ABSTRACT
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.
Subject(s)
Caroli Disease/surgery , Hepatectomy/methods , Aged , Caroli Disease/physiopathology , Female , Humans , Male , Middle Aged , Treatment OutcomeABSTRACT
La enfermedad de Caroli consiste en una malformación congénita de la vía biliar intrahepática caracterizada por dilataciones saculares, segmentarias múltiples y quísticas, cuyas manifestaciones clínicas se centran en crisis repetitivas de colangitis. Esta enfermedad tiene una base hereditaria incierta y puede asociarse a otras enfermedades de carácter hereditario como la enfermedad poliquística renal del adulto (EPRAD). La mejor opción terapéutica en los casos sintomáticos unilobares hepáticos es la exéresis hepática del lóbulo afecto correspondiente; en caso de afección bilobar, debe plantearse el trasplante hepático. Presentamos por su rareza 3 casos de enfermedad de Caroli resueltos con éxito mediante una lobectomia hepática del lado afecto: 2 pacientes manifestaron la enfermedad en forma de episodios de colangitis de repetición y 1 paciente, con asociación a EPRAD (AU)
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 (AU)
