von Meyenburg complex

Case contributed by Dr Jan Frank Gerstenmaier

Presentation

Query cirrhosis secondary to NAFLD

Patient Data

Age: 51
Gender: Male

Ultrasound including elastogrpaphy

Modality: Ultrasound

The liver is coarsened in echotexture with a subtly nodular surface. The walls of the hepatic veins are slightly wavy. These features suggest cirrhosis. Multiple small hypoechoic lesions are seen within the liver which have features consistent with simple hepatic cysts. The two largest measure 9 mm and lie in segments IVa and V/VI. No other focal hepatic lesion is seen. The main portal vein is mildly enlarged measuring 14mm in maximum transverse diameter. It demonstrates flow in a normal direction. No flow is seen within the ligamentum teres. Numerous small, mobile gallstones are seen within the gallbladder. The common bile duct although not seen in its entirety due to bowel gas, is not dilated measuring 4 mm and there is no evidence of intrahepatic duct dilation.

ARFI - Median velocities and IQRs: 1) 1.52; 0.232  2) 1.27; 0.163 3) 1.39; 0.42

Conclusion:

1. The ultrasound features suggest cirrhosis with enlargement of the main portal vein suggestive of portal hypertension.

2. Multiple small hepatic lesions suggest multiple cysts.

3. There is discrepancy in the ARFI values, Two lying in the F2 range and the other in the F0/F1 range.

 

ARFI reference values:

  • 1.35 - absent or mild fibrosis (F0 or F1)
  • 1.35-1.55 - significant fibrosis (F2)
  • 1.55- 1.80 - severe fibrosis (F3)
  • >1.80 - cirrhosis (F4)

These thresholds are not absolute.

Values with an IQR of <0.3 x median velocity should be either discounted or interpreted with caution (depending on how close to this threshold they are). ie IQR/median velocity should be ideally >0.3

Based on meta analysis of pooled data which included a predominance of HCV patients - Friedrich-Rust et al J Viral hepatitis 2012, 19 e212-1219

Results should be interpreted in clinical context. Such things as inflammatory activity, venous congestion can elevate the ARFI value.

CT liver

Modality: CT

Reference is made to the ultrasound examination.The liver is slightly enlarged, but there is no morphological evidence of cirrhosis. The liver parenchyma is of normal density without evidence of fatty infiltration. There is no evidence of portal venous hypertension. The liver contains numerous circumscribed low density lesions, ranging in size between pinpoint and 10 mm. None of these lesions demonstrate enhancement. Some of the larger lesions are circumscribed and anechoic at ultrasound with some posterior acoustic enhancement and in keeping with cysts. The small and intermediate sized lesions are not shown to be cysts on ultrasound.

The hepatic vasculature is patent. The biliary tree is non-dilated.A number of renal cysts are shown. Adrenal glands, spleen, pancreas are normal in appearance. There is no lymphadenopathy or free fluid in the upper abdomen.Old right lower lobe rib fractures.

Conclusion: No CT evidence of cirrhosis or fatty infiltration. No evidence of portal venous hypertension. Mild hepatomegaly. Numerous low density lesions throughout the liver - the overall appearances favour multiple biliary hamartomas (von Meyenburg Complex) which could be confirmed with MRCP if clinically indicated.

MRCP

Modality: MRI

Scattered throughout the liver, there are innumerable high T2 signal lesions. Some of these are in keeping with cysts, particularly in segments 6 and 7, but the vast majority are less intense than cysts, and slightly ill-defined. There is no communication with the biliary tree. The common bile duct measures 7 mm in diameter. There is no intra or extrahepatic duct dilatation. No gallbladder is identified. The main pancreatic duct is not dilated. The pancreas is normal on non-contrast imaging. Left renal cyst, otherwise both kidneys, adrenal glands, and spleen within normal limits. No free fluid or lymphadenopathy.

Conclusion:
Multiple biliary hamartomas (von Meyenburg Complex) confirmed. No evidence of PSC. No evidence of malignancy.

Case Discussion

Typical appearances of multiple biliary hamartomas on US, CT and MR

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Case Information

rID: 38192
Case created: 9th Jul 2015
Last edited: 3rd Nov 2016
Inclusion in quiz mode: Included

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