caroli disease

Summary

Summary: 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.

Top Publications

  1. Wang Z, Yan L, Li B, Zeng Y, Wen T, Wang W. Orthotopic liver transplantation for patients with Caroli's disease. Hepatobiliary Pancreat Dis Int. 2008;7:97-100 pubmed
    ..But liver transplantation has been a curative option. 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...
  2. Levy A, Rohrmann C, Murakata L, Lonergan G. Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol. 2002;179:1053-7 pubmed
    ..The purpose of our study was to describe the spectrum of radiologic and pathologic features of Caroli's disease...
  3. Ulrich F, Pratschke J, Pascher A, Neumann U, Lopez Hanninen E, Jonas S, et al. Long-term outcome of liver resection and transplantation for Caroli disease and syndrome. Ann Surg. 2008;247:357-64 pubmed publisher
    ..preoperative disease characteristics as well as the rate of postoperative complications, patient survival, and course of symptoms after liver resection or orthotopic liver transplantation (OLT) for Caroli disease (CD) or syndrome (CS).
  4. Abouchacra S, Toumeh S, Boobes Y, Bernieh B, Gorka W. An atypical course of Caroli's disease in a renal transplant patient--case report and review of the literature. Clin Nephrol. 2004;61:282-8 pubmed
    ..Once confirmed, affected patients with end-stage renal disease such as our patient, should ideally undergo combined liver-kidney transplantation...
  5. Ninan V, Nampoory M, Johny K, Gupta R, Schmidt I, Nair P, et al. Caroli's disease of the liver in a renal transplant recipient. Nephrol Dial Transplant. 2002;17:1113-5 pubmed
  6. Wu K, Changchien C, Kuo C, Chuah S, Chiu Y, Kuo C. Caroli's disease - a report of two siblings. Eur J Gastroenterol Hepatol. 2002;14:1397-9 pubmed
    ..An ultrasonography survey was carried out on the family. Reports of Caroli's disease occurring in two siblings are rare in the literature...
  7. Espinoza R, San Martín S, Court F, Vera E, Ferreira R, Croxatto H. [Hepatic resection in localized Caroli disease]. Rev Med Chil. 2003;131:183-9 pubmed
    b>Caroli disease is a cystic congenital malformation of the intrahepatic biliary tract, frequently associated with lithiasis. Commonly, cystic dilatations are bilateral and infrequently they affect only one hepatic lobule or segment...
  8. Rault R. Renal transplantation in Caroli's disease. Clin Nephrol. 2005;63:415-6 pubmed
  9. Shedda S, Robertson A. Caroli's syndrome and adult polycystic kidney disease. ANZ J Surg. 2007;77:292-4 pubmed
    ..Three cases have been reported in the English Medline search. The presentation of this fourth case discusses the issues surrounding the treatment of Caroli's disease in the setting of a renal transplant...

More Information

Publications62

  1. Yasoshima M, Sato Y, Furubo S, Kizawa K, Sanzen T, Ozaki S, et al. Matrix proteins of basement membrane of intrahepatic bile ducts are degraded in congenital hepatic fibrosis and Caroli's disease. J Pathol. 2009;217:442-51 pubmed publisher
  2. Waechter F, Sampaio J, Pinto R, Alvares da Silva M, Cardoso F, Francisconi C, et al. The role of liver transplantation in patients with Caroli's disease. Hepatogastroenterology. 2001;48:672-4 pubmed
    ..The authors present the course of two cases of this disease, associated with congenital fibrosis of the liver worsened by repeated episodes of cholangitis, submitted to orthotopic liver transplantation...
  3. Joshi D, Dungu J, Dunga J, James A, Yaqoob M. An unusual case of hepatosplenomegaly. Gut. 2005;54:1272, 1331 pubmed
  4. Giovanardi R. Monolobar Caroli's disease in an adult. Case report. Hepatogastroenterology. 2003;50:2185-7 pubmed
    ..Histologically there was no evidence of malignancy. Liver resection is the treatment of choice for Caroli's disease confined to a single lobe or segment, eliminating the potential for cholangitis, lithiasis and carcinoma...
