The anesthetic implications of Crigler-Najjar syndrome.

Abstract

Crigler-Najjar syndrome is a hereditary condition of unconjugated hyperbilirubinemia due to a deficiency of the enzyme, uridine diphosphate glucuronosyltransferase. Exacerbations of the disease can occur whenever there is either an increase in free serum bilirubin and/or a decrease in serum albumin. The exacerbations can lead to bilirubin encephalopathy and severe brain damage. The goal of anesthetic management in these patients is to prevent an imbalance in the serum bilirubin to serum albumin molar ratio, thereby avoiding neurologic sequelae.