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.