Long-term outcome after partial external biliary diversion for progressive familial intrahepatic cholestasis

BackgroundThough patients with progressive familial intrahepatic cholestasis (PFIC) typically require liver transplantation, initial surgical treatment includes partial biliary diversion (PBD) to relieve jaundice-associated pruritus. This study was undertaken to describe long-term PFIC outcome data, which are currently sparsely reported.MethodsRetrospective review of 7 patients diagnosed with PFIC who underwent PBD between 2004 and 2008 was directed toward long-term postoperative outcome including resolution of jaundice/pruritus, stoma complications, interval to transplantation, and death.ResultsSix patients who underwent PBD experienced short-term resolution of jaundice and pruritus. Four patients experienced persistent stoma-related complications requiring a total of 14 revisions. Three symptom-free patients have not yet required liver transplantation post-PBD (average, 70 months; range, 59-78 months). Two patients underwent orthotopic liver transplantation (average, 44 ± 18 months post-PBD). Two patients died at home because of gastroenteritis-associated dehydration before transplantation.ConclusionPartial biliary diversion for PFIC is effective as a bridge to liver transplantation in improving jaundice and pruritus but may be associated with a high incidence of stoma-related complications. Persistent or recurrent pruritus after PFIC is associated with an increased risk of stoma prolapse or reflux. Insufficiently replaced stomal losses over time may increase the risk of dehydration-related complications in association with gastroenteritis.