Caroli’s disease

Gupta, Ashish; Gupta, Anuj; Bhardwaj, Vaishali; Chansoria, M

doi:10.1007/bf02825490

Cited by 27 publications

(22 citation statements)

References 12 publications

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“…More than 200 cases of Caroli's disease have been reported in the literature [3] and the incidence of Caroli's syndrome is more than the pure form of Caroli's disease [6] .…”

Section: Epidemiologymentioning

confidence: 99%

Clinical characteristics of Caroli’s syndrome

Yönem

Bayraktar²

2007

WJG

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Caroli's syndrome is characterized by multiple segmental cystic or saccular dilatations of intrahepatic bile ducts associated with congenital hepatic fibrosis. The clinical features of this syndrome reflect both the characteristics of congenital hepatic fibrosis such as portal hypertension and that of Caroli's disease named as recurrent cholangitis and cholelithiasis. The diagnosis depends on both histology and imaging methods which can show the communication between the sacculi and the bile ducts. Treatment consists of symptomatic treatment of cholangitis attacks by antibiotics, some endoscopic, radiological and surgical drainage procedures and surgery. Liver transplantation seems the ultimate treatment for this disease. Prognosis is fairly good unless recurrent cholangitis and renal failure develops.

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“…More than 200 cases of Caroli's disease have been reported in the literature [3] and the incidence of Caroli's syndrome is more than the pure form of Caroli's disease [6] .…”

Section: Epidemiologymentioning

confidence: 99%

Clinical characteristics of Caroli’s syndrome

Yönem

Bayraktar²

2007

WJG

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“…If the defective remodelling involves the larger intrahepatic ducts Caroli's disease develops, but when the entire intrahepatic bilary tree is involved the condition is Caroli's syndrome [15] . Incidence of Caroli's syndrome is more than Caroli's disease [2] . In addition, various renal disorders may be ssen in conjunction with these hepatic diseases, including autosomal polycystic kidney disease (both dominant and recessive forms), medullary sponge kidney and medullary cystic disease [7] .…”

Section: Associated Conditionsmentioning

confidence: 99%

“…Mode of inheritance is still unclear but in majority of cases it is transmitted in autosomal recessive fashion [2] . Review of the literature suggests that there are no large series of this disease [3] .…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics of Caroli’s disease

Yönem

Bayraktar²

2007

WJG

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Caroli's disease is a rare congenital condition characterized by non-obstructive saccular or fusiform dilatation of larger intrahepatic bile ducts. Cholangitis, liver cirrhosis, and cholangiocarcinoma are its potential complications. The diagnosis of Caroli's disease depends on demonstrating that the cystic lesions are in continuity with the biliary tree which can be showed by ultrasonography, computerized tomography, endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography or magnetic resonance cholangiopancreatography. Treatment of Caroli's disease relies on the location of the biliary abnormalities. While localized forms confined to one lobe can be treated with surgery, liver transplantation is the only effective modality for diffuse forms. Although a rare disorder; Caroli's disease should always be considered in the differential diagnosis of chronic cholestasis of unknown cause.

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“…Most patients first present in the second or third decades of life 5 with CS being more common than CD in the majority of case series. 20 Patients with CD/CS are prone to hepatolithiasis, and this may lead to obstructive jaundice or acute pancreatitis, but their most common manifestation is recurrent cholangitis. Hepatic abscesses and/or sepsis can complicate the clinical course.…”

Section: Discussionmentioning

confidence: 99%