Caroli Disease (Caroli's Disease)

Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease.

Also Known As:

Caroli's Disease; Caroli's Syndrome; Caroli Syndrome; Carolis Disease; Carolis Syndrome; Disease, Caroli; Disease, Caroli's; Syndrome, Caroli's

Networked: 152 relevant articles (3 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Cholangitis
2.	Carcinoma (Carcinomatosis)
3.	Cysts
4.	Congenital Hepatic Fibrosis
5.	Autosomal Recessive Polycystic Kidney (Polycystic Kidney Disease, Infantile, Type I)

Experts

1.	Harada, Kenichi: 3 articles (08/2021 - 02/2009)
2.	Sato, Yasunori: 3 articles (08/2021 - 02/2009)
3.	Sasaki, Motoko: 2 articles (08/2021 - 10/2018)
4.	Yamamura, Minako: 2 articles (08/2021 - 10/2018)
5.	Goldberg, Itzhak D: 2 articles (06/2020 - 04/2019)
6.	Li, Jingsong: 2 articles (06/2020 - 04/2019)
7.	Settmacher, U: 2 articles (04/2019 - 09/2002)
8.	Rawat, Dinesh: 2 articles (08/2016 - 08/2013)
9.	Lerut, Jan: 2 articles (01/2016 - 05/2006)
10.	Enne, Marcelo: 2 articles (01/2011 - 01/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Caroli Disease:

