ORIGINAL ARTICLE An outstanding non-transplant surgical intervention in progressive familial intrahepatic cholestasis: partial internal biliary diversion F. Gu ¨n • B. Erginel • O ¨ . Durmaz • S. So ¨ku ¨cu ¨ • T. Salman • A. C ¸ elik Accepted: 8 June 2010 / Published online: 20 June 2010 Ó Springer-Verlag 2010 Abstract Aim Progressive familial intrahepatic cholestasis (PFIC) is a hereditary disease with severe cholestasis progressing to cirrhosis and chronic renal failure usually during the first decade. An alternative approach is partial diversion of bile. The aim of this study is to describe four patients with PFIC who underwent partial internal biliary diversion (PIBD). Methods Review of three patients, their clinical, labora- tory and histologic workups to evaluate the short-term effects of PFIC, a 1-year follow-up. For PIBD, a conduit is performed between the terminolateral side of the gall bladder and distal colon using a segment of jejunum, to divert the biliary flow from the enterohepatic cycle without any external stoma. Results All four patients were presented with jaundice, pruritus, hepatomegaly, sleep disturbance. They fulfilled the criteria for PFIC. The surgery was uneventful. At follow-up, biochemical parameters improved significantly, growth was regained, relief in pruritus, sleeping pattern was normalized. Conclusions Partial internal biliary diversion had a dra- matic effect on cholestasis, growth, sleeping and bio- chemical parameters. It also avoids the disadvantages of a permanent stoma. We believe that it is one of the best surgical procedures ever described for PFIC. Since long- term results of partial external biliary diversion on liver histopathology are successful, we hope that our long-term results will also be similar. Keywords Progressive familial intrahepatic cholestasis Á Biliary diversion Introduction Progressive familial intrahepatic cholestasis (PFIC) type 1 (Byler disease) is a rare autosomal recessive chole- static disease of childhood that becomes manifest in the first months of life. In *10–15 of jaundice in the first month of life, the cause is PFIC. The disease is char- acterized by the defects of the genes affiliated in bile transportation and affects the ability of the hepatocyte to transport bile salts into the biliary tree. Progressive hepatocellular cholestasis, subsequent retension and accumulation of bile salts with in the hepatocyte leads to progressive liver damage [1]. PFIC patients are mostly of European or Mediterranean descent [2]. Before 1990s, liver transplantation was the only therapeutic option. Patients with transplantation would continue to suffer morbidities associated with life-long immunosuppression. During the last 15 years, two alternative methods of surgical treatment have been proposed: partial biliary diversion (external or internal) and ileoileal by-pass procedure. The purpose of the both procedures was to reduce the reabsorption of bile acids from terminal ileum. The aim of this study is to evaluate the patients with PFIC who underwent partial internal biliary diver- sion (PIBD). F. Gu ¨n (&) Á B. Erginel Á O ¨ . Durmaz Á S. So ¨ku ¨cu ¨ Á T. Salman Á A. C ¸ elik Department of Pediatric Surgery, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey e-mail: gunferyal@yahoo.com O ¨ . Durmaz Á S. So ¨ku ¨cu ¨ Department of Pediatric Gastroenterology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey 123 Pediatr Surg Int (2010) 26:831–834 DOI 10.1007/s00383-010-2638-x