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

Abstract

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

Diffusion tensor magnetic resonance imaging in the grading of liver fibrosis associated with congenital ductal plate malformations

“…The patient may present with oesophageal varices, splenomegaly, or spontaneous luminal gastrointestinal bleeding [ 4 ]. Some patients may present with cholangitis [ 5 , 6 ]. Associated renal anomalies have also been reported, including polycystic kidney disease (PCKD), nephronophthisis, and medullary sponge kidney [ 2 ].…”