A starry sky by Von Meyenburg: Biliary hamartomatosis

An 82-year-old male with a history of abdominal aorta aneurysm with endovascular correction who went to accident and emergency with abdominal epigastric pain radiating to the dorsal region following physical effort. The physical examination showed abdominal pain in the right upper quadrant and jaundice. Blood tests revealed leukocytosis (12,000 ml), elevated ALT 63 U/l, AST 175 U/l, total bilirubin 6.7 mg/dl and alkaline phosphatase 163 U/l.

An abdominal CT scan was performed due to the patient's history, showing a grade II prosthetic leak. The abdominal ultrasound showed thickened gallbladder walls. The magnetic resonance cholangiopancreatography showed images suggesting Von Meyenburg complexes and the absence of biliary obstruction (Figs. 1 and 2). The patient progressed favourably and continued with outpatient clinical monitoring.

Figure 1.

Von Meyenburg complexes produce a starry sky appearance.

Figure 2.

Multiple images of microcystic appearance in the liver parenchyma.

Multiple biliary hamartomas (MBH) are rare benign malformations of the intrahepatic bile ducts, first described by Von Meyenburg in 1955.¹ MBH appear in 0.9% of children and 5.6% of adults. A ‘twinkling’ artifact can be seen, caused by the reverberations produced by the cholesterol crystals that fill the dilated ducts. Magnetic resonance provides greater sensitivity and specificity.² MBH is an asymptomatic, benign disease³ that does not require specific treatment.