Congenital Budd-Chiari Syndrome

Budd-Chiari syndrome, due to thrombotic occlusion of hepatic veins or ostia, is unusual in infants. Review of the literature reveals no documented case symptomatic at birth. We describe a male infant with ascites present at birth. A liver biopsy at 7 weeks of age showed sinusoidal dilatation, but the diagnosis of Budd-Chiari syndrome was made only at autopsy, 4 months later. The cause was not determined although maternal drug abuse was suspected.