ABSTRACT: A select group of children with short bowel syndrome (SBS) and intestinal failure associated liver disease (IFALD) fulfil the criteria for isolated liver transplantation (iLTx). METHODS:: A retrospective study of the medical records of 8 survivors out 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). 5/8 children were weaned from parenteral nutrition (PN) to enteral nutrition (EN) in a median of 10 months after iLTx (range 3-32 months). 3/5 children were subsequently weaned from EN to full oral feeding in 13, 24 and 53 months after stopping PN while the remaining 2 are still on 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 pre-transplant z-score, whereas the height z-sores show fluctuations over the years with a median change of 0.12 (range -0.29 to 0.36). The other 3/8 children developed progressive intestinal failure; 2 undergone isolated small bowel transplantation 112 and 84 months after iLTx and the third is on PN. CONCLUSION:: Children with SBS and IFALD who have a 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 non-transplant surgery in the long-term.
|Journal||Journal of Pediatric Gastroenterology and Nutrition|
|Publication status||Published - 12 Dec 2011|