Congenital hepatic fibrosis associated with Cruveilhier-Baumgarten syndrome.

概要

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32 years old male was admitted to the Toho University Hospital with the chief complaint of hematemesis and was diagnosed as rupture of esophageal varices. No liver disease was found in his past or family history. Laboratory findings showed no hepatic disoder. Various imaging diagnostics revealed hepatosplenomegaly and an ascending collateral running from the umbilical portion into intrathoracic veins.<BR>Moreover, he had C-B syndrome, manifesting a continuous venous hum, which was the strongest point at the xiphoid process. By the diagnosis of ideopathic portal hypertension (IPH), esophageal transection and splenectomy were performed. However, histopasological findings revealed the development of fibrous septum containing many dilated bile ducts. The liver lobules were otherwise normal, which was the feature of congenital hepatic fibrosis (CHF). This case did not show polycystic kidney, and histopathological findings alone was a finding to discriminate it from IPH.<BR>Only 31 cases has been reported in Japan, including our case, and this was the 3rd case complicated with C-B syndrome in the world literature.
社団法人日本肝臓学会の論文