Cystic disease of the biliary tract.
Cystic disease of the biliary tract is rare in Western countries with an incidence of 1/2,000,000 live births. Five anatomical types are recognized. The majority (80%) become symptomatic before the age of 30 years. The cysts rarely have an intact epithelial lining and this often exhibits metaplasia. The risk of cancer developing in a choledochal cyst has been variously estimated at 2.5-28%. Malignancy is often associated with an abnormal pancreaticobiliary junction. Surgical treatment is indicated for types I-IV and is by excision rather than drainage. The treatment of Type V is difficult and should be conservative in the first instance. However, patients with severe symptomatic diffuse hepatic disease and progressive deterioration of liver function are best managed by hepatic transplantation.
Cuschieri
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Department of Surgery, Ninewells Hospital and Medical School, University of Dundee, Scotland, U.K.
Byrne
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D000328:Adult; D001660:Biliary Tract Diseases / Q000453:epidemiology / Q000601:surgery; D002648:Child; D003560:Cysts / Q000453:epidemiology / Q000601:surgery; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male