[Anesthesia in intrahepatic cholestasis: Alagille syndrome]

A 42-year-old patient, diagnosed with Alagille syndrome when he was 1 month old, was admitted to the general surgery department to undergo right hepatectomy for right lobe carcinoma. Alagille syndrome, also known as biliary duct hypoplasia, is an extremely rare genetic condition with multiorganic implications. Liver and heart dysfunction will play key roles in the course of the disease and its prognosis. An exhaustive preoperative assessment of liver and heart function, blood chemistry and the skeleton will be essential for managing these patients. This article reviews the main principles underlying the anesthetic management of patients with Alagille syndrome.