Multiple biliary hamartomas
Citation, DOI and article data
Multiple biliary hamartomas (MBHs) are a rare cause of multiple benign hepatic lesions. The condition is also known as von Meyenburg complexes, multiple bile duct hamartomas or biliary microhamartomas. Multiple biliary hamartomas are asymptomatic and usually found incidentally, when it is important to differentiate them from other causes of multiple liver lesions, particularly metastases.
The prevalence of multiple biliary hamartomas is ~3% at autopsy 1; however, prevalence on imaging is <1% as most hamartomas are <5 mm in size and often remain undetected. Multiple biliary hamartomas are reportedly three times more common in women than men 2.
Multiple biliary hamartomas are known to be associated with:
- autosomal dominant polycystic kidney disease (ADPKD) 3
- autosomal recessive polycystic kidney disease (ARPKD) 4
- polycystic liver disease 3
Biliary hamartomas are composed of small disorganized clusters of dilated cystic bile ducts lined by a single layer of cuboidal cells and surrounded by an abundant fibrocollagenous stroma 1,7,8. Although they may communicate with the biliary tree, they generally do not. They are thought to arise from embryonic bile duct remnants that have failed to involute.
Multiple small round or irregular lesions throughout the liver, with some predilection for subcapsular regions 1. Usually, 5-30 mm in size when detected by imaging (however most lesions are <5 mm when detected by pathologists). Radiographic findings can be non-specific and may be hard to differentiate from metastases and microabscesses.
Small hamartomas are usually echogenic if discretely seen. Often tiny individual hamartomas cannot be resolved and are instead interpreted as diffuse heterogenous liver echotexture. Larger hamartomas (>10 mm) may appear hypoechoic or anechoic and comet-tail artifact may be seen 9. The appearances may mimic metastases (see ultrasound appearances of liver metastases).
Biliary hamartomas are hypoattenuating and often show no enhancement 7. Occasionally an enhancing nodule or rim may be identified in a small number of lesions.
Only a limited number of the many lesions that are actually present can be seen as grape-like clusters of abnormal vascularity with contrast persisting into the venous phase 10.
Most biliary hamartomas are:
- T1: hypointense compared to liver parenchyma
- depending on TE, may approach CSF-signal intensity
T1 C+ (Gd)
- usually no enhancement
- thin peripheral rim enhancement has been described (see below), this may represent compressed normal liver parenchyma 14,15
However, in ~90% of cases an occasional hamartoma can be found containing a mural nodule (fibrocollagenous stroma, see Pathology above) or rim (see above) that is T1 isointense and T2 intermediate, of which ~90% will enhance. Hamartomas show no diffusion restriction on DWI sequences 2,9,11.
Cholescintigraphy shows delayed uptake and delayed emptying of tracer within larger biliary hamartomas 12.
Treatment and prognosis
Aside from the possible risk of malignant transformation (see Associations above), multiple biliary hamartomas are a benign asymptomatic condition with no long-term consequences, and no treatment is required.
History and etymology
Hans von Meyenburg (1887-1971) was a German-born Swiss pathologist, who first described the pathology of biliary hamartomas in an article published in 1918 13,16.
General imaging differential considerations include:
- 1. Chung EB. Multiple bile-duct hamartomas. Cancer. 1970;26 (2): 287-96. - Pubmed citation
- 2. Tohmé-noun C, Cazals D, Noun R et-al. Multiple biliary hamartomas: magnetic resonance features with histopathologic correlation. Eur Radiol. 2008;18 (3): 493-9. doi:10.1007/s00330-007-0790-z - Pubmed citation
- 3. Karhunen PJ. Adult polycystic liver disease and biliary microhamartomas (von Meyenburg's complexes). Acta Pathol Microbiol Immunol Scand A. 1986;94 (6): 397-400. - Pubmed citation
- 4. Pech L, Favelier S, Falcoz MT, Loffroy R, Krause D, Cercueil JP. Imaging of Von Meyenburg complexes. (2016) Diagnostic and interventional imaging. 97 (4): 401-9. doi:10.1016/j.diii.2015.05.012 - Pubmed
- 5. Xu AM, Xian ZH, Zhang SH et-al. Intrahepatic cholangiocarcinoma arising in multiple bile duct hamartomas: report of two cases and review of the literature. Eur J Gastroenterol Hepatol. 2009;21 (5): 580-4. doi:10.1097/MEG.0b013e3282fc73b1 - Pubmed citation
- 6. Heinke T, Pellacani LB, Costa Hde O et-al. Hepatocellular carcinoma in association with bile duct hamartomas: report on 2 cases and review of the literature. Ann Diagn Pathol. 2008;12 (3): 208-11. doi:10.1016/j.anndiagpath.2006.12.003 - Pubmed citation
- 7. Cooke JC, Cooke DA. The appearances of multiple biliary hamartomas of the liver (von Meyenberg complexes) on computed tomography. Clin Radiol. 1987;38 (1): 101-2. Clin Radiol (link) - Pubmed citation
- 8. Cotran RS, Kumar V, Collins T et-al. Robbins pathologic basis of disease. W.B. Saunders Company. (1999) ISBN:072167335X. Read it at Google Books - Find it at Amazon
- 9. Zheng RQ, Zhang B, Kudo M et-al. Imaging findings of biliary hamartomas. World J. Gastroenterol. 2005;11 (40): 6354-9. World J. Gastroenterol. (link) - Pubmed citation
- 10. Dähnert W. Radiology Review Manual. Lippincott Williams & Wilkins. (2007) ISBN:0781766206. Read it at Google Books - Find it at Amazon
- 11. Slone HW, Bennett WF, Bova JG. MR findings of multiple biliary hamartomas. AJR Am J Roentgenol. 1993;161 (3): 581-3. AJR Am J Roentgenol (citation) - Pubmed citation
- 12. Liu CH, Yen RF, Liu KL et-al. Biliary hamartomas with delayed 99mTc-diisopropyl iminodiacetic acid clearance. J. Gastroenterol. 2005;40 (5): 540-4. doi:10.1007/s00535-004-1581-9 - Pubmed citation
- 13. H. von Meyenburg. Über die Zystenleber. Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, Jena, 1918, 64: 477-532.
- 14. Lev-toaff AS, Bach AM, Wechsler RJ et-al. The radiologic and pathologic spectrum of biliary hamartomas. AJR Am J Roentgenol. 1995;165 (2): 309-13. AJR Am J Roentgenol (abstract) - Pubmed citation
- 15. Brancatelli G, Federle MP, Vilgrain V et-al. Fibropolycystic liver disease: CT and MR imaging findings. Radiographics. 25 (3): 659-70. doi:10.1148/rg.253045114 - Pubmed citation
- 16. Giles W Boland. Gastrointestinal Imaging: The Requisites E-Book. (2013) ISBN: 9780323170116
- 17. Piero Boraschi, Paola Scalise, Gaia Tarantini, Piero Colombatto, Francescamaria Donati. MR imaging features of multiple biliary hamartomas (Von Meyenburg Complex): A pictorial review and differential diagnosis. (2021) Journal of Medical Imaging and Radiation Oncology. 65 (3): 323. doi:10.1111/1754-9485.13173