MULTIPLE FOCAL NODULAR HYPERPLASIA OF THE LIVER ASSOCIATED WITH HEMIHYPERTROPHY AND VASCULAR MALFORMATIONS

A case of multiple focal nodular hyperplasia of the liver occurring in a 22-year-old woman with musculoskeletal hemihypertrophy and anomalou s vascular supply to the liver is described. The patient had Klippel-T renaunay-Weber syndrome and abdominal pain and tender massive hepatome galy. Visceral angiography showed marked dilatation of the celiac axis and both the main trunk and peripheral branches of the hepatic artery . Large abdominal veins drained from the dome of the liver into the he patic veins. The vascular anomalies were evident on contrast-enhanced computed tomography and magnetic resonance imaging. Multiple focal nod ular hyperplasia was confirmed by laparoscopic liver biopsy. The findi ngs in this patient support the concept that multiple focal nodular hy perplasia characteristically occurs in a syndromic form and is induced by an irregular arterial supply in the liver, with localized hyperfus ion that leads to nodular areas of hepatocyte hyperproliferation.