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

Cholestasis progressing to end-stage liver disease (ESLD) is common in intestinal failure (IF) in infants. Isolated liver transplantation (OLT) is performed when eventual enteral sufficiency is expected. We reviewed our experience with OLT for ESLD in patients with residual IF. From 1998 to 2004, four IF patients underwent OLT for ESLD at our institution. Three were performed as UNOS status I for acute decompensation of chronic liver failure; one other with severe cholestasis with a living donor. The living donor recipient died within months after OLT of chronic respiratory failure despite normal liver function. One recipient remains on parenteral nutrition (PN) and continues to receive partial enteral feeds with normal liver function. Two other recipients became enterally sufficient after the OLT. Biliary complications occurred in two patients, one with late hepatic artery thrombosis. Resolution was achieved with serial balloon dilatations and the other by conversion from duct-to-duct anastomosis to a choledochoduodenostomy. The morbidity of OLT for this indication is higher than for others likely because of comorbidities like sepsis and pulmonary insufficiency. OLT is complicated further by the attempt to maximally preserve residual bowel length for the biliary reconstruction. OLT can be an emergent life-saving procedure in IF patients despite higher morbidity. Improved liver function and diminished portal pressures may shorten the time to enteral sufficiency. Management of parenteral nutrition after OLT can avoid ESLD and eliminate small bowel transplantation in selected patients.