Difficult Management Choices For Infants With Short Bowel Syndrome and Liver Failure

Intestinal Failure‐Associated Liver Disease

“…Reserved for short bowel syndrome iLTx has 60-80% 1-year survival in patients with short bowel who are progressing with weaning from PN before the onset of liver failure; avoid in patients with long-term need for PN especially if tolerance of enteral feed is poor (less than 50%) as IFALD recurs [34,36].…”

“…The decision to embark on further abdominal surgery in a child or adult with evolving liver failure is always a difficult one, but intestinal plication or tapering and lengthening operations for well selected patients with short bowel syndrome, can produce improvements in intestinal function and resolution of IFALD if the PN is, thereby, reduced or discontinued [20,34,35] (see also Chapter 26 of this volume for more detailed discussion of this important topic). The option of isolated liver transplant is available for patients (almost always young children aged less than 2 years with short bowel syndrome) with a good prognosis for future intestinal autonomy, but in whom liver failure has supervened [36].…”

“…The option of isolated liver transplant is available for patients (almost always young children aged less than 2 years with short bowel syndrome) with a good prognosis for future intestinal autonomy, but in whom liver failure has supervened [36]. The rehabilitation of these patients is more protracted than after small bowel transplantation so it is advisable to refer to an experienced unit [20,34].…”

Isolated liver transplantation in pediatric short bowel syndrome: is there a role?

“…Although bowellengthening procedures have shown promise in infants with SBS [2], the concomitant liver failure may make these infants unsuitable candidates for these procedures. Also, small bowel transplantation is still an evolving field [3] with lower survival rates than liver transplantation [4,5] and with mortality on the waitlist exceeding that of other organ transplants [6].As a result of this, some have advocated isolated liver transplantation in infants with SBS and PNAC who develop liver failure before full expected gut adaptation [1,[7][8][9][10][11]. The aim of this article is to present our experience with isolated liver transplantation in infants with short bowel syndrome and to review the literature.…”

“…As a result of this, some have advocated isolated liver transplantation in infants with SBS and PNAC who develop liver failure before full expected gut adaptation [1,[7][8][9][10][11]. The aim of this article is to present our experience with isolated liver transplantation in infants with short bowel syndrome and to review the literature.…”