{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T03:33:33Z","timestamp":1785900813006,"version":"3.56.0"},"reference-count":21,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":3471,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2006,3]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Caroli's disease is a rare congenital hepatic disease, characterized by segmental dilatation of the biliary tree. Patients who have recurrent bouts of biliary infection, particularly those with complications related to portal hypertension, may require orthotopic liver transplantation (OLT). Few case reports have described the outcome of OLT in patients with Caroli's disease and to date there is no large series reported in the literature. We retrospectively analyzed the outcome of OLT in patients with Caroli's disease who underwent OLT between 1982 and 2002 at Starzl Transplantation Institute, University of Pittsburgh. Patients were identified and data was collected by computerized search of the electronic database system. All patients had confirmation of diagnosis by histopathology of explanted liver. A total of 33 patients with Caroli's disease were listed for liver transplantation, 3 of whom were excluded, as they were not transplanted. A total of 90% had signs of hepatic decompensation at the time of OLT. Median posttransplantation follow-up was 7.7 yr. Short-term graft and patient survival at 1 month was 83% and 86%, whereas overall long-term graft survival rates at 1, 5, and 10 yr were 73%, 62%, and 53%, respectively, and patient survival rates were 76%, 65%, and 56%, respectively. Long-term outcome in patients who survived the first year after transplantation was significantly better. Their survival rate at 5 and 10 yr was 90% and 78%. On univariable analysis, recipient age, donor male gender, coexistent congenital hepatic fibrosis, and re-OLT were associated with poor patient survival. Eight patients were retransplanted, 3 of whom had primary nonfunction. A total of 13 patients died; the most common cause of death being sepsis and cardiovascular complications. Patients who died of sepsis had cholangitis pre-OLT. In conclusion, OLT is a form of curative and life-saving therapy in patients with Caroli's disease, especially in those with decompensated liver disease. Overall survival is better with liver transplantation and is comparable with the survival of recipients who undergo OLT for other etiologies of chronic liver disease. Survival was poor in patients with congenital hepatic fibrosis (Caroli's syndrome) and in those who had cholangitis at the time OLT.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/lt.20719","type":"journal-article","created":{"date-parts":[[2006,2,23]],"date-time":"2006-02-23T23:17:50Z","timestamp":1140736670000},"page":"416-421","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":68,"title":["Caroli's disease and orthotopic liver transplantation"],"prefix":"10.1097","volume":"12","author":[{"given":"Shahid","family":"Habib","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Obaid","family":"Shakil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Osvaldo F.","family":"Couto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony J.","family":"Demetris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John J.","family":"Fung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amadeo","family":"Marcos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kapil","family":"Chopra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-13-20250128","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1136\/bmj.1.2356.434","article-title":"Case of intrahepatic calculi.","volume":"1","author":"Vachell","year":"1906","journal-title":"Br Med J"},{"key":"R3-13-20250128","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/0002-9610(77)90359-2","article-title":"Congenital bile duct cysts: classification, operative procedures and review of thirtyseven cases, including cancer arising from choledochal cyst.","volume":"134","author":"Todani","year":"1977","journal-title":"Am J Surg"},{"key":"R4-13-20250128","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1002\/ajmg.1320290323","article-title":"Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family.","volume":"29","author":"Kaplan","year":"1988","journal-title":"Am J Med Genet"},{"key":"R5-13-20250128","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1038\/ki.1997.300","article-title":"Autosomal dominant polycystic kidney disease with anticipation and Carolis disease associated with a PKD1 mutation. Rapid communication.","volume":"52","author":"Torra","year":"1997","journal-title":"Kidney Int"},{"key":"R7-13-20250128","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1001\/archsurg.1977.01370010084017","article-title":"Congenital cystic dilatation of the bile duct associated with LaurenceMoonBiedlBardet