Partial Internal Biliary Diversion for Progressive Familial Intrahepatic Cholestasis: Clinical Outcomes in Three Cases.

Internal biliary diversion surgery is a promising treatment modality for relieving pruritus in patients with progressive familial intrahepatic cholestasis (PFIC). Other treatment modalities include medical management and liver transplantation. We report three children with PFIC and preserved synthetic liver function who underwent successful partial internal biliary diversion surgery at a tertiary care centre in North India. All three children had a good postoperative recovery with significant improvement in pruritus, as documented by the 5-D itch scale, associated with a reduction in serum bile acid levels over a median follow-up of 23 months. Therefore, in appropriately selected patients with PFIC, partial internal biliary diversion can result in favourable clinical outcomes, and potentially avoid or delay the need for liver transplantation.