RECURRENT SEPSIS IN A PATIENT WITH BILIARY HAMARTOMAS

Biliary hamartomas or von Meyenburg complexes (VMCs) are hepatic tumour-like lesions related to congenital malformation of the ductal plate, and are part of the ciliopathy spectrum of disorders. The exact pathogenesis of VMCs is unclear and it remains controversial whether they have the potential for malignant transformation. Patients are often asymptomatic and VMCs are usually encountered as an incidental finding on imaging. We report a case of recurrent sepsis with an unidentified focus. It was later confirmed that biliary hamartomas were acting as a sanctuary for the persistent pathogenic agent. The authors hope to draw attention to the existence of this unusual focus of recurrent sepsis.

Hepatobiliary sepsis is an unusual clinical presentation of biliary hamartomas.Clinicians should be aware of the infectious complications of these diffuse structural biliary ductal abnormalities.Early recognition of this atypical life-threatening clinical presentation is important for the prognosis.