The hepatic von Meyenburg complex: prevalence and association with hepatic and renal cysts among 2843 autopsies [corrected]

Abstract

von Meyenburg complexes (VMC) are dilated small bile ducts surrounded by fibrous stroma. These lesions are frequently seen at autopsy, especially in association with polycystic disease of the kidney and/or liver. The purpose of this study was to quantitate the prevalence of VMC and associated lesions, with a view to clarifying the nature of VMC. We examined the liver slides from 2843 autopsies and found 157 patients having VMC or cysts in the liver. For each of the 157 patients, and age- and gender-matched controls, VMC, hepatic cysts, and gross and microscopic renal cysts were counted and measured and autopsy reports were reviewed. VMC were found in 5.6% of adults and in 0.9% of children. Macroscopic hepatic cysts were found in 16.9% of livers that also had VMC. Of livers with hepatic cysts, 73.5% also had VMC. Adult polycystic kidney disease (APKD) was found in 11% of adults with at least one VMC and in 40% of those with four or more VMC. Among adults with APKD, VMC were found in 97% and hepatic cysts in 88%. Because APKD could account for only 11% of the patients with VMC, we suggest that VMC, in the absence of APKD, are a manifestation of a different disease, which could be genetic or secondary to inflammation or ischemia.