"Starry liver" - Von Meyenburg complex clinical case presentation and differential diagnosis discussion: A case report

Abstract

Background: Von Meyenburg complex (VMC) (i.e., biliary hamartoma) is a rare congenital disorder characterized by multiple dilated cystic bile ducts, without clear trends in sex or age predominance. Due to the low number of published cases and the lack of recognized guidelines, the management of such patients remains a clinical challenge.

Case summary: We present a case of symptomatic VMC that was diagnosed after imaging and histopathological examinations. Considering the patient's condition, a conservative treatment strategy was chosen. Instrumental, laboratory, and clinical follow-up demonstrated the stable condition of the patient receiving conservative treatment.

Conclusion: VMC is a potentially non-life threatening condition, but its recognition is crucial for the management of patients.

Keywords: Biliary hamartoma; Case report; Liver polycystic disease; Magnetic resonance imaging; Ultrasonography imaging; Von Meyenburg complex.

Figure 1

Abdominal contrast-enhanced ultrasound imaging of the patient. Marked echostructural inhomogeneity of the liver.

Figure 2

Ultrasound-controlled multiple needle liver biopsy. The procedure was performed to obtain hepatic tissue for histopathological examination.

Figure 3

Histopathological examination of the punctured liver biopsy specimen. Microphotograph of histological appearance of liver biopsy specimen showing in the peri-portal region a group of ductal structures embedded in a hyalinized stroma. The ductal structures appear variably dilated and focal microcystic. These ducts are lined with a cubic or flattened biliary epithelium. (hematoxylin-eosin staining, magnification × 20).

Figure 4

Gadolinium contrast-enhanced magnetic resonance cholangiography. Multiple lesions were hyperintense on coronal thick slab T2 MIP. No communication between hamartomas and biliary ducts; noticeable typical formation of a “starry sky.”