  5. Mumoli N, Cei M. Caroli disease. Mayo Clin Proc. 2007;82:208 pubmed
  6. De Kerckhove L, De Meyer M, Verbaandert C, Mourad M, Sokal E, Goffette P, et al. The place of liver transplantation in Caroli's disease and syndrome. Transpl Int. 2006;19:381-8 pubmed
    ..In case of associated ARPKD, renal transplantation is often indicated early on because of the more rapid progression of the renal component of the disease...
  7. Jeha G, Tatevian N, Heptulla R. Congenital hypothyroidism in association with Caroli's disease and autosomal recessive polycystic kidney disease: patient report. J Pediatr Endocrinol Metab. 2005;18:315-8 pubmed
    ..To our knowledge this is the first reported case of congenital hypothyroidism in an infant with ARPKD...
  8. Kayhan B, Akdogan M, Parlak E, Ozarslan E, Sahin B. Hepatolithiasis: a Turkey experience. Turk J Gastroenterol. 2007;18:28-32 pubmed
    ..The aim of this study was to report our experience of hepatolithiasis, diagnosed in 55 patients between June 1995 and March 2003...
  9. Cetinkaya S, Alikasifoglu A. Central diabetes insipidus associated with Caroli syndrome. Indian J Pediatr. 2007;74:419-20 pubmed
    b>Caroli disease is a rare congenital malformation characterized by cystic dilatations of large bile ducts. There are two forms of Caroli disease. Simple form entails the bile duct dilatation or ectasia...
  10. Tallón Aguilar L, Sanchez Moreno L, Barrera Pulido L, Pareja Ciuró F, Suarez Artacho G, Alamo Matinez J, et al. Liver transplantation consequential to Caroli's syndrome: a case report. Transplant Proc. 2008;40:3121-2 pubmed publisher
  11. Esmer C, Alvarez Mendoza A, Lieberman E, Del Castillo V, Ridaura Sanz C. Liver fibrocystic disease and polydactyly: proposal of a new syndrome. Am J Med Genet. 2001;101:12-6 pubmed
    ..Our patients' malformations might represent a new entity where autosomal recessive inheritance is probable, but other patterns cannot be ruled out...
  12. Ko J, Yi N, Suh K, Seo J. Pediatric liver transplantation for fibropolycystic liver disease. Pediatr Transplant. 2012;16:195-200 pubmed publisher
    ..91 mg/dL at the last follow-up (p = 0.01). LT is an excellent option for children with complications from fibropolycystic liver disease. Renal function should be monitored cautiously after LT in the patients with ARPKD...
  13. Kumar K, Nair S, Hertan H, Grover H. Isolated intrahepatic biliary dilatation in a patient with acquired immune deficiency syndrome (AIDS): AIDS cholangiopathy versus incidental unilobar Caroli's disease. J Clin Gastroenterol. 2001;32:79-81 pubmed
    ..Special stain results for fungi and acid-fast organisms were negative. The presence of advanced AIDS in this patient raised the possibility of this being a possible manifestation of AIDS cholangiopathy...
  14. Erlinger S. [Cystic dilatation of the biliary tract]. Rev Prat. 2000;50:2136-41 pubmed
    ..Choledocal cyst is characterized by a cystic dilatation of the common bile duct or hepatic duct. The main manifestations are jaundice and cholangitis. Cholangiocarcinoma may occur. Treatment is surgical resection...
  15. Boopathy Vijayaraghavan S, Kamalam M, Raman M. Prenatal sonographic appearance of congenital bile duct dilatation associated with renal-hepatic-pancreatic dysplasia. Ultrasound Obstet Gynecol. 2004;23:609-11 pubmed
    ..It is also the first report of a case in which the features of dysplasia were evident in all three of the organs which may be affected, the kidneys, liver and pancreas...
