Caroli disease communicating cavernous ectasia, or congenital cystic dilatation of the intrahepatic biliary tree is a rare inherited disorder characterized by cystic dilatation or ectasia of the bile ducts within the liver. The more common variant is caroli syndrome in which bile duct dilatation is associated with congenital hepatic fibrosis. Request pdf on oct 1, 2007, nk biswas and others published caroli s disease find, read and cite all the research you need on researchgate. Caroli s disease cd is an uncommon congenital malformation, first described by jacques caroli in 1958. Diffuse carolis disease with atypical presentation. Its incidence is extremely low 1 in 1,000,000 population, and comprises two entities, namely cd and caroli s syndrome. Liver transplantation consequential to carolis syndrome. Request pdf on oct 1, 2007, nk biswas and others published carolis disease find, read and cite all the research you need on researchgate. Aug 25, 2015 carolis disease is a rare congenital condition characterized by nonobstructive dilatation of intrahepatic ducts.
Caroli disease is limited to the dilatation of larger intrahepatic bile ducts, whereas caroli syndrome describes the combination of small bile ducts dilatation and congenital hepatic fibrosis however, some series show that extrahepatic duct. Aunque existen datos diferenciales entre ambas enfermedades renales. Foi descrita primeiramente no ano de1958, em paris, pelo gastroenterologista jacques caroli. Caroli disease is the less common form and is characterized by bile ductular ectasia without other apparent hepatic abnormalities. Caroli s disease is defined as a abnormal dilatation of the intrahepatica bile ducts. Caroli initially described two variants, which has led to some confusion in terminology. Jan 14, 20 eales disease, first described by the british ophthalmologist henry eales in 1880, is characterized by three overlapping stages of venous inflammation vasculitis, occlusion, and retinal neovascularization. Eales disease current concepts in diagnosis and management. Carolis disease is an autosomal recessive disease characterized by nonobstructive dilatation of the intrahepatic bile ducts.
Caroli disease carolis disease carolis syndrome caroli syndrome carolis disease carolis syndrome disease, caroli. Laparoscopic left liver sectoriectomy of carolis disease. Caroli disease and caroli syndrome are congenital disorders comprising of multifocal cystic dilatation of segmental intrahepatic bile ducts. In case of dilatation limited to the left or right lobe, liver resection can be performed. Perla davidmurillo 1, samuel lagosrivera 1, daniel suazovillalobos 1, dr. Abstract caroli disease is a nonobstructive dilatation of intrahepatic bile ducts. Nine patients were identified, seven males and two females, with a median age of 40. Caroli s syndrome is more common than cd and consists of multiple cystic or saccular dilatation of intrahepatic biliary ducts ihbd associated.
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