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Multiple comet tail artifacts in the liver: a case of congenital hepatic fibrosis showing unusual biliary appearance

Congenital hepatic fibrosis (CHF) is a form of autosomal recessive polycystic kidney disease. Because of the common underlying pathophysiology of ductal plate malformation, CHF can be accompanied by an abnormal biliary appearance, which is characterized by a saccular or fusiform dilatation of the bi...

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Bibliographic Details
Published in:Journal of medical ultrasonics (2001) 2014-07, Vol.41 (3), p.377-380
Main Authors: Kuwano, Michiko, Maruyama, Hitoshi, Kondo, Takayuki, Sekimoto, Tadashi, Shimada, Taro, Yamamoto, Shuichi, Tuyuguchi, Toshio, Yokosuka, Osamu, Nomura, Fumio
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Language:English
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Summary:Congenital hepatic fibrosis (CHF) is a form of autosomal recessive polycystic kidney disease. Because of the common underlying pathophysiology of ductal plate malformation, CHF can be accompanied by an abnormal biliary appearance, which is characterized by a saccular or fusiform dilatation of the bile ducts. We encountered the case of a 35-year-old man suffering from CHF concomitant with esophageal varices, which were treated by endoscopic sclerotherapy. The patient had elevated serum concentrations of alkaline phosphatase and γ-glutamyl transpeptidase without apparent biliary disease, including hepatolithiasis or a history of cholangitis. Magnetic resonance cholangiography showed an abnormal biliary appearance, which was not saccular or fusiform but had multiple stenosis with unknown causes. B-mode sonogram showed multiple comet tail artifacts in the liver parenchyma, probably corresponding to the compact fibrosis bands and bile in the bile duct as well as peripheral bile duct dilatation, which was proven pathologically. We propose that multiple comet tail artifacts in the liver may suggest the presence of a bile duct abnormality in patients with CHF, suggesting the potential risk for developing biliary complications.
ISSN:1346-4523
1613-2254
DOI:10.1007/s10396-014-0519-y