Is Congenital Hepatic Fibrosis a Pure Liver Disease?

Yönem, Özlem; Ozkayar, Nihal; Balkancı, Ferhun; Harmancı, Özgür; Sökmensüer, Cenk; Ersoy, Osman; Bayraktar, Yusuf

doi:10.1111/j.1572-0241.2006.00642.x

Cited by 41 publications

(23 citation statements)

References 18 publications

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“…In the presence of congenital hepatic fibrosis and portal hypertension, it is often termed Caroli's syndrome [63,64] . US shows intrahepatic cystic anechoic areas in which fibrovascular bundles, stones and linear bridging or septum may be present [65] .…”

Section: Other Cystic Lesionsmentioning

confidence: 99%

Current management of noninfectious hepatic cystic lesions: A review of the literature

Macedo¹

2013

WJH

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Nonparasitic hepatic cysts consist of a heterogeneous group of disorders, which differ in etiology, prevalence, and manifestations. With improving diagnostic techniques, hepatic cysts are becoming more common. Recent advancements in minimally invasive technology created a new Era in the management of hepatic cystic disease. Herein, the most current recommendations for management of noninfectious hepatic cysts are described, thereby discussing differential diagnosis, new therapeutic modalities and outcomes. Key words: simple hepatic cyst; Noninfectious; Polycystic liver disease; Cystadenoma; Cystadenocarcinoma; Caroli's disease; sclerotherapy; Fenestration Core tip: Nonparasitic hepatic cysts consist of a broad spectrum of entities ranging from benign developmental cysts to malignant neoplasms. With recent advancements in diagnostic studies, hepatic cysts are becoming more frequent and better understanding of risk factors, management and long-term outcomes, and further development of current therapeutic modalities are needed.

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Section: Other Cystic Lesionsmentioning

confidence: 99%

Current management of noninfectious hepatic cystic lesions: A review of the literature

Macedo¹

2013

WJH

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“…In our study; between the time period 1971-2003 we followed-up 6 cases of Caroli's syndrome in whom, 2 patients had cavernomatous transformation of portal vein and 2 had polycystic kidney disease as an associated condition. One of these patients died due to sepsis and two of them underwent liver transplantataion because of the development of secondary biliary cirrhosis [14] . Over the past years there has been an increasing recognition of the association of congenital hepatic fibrosis with not only fibropolycystic disease family related diseases but also with abnormalities in other organs such as pulmonary fibrosis, congenital heart disease, pulmonary hypertension with arteriovenous fistula, cavernomatous transformation of the portal vein and with Joubert's syndrome [15][16][17] .…”

Section: Associated Conditionsmentioning

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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“…Among the patients with Caroli's syndrome; 2 patients had Cavernomatous transformation of portal vein and 2 had polycystic kidney disease as an associated condition. One of these patients died due to sepsis and two of them underwent liver transplantation because of the development of secondar y biliar y cirrhosis [16] .…”

Section: Associated Conditionsmentioning

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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Is Congenital Hepatic Fibrosis a Pure Liver Disease?

Abstract: In this prospective study, it seems that CHF is not a pure liver disease but rather a multiorgan disorder involving the brain, portal vein, kidneys, and bile ducts. In most cases, the clinical picture includes other organ involvement, rather than purely the liver parenchyma.

Cited by 41 publications

References 18 publications

Current management of noninfectious hepatic cystic lesions: A review of the literature

Current management of noninfectious hepatic cystic lesions: A review of the literature

Clinical characteristics of Caroli’s syndrome

Clinical characteristics of Caroli’s disease

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