Isolated liver transplantation for decompensated end-stage liver disease in children with intestinal failure

K Ventura1, Y Rekhtman, D Jan

  • 1Columbia University Medical Center, New-York Presbyterian Hospital, 622 W. 168th Street, New York, NY 10032, USA.

Insights

Isolated liver transplantation (OLT) can be a life-saving option for infants with intestinal failure (IF) and end-stage liver disease (ESLD), potentially leading to enteral sufficiency despite higher risks.

Area of Science:

  • Hepatology
  • Pediatric Surgery
  • Gastroenterology

Background:

  • Cholestasis leading to end-stage liver disease (ESLD) is a frequent complication in infants with intestinal failure (IF).
  • Isolated liver transplantation (OLT) is considered for IF patients anticipating eventual enteral feeding independence.

Purpose of the Study:

  • To review the institutional experience with OLT for ESLD in patients with residual IF.
  • To evaluate the outcomes and complications of OLT in this specific patient population.

Main Methods:

  • Retrospective review of four IF patients who underwent OLT for ESLD between 1998 and 2004.
  • Analysis of patient demographics, indications for OLT, surgical procedures, complications, and long-term outcomes, including enteral feeding status.

Main Results:

  • Two of the four OLT recipients achieved enteral sufficiency post-transplantation.
  • One patient remained on parenteral nutrition with partial enteral feeds, while another died from respiratory failure.
  • Biliary complications occurred in two patients, with one experiencing late hepatic artery thrombosis; both were successfully managed.

Conclusions:

  • OLT is a viable, emergent, life-saving procedure for IF patients with ESLD, despite associated higher morbidity due to comorbidities.
  • Improved liver function and reduced portal pressures post-OLT may facilitate enteral independence.
  • Careful management of parenteral nutrition after OLT can potentially prevent the need for small bowel transplantation in selected cases.