Caroli`s Disease: surgical treatment, hepatic ressection and transplantation in seven years
Abstract
Objectives: We review seven years of experience of surgical treatment of CD (Caroli Disease) in the HUC (Hospitais da Unversidade de Coimbra).
Material and methods: Histopathology records from July 2000 to July 2007 were scanned CD. Patient files were retrieved and reviewed for demographic and clinical data, comorbidity and exam results. Previous diagnosis, medical treatments and other data pertaining to congenital biliary tree malformations were sought.
Results: Between July 2000 and July 2007, eight patients with histologically confirmed CD were treated in the HUC. The average age was 41,2 years. Seven patients have a history of previous cholangitis. Six patients had hepatic resection and the remaining two had liver transplantation. At present all eight patients are alive and experience clinical improvement.
Conclusion: Hepatic resection is the choice treatment for localized CD, and it is potentially curative. However, for diffuse disease liver transplant may be the only option, and it may provide good long term results.
Keywords: Caroli disease; biliar tree dilation; cholangitis
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