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Caroli's disease in two sisters. Diagnosis by ultrasonography and computed tomography.

Acta Radiol

March 1990

Department of Diagnostic Radiology, Sabbatsberg's Sjukhus, Stockholm, Sweden.

Caroli's disease is a rare congenital disease with multiple segmental dilatations of the bile ducts. It may present in two forms: one (the simple form) with segmental dilatations only, and the other with dilatations combined with fibroangioadenomatosis, frequently complicated by portal hypertension. The disease is thought to be recessively inherited, but few familial cases have been reported.

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