  16. Dhanjal N, Sharif A, Rosenfelder N, Lim A, Taylor Robinson S. Cystic duct dilatation after cholecystectomy in fibropolycystic liver disease. J Hepatol. 2005;43:192 pubmed
  17. Alvarez Navascués R, Quinones L, Guinea O, Guerediaga F. [Caroli disease in a kidney transplant patient with polycystic kidney disease]. Nefrologia. 2005;25:336-7 pubmed
  18. Chapal M, Debout A, Dufay A, Salomon R, Roussey G, Burtey S, et al. Kidney and liver transplantation in patients with autosomal recessive polycystic kidney disease: a multicentric study. Nephrol Dial Transplant. 2012;27:2083-8 pubmed publisher
    ..Here, we report the results and outcome of renal and/or liver transplantation in a series of patients with ARPKD...
  19. Narsanska A, Treska V, Mirka H, Mukensnabl P, Chlumska A. [Caroli disease--dilatation of intrahepatic bile ducts]. Rozhl Chir. 2011;90:281-4 pubmed
    b>Caroli disease is a rare congenital condition characterized by a non-obstructive saccular or fusiform multi-focal segmental dilatation of the intrahepatic bile ducts and the frequent formation of the intrahepatic calculi...
  20. Terada T, Moriki T. Monolobar hepatobiliary fibropolycystic disease. Pathol Oncol Res. 2011;17:159-65 pubmed publisher
    ..The biliary cysts, persistent ductal plate, and microhamartomas were positive for fetal apomucin antigen MUC1...
  21. Naraynsingh V, Maharaj D, Busby G, Raju G, Jankey N. Caroli's disease associated with a gastric diverticulum. West Indian Med J. 2000;49:175-6 pubmed
    ..A deficiency in the fibromuscular matrix of both the bile ducts and the gastric wall may explain why these two pathologies may coexist in a single patient...
  22. Hillingsø J, Kirkegaard P. [Cysts of the liver]. Ugeskr Laeger. 2008;170:1350-3 pubmed
    ..It is concluded that up to 25% of cysts must be treated surgically, because recurrence after percutaneous or laparoscopic treatment is between 5% and 71%, and only resection or liver transplantation are curative...
  23. Pinto R, Lima J, da Silveira T, Scholl J, de Mello E, Silva G. Caroli's disease: report of 10 cases in children and adolescents in southern Brazil. J Pediatr Surg. 1998;33:1531-5 pubmed
    ..The aim of this study was to describe the authors' experience with Caroli's disease in children and adolescents...
  24. Ikeda H, Sasaki M, Ishikawa A, Sato Y, Harada K, Zen Y, et al. Interaction of Toll-like receptors with bacterial components induces expression of CDX2 and MUC2 in rat biliary epithelium in vivo and in culture. Lab Invest. 2007;87:559-71 pubmed
  25. Keskin Z, Keskin S, Yesildag M, Yesildag A. Caroli disease with bilateral severe bullous emphysema. An unknown component. Saudi Med J. 2012;33:1227-8 pubmed
    We suspected that the multi-bullous parenchymal disease of our patient could be related to Caroli disease (CD) because he had no pulmonary pathology before the diagnosis of CD...
  26. Bawany M, Alaradi O, Nawras A. Caroli's syndrome in a post renal transplant patient: case report and review of the literature. Saudi J Gastroenterol. 2012;18:59-61 pubmed publisher
    ..However, there has been only few case reports published with Caroli's syndrome diagnosed postrenal transplantation...
  27. Aguilera V, Rayon M, Pérez Aguilar F, Berenguer J. Caroli's syndrome and imaging: report of a case. Rev Esp Enferm Dig. 2004;96:74-6 pubmed
  28. Maxien D, Reiser M, Wirth S. [Upper abdominal pain with recurrent cholangitis and pyelonephritis]. Radiologe. 2010;50:372-6 pubmed publisher
    ..Depending on the grade of liver involvement the treatment is partial hepatectomy or, as in our case transplantation, which also eliminates the risk of associated cholangiocarcinoma...
