Introduction: Parenteral Nutrition Associated Liver Disease (PNALD) is a devastating complication commonly seen in patients with short gut syndrome (SGS). Most of these patients are referred for combined liver and intestinal transplantation, but in certain cases enteral adaptation might be achieved without the need of an intestinal transplant.

Methods: We performed a retrospective analysis of pediatric patients with IFALD who underwent an isolated liver transplant (LT).

Results: From 2003 to 20011 we performed 100 pediatric LTs. Six transplants were done for PNALD (5 cadaveric whole organs, 1 living donor graft). Median age was 11.6 months (9 -19). We had no vascular or biliary complications. Etiology of SGS was Necrotizing Enterocolitis in 4 cases and intestinal atresia in 2. Five patients are alive at a median follow-up of 5.7 years. Two patients were weaned off TPN in the perioperative period. Three patients required transient intravenous nutritional support achieving enteral sufficiency at a median of 101 days (range 65 to 150). One patient was never able to be weaned off TPN, requiring a multivisceral transplant 2.7 years after the original liver transplant. One patient required reestablishment of TPN 11 months after the LT and he subsequently died of sepsis 2.5 months later secondary to a central line infection.

Conclusions: Isolated LT for children with IFALD represents a reasonable strategy for selected patients who have good potential for intestinal rehabilitation. Failure to wean from TPN after liver transplantation was associated with a poor prognosis in our series.

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