A Liver Full of Stars: Hepatostellular!

Abstract

Question: A 37-year-old white man with no past medical history was referred to our clinic for further evaluation of multiple incidental hepatic lesions. The patient initially presented to an outside hospital weeks prior for an episode of acute cholecystitis and underwent an uncomplicated laparoscopic cholecystectomy. During this hospitalization, hepatic lesions were incidentally noted on abdominal ultrasound and magnetic resonance imaging (MRI).Physical examination revealed a young, obese male (body mass index of 31 kg/m 2 ). Abdominal examination revealed healing laparoscopic scars, but otherwise was normal. Basic laboratory data including hepatic function panel were normal. Tumor markers for a-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 were normal. Viral studies for hepatitis B and C viruses were negative.Abdominal MRI with use of Gadobenate dimeglumine (Multihance) revealed a noncirrhotic liver with no evidence of portal hypertension. "Too numerous to count" small lesions, measuring up to 1.5 cm, were seen scattered uniformly throughout the liver. The lesions were T2 hyperintense ( Figure A) and T1 hypointense, without appreciable enhancement ( Figure B). The lesions did not show evidence of mass effect on hepatic vasculature. There were no communications between the biliary tree and lesions on magnetic resonance cholangiopancreatography (MRCP) ( Figure C). MRCP with 3-dimensional reconstruction demonstrates details of lesions ( Figure D).What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.

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

“…Biliary hamartoma or Von Meyenburg complex (VMC) is a congenital disorder that is characterized by multiple dilated cystic bile ducts. The discovery of such lesions in most cases is incidental, with an estimated incidence in the general population of 6%[ 1 ]. The formation of hamartomas has a genetic background and consists of remodeling of primitive ductal plate configurations[ 1 ].…”

“…The discovery of such lesions in most cases is incidental, with an estimated incidence in the general population of 6%[ 1 ]. The formation of hamartomas has a genetic background and consists of remodeling of primitive ductal plate configurations[ 1 ]. The distinctive feature of these cystic hepatic lesions is that they do not communicate with biliary tracts, and are usually small (up to 1.5 cm), dimensionally similar with each other, and countless, producing a “starry sky” configuration[ 1 ].…”

“…The formation of hamartomas has a genetic background and consists of remodeling of primitive ductal plate configurations[ 1 ]. The distinctive feature of these cystic hepatic lesions is that they do not communicate with biliary tracts, and are usually small (up to 1.5 cm), dimensionally similar with each other, and countless, producing a “starry sky” configuration[ 1 ]. Although in most cases hamartomas do not cause symptoms, VMC can have diverse clinical presentations, such as abdominal pain, fever, jaundice[ 2 ], and in rare cases, severe portal hypertension[ 3 ].…”

“…Multiple liver cystic lesions may cause diagnostic uncertainties, such as when mimicking liver metastasis on imaging[ 4 ]. In such cases, liver biopsy with subsequent histopathological evaluation is recommended[ 1 ]. Notably, blood analysis has not proven to be very useful due to the variability of findings and low specificity.…”

“…Complications of the condition include possible calcifications[ 5 ], portal hypertension[ 3 ], recurrent cholangitis with infectious complications[ 2 ] and malignization[ 1 ]. Currently no specific treatment is available, except treatment for symptomatic patients developing the abovementioned complications.…”