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Caroli's syndrome is a rare disease, six cases of which were under the authors' care between 1979 and 1986. None had associated congenital hepatic fibrosis; one had an associated choledochal anomaly. Endoscopic retrograde cholangiography proved to be the most sensitive diagnostic method. Liver abscess and secondary biliary cirrhosis of the liver developed as complications. Partial liver resection was possible in two patients in whom only one lobe had been affected.
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