Premium
Caroli's disease in children: Is it commonly misdiagnosed?
Author(s) -
Senyüz O. F.,
Yesildag E.,
Kuruoglu S.,
Yeker Y.,
Emir H.
Publication year - 2005
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.2005.tb01798.x
Subject(s) - medicine , portal hypertension , ectasia , differential diagnosis , disease , presentation (obstetrics) , liver disease , intrahepatic bile ducts , surgery , bile duct , pediatrics , gastroenterology , pathology , cirrhosis
Aim: Caroli's disease is a simple form of intrahepatic bile duct ectasia. It can be complicated with the involvement of liver parenchyma and portal hypertension. Herein, the difficult management of delayed presentation of Caroli's disease is reported. Methods and results: We report on four different forms of clinical presentation of Caroli's disease: an infant with fulminant liver failure, a teenager with persistent biliary fistula, a boy with hypersplenism in the face of portal hypertension and a girl with variceal bleeding. Conclusions: Caroli's disease must be included in the differential diagnosis of cystic lesions in the liver. Delayed diagnosis of Caroli's disease is difficult to manage and appropriate investigations are warranted before planning a surgical approach.