Congenital dilatation of the large and segmental intrahepatic bile ducts (Caroli's disease) in two Golden retriever littermates : clinical communication

Two, sibling, male Golden retriever puppies, 13 weeks of age, were presented with congenital biliary cysts of the liver involving both hepatic and segmental bile ducts, as well as bilateral polycystic kidney disease. Ultrasonography of the livers of both pups demonstrated segmental cystic lesions that were contiguous with the bile ducts. Histopathology revealed cystic ectatic bile duct hyperplasia and dysplasia with variable portal fibrosis in the liver, while in the kidneys there were radially arranged, cylindrically dilated cysts of the collecting ducts, which extended through the medulla and cortex. This pathology was compatible with that of congenital dilatation of the large and segmental bile ducts (Caroli's disease) described in humans, dogs and rats. In humans Caroli's disease has an autosomal recessive inheritance pattern, while in rats activation of the MEK5/ERK cascade initiates the biliary dysgenesis of Caroli's disease in this species. However, the exact mode of inheritance and pathogenesis of Caroli's disease in dogs is as yet unknown. Previous reports on congenital hepatic cystic diseases of the dog have described Caroli's disease like lesions in various breeds, but these are believed to be the 1st reported cases in the Golden retriever breed.

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Congenital cystic dilation of the intrahepatic bile ducts (Caroli's disease): Report of a case and review of the literature

Journal of Pediatric Surgery ◽

10.1016/s0022-3468(79)80335-8 ◽

1979 ◽

Vol 14 (6) ◽

pp. 863

Author(s):

Eugene S. Wiener

Keyword(s):

Bile Ducts ◽

Review Of The Literature ◽

Caroli's Disease ◽

Caroli’S Disease ◽

Intrahepatic Bile Ducts

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Diffuse Oligosymptomatic Caroli’s Disease: Case Report

Bangladesh Journal of Medicine ◽

10.3329/bjm.v31i2.48540 ◽

2020 ◽

Vol 31 (2) ◽

pp. 107-110

Author(s):

Lemfadli Y ◽

Bouchrit S ◽

Ait Errami A ◽

Oubaha S ◽

Samlani Z ◽

...

Keyword(s):

Case Report ◽

Female Patient ◽

Bile Ducts ◽

Clinical Case ◽

Caroli's Disease ◽

Caroli’S Disease ◽

Caroli Disease ◽

Intrahepatic Bile Ducts ◽

Disease Case

This article describes a case of Caroli’s disease in a 53-year-old female patient who complained nonspecificabdominal pain without cholestasis or cholangitis. Ultrasound and hepatic magnetic resonanceimaging showed segmental saccular dilations connected to intrahepatic bile ducts without hepaticfibrosis. This clinical case shows the possibility of having oligosymptomatic forms in the diffuse formsof Caroli disease, therefore the interest to consider this diagnosis in case of non-specific abdominalsigns and to request a hepatic ultrasound. Bangladesh J Medicine July 2020; 31(2) : 107-110

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Congenital Dilatation of the Intrahepatic Bile Ducts (Caroli's Disease)

Radiology ◽

10.1148/127.3.746 ◽

1978 ◽

Vol 127 (3) ◽

pp. 746-746 ◽

Cited By ~ 13

Author(s):

Javier Lucaya ◽

Jose Luis Gomez ◽

Ceferino Molino ◽

Juan Gonzalez Atienza

Keyword(s):

Bile Ducts ◽

Caroli's Disease ◽

Caroli’S Disease ◽

Intrahepatic Bile Ducts

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Caroli's disease (clinical observation)

Journal Infectology ◽

10.22625/2072-6732-2021-13-3-150-152 ◽

2021 ◽

Vol 13 (3) ◽

pp. 150-152

Author(s):

G. G. Kuttykuzhanova ◽

A. Zh. Tanirbergenova ◽

S. Zh. Abdirazakova ◽

Z. Zh. Urikbaeva

Keyword(s):

Early Diagnosis ◽

Rare Diseases ◽

Medical Practice ◽

Bile Ducts ◽

Clinical Observation ◽

Diagnostic Methods ◽

Caroli's Disease ◽

Caroli’S Disease ◽

Intrahepatic Stones ◽

Intrahepatic Bile Ducts

In their medical practice doctors deal with rare diseases which present difficulties in making a clinical diagnosis, in this case additional diagnostic methods are required. One of these diseases is Caroli's disease (syndrome), characterized by cystic enlargement of the intrahepatic bile ducts with frequent formation of intrahepatic stones. Taking into account the difficulties of early diagnosis, we present our own observation.

