Fatal Hemoperitoneum Due to Rupture of a Hepatic Vascular Malformation With Focal Absence of Hepatic Parenchyma: A Case Report

We describe an exceptionally rare case of fatal hemoperitoneum resulting from rupture of a hepatic vascular malformation associated with focal absence of hepatic parenchyma. A 60-year-old man with alcoholic cirrhosis died of intra-abdominal bleeding. Autopsy revealed a sharply depressed area on the dorsal surface of the right hepatic lobe. Histologically, this depression corresponded to a complete defect of hepatic parenchyma, replaced only by scant fibro-fatty tissue abutting large portal tracts. Prominent branches of the hepatic artery and portal vein were exposed at the base, accompanied by clusters of dilated CD31-positive vessels and D2-40-positive lymphatics. No tumor was identified. To our knowledge, this is among the first detailed descriptions of a congenital-type hepatic vascular anomaly characterized by focal parenchymal absence, which can culminate in catastrophic hemorrhage.