The “von Meyenburg complex”

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  • Potential conflict of interest: Nothing to report.

A 67-year-old woman was admitted to our hospital with weakness, fatigue, fever, and persistent vomiting for 2 days. Physical examination showed reduced general condition and adiposity, but no abdominal tenderness. Laboratory tests revealed elevated levels of serum gamma glutamyltransferase (50 U/L [normal < 28 U/L]) and C-reactive protein (16 mg/dL [normal < 1 mg/dL]). Serum levels of total bilirubin and direct bilirubin were normal. Blood cultures were negative. Ultrasound examination of the abdomen showed multiple hyperechoic and hypoechoic liver lesions accentuated in the right liver lobe. The further diagnostic workup included a magnetic resonance cholangiopancreatography (MRCP) which showed multiple hyperintense liver lesions. There was no visible communication between the cystic lesions and the normal biliary system (Fig. AB). In some parts of the liver, the lesions were surrounded by fibrosis. Due to persisting uncertainty of the pathology, the patient underwent ultrasound-guided fine-needle biopsy, which showed chronic portal and periportal inflammation. In addition, septal fibrosis with bile duct proliferation and metaplasia could be observed. There was no evidence of malignancy. The histological findings were compatible with bile duct hamartomas embedded in a fibrous stroma (Fig. C).

Figure  .
Abbreviations:

MRCP, magnetic resonance cholangiopancreatography; VMC, von Meyenburg complex.

The numerous, disseminated cystic lesions in MRCP, smaller than 10 mm in size and without communication to the normal biliary system, is a highly suggestive imaging feature of multiple biliary hamartomas.1 The entity of multiple biliary hamartomas was first described by von Meyenburg in 1918 and is hence also known as the “von Meyenburg complex” (VMC).2 Although VMC is a rare clinical diagnosis, the prevalence of VMC is up to 5.6% in large autopsy series.3 Because VMC often lacks clinical symptoms, it is typically an incidental finding. On the other hand, there are single case reports of VMC associated with clinical symptoms of jaundice, epigastric pain, cholangitis, and fever.4 Thus, it can be easily confused with a variety of multifocal liver lesions, e.g., Caroli syndrome, cysts, or metastases.5 Differential diagnosis of VMC in MRCP depends on the number of lesions and their uniformity and dissemination. In addition, a normal-sized biliary tree and accompanying fibrosis are main diagnostic criteria. In asymptomatic patients with VMC, no treatment or follow-up examinations are required. Therefore, the knowledge of this distinctive imaging feature is important and can help physicians avoid unnecessary examinations and biopsies.

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