Global Advanced Research Journal of Medicine and Medical Sciences (GARJMMS) ISSN: 2315-5159
July 2017 Vol. 6(7), pp. 149-155
Copyright © 2017 Global Advanced Research Journals


Case Report 

Caroli’s disease: management of a rare condition during pregnancy

Nisa Félix* and Fátima Palma

Department of Maternal-Fetal Medicine, Dr. Alfredo da Costa Maternity, Lisbon, Portugal.

*Corresponding Author E-mail:

Accepted 17 July, 2017


Caroli’s disease is a rare congenital condition characterized by dilation of the intrahepatic biliary tree. There are about 200 cases described in the international literature and only three in pregnant women. In this article we describe a forth case dominated by the presence of severe cholestasis, with initial bile acids (BA) higher than 400μmol/L. We purposed a monitoring plan based on our knowledge about intrahepatic cholestasis of pregnancy (ICP), given its high prevalence in the obstetric population.

Keywords: Caroli’s disease, Caroli’s syndrome, cholestasis, bile acids, pregnancy 


List of Abbreviations

AF, alkaline phosphatase; BA, bile acids; CPAP, continuous positive airway pressure; CT, computed tomography; CTG, cardiotocography; ERCP, endoscopic cholangiopancreatography; GGT, gamma-glutamyl transpeptidase; H, hours; ICP, intrahepatic cholestasis of pregnancy; IM, intramuscular; MRCP, resonance cholangiopancreatography; NICU, neonatal intensive care unit; RDS, respiratory distress syndrome; UDCA, ursodeoxycholic acid; W, weeks



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