Taha Ahmed¹, Khalid Sharif¹, Tracey Johnson^2,3, Sara Clarke^1,3, Stephen Murphy², Girish Gupte¹

¹Liver Unit (including small bowel transplantation), Birmingham Children's Hospital, Birmingham, United Kingdom; ²Department of Gastroenterology, Birmingham Children's Hospital, Birmingham, United Kingdom; ³Department of Dietetics, Birmingham Children's Hospital, Birmingham, United Kingdom

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). We report on the long-term outcomes of children undergoing iLTx for the above indication, managed by a multidisciplinary intestinal rehabilitation team. Of the 14 transplants performed, 4 died in the immediate post-operative period. Two further children died at 12.3 and 46 months after transplantation. Eight children survived in the long-term with a median follow-up of 107.5 months (range 89-155 months). 5/8 children were weaned from parenteral nutrition (PN) into enteral nutrition (EN) in a median of 10 months after iLTx (range 3-32 months). Three of those 5 children were subsequently weaned from EN into full oral feeding in 13, 24 and 53 months after stopping PN while the other 2 are still on EN 118 and 74 months after stopping PN. The 5 children maintain their weight median z-score with a median increase of 1.59 (range 1.24-1.79) compared with the pre-transplant z-scores, 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 irreversible 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 need close follow-up with an experienced multidisciplinary team to monitor long term nutritional outcome and may need consideration for non-transplant/transplant surgery in the follow-up period.