Caroli disease and caroli syndrome are congenital disorders comprising of multifocal cystic dilatation of segmental intrahepatic bile ducts. Caroli disease carolis disease carolis syndrome caroli syndrome carolis disease carolis syndrome disease, caroli. Caroli initially described two variants, which has led to some confusion in terminology. In carolis syndrome, there is additionally an associated congenital hepatic fibrosis. Eales disease current concepts in diagnosis and management. Foi descrita primeiramente no ano de1958, em paris, pelo gastroenterologista jacques caroli.
Liver transplantation consequential to carolis syndrome. Aunque existen datos diferenciales entre ambas enfermedades renales. Carolis disease is an autosomal recessive disease characterized by nonobstructive dilatation of the intrahepatic bile ducts. Its incidence is extremely low 1 in 1,000,000 population and in most of the cases the whole liver is interested and liver transplantation is the treatment of choice. Caroli s syndrome is more common than cd and consists of multiple cystic or saccular dilatation of intrahepatic biliary ducts ihbd associated. Diffuse carolis disease with atypical presentation. Laparoscopic left liver sectoriectomy of carolis disease. Perla davidmurillo 1, samuel lagosrivera 1, daniel suazovillalobos 1, dr. 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. Abstract caroli disease is a nonobstructive dilatation of intrahepatic bile ducts.
Request pdf on oct 1, 2007, nk biswas and others published carolis disease find, read and cite all the research you need on researchgate. The more common variant is caroli syndrome in which bile duct dilatation is associated with congenital hepatic fibrosis. 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. Diagnosis is mostly clinical and requires exclusion of other systemic or ocular conditions that could present with similar retinal features. In case of dilatation limited to the left or right lobe, liver resection can be performed.
Caroli s disease is defined as a abnormal dilatation of the intrahepatica bile ducts. Its incidence is extremely low 1 in 1,000,000 population, and comprises two entities, namely cd and caroli s syndrome. Aug 25, 2015 carolis disease is a rare congenital condition characterized by nonobstructive dilatation of intrahepatic ducts. Nine patients were identified, seven males and two females, with a median age of 40. Caroli disease is the less common form and is characterized by bile ductular ectasia without other apparent hepatic abnormalities.
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. Liver transplantation in patients with caroli s disease and cholangitis. Caroli s disease cd is an uncommon congenital malformation, first described by jacques caroli in 1958. 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.
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