Adult presentation of Caroli's syndrome treated with orthotopic liver transplantation

Thomas D. Schiano, M. Isabel Fiel, Charles M. Miller, Henry C. Bodenheimer, Albert D. Min

Research output: Contribution to journalArticlepeer-review

31 Scopus citations

Abstract

When Caroli's disease, defined as a congenital dilation and ectasia of segmental intrahepatic bile ducts in the absence of other histological abnormalities, is associated with periportal fibrosis, it is termed Caroli's syndrome. We describe the case of a 35-yr-old woman with Caroli's syndrome without clinical manifestation of portal hypertension despite diffuse involvement of the liver who was successfully treated with orthotopic liver transplantation after recurrent nearly fatal episodes of cholangitis.

Original languageEnglish
Pages (from-to)1938-1940
Number of pages3
JournalAmerican Journal of Gastroenterology
Volume92
Issue number10
StatePublished - Oct 1997

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