Curative partial hepatectomy in adult Caroli's disease

Caroli's disease consists of a congenital malformation of the intrahepatic bile ducts characterized by saccular, segmental, cystic dilatations giving rise to recurrent cholangitis. The inheritance pattern is unclear and the disease may be associated with other hereditary diseases such as adult...

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Bibliographic Details
Published inCirugia Española Vol. 81; no. 4; p. 218
Main Authors Medrano-Caviedes, Rodrigo, Artigas, Vicente, Sancho, Francisco J, Marín-Hargreaves, Guillermo, Rodríguez, Manuel, Trías, Manuel
Format Journal Article
LanguageSpanish
Published Spain 01.04.2007
Subjects
Aged
Caroli Disease - physiopathology
Caroli Disease - surgery
Female
Hepatectomy - methods
Humans
Male
Middle Aged
Treatment Outcome
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Summary:Caroli's disease consists of a congenital malformation of the intrahepatic bile ducts characterized by saccular, segmental, cystic dilatations giving rise to recurrent cholangitis. The inheritance pattern is unclear and the disease may be associated with other hereditary diseases such as adult polycystic kidney disease. The most effective therapeutic option in symptomatic unilobar Caroli's disease is resection of the affected lobe. In bilobar involvement, liver transplantation should be considered. Due to their rarity, we report three cases of Caroli's disease successfully resolved through lobectomy of the affected lobe: two patients with recurrent cholangitis and one patient with associated adult polycystic kidney disease.
ISSN:0009-739X
1578-147X