Cholecysto-appendicostomy as partial internal biliary drainage in Progressive Familial Intrahepatic Cholestasis Type 1: A case report and review of literature

• PFIC required liver transplant before the consideration of biliary diversion.
• Children with PFIC often have poor quality of life.
• Option for biliary diversion is either external (PEBD) or internal (PIBD).
• PIBD has less complications than PEBD.
• Cholecysto-appendicostomy involves only single anastomosis with promising outcome.

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 (cholecysto-appendicostomy).