New Trends in Management of Epilepsy in Patients with Cerebral Venous Malformations: Our Experience

Ivan P. Artyukhov, PhD, ScD¹; Diana V. Dmitrenko, PhD, ScD¹*; Natalia A. Shnayder, PhD, ScD¹; Ekaterina V. Gurenova¹; Alexander A. Molgachev²; Olga S. Shilkina¹; Evgenia A. Dontceva, PhD¹

¹Krasnoyarsk State Medical University; ²Krasnoyarsk Diagnostic-Treatment Center of International Institution of Biological Systems, Krasnoyarsk, the Russian Federation

*Corresponding author: Diana V. Dmitrenko, MD, PhD, ScD, Associate Professor of the Department of Medical Genetics and Brain Research of Postgraduate Education Institute, Krasnoyarsk State Medical University; Krasnoyarsk, Russia. E-mail:

Published: September 12, 2016.  DOI: 10.21103/Article6(3)_OA10


Background: Venous vascular malformations, also known as venous angiomas or, more exactly, developmental venous anomalies (DVAs), represent congenital, anatomically variant pathways in the normal venous drainage of the brain area. In general neurological practice, DVAs are not considered epileptogenic, therefore, in conducting neuroimaging as a rule, not taken into account. A positive correlation, however, between the location of the DVAs and the electroencephalographic seizure focus is debated.
Materials and Methods: The present study provides a complete analysis of clinical and MRI characteristics of symptomatic epilepsies associated with cerebral venous malformations in children and adults. Patients were selected after a retrospective search through the database of the Neurological Center of Epileptology, Neurogenetics and Brain Research of the University Clinic into which, since 2016, patients were prospectively entered. To date (February 2016), there is a total of 5,856 patients with epilepsy of which there are 105 patients with congenital malformations of the brain, and 32 of them were found to have principal diagnosis of DVA.
Results: Cavernous angiomas prevailed among venous anomalies (53.1%); DVAs were registered in 46.9% of cases. The associated analysis of DVA localization and the epileptic seizure types showed a direct relationship in 60.0% cases.
Conclusion: DVAs as a cause of seizures are important to consider when examining patients with epileptic seizures.