  29. Shorbagi A, Bayraktar Y. Experience of a single center with congenital hepatic fibrosis: a review of the literature. World J Gastroenterol. 2010;16:683-90 pubmed
    ..It is one of the fibropolycystic diseases, which also include Caroli disease, autosomal dominant polycystic kidney disease, and autosomal recessive polycystic kidney disease...
  30. Potenza L, Luppi M, Barozzi P, Rossi G, Cocchi S, Codeluppi M, et al. HHV-6A in syncytial giant-cell hepatitis. N Engl J Med. 2008;359:593-602 pubmed publisher
    ..Neither HHV-6B DNA nor late protein was identified in the same follow-up samples from the patient. Thus, HHV-6A may be a cause of syncytial giant-cell hepatitis...
  31. Patil S, Das H, Desai N, Manjunath S, Thakur B, Sawant P. Caroli's syndrome--a rare cause of portal hypertension. J Assoc Physicians India. 2004;52:261 pubmed
  32. Iyer V. A young man with generalized oedema, vomiting and easy fatiguability. Natl Med J India. 2004;17:152-9 pubmed
  33. Furubo S, Sato Y, Harada K, Nakanuma Y. Roles of myofibroblasts and notch and hedgehog signaling pathways in the formation of intrahepatic bile duct lesions in polycystic kidney rats. Pediatr Dev Pathol. 2013;16:177-90 pubmed publisher
  34. Rawat D, Kelly D, Milford D, Sharif K, Lloyd C, McKiernan P. Phenotypic variation and long-term outcome in children with congenital hepatic fibrosis. J Pediatr Gastroenterol Nutr. 2013;57:161-6 pubmed publisher
    ..They have a variable clinical course, and the natural history is not well defined despite molecular advances. Our study describes the clinical manifestations and long-term outcome in children with this disorder...
  35. Lendoire J, Raffin G, Grondona J, Bracco R, Russi R, Ardiles V, et al. Caroli's disease: report of surgical options and long-term outcome of patients treated in Argentina. Multicenter study. J Gastrointest Surg. 2011;15:1814-9 pubmed publisher
    ..Caroli's disease (CD) management is still controversial...
  36. Ozkurt S, Canaz F, Temiz G, Sahin G, Yalcin A. Unusual renal manifestation of Caroli disease: AA amyloidosis. Ren Fail. 2012;34:930-3 pubmed publisher
    ..Renal cystic disorders are common complications of Caroli disease (CD), but renal amyloidosis is fairly uncommon...
  37. Hasegawa E, Sawa N, Hoshino J, Suwabe T, Hayami N, Yamanouchi M, et al. Recurrent Cholangitis in a Patient with Autosomal Dominant Polycystic Kidney Disease (ADPKD) and Caroli's Disease. Intern Med. 2016;55:3009-3012 pubmed
    ..Hepatobiliary system abnormalities such as Caroli's disease should be considered in febrile ADPKD patients, even in the absence of typical clinical signs or symptoms...
  38. Habib S, Shaikh O. Caroli's disease and liver transplantation. Liver Transpl. 2008;14:2-3 pubmed
  39. Makino I, Tani T, Shimizu K, Takamura H, Fujimura T, Nishimura G, et al. [A case of monolobar Caroli's disease]. Nihon Shokakibyo Gakkai Zasshi. 2006;103:316-21 pubmed
    ..Finally, it was diagnosed as pure type monolobar Caroli's disease by pathological examination. This was a rare case of Caroli's disease which arose limitedly in monolobe. And we could curatively resect it by hepatectomy...
  40. Romanowicz T, Waga Z, Słomian M, Słomian M, Marczewski K. [Caroli's disease--if so rare clinical problem?]. Pol Merkur Lekarski. 2004;17:92-4 pubmed
    ..Basic treatment is hepatic resection (focal form) and internal bypass of the biliary tree (diffuse form). The prognosis of the disease is reserved one...
