Multiple biliary hamartomas “von Meyenberg complexes” in a newborn: a unique observation

Abstract

Background: Biliary hamartomas are rare benign bile duct malformations consisting of multiple collections of localized duct-like structures that are lined by biliary epithelium and embedded in a fibrous stroma. In general, these malformations are asymptomatic and are usually detected incidentally during imaging, surgical exploration, or autopsy. Previously, the definitive diagnosis of these lesions was possible only with liver biopsy. The use of advanced imaging modalities made it possible to establish the diagnosis when undoubtful imaging findings are evident. Case presentation: We present a 25-day-old male newborn who was referred to our institution with jaundice, abdominal distension, and abdominal U/S that revealed hepatomegaly and involvement of both lobes of the liver with diffuse cystic lesions of variable sizes and echogenicity without a diagnostic suggestion. We discuss the details of imaging findings that enabled to establish the diagnosis of multiple biliary hamartomas and brief the patient's status on follow-up. Conclusion: Up to our best knowledge, the diagnosis of multiple biliary hamartomas has not been previously reported among newborns, making this report an extremely rare if not a unique observation.