Maitham A Moslim1, Ganesh Gunasekaran2, David Vogt3, Michael Cruise4, Gareth Morris-Stiff3. 1. Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA. moslimm@ccf.org. 2. Recanati Miller Transplant Institute, Mount Sinai Hospital, 1425 Madison Avenue, New York, NY, 10029, USA. 3. Department of HPB Surgery, Digestive Disease Institute, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA. 4. Department of Anatomic Pathology, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.
Abstract
BACKGROUND: Caroli's disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli's syndrome, there is additionally an associated congenital hepatic fibrosis. METHODS: With institutional review board approval, we identified all patients with Caroli's disease and syndrome. RESULTS: Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli's disease (n = 6) and Caroli's syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli's syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10-134 months). CONCLUSIONS: Caroli's disease and syndrome have a varied presentation. Most individuals with Caroli's disease may be adequately treated by resection, but transplantation is required for Caroli's syndrome patients due to the associated hepatic fibrosis.
BACKGROUND:Caroli's disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli's syndrome, there is additionally an associated congenital hepatic fibrosis. METHODS: With institutional review board approval, we identified all patients with Caroli's disease and syndrome. RESULTS: Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli's disease (n = 6) and Caroli's syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli's syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10-134 months). CONCLUSIONS:Caroli's disease and syndrome have a varied presentation. Most individuals with Caroli's disease may be adequately treated by resection, but transplantation is required for Caroli's syndromepatients due to the associated hepatic fibrosis.
Authors: M Shimoda; D G Farmer; S D Colquhoun; M Rosove; R M Ghobrial; H Yersiz; P Chen; R W Busuttil Journal: Liver Transpl Date: 2001-12 Impact factor: 5.799
Authors: Sarwa Darwish Murad; W Ray Kim; Denise M Harnois; David D Douglas; James Burton; Laura M Kulik; Jean F Botha; Joshua D Mezrich; William C Chapman; Jason J Schwartz; Johnny C Hong; Jean C Emond; Hoonbae Jeon; Charles B Rosen; Gregory J Gores; Julie K Heimbach Journal: Gastroenterology Date: 2012-04-12 Impact factor: 22.682
Authors: Kevin C Soares; Seth D Goldstein; Mounes A Ghaseb; Ihab Kamel; David J Hackam; Timothy M Pawlik Journal: Pediatr Surg Int Date: 2017-03-31 Impact factor: 1.827