Purpose: Total parenteral nutrition (TPN) has prolonged survival in children with intestinal failure; however, end-stage liver disease owing to TPN-induced cholestasis (ESLD-TPN) may preclude its use. ESLD-TPN is an indication for isolated liver transplantation (ILT) or multivisceral transplantation (MVT). Isolated liver transplantation for ESLD-TPN should only be considered in patients who have the potential for enteral autonomy.
Methods: We retrospectively reviewed the records of patients with ESLD-TPN who underwent ILT (n = 7) or MVT (n = 5) between 1994 and 2005. The median age at the time of transplantation was 10.0 months. Intestinal failure followed necrotizing enterocolitis (n = 3), gastroschisis (n = 3), gastroschisis with volvulus (n = 3), gastroschisis with atresia (n = 1), malrotation (n = 1), and megacystis microcolon intestinal hypoperistalsis syndrome (n = 1).
Results: Isolated liver transplant patients had a median length of small bowel of 70 cm and tolerated a median of 50% of enteral calories. The median length of small bowel in patients who underwent MVT was 29 cm, and none tolerated more than 30% of goal enteral feeds. Reduced-size (n = 5) and whole-liver (n = 2) allografts were used for patients undergoing ILT. Patients undergoing MVT received liver-small bowel-pancreas (n = 4) or liver-small bowel-pancreas-colon (n = 1). Overall patient survival was 57% in ILT (median follow-up = 25.1 months); 3 survivors are TPN independent, and the fourth patient requires TPN 3 days/wk. Patient survival was 40% after MVT (median follow-up = 13.0 months); 1 MVT patient died of abuse 16.9 months after transplant and was TPN independent at the time of death. Both survivors are TPN independent. Bilirubin levels are within normal range in all survivors.
Conclusion: Isolated liver transplantation for ESLD-TPN in the setting of intestinal failure is a viable option in patients who have the potential for enteral autonomy. Multivisceral transplantation is the only alternative in patients without the potential for intestinal recovery. Survival can be achieved in patients with ESLD-TPN, but mortality remains high for both procedures.