Open AccessMonolobar Form of Caroli’s Disease with Intrahepatic Stone (Clinical Case)
Author(s) -
Т. Н. Галян,
Н. С. Тарба,
В. В. Ховрин,
L O Polishchuk,
Н Н Багмет,
Elena P. Fisenko
Publication year - 2017
Publication title -
medicinskaâ vizualizaciâ
Language(s) - English
Resource type - Journals
eISSN - 2408-9516
pISSN - 1607-0763
DOI - 10.24835/1607-0763-2017-2-85-89
Subject(s) - congenital hepatic fibrosis , intrahepatic bile ducts , medicine , polycystic liver disease , differential diagnosis , disease , gastroenterology , dilation (metric space) , pathology , primary sclerosing cholangitis , portal hypertension , bile duct , liver transplantation , cirrhosis , transplantation , mathematics , combinatorics
Caroli’s disease is a rare inherited disorder characterized by nonobstructive saccular or fusiform dilation of the intrahepatic bile ducts. Two main types of Caroli’s disease are reported, the true type with isolated dilation of intrahepatic bile ducts of single liver segment with formation of stones and development of cholangitis. The second type is combined with a congenital hepatic fibrosis. The true type of Caroli’s disease is distinct from primary sclerosing cholangitis, polycystic disease, cholangiolithiasis and other diseases. This paper presents a literature review and a case of monolobar Caroli’s disease with intrahepatic stones. The difficulties of preoperative differential diagnosis are shown.