Von Meyenburg Complexes Associated with Polycystic Kidney Disease and The Endocystic Mural Nodule Sign: A Case Report
- 1. Department of Radiology, Mehmet Akif Inan Education and Research Hospital, University of Health Sciences, Şanlıurfa, Turkey.
- 2. Department of Radiology, Ankara Dr. Sami Ulus Education and Research Hospital, University of Health Sciences, Ankara, Turkey.
- 3. Department of General Surgery, Ankara Numune Training and Research Hospital, University of Health Sciences, Ankara, Turkey.
Description
Abstract
Von Meyenburg complexes (VMCs) are one of the rare, congenital, fibropolycystic benign diseases of the liver. We report a 40-year-old woman with characteristic imaging findings for diagnosis of VMCs associated with polycystic kidney disease. Multiple micro-nodular lesions, many of which were cystic, were detected on ultrasound (US). There were echogenic mural nodules and comet tail artifacts in their internal structures. In magnetic resonance imaging (MRI), the mural nodular enhancement and starry sky appearance were observed. The presence of endocystic mural nodules in both US and MRI, as well as the starry sky appearance that shows VMCs, are very important findings for the diagnosis of this condition.
Özet
Von Meyenburg kompleksleri (VMC'ler), karaciğerin nadir görülen doğuştan, fibropolikistik iyi huylu hastalıklarından biridir. Polikistik böbrek hastalığı ile ilişkili VMC'lerin tanısı için karakteristik görüntüleme bulguları olan 40 yaşında bir kadın olguyu sunuyoruz. Ultrasonografik (US) görüntülemede çoğu kistik yapıda olan çok sayıda mikronodüler lezyon tespit edildi. İç yapılarında ekojenik mural nodüller ve kuyruklu yıldız artefaktları vardı. Manyetik rezonans görüntülemede (MRG), mural nodüler kontrastlanma ve yıldızlı gökyüzü görünümü gözlemlendi. Hem US hem de MRG'de endokistik mural nodüllerin varlığı ve VMC'leri gösteren yıldızlı gökyüzü görünümü bu durumun tanısı için çok önemli bulgulardır.
Notes
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Additional details
References
- 1. Pech L, Favelier S, Falcoz MT, Loffroy R, Krause D, Cercueil JP. Imaging of Von Meyenburg complexes. Diagn Interv Imaging 2016; 97(4): 401-9.
- 2. Venkatanarasimha N, Thomas R, Armstrong EM, Shirley JF, Fox BM, Jackson SA. Imaging features of ductal plate malformations in adults. Clin Radiol 2011; 66(11): 1086-93.
- 3. Tohmé-Noun C, Cazals D, Noun R, Menassa L, Valla D, Vilgrain V. Multiple biliary hamartomas: magnetic resonance features with histopathologic correlation. Eur Radiol 2008; 18(3): 493-9.
- 4. Sasaki M, Nakanuma Y. Abnormal expression of MUC1 apomucin and mature MUC1 mucin in biliary epithelial cells in various cystic liver diseases. Hepatology 1996; 24(3): 539-43.
- 5. Lin S, Shang TY, Wang MF, Lin J, Ye XJ, Zeng DW, et al. Polycystic kidney and hepatic disease 1 gene mutations in von Meyenburg complexes: Case report. World J Clin Cases 2018; 6(9): 296-300.
- 6. Esseghaier S, Aidi Z, Toujani S, Daghfous MH. A starry sky: Multiple biliary hamartomas. Presse Med 2017; 46(7-8 Pt 1): 787-8.
- 7. Zheng RQ, Zhang B, Kudo M, Onda H, Inoue T. Imaging findings of biliary hamartomas. World J Gastroenterol 2005; 11(40): 6354-9.
- 8. Jain D, Ahrens W, Finkelstein S. Molecular evidence for the neoplastic potential of hepatic Von-Meyenburg complexes. Appl Immunohistochem Mol Morphol 2010; 18(2): 166-71.