부산대학교 도서관

The von Meyenburg complex (VMC) is a rare, congenital malformation of the ductal plate. It is typically asymptomatic and usually discovered incidentally. We report a unique case of recurrent life-threatening hepatobiliary sepsis caused by VMC and a review of the literature. A 62-year-old man presented with recurrent episodes of life-threatening hepatobiliary sepsis. Extensive investigations only showed that he has VMCs without any other source of sepsis or underlying immunodeficiency states. Despite prolonged courses of antibiotics which resolved each episode of sepsis, he suffers repeated recurrences of hepatobiliary sepsis. Liver transplantation is now being considered in view of his refractoriness to medical therapy. As VMC can present with severe hepatobiliary sepsis, it is therefore essential to recognise its presence. This case adds to the literature the atypical but life-threatening clinical presentation of VMC.