Esophagogastric variceal bleeding as debut of Caroli's syndrome

Caroli's disease is a congenital disease characterized by saccular or cystic dilatations of intrahepatic bile ducts. It is called Caroli's syndrome (CS) when intrahepatic dilations are associated with congenital hepatic fibrosis. CS is an infrequent cause of gastrointestinal bleeding in yo...

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Bibliographic Details
Published inRevista española de enfermedades digestivas Vol. 114; no. 11; p. 678
Main Authors Izquierdo, Julia del Rio, Belinchon, Clara Ramos, Cahizares, Rafael Bahares, Ma
Format Journal Article
LanguageEnglish
Spanish
Published Spain Sociedad Espanola de Patologia Digestivas 01.11.2022
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Summary:Caroli's disease is a congenital disease characterized by saccular or cystic dilatations of intrahepatic bile ducts. It is called Caroli's syndrome (CS) when intrahepatic dilations are associated with congenital hepatic fibrosis. CS is an infrequent cause of gastrointestinal bleeding in young people that should be considered when saccular dilatations of the bile duct are associated with portal hypertension (PHT). We present a rare case with gastrointestinal bleeding as first manifestation of CS: A 13-year-old woman with no relevant medical history was admitted to the hospital with an episode of hematemesis.
ISSN:1130-0108
DOI:10.17235/reed.2022.8813/2022