  41. Hara H, Morita S, Ishibashi T, Sako S, Otani M, Tanigawa N. Surgical treatment for congenital biliary dilatation, with or without intrahepatic bile duct dilatation. Hepatogastroenterology. 2001;48:638-41 pubmed
    ..The incidence of postoperative cholangitis differs between patients with and those without congenital intrahepatic bile duct dilatation. The aim of this study was to evaluate comparatively the treatment results in these two patients groups...
  42. Hogan M, Torres V. What the similarities of specific polycystic liver and kidney diseases can teach us about both. Nephrol News Issues. 2008;22:29-31 pubmed
  43. Agustsson A, Cariglia N. [Caroli's disease, case report and review of the literature]. Laeknabladid. 2007;93:603-5 pubmed
    ..He later developed cholangiocarcinoma. Caroli's disease has not, to our knowledge, been reported in Iceland before...
  44. Yonem O, Bayraktar Y. Clinical characteristics of Caroli's disease. World J Gastroenterol. 2007;13:1930-3 pubmed
    ..Although a rare disorder; Caroli's disease should always be considered in the differential diagnosis of chronic cholestasis of unknown cause...
  45. Kerkar N, Norton K, Suchy F. The hepatic fibrocystic diseases. Clin Liver Dis. 2006;10:55-71, v-vi pubmed
    ..The treatment is supportive, with careful attention to associated renal disease. Liver transplantation is an option in selected patients...
  46. Desmet V. [Cystic diseases of the liver. From embryology to malformations]. Gastroenterol Clin Biol. 2005;29:858-60 pubmed
    ..The genetic or non genetic factors leading to these ductal plate malformations are unknown...
  47. Loreno M, Bo P, Senzolo M, Cillo U, Naoumov N, Burra P. Successful pregnancy in a liver transplant recipient treated with lamivudine for de novo hepatitis B in the graft. Transpl Int. 2005;17:730-4 pubmed
    ..The course of gestation was uneventful and caesarean section was performed after 36 weeks. The newborn infant was a healthy male weighing 3,080 g and measuring 50 cm...
  48. La Barba G, Vivarelli M, Golfieri R, Tame M, Caputo M, Piscaglia F, et al. Hepatic artery thrombosis and graft ischemia in the presence of preserved arterial inflow: not a contradiction but a real possibility. Liver Transpl. 2004;10:710-1 pubmed
  49. Osteaux M, Op de Beeck B, Hoorens A, Dujardin M, Goes E. [Radiological differential diagnosis of focal liver lesions with anatomo-pathological correlations]. J Radiol. 2002;83:269-91 pubmed
    ..This study applies to the following focal lesions: angioma, focal hyperplasia, adenoma, regeneration nodule, hepatocarcinoma, metastasis...
  50. Kuipers E, de Man R, van Buuren H. [Clinical thinking and decision-making in practice. 4 times ERCP, 6 times echography of the upper abdomen and 3 CT scans in a woman with recurrent fever and bacteremia]. Ned Tijdschr Geneeskd. 2002;146:438; author reply 438-9 pubmed
  51. Halbritter J, Porath J, Diaz K, Braun D, Kohl S, Chaki M, et al. Identification of 99 novel mutations in a worldwide cohort of 1,056 patients with a nephronophthisis-related ciliopathy. Hum Genet. 2013;132:865-84 pubmed publisher
    ..In addition, we present the first case of Caroli disease due to mutations in WDR19/NPHP13 and the second case ever with a recessive mutation in GLIS2/NPHP7...
  52. Lai Q, Lerut J. Proposal for an algorithm for liver transplantation in Caroli's disease and syndrome: putting an uncommon effort into a common task. Clin Transplant. 2016;30:3-9 pubmed publisher
    ..This review aims at analyzing highlighting recent transplant experiences in this field and also at focusing on the role of LT in case-specific comorbidities such as development of cholangiocellular cancer or renal failure are present. ..
  53. Carrera C, Castiella A, Fernandez J, Cosme A, García Bengoechea M. Caroli's disease diagnosed by magnetic resonance cholangiopancreatography. Eur J Gastroenterol Hepatol. 2002;14:577 pubmed