Isolated Liver Transplantation in Children With Intestinal Failure–associated Liver Disease: A Still‐debated Matter

Abstract

Although combined liver and small bowel transplantation is considered a lifesaving treatment option for children with intestinal failure and associated liver disease (IFALD), the survival rate of children undergoing combined liver and small bowel transplantation is low, with a mortality rate of 65% at 6 months (1-6). However, compared with isolated small bowel transplantation, combined transplantation is associated with better intestinal graft survival and a lower incidence and severity of rejection (7-9). In particular, there is a lower incidence and severity of acute intestinal graft rejection in recipients of combined transplantation than in recipients of isolated small bowel transplantation, probably as a consequence of immune tolerance induced by the liver. Liver failure is a frequent indication for combined transplantation in children with intestinal failure (6). In other words, if intestinal failure-associated liver disease needs liver transplantation, then it also represents one of the criteria to select candidates for intestinal transplantation (8,9). The impact of isolated liver transplantation (iLTx) on the survival and quality of life of patients with intestinal failure and associated liver disease is unclear (10)(11)(12)(13).In this issue of JPGN, Gupte et al report that iLTx may be lifesaving for selected children with short bowel syndrome and IFALD. The authors propose new criteria for iLTx in children with intestinal failure-associated liver disease. In their experience, 9 (64%) out of 14 children receiving iLTx were alive after a median of 35.6 months (range 21.2-86.8 months), and 8 of them were successfully weaned from parenteral nutrition (PN) after a median of 15 months. If these patients had received a combined liver-intestinal transplant rather than iLTx during the same period of time, most of them would have died, on the basis of available survival rates.

IFALD in children: What's new? A narrative review

“…The outcome of isolated liver transplantation in patients with intestinal failure and IFALD is better than the outcome of patients who undergo either isolated intestinal transplantation or combined liver and small bowel transplantation. Furthermore, isolated liver transplantation may improve intestinal adaptation reducing the need for small bowel transplantation ( 83 ). Because of the good survival rate and overall results in children with IF due to SBS managed with home PN, isolated liver transplantation should not become the SOC, should be considered with extreme caution, and proposed when liver disease with portal hypertension is considered the major obstacle to the enteral autonomy ( 3 , 84 , 85 ).…”

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

“…I solated liver transplantation (iLTx) in children with short bowel syndrome (SBS) and intestinal failure-associated liver disease (IFALD) has been reported from our centre in 14 children with proposed selection criteria (1). The key determining factor in proposing this option in SBS and IFALD is the predicted ability of the small bowel to adapt and allow weaning from parenteral nutrition (PN) to enteral nutrition (EN) (2,3) and the shortage of size-matched organs in the United Kingdom that precludes them from combined liver and small bowel transplantation (iSBTx). The major difficulties in the initial care and short to medium term follow-up have been outlined in the previous report (1).…”

Avaliação da incidência e evolução da colestase em pacientes portadores de gastrosquise com diferentes emulsões lipídicas na nutrição parenteral

“…O amplo espectro de alterações da função hepática induzidas pela NP é nomeado intercambiavelmente com os termos de: doença colestática, lesão hepática associada à NP ou doença hepática associada à insuficiência intestinal (DHAII), quando a doença hepática está especificamente associada à insuficiência intestinal em lactentes e crianças dependentes de NP (Orso et al, 2016 Nos casos em que a NP em longo prazo é necessária, um amplo espectro de mudanças hepato-biliares pode ocorrer (Fitzgibbons et al, 2010;Naini & Lassman, 2012;Spagnuolo et al, 2009). Estas mudanças são vistas principalmente na população pediátrica, variando da esteatose à colestase, colelitíase e fibrose hepática, progredindo para cirrose, hipertensão portal e "estágio final" do fígado, podendo até exigir transplante hepático.…”