Long-term Outcomes of Isolated Liver Transplantation for Short Bowel Syndrome and Intestinal Failure-associated Liver Disease

Background and Aim: A select group of children with short bowel syndrome (SBS) and intestinal failure-associated liver disease (IFALD) fulfill the criteria for isolated liver transplantation (iLTx). Long-term results in this group of patients have not been reported. Methods: A retrospective study of the medical records of 8 survivors of 14 children who underwent iLTx for SBS and IFALD from 1998 to 2005, managed by a multidisciplinary intestinal rehabilitation team at our institution. Results: Median follow-up is 107.5 months (range 89-153 months). Five of 8 children were weaned from parenteral nutrition (PN) to enteral nutrition (EN) in a median of 10 months after iLTx (range 3-32 months). Three of 5 children were subsequently weaned from EN to full oral feeding in 13, 24, and 53 months after stopping PN, whereas the remaining 2 are still receiving EN 118 and 74 months after stopping PN. These 5 children maintain their weight median z scores with a median increase of 1.59 (range 1.24-1.79) compared with the pretransplant z score, whereas the height z scores show fluctuations through the years with a median change of 0.12 (range -0.29 to 0.36). The other 3 of 8 children developed progressive intestinal failure; 2 underwent isolated small bowel transplantation 112 and 84 months after iLTx and the third is receiving PN. Conclusions: Children with SBS and IFALD who have the potential for adaptation in the residual bowel can undergo iLTx, but it is a treatment option to be exercised with extreme caution. These children need close follow-up with an experienced multidisciplinary team to monitor nutritional outcomes and may need consideration for transplant or nontransplant surgery in the long term.