Nodular regenerative hyperplasia of the liver. A review of 14 cases.

BACKGROUND/AIMS: Nodular regenerative hyperplasia of the liver, is a noncirrhotic liver disease, characterized by nodules in the hepatic parenchyma, which clinically presents primarily with manifestations of portal hypertension. The aims of this study are i) to review the clinical, histological and diagnostic aspects of 14 documented cases of NRHL, and ii) to assess the evolution and management of this condition in the cases reviewed.
METHODOLOGY: The diagnosis of nodular regenerative hyperplasia of the liver was based on liver biopsy in all cases. Imaging studies (ultrasonography, computed tomography scan and magnetic resonance imaging scan) were performed as part of the diagnostic evaluation. Clinical manifestations and biochemical tests were recorded at the time of diagnosis. Management and prognosis were also reviewed.
RESULTS: The most common clinical manifestations were those of portal hypertension, namely splenomegaly, esophageal varices and variceal bleeding. The histological findings were nodules in the hepatic parenchyma, the typical histologic feature of nodular regenerative hyperplasia of the liver, with mild periportal fibrosis and intraportal lymphocytic infiltration. Biochemical tests showed normal synthetic liver function, as evidenced by normal serum albumin, bilirubin and prothrombin time. Elevation of gamma-glutamyl transpeptidase and alkaline phosphatase due to cholestasis was noted. Management was directed to portal hypertension and variceal bleeding, with beta-blockers, sclerotherapy, mesenteric-caval shunt and transjugular intrahepatic portosystemic shunt with satisfactory results.
CONCLUSIONS: Nodular regenerative hyperplasia of the liver is an uncommon condition but it should be considered in patients with unexplained portal hypertension and distinguished from liver cirrhosis, in view of the differences in the natural history and prognosis. Liver biopsy confirms the diagnosis. Management is directed primarily to portal hypertension and variceal bleeding, which is the main source of mortality. Liver failure is uncommon due to satisfactory preservation of liver function.