1.	BilirubinIBA 05/01/2010 - "The intrahepatic bile ducts were dilated (Caroli's disease), and showed small calcium bilirubinate hepatoliths. " 09/01/1996 - "The spiral CT was not diagnostic in 2 patients: a case of choledocal cyst, the study was of poor quality due to inadequate sedation and a case of Caroli's disease with a high serum bilirubin level. " 05/01/2010 - "Microscopically, the intrahepatic bile duct showed non-obstructive segmental dilations (Caroli's disease), numerous peribiliary cysts, numerous ductal plate malformations, proliferation of intrahepatic peribiliary glands, and calcium bilirubinate hepatolithiasis. " 10/20/2020 - "The total bilirubin [6.7 (4.7, 15.0) vs 16.0(10.9, 33.0)μmol/L] and direct bilirubin [1.3(0.9,6.4)vs 3.5(2.7, 16.2)μmol/L] were significantly lower in Caroli disease group in comparison to those in Caroli syndrome group(both P<0.05). "
2.	Collagen Type IV (Type IV Collagen)IBA 02/01/2009 - "To clarify the involvement of basal laminar components in the ductal plate malformation, this study examined the immunohistochemical expression of laminin and type IV collagen in the livers of CHF and Caroli's disease. " 02/01/2009 - "Immunostaining showed that basement membrane expression of laminin and type IV collagen around intrahepatic bile ducts was degraded in CHF, Caroli's disease, and the PCK rats. " 02/01/2009 - "The degradation of laminin and type IV collagen around bile ducts was also observed in foci of cholangiocarcinoma in situ of Caroli's disease. "
3.	Meglumine FDA Link 12/01/1992 - "Computed tomography (CT) with meglumine lotroxinate (biliscopin) infusion study and hepatobiliary scintigraphy confirmed the diagnosis of Caroli's disease. "
4.	Laminin (Merosin)IBA 02/01/2009 - "To clarify the involvement of basal laminar components in the ductal plate malformation, this study examined the immunohistochemical expression of laminin and type IV collagen in the livers of CHF and Caroli's disease. " 02/01/2009 - "Immunostaining showed that basement membrane expression of laminin and type IV collagen around intrahepatic bile ducts was degraded in CHF, Caroli's disease, and the PCK rats. " 02/01/2009 - "The degradation of laminin and type IV collagen around bile ducts was also observed in foci of cholangiocarcinoma in situ of Caroli's disease. "
5.	meglumine iotroxinate (SH 273)IBA 12/01/1992 - "Computed tomography (CT) with meglumine lotroxinate (biliscopin) infusion study and hepatobiliary scintigraphy confirmed the diagnosis of Caroli's disease. "
6.	Proteins (Proteins, Gene)FDA Link 02/01/2009 - "Matrix proteins of basement membrane of intrahepatic bile ducts are degraded in congenital hepatic fibrosis and Caroli's disease." 08/01/2005 - "Congenital hepatic fibrosis whether it is accompanied by bile duct dilatations (Caroli's syndrome) or not, may be associated with autosomal recessive polycystic kidney disease, which is caused by mutations in PKHD1 that encodes fibrocystin, a protein of primary cilia. " 10/01/2018 - "An immunohistochemical analysis using liver sections confirmed the overexpression of Smoothened and cyclin D1, and the nuclear expression of the Gli proteins in the biliary epithelium of PCK rats as well as human Caroli disease. "
7.	CholesterolIBA 01/01/1981 - "A patient with the Caroli syndrome and multiple intrahepatic and extrahepatic cholesterol stones." 06/01/1979 - "A case of monolobar localization of Caroli's disease with intrahepatic cholesterol gallstones is described. " 12/31/2009 - "hepatocellular carcinoma or liver metastasis) or infectious diseases; (3) For differentiation diagnosis between biliary cystadenoma and biliary cystadenocarcinoma; (4) To detect malignant change in Caroli's disease; (5) To depict the extent of Klatskin's tumor with greater clarity; (6) To make a distinction between gallbladder cholesterol polyp, adenoma and polypoid cancer; (7) To make a distinction between chronic cholecystitis with thickened wall and gallbladder cancer; (8) For differentiation diagnosis between motionless sludge and gallbladder cancer; (9) For differentiation diagnosis between common bile duct cancer and sludge or stone without acoustic shadowing; and (10) In patients who are suspected of having a drop of their percutaneous transhepatic cholangiodrainage tube, US contrast agent can be administered to through the tube detect the site of the tube."
8.	Bile Acids and Salts (Bile Acids)IBA 03/01/1999 - "The role of therapeutic endoscopy associated with extracorporeal shock-wave lithotripsy and bile acid treatment in the management of Caroli's disease." 08/01/1998 - "The role of therapeutic endoscopy associated with extracorporeal shock-wave lithotripsy and bile acid treatment in the management of Caroli's disease." 01/01/2020 - "These include diseases such as biliary atresia, Alagille syndrome, progressive intrahepatic cholestasis entities, ductal plate abnormalities including Caroli syndrome and congenital hepatic fibrosis, primary sclerosing cholangitis, bile acid synthesis defects, and certain metabolic disease. "
9.	Anti-Bacterial Agents (Antibiotics)IBA 12/01/1992 - "As far as we know, this is the first reported case of ABN in a patient with PKD and Caroli's disease, and it showed good response to antibiotic therapy." 01/01/1975 - "Cholecystectomy and prolonged courses of antibiotics failed to eradicate the infection, and the presence of multiple intrahepatic biliary cysts (Caroli's syndrome) was eventually discovered by endoscopic retrograde cholangiography." 04/01/2007 - "The treatment for Caroli's disease includes supportive care with antibiotics for cholangitis and ursodeoxycholic acid for hepatolithiasis. "
10.	lidofenin (HIDA)IBA 09/01/2000 - "The authors report on an infant who had a multiloculated cystic lesion located in segment IV of the liver, consistent with Caroli's disease diagnosed, by routine prenatal ultrasound at 25 weeks' gestation, and confirmed by hepatobiliary HIDA scan and computed tomography soon after birth. " 12/01/2010 - "Unexpectedly Diagnosed Caroli's Disease on HIDA Scintigraphy in a Patient with Calculous Cholecystitis." 05/01/1986 - "[Caroli's disease: usefulness of gammagraphy with TC-99m HIDA before the suspicion of hepatic abscess. "

