suk-Ru Emre, Dominic Olio, Girish Gupte, Khalid Sharij, Deirdre Kelly, Carla Lloyd, Sue Protheroe, Stephen Murphy, Mckiman Patick, Indra Vanmourik, Darius Mina, David Mayer, Alastair Millar, Susan Beath, Cirish Gupte, Khalid Sharif, Sue Kotheroe, Mckiernan Patick, Darius Mirza, Transplantation Yuliya Rekhtman, Sarah Bellemare, Patricia Harren, Jean Emond, Dominique An, Steven Lobrifto, Sanjay Bansal, Anita Verma, Rachel Taylor, Paul Cheeseman, Marianne Samyn, Nigel Heaton, Mohammed Rela, Giorgina Mieli- V Q A N I, Anil Dhawan, Groupa Groupb, Giorgina Mieli-Vergani, Timothy Lee, Neal Barshes, Liz Nguyen, Sasi Yallampalli, Saul Knrpen, Beth Carter, Ruben Quims-Tejeira, Lisa Bristow, Jaymee Scoff, John Goss, Saul Karpen, Ruben Quiros-Tejeira, Jaymee Scott, John Coss, Todd Pillen, Rene Romero, David Welch, Carlos Fasola, Greg Smallwood, Thomas Hefbn, Hypertension Wiest, Lars Junik, Matthias Froh, Erwin Gabele, Claus Hellerbrand, Markus Miihlbauer, Bernd Schnabl, Jueqen Schol~nm'ch, Rainer Straub
Journal: Hepatology (2004)

Introduction:In infants with SBS and IFALD, intestinal adaptation and the transition to enteral feeding may not be achieved due to rapidly progressive liver disease.These infants may die on the waiting list awaiting a combined liver and small bowel transplant (LSBTx) and even after LSBTx , the long term outcome is poor.ILTx has been proposed as a life saving treatment in infants when intestinal function is expected to improve in the future.Aim: To describe experience with ILTx in children with SBS and IFALD .Subjects and Methods: Eleven children (7M.G) undergoing ILTx for IFALD were identified following a retrospective review of the database.Isolated liver transplantation was performed in children with SBS and IFALD, if at least 50% of estimated daily requirements enterally had been tolerated and at least 30cm residual small bowel remained intact. Results:The median age at assessment was 9.7 months ( range 5.1-19.3months).Table 1 outlines the patient characteristics and outcome of children undergoing ILTX expressed as median (range).Discussion: Of the five children weaned off PN , one child had intestinal lengthening and one child had intestinal tapering procedure, facilitating weaning off PN.Three children are being weaned off PN (three nightslweek) and two children continue on home PN.One child with pre-existing respiratory disease developed respiratory distress syndrome and died in ITU 7 weeks after transplant.Summary: The children were on the waiting list for shorter time ( 8-100 days) as compared to children awaiting small bowel transplant ( 90-365 days).Split grafts have been assodated with biliary complications and hence reduced grafts are being used in our programme for children with SBS and IFALD.Rehabilitation folloing ILTx involved a multidisciplinary team (dietitian + gastroenterologist + surgeons + hepatologists + nutritional care team + anaesthetists) requiring hospital care for longer period of time than liver transplantation for infants with normal intestinal function. Conclusion:ILTx in young infants with SBS and IFALD is a viable option resulting in good medium term survival, but management by a well established multidisciplinary team is essential Table 1: PaUent chmcterirtkr and outcane following ILTX Resldwl IenaIh of bowel In em 60 13080) Bilhry complltlons B(k leak Cho*ngith -prrfMtlon 2 (split grab) Weaned from parenteml nutrim (45%) Uon Time to waning OR PN in day8 273 (62-768 ) Tlm to discharge from hospbl , 4 (31-144) in dryt Overall rurvivll 91% 2 (split graft.dud to intesline without rwx) 2 (duct to intestine without rwx)