Starry Liver: An Unexpected Diagnosis

A 65-year-old man presented with mildly elevated gamma-glutamyltranspeptidase (GGT) serum level on blood tests done for general check-up, and the finding of hepatomegaly and diffuse heterogeneity of the hepatic parenchyma on abdominal ultrasound. The patient’s history included essential hypertension treated with angiotensin converting enzyme-inhibitors. The patient had no other features of the metabolic syndrome and did not drink alcohol. Physical examination revealed a mildly enlarged liver with a smooth, non-tender margin and a regular surface. Serum GGT was 75 IU/L (normal: 11-50 IU/L); liver enzymes and testing for viral and autoimmune hepatitis were normal. Abdominal ultrasound showed diffuse hyperechoic liver echotexture with multiple, small hypoechoic lesions in both hepatic lobes, particularly evident in the hepatic dome. It also showed one 8-mm gallbladder stone with no intrahepatic and main bile duct dilation, and no signs of portal hypertension. Contrast-enhanced computed tomography showed small, multiple, round and irregular hypodense lesions in both hepatic lobes that did not enhance with contrast. Magnetic resonance imaging (MRI) and MR-cholangiography showed the presence of numerous small (<1.0–1.5-cm diameter) liver lesions hypointense on T1-weighted scans and hyperintense on heavily T2-weighted scans, not communicating with the bile ducts, giving the liver a “starry sky” appearance (Figure 1). Findings were consistent with the diagnosis of bile duct hamartomas (Von Meyenburg complexes; VMC).