Therapies and Procedures

1.	Liver Transplantation 05/01/2001 - "Orthotopic liver transplantation appears to be an effective curative option for the treatment of patients with Caroli's disease associated to complications. " 01/01/2010 - "Nine of 11 patients underwent initial operations before liver transplantation while 2 patients with Caroli's disease received liver transplantation as initial surgery, with good outcomes. " 02/01/2008 - "The aim of this study was to investigate the feasibility and rationality of orthotopic liver transplantation as an indication for patients with diffuse Caroli's disease. " 01/01/2008 - "The aim of this study was to determine the outcomes of liver transplantation in unselected patients with Caroli's disease and recommend an evidence-based therapeutic algorithm for the management of Caroli's disease. " 01/01/2016 - "Liver transplantation (LT) represents an uncommon indication for Caroli's disease (CD) or syndrome (CS). "
2.	Hepatectomy 10/01/2023 - "Right hepatectomy with a cholangiojejunostomy and hepaticojejunostomy for unilobar Caroli's syndrome." 04/01/2023 - "Therefore, a robotic left hepatectomy was carried out for the unilobar involvement of Caroli's syndrome using the Da Vinci Xi-system. " 04/01/2023 - "[Robotic Left Hepatectomy Using the Glissonean Pedicle Approach for the Treatment of Caroli's Syndrome]." 03/01/2018 - "Laparoscopic hepatectomy could be a feasible, safe treatment option for localized Caroli disease." 03/01/2018 - "Laparoscopic hepatectomy for the treatment of Caroli's disease: a case report."
3.	Kidney Transplantation 12/01/2017 - "This case describes a patient being considered for combined liver-kidney transplantation for Caroli's disease with a failed renal transplant. " 01/01/2008 - "Of the 78,124 patients transplanted in the United States between 1987 and 2006, 104 had Caroli's disease; 96 of these underwent liver alone, and 8 underwent combined liver/kidney transplantation. " 05/01/2005 - "Renal transplantation in Caroli's disease." 05/01/2012 - "Our data suggest that ARPKD patients evaluated for renal transplantation should be carefully screened for severe Caroli's disease. " 04/01/2004 - "This is a rare case of Caroli's disease, diagnosed following renal transplantation in a patient with autosomal recessive polycystic kidneys. "
4.	Endoscopic Sphincterotomy 08/01/1998 - "The aim of this study was to evaluate the role of therapeutic endoscopy, and especially endoscopic sphincterotomy (ES), in the management of Caroli's disease. " 02/01/1998 - "Three cases with Caroli's disease were treated with endoscopic sphincterotomy and stone extraction. " 03/01/1985 - "We suggest that endoscopic papillotomy should be considered in selected patients with Caroli's disease. " 03/01/1985 - "Endoscopic papillotomy in Caroli's disease twenty-year follow-up of a previously reported case." 03/01/1998 - "A case of recurrent pancreatitis in Caroli's disease associated with renal policystic disease is presented: the rareness of this manifestation is stressed and the possible pathogenetic hypotheses are discussed, as well as indications for the selected treatment by endoscopic sphincterotomy."
5.	Therapeutics 01/01/2023 - "[Caroli disease: optimizing the choice of surgical strategy using 3D modeling, 3D printing and therapy]." 04/01/2019 - "Morphopathogenetic mechanisms of the Caroli syndrome can be targets for a potential pathogenetic therapy and prevention of its manifestations and complications." 08/01/2012 - "To analyse and discuss diagnostic and therapeutics difficulties through 16 patients with Caroli's disease. " 06/01/2006 - "Surgery can offer a definite therapy, with an acceptable morbidity and virtually no mortality in localized Caroli's disease. " 01/01/1997 - "Other therapies, including internal or external biliary drainages and various surgical or endoscopic procedures, have been used in the treatment of Caroli's disease, with poor results. "