syndrome.","volume":"112","author":"Tsuchiiya","year":"1977","journal-title":"Arch Surg"},{"key":"R8-13-20250128","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1136\/gut.13.8.604","article-title":"Congenital dilatation of the intrahepatic bile ducts associated with the development of amyloidosis.","volume":"13","author":"Fevery","year":"1972","journal-title":"Gut"},{"key":"R9-13-20250128","first-page":"241","article-title":"Neurofibromatosis and Carolis disease: an extremely rare association.","volume":"14","author":"Arfan","year":"2004","journal-title":"J Coll Physicians Surg Pak"},{"key":"R10-13-20250128","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1002\/hep.1840160434","article-title":"Congenital diseases of intrahepatic bile ducts: variations on the theme ductal plate malformation.","volume":"16","author":"Desmet","year":"1992","journal-title":"Hepatology"},{"key":"R11-13-20250128","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1097\/00042737-199802000-00001","article-title":"Carolis disease.","volume":"10","author":"Taylor","year":"1998","journal-title":"Eur J Gastroenterol Hepatol"},{"key":"R12-13-20250128","doi-asserted-by":"crossref","first-page":"333","DOI":"10.2214\/ajr.165.2.7618550","article-title":"Imaging findings in Carolis disease.","volume":"165","author":"Miller","year":"1995","journal-title":"Am J Roentgenol"},{"key":"R13-13-20250128","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1016\/0140-6736(93)92817-D","article-title":"Ursodeoxycholic acid treatment of primary hepatolithiasis in Carolis syndrome.","volume":"342","author":"Ros","year":"1993","journal-title":"Lancet"},{"key":"R14-13-20250128","first-page":"134","article-title":"Surgical treatment of congenital dilatation of the biliary system.","volume":"40","author":"Moreno Gonzalez","year":"1993","journal-title":"Hepatogastroenterology"},{"key":"R15-13-20250128","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1007\/s00268-001-0250-x","article-title":"Surgical strategy for cystic diseases of the liver in a western hepatobiliary center.","volume":"26","author":"Ammori","year":"2002","journal-title":"World J Surg"},{"key":"R16-13-20250128","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1007\/s003839900323","article-title":"Harjai, MM, Bal, RK. Caroli syndrome.","volume":"16","year":"2000","journal-title":"Pediatr Surg Int"},{"key":"R17-13-20250128","doi-asserted-by":"crossref","first-page":"2279","DOI":"10.1016\/S0041-1345(02)03235-9","article-title":"Therapy of Carolis disease by orthotopic liver transplantation.","volume":"34","author":"Ulrich","year":"2002","journal-title":"Transplant Proc"},{"key":"R18-13-20250128","first-page":"672","article-title":"The role of liver transplantation in patients with Carolis disease.","volume":"48","author":"Waechter","year":"2001","journal-title":"Hepatogastroenterology"},{"key":"R19-13-20250128","first-page":"1938","article-title":"Adult presentation of Carolis syndrome treated with orthotopic liver transplantation.","volume":"92","author":"Schiano","year":"1997","journal-title":"Am J Gastroenterol"},{"key":"R20-13-20250128","first-page":"241","article-title":"Liver transplantation in patients with Carolis disease and recurrent cholangitis.","volume":"10","author":"Sans","year":"1997","journal-title":"Transpl Int"},{"key":"R22-13-20250128","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1007\/s005340170030","article-title":"Livingdonor liver transplantation for Carolis disease with intrahepatic adenocarcinoma.","volume":"8","author":"Takatsuki","year":"2001","journal-title":"J Hepatobiliary Pancreat Surg"},{"key":"R23-13-20250128","first-page":"175","article-title":"et al. Evaluation of long term results of Carolis disease: 21 years observation of a family with autosomal dominant inheritance, and review of literature.","volume":"42","author":"Tsuchida","year":"1995","journal-title":"Hepatogastroenterology"},{"key":"R24-13-20250128","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1097\/00042737-199802000-00002","article-title":"Carolls disease: 1977-1995 experiences.","volume":"10","author":"Dagli","year":"1998","journal-title":"Eur J Gastroenterol Hepatol"}],"container-title":["Liver Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Flt.20719","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.lww.com\/01445473-200603000-00013","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,28]],"date-time":"2025-01-28T20:01:36Z","timestamp":1738094496000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/01445473-200603000-00013"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,3]]},"references-count":21,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2006]]}},"URL":"https:\/\/doi.org\/10.1002\/lt.20719","relation":{},"ISSN":["1527-6465"],"issn-type":[{"value":"1527-6465","type":"print"}],"subject":[],"published":{"date-parts":[[2006,3]]}}}