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Monolobar Form of Caroli’s Disease with Intrahepatic Stone (Clinical Case)

Medical Visualization ◽

10.24835/1607-0763-2017-2-85-89 ◽

2017 ◽

pp. 85-89

Author(s):

T. N. Galyan ◽

N. S. Tarba ◽

V. V. Khovrin ◽

L. O. Polizhuk ◽

N. N. Bagmet ◽

...

Keyword(s):

Sclerosing Cholangitis ◽

Bile Ducts ◽

Clinical Case ◽

Caroli's Disease ◽

Liver Segment ◽

Caroli’S Disease ◽

Intrahepatic Stones ◽

Intrahepatic Bile Ducts ◽

Polycystic Disease ◽

True Type

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.

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Matrix proteins of basement membrane of intrahepatic bile ducts are degraded in congenital hepatic fibrosis and Caroli's disease

The Journal of Pathology ◽

10.1002/path.2472 ◽

2009 ◽

Vol 217 (3) ◽

pp. 442-451 ◽

Cited By ~ 24

Author(s):

Mitsue Yasoshima ◽

Yasunori Sato ◽

Shinichi Furubo ◽

Kazuo Kizawa ◽

Takahiro Sanzen ◽

...

Keyword(s):

Basement Membrane ◽

Hepatic Fibrosis ◽

Bile Ducts ◽

Matrix Proteins ◽

Congenital Hepatic Fibrosis ◽

Caroli's Disease ◽

Caroli’S Disease ◽

Intrahepatic Bile Ducts

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Monolobar Caroli's Disease and Cholangiocarcinoma

HPB Surgery ◽

10.1155/1999/70985 ◽

1999 ◽

Vol 11 (4) ◽

pp. 271-277 ◽

Cited By ~ 31

Author(s):

Eddie K. Abdalla ◽

Christopher E. Forsmark ◽

Gregory Y. Lauwers ◽

J. Nicolas Vauthey

Keyword(s):

Biliary Tract Cancer ◽

Bile Ducts ◽

Cystic Dilatation ◽

Endoscopic Retrograde ◽

Caroli's Disease ◽

Caroli’S Disease ◽

Tract Cancer ◽

Intrahepatic Bile Ducts ◽

Rare Congenital Disorder ◽

One Year

Caroli's Disease (CD) is a rare congenital disorder characterized by cystic dilatation of the intrahepatic bile ducts. This report describes a patient with cholangiocarcinoma arising in the setting of monolobar CD. In spite of detailed investigations including biliary enteric bypass and endoscopic retrograde cholangiography, the diagnosis of mucinous cholangiocarcinoma (CCA) was not made for almost one year. The presentation, diagnosis and treatment of monolobar CD and the association between monolobar CD and biliary tract cancer are discussed. Hepatic resection is the treatment of choice for monolobar CD.

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Adenocarcinoma in Caroli's Disease Treated by Liver Transplantation

HPB Surgery ◽

10.1155/1993/61048 ◽

1993 ◽

Vol 7 (1) ◽

pp. 81-87 ◽

Cited By ~ 16

Author(s):

J. Balsells ◽

C. Margarit ◽

E. Murio ◽

J. L. Lazaro ◽

R. Charco ◽

...

Keyword(s):

Liver Transplantation ◽

Bile Ducts ◽

Normal Liver ◽

Abdominal Ultrasound ◽

Papillary Adenocarcinoma ◽

Pathological Examination ◽

Caroli's Disease ◽

Caroli’S Disease ◽

Intrahepatic Bile Ducts ◽

First Case

Caroli's disease is characterized by congenital cystic dilatation of the intrahepatic bile ducts. In 7% of casea a malignant tumor develops complicating the course of the disease.We report the case of a 25 year-old woman in whom Caroli's disease was diagnosed at the age of 11. From that time on, she had several episodes of cholangitis. In 1989, the abdominal ultrasound and CT scan showed dilatation of the intrahepatic bile ducts, intracystic lithiasis and a solid mass. FNA cytology showed a papillary adenocarcinoma. At laparotomy a tumor was found occupying both hepatic lobes, and intraoperative US showed another two nodules in the left lobe. The tumor was considered unresectable. Examination of the hilar lymph nodes was tumor-negative. Two weeks later, the patient underwent an ortothopic liver transplantation (OLT). The pathological examination confirmed Caroli's disease with adenocarcinoma. Two years after OLT, the patient is alive with normal liver function and no evidence of disease.To our knowledge this is the first case report of adenocarcinoma in Caroli's disease treated by OLT.

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