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

Abstract

BACKGROUND Von Meyenburg complex (VMC) ( i.e., biliary hamartoma) is a rare congenital disorder characterized by multiple dilated cystic bile ducts, without clear trends in sex or age predominance. Due to the low number of published cases and the lack of recognized guidelines, the management of such patients remains a clinical challenge. CASE SUMMARY We present a case of symptomatic VMC that was diagnosed after imaging and histopathological examinations. Considering the patient’s condition, a conservative treatment strategy was chosen. Instrumental, laboratory, and clinical follow-up demonstrated the stable condition of the patient receiving conservative treatment. CONCLUSION VMC is a potentially non-life threatening condition, but its recognition is crucial for the management of patients.

Multiple biliary duct hamartomas in a cat resulting in a hepatic mass: A case report

“…Human case reports frequently discuss the advantages of magnetic resonance imaging (MRI), particularly contrast-enhanced cholangiopancreatography (MRCP), where BDHs manifest as numerous, small irregularly shaped cystic hepatic lesions (up to 1.5 cm) that lack communication with the biliary tree, resulting in a 'starry sky' or 'honeycomb' configuration (Priadko et al, 2022;Ryu et al, 2010). When paired with additional T1-and T2-weighted imaging or the use of contrast medium, MRCP highlights fluid within the biliary tract and pancreatic ducts, helping to better identify both intra-and extrahepatic disorders, such as inflammation, obstruction, or fluid-filled structures (Marolf, 2017;Vidal et al, 2020).…”

“…Rarely, cases can present with nonspecific clinical signs, such as pain on abdominal palpation, jaundice or fever. Although the majority of patients have normal laboratory results, some may develop minor haematologic abnormalities, most frequently increased liver enzymes (Carmona et al., 2022 ; Lanser & Puckett, 2023 ; Priadko et al., 2022 ; Sheikh et al., 2022 ).…”

“…These benign lesions may be difficult to differentiate between other ductal‐plate malformations (DPM), such as Caroli's disease and polycystic liver disease. BDHs can also be mistaken for more sinister diseases: biliary cystadenocarcinomas, cholangiocarcinoma, lymphangioma, hepatocellular carcinoma or cystic change of solid hepatic tumours (Priadko et al., 2022 ; Ryu et al., 2010 ; Soares et al., 2014 ; Strasberg & Chapman, 2021 ). In an animal with extrahepatic malignant neoplasia, establishing a diagnosis is of further importance due to BDHs bearing similar resemblance to metastatic lesions of the liver (Lanser & Puckett, 2023 ).…”

“…Although BDHs are clinically benign malformations, many patients have undergone surgery due to diagnostic uncertainty (Kakar et al., 2017 ; Priadko et al., 2022 ). Due to the low number of published cases and the lack of recognised guidelines, the management of such patients remains a clinical challenge (Lanser & Puckett, 2023 ; Priadko et al., 2022 ). Oftentimes, BDHs are treated conservatively; however, further investigation with biopsy may be required if progression is detected.…”

“…Several studies in human literature have shown evidence of BDHs transitioning to intrahepatic cholangiocarcinoma or, less commonly, hepatocellular carcinomas (Carmona et al., 2022 ; Lanser & Puckett, 2023 ; Sheikh et al., 2022 ; Vijayan et al., 2022 ). Once diagnosed with BDHs, routine surveillance via diagnostic imaging is necessary, but there are no set guidelines or clinical recommendation on the frequency of rechecks in the human literature (Kakar et al., 2017 ; Priadko et al., 2022 ).…”