Avoiding Liver Transplantation in Progressive Familial Intrahepatic Cholestasis: Role of Partial Biliary Diversion

Abstract

Progressive familial intra-hepatic cholestasis (PFIC) is a rare cholestatic disorder affecting children often resulting in end stage liver failure. Patients usually present with unremitting pruritus as the most distressing symptoms in addition to jaundice. Its treatment is difficult and varies from medical therapy which is often only symptomatic and provides only brief relief. Non transplant surgical options aim at interrupting the entero-hepatic bile salt circulation and thereby palliating symptoms, delaying disease progression. There are however few long term reports regarding the results of these biliary diversion procedures. We report six cases of PFIC who did not respond to medical therapy but had normal liver and underwent partial biliary diversion, at 5 months, 8 months, 13 months 18 months, 22 months and 2 years of age. Four patients had PFIC type 2, all of whom underwent partial internal biliary diversion (PIBD) and two patients had PFIC type 1 in whom we performed a partial external biliary diversion (PEBD) in one and partial internal biliary diversion in the other. One patient died at 9 months after surgery due to unrelated cause, 3 patients were lost to follow up. Remaining two patients are asymptomatic and doing well without need for Liver transplantation at long term follow up of 10 years. Internal diversion appears to have an advantage in terms of having better control of symptoms and quality of life without a stoma. In our experience, partial biliary internal diversion is a safe and easy procedure with good results in patients without fibrosis.