The Turkish Journal of Pediatrics 2003 , Vol 45 , Num 1
Byler's disease and anesthetic consideration

Department of Anesthesiology and Reanimation, Hacettepe University Faculty of Medicine, Ankara, Turkey


Bylers disease is an autosomal recessive condition characterized by intrahepatic cholestasis, progressive fibrotic changes and finally cirrhosis that leads to death during childhood. This is a report of a six-year-old girl with Bylers disease and retrobulbar hematoma as a result of trauma who underwent enucleation and implantation. This case report describes the anesthetic features of a patient with Bylers disease in which anesthetic agents with no or minimal hepatotoxic effect should be used to avoid deterioration of liver function.

Keywords : Byler's disease familial intrahepatic cholestasis general anesthesia
Copyright © 2016