Congenital Hepatic Fibrosis as a Cause of Recurrent Cholangitis: A Case Report and Review of the Literature

Rare liver diseases caused by ductal plate malformation, such as congenital hepatic fibrosis (CHF), Caroli syndrome, and polycystic disease, can have clinical manifestations recurrent cholangitis—frequently involving multidrug-resistant microorganisms—leading to difficulties in selecting the optimal antimicrobial treatment. Without prompt recognition, these infections severely hamper patient’s quality of life develop into life-threatening complications. We report here case a 50-year-old woman with history recurring cholangitis occasional systemic involvement leading bloodstream infection, who ultimately received diagnosis CHF was put on chronic suppressive antibiotic therapy while waiting list for transplant. also reviewed literature collecting cases occurring patients malformation.