Long-term Outcomes of Isolated Liver Transplantation for Short Bowel Syndrome and Intestinal Failure-associated Liver Disease Journal of Pediatric Gastroenterology and Nutrition, 03/30/2012

Taha AMI et al. – Children with short bowel syndrome (SBS) and intestinal failure–associated liver disease (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.

• 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 institution.

• 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.