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Isolated liver transplantation in pediatric short bowel syndrome: is there a role?
Ivan R Diamond1, Paul W Wales, David R Grant
1Group for the Improvement of Intestinal Function and Treatment (GIFT) and the Pediatric Academic Multi-organ Transplant Program, Division of General Surgery, The Hospital for Sick Children, Toronto, Canada M5G 1X8.
Insights
Isolated liver transplantation is a viable option for infants with parenteral nutrition-associated cholestasis (PNAC) and short bowel syndrome (SBS). Survival rates are promising, with many patients achieving independence from parenteral nutrition (PN).
Area of Science:
- Pediatric surgery
- Hepatology
- Gastroenterology
Background:
- Parenteral nutrition-associated cholestasis (PNAC) in infants with short bowel syndrome (SBS) presents a significant clinical challenge.
- The efficacy of isolated liver transplantation for PNAC in this population remains incompletely understood.
Purpose of the Study:
- To evaluate the outcomes of isolated liver transplantation in infants diagnosed with PNAC and SBS.
- To assess graft function, survival rates, and the potential for independence from parenteral nutrition post-transplant.
Main Methods:
- A retrospective descriptive study was conducted on infants who underwent isolated liver transplantation for PNAC and SBS.
- A comprehensive review of existing literature on similar cases was also performed.
Main Results:
- Three infants (7-13 months) with SBS and PNAC underwent isolated liver transplantation.
- Two patients showed good graft function post-transplant (41 and 58 months), with one experiencing mortality due to PNAC.
- Literature review identified 22 cases; overall survival was 77%, with 76% of survivors achieving PN independence.
Conclusions:
- Isolated liver transplantation can be considered an acceptable treatment for select infants with PNAC and SBS.
- The procedure offers potential for improved outcomes when further intestinal adaptation and reduced reliance on PN are anticipated.
Background:
The role of isolated liver transplantation in infants with parenteral nutrition-associated cholestasis (PNAC) associated with short bowel syndrome (SBS) is unclear.
Methods:
We performed a retrospective descriptive study of infants with PNAC and SBS who received an isolated liver transplant at our institution. Review of the literature was also performed.
Results:
Three infants (aged 7, 8, and 13 months) with SBS and PNAC received an isolated liver transplant. Etiology of SBS was necrotizing enterocolitis, gastroschisis, and volvulus. Two patients with pretransplant small bowel length of 40 and 80 cm, who were receiving 65% and 79% of intake enterally, demonstrated good graft function (bilirubin, 0 and 7 micromol/L) at 41 and 58 months posttransplant. Despite full tolerance of enteral feeds, both remain on parenteral nutrition (PN) (4 and 7 nights per week) for poor weight gain. One child with 25 cm of small bowel, who received 65% of energy enterally pretransplant, died 7 months posttransplant from PNAC. A literature review revealed 22 cases of isolated liver transplant for PNAC associated with SBS. Overall survival was 77%, with 76% of survivors demonstrating independence from PN.
Conclusions:
Isolated liver transplantation is an acceptable option for select infants with PNAC associated with SBS when further intestinal adaptation and freedom from PN are anticipated.