brain; developmental venous anomalies; cavernous malformation; epilepsy; management
  1. Truwit CL. Venous angioma of the brain: history, significance, and imaging findings. AJR Am J Roentgenol. 1992; 159: 1299–307.
  2. Ostertun B, Solymosi L. Magnetic resonance angiography of cerebral developmental venous anomalies: its role in differential diagnosis. Neuroradiology. 1993; 35: 97–104.
  3. Zimmer A, Hagen T, Ahlhelm F, Viera J , Reith W ,  Schulte-Altedorneburg G. Developmental venous anomaly (DVA). Radiologe. 2007; 47(10): 868, 870-4.
  4. Jones BV, Linscott L, Koberlein G,  Hummel TR, Leach JL.  Increased prevalence of developmental venous anomalies in children with intracranial neoplasms. AJNR Am J Neuroradiol. 2015; 36 (9):1782-5
  5.  Koc K, Anik I, Akansel Q, Anik Y, Ceylan S. Massive intracerebral haemorrage due to developmental venous anomaly. Br J Neurosurg. 2007; 21: 403–5.
  6. Uchino A, Hasuo K, Matsumoto S, Fujii K, Fukui M, Horino K, et al. Cerebral venous angiomas associated with hemorrhagic lesions. Their MRI manifestations. Clin Imaging. 1996; 20: 157–63.
  7. Baumann CR, Schuknecht B, Lo Russo G,  Cossu M, Citterio A, Andermann F, et al. Seizure outcome after resection of cavernous malformations is better when surrounding hemosiderin-stained brain also is removed. Epilepsia. 2006; 47: 563–6.
  8. Upchurch K, Stern JM, Salamon N,  Dewar S, Engel J Jr, Vinters HV, et al. Epileptogenic temporal cavernous malformations: Operative strategies and postoperative seizure outcomes. Seizure. 2010; 19 (2): 120–8.
  9.  Morioka T, Hashiguchi K, Nagata S, Miyagi Y, Yoshida F, Mihara F, et al. Epileptogenicity of supratentorial medullary venous malformation. Epilepsia. 2006; 47: 365–70.
  10. Lee C, Pennington MA, Kenney CM. III. MR evaluation of developmental venous anomalies: medullary venous anatomy of venous angiomas. AJNR Am J Neuroradiol. 1996; 17: 61–70.
  11. Lee BC, Vo KD, Kido DK. MR high-resolution blood oxygenation level-dependent venography of occult (low-flow) vascular lesions. AJNR Am J Neuroradiol. 1999. 20(7): 1239-42.
  12. Zhu WZ, Qi JP, Zhan CJ, Shu HG, Zhang L, Wang CY, et al. Magnetic resonance susceptibility weighted imaging in detecting intracranial calcification and hemorrhage. Chin. Med. J. 2008; 121 (20): 2021-5.
  13. Shnayder NA, Dmitrenko DV, Sadykova AV, Sharavii LK, Shulmin AV, Shapovalova EA, et al. Epidemiological studies on epilepsy in Siberia. Medical and Health Science Journal. 2011; 6: 35-42.
  14. Dmitrenko DV, Shnayder NA, Kiselev IA, Shulmin AV, Zhirova NV, Shapovalova EA, et al. Problems of rational therapy for epilepsy during pregnancy. Open Journal of Obstetrics and Gynecology. 2014; 4: 506-15.
  15. Striano S, Nocerino C, Striano P, Boccella P, Meo R, Bilo L, et al. Venous angiomas and epilepsy. Neurol. Sci. 2000; 21: 151–5.
  16. Awad IA, Robinson JR Jr, Mohanty S, Estes ML. Mixed vascular malformations of the brain: Clinical and pathogenic considerations. Neurosurgery. 1993; 33: 179–88.
  17. Naff NJ, Wemmer J, Hoenig-Rigamonti K, Rigamonti DR. A longitudinal study of patients with venous malformations: Documentation of a negligible hemorrhage risk and benign natural history. Neurology. 1998; 50: 1709–14.
  18. Abdulrauf SI, Kaynar MY, Awad IA. A comparison of the clinical profile of cavernous malformations with and without associated venous malformations. Neurosurgery.  1999; 44: 41–6, discussion 46–7.
  19. Chandra PS, Manjari T, Chandramouli BA,  Jayakumar PN,   Shankar SK. Cavernous-venous malformation of brain stem: Report of a case and review of literature. Surg Neurol. 1999; 52: 280–5.
  20. Wurm G, Schniyer M, Fellner FA. Cerebral cavernous malformations associated with venous anomalies: surgical considerations. Neurosurgery. 2005; 57 (1 Suppl): 42–58.
  21. Sarwar K, McCormick WF. Intracerebral venous angioma. Case report and review. Arch Neurol. 1978; 35: 323–5.
  22. Pereira VM, Geibprasert S, Krings T, Aurboonyawat T, Ozanne A, Toulgoat F, et al. Pathomechanisms of symptomatic developmental venous anomalies. Stroke. 2008; 39: 3201–15.
  23. Tomycz ND, Vora NV, Kanal E, Horowitz MB, Jovin TG. Intracranial arterialized venous angioma: case report with new insights from functional brain MRI. Diagn Interv Radiol. 2010; 16: 13–5.
  24. San Millán Ruíz D, Delavelle J, Yilmaz H, Gailloud P, Piovan E, Bertramello A, et al. Parenchymal abnormalities associated with developmental venous anomalies. Neuroradiology 2007; 49: 987–95.
  25. Rammos SK, Maina R, Lanzino G. Developmental venous anomalies: current concepts and implications for management. Neurosurgery. 2009; 65: 20-9; discussion 29-30.
  26. Saito Y, Kobayashi N. Cerebral venous angiomas: clinical evaluation and possible etiology. Radiology. 1981; 139: 87–94.
  27. Hong Y, Chung T-S, Suh S, Park CH, Tomar G, Seo KD, et al. The angioarchitectural factors of the cerebral developmental venous anomaly; can they be the causes of concurrent sporadic cavernous malformation? Neuroradiology. 2010; 52: 883–91.
  28. Ruíz DSM, Yilmaz H., Gailloud P. Cerebral developmental venous anomