Atlas of Gastroenterological Endoscopy
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Caroli-syndrome
Caroli-syndrome Caroli-syndrome
Incomplete Caroli´s syndrome confined to the left hepatic duct. The disease manifested with jaandice at the age of 28. The left hepatic duct is dilated sac- like on the left x-ray after extraction of multiple stones. Right: The left hepatic duct is still considerably dilated after 6 weeks. Liver biopsy fails to show fibroadenomatous changes. Classic Caroli´s syndrome is a rare autosomal- recessive disorder with sac- like, segmental dilatation of the biliary tree with concomitant concrements. Hepatic blood vessels may be affected in the same way.