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Liver resection and transplantation in Caroli disease and syndrome
- Source :
- Journal of Visceral Surgery. 156:91-95
- Publication Year :
- 2019
- Publisher :
- Elsevier BV, 2019.
-
Abstract
- Summary Introduction Caroli disease (CD) is a congenital dilatation of the intrahepatic bile ducts. In combination with liver fibrosis or cirrhosis, it is called Caroli syndrome (CS). Infectious complications and intrahepatic cholangiocarcinoma are secondary problems. The aim of this study was to analyse the clinical pattern and outcome in patients with CD/CS who underwent liver surgery. Methods Between January 2004 and December 2016, 21 patients with CD/CS were treated with liver resection or transplantation (LTX) and post-operative data of patients with CD/CS were retrospectively analysed in a database. Results Two patients underwent LTX, and 19 patients underwent liver resection due to CD/CS. During follow-up, one patient developed lung cancer nine years after LTX. Patients resected due to CD/CS were predominantly females (74%) with an overall low incidence of co-morbidities. The median post-operative Clavien-Dindo score was 1 (range: 0–3). There was no death during a median follow-up period of over five years. In four patients, cholangiocarcinoma was confirmed. Tumor recurrence was seen in three patients, and was treated with chemotherapy or repeated liver resection. Conclusions LTX and liver resections due to CD/CS are rare and associated with an acceptable post-operative morbidity and low mortality. Surgical treatment should be performed as early as possible to avoid recurrent episodes of cholangitis or carcinogenesis.
- Subjects :
- Liver Cirrhosis
Male
medicine.medical_specialty
Time Factors
Cirrhosis
Caroli disease
medicine.medical_treatment
Intrahepatic bile ducts
Liver transplantation
Gastroenterology
Cholangiocarcinoma
03 medical and health sciences
0302 clinical medicine
Internal medicine
medicine
Hepatectomy
Humans
Lung cancer
Intrahepatic Cholangiocarcinoma
Retrospective Studies
Chemotherapy
business.industry
Syndrome
General Medicine
Middle Aged
medicine.disease
Caroli Disease
Liver Transplantation
Transplantation
Treatment Outcome
Bile Duct Neoplasms
030220 oncology & carcinogenesis
Female
030211 gastroenterology & hepatology
Symptom Assessment
business
Follow-Up Studies
Subjects
Details
- ISSN :
- 18787886
- Volume :
- 156
- Database :
- OpenAIRE
- Journal :
- Journal of Visceral Surgery
- Accession number :
- edsair.doi.dedup.....a66410e74d4242fdab5555ac0686864f