Cholecysto-appendicostomy as partial internal biliary drainage inProgressive Familial Intrahepatic Cholestasis Type 1: A case reportand review of literature

Intractable pruritus secondary to bile salts retention in Progressive Familial Intrahepatic Cholestasis (PFIC) can be relieved surgically by diverting bile drainage from ileum to reduce bile salts reabsorption into entero-hepatic circulation. We are reporting on the successful biliary diversion in a child with PFIC, with the use of the appendix as a conduit to drain bile from gallbladder to the colon (cholecystoappendicostomy). 2016 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Progressive Familial Intrahepatic Cholestasis (PFIC) is an autosomal recessive disorder, typically presented during infancy with jaundice, pruritus and failure to thrive [1]. It is characterized by impaired bile acid metabolism and excretion causing intrahepatic cholestasis [1,2]. The concentrated biliary compounds lead to disabling pruritus and hepatocellular damage, subsequently progresses to liver failure and death in early childhood or adolescence [2,3]. Mutation in the ATP8B1 gene, a regulator of intestinal bile acid absorption, has been identified in PFIC Type 1, also known as Byler disease [4]. Before 1990, liver transplant (LTX) was the only treatment option for PFIC [2]. However, in the past 2 decades, surgical intervention with biliary diversion (BD) had been considered for symptomatic relief and was found to curtail liver impairment, and delaying the need for LTX [2,3]. In this report, we share our experience of cholecysto-appendicostomy (CA, a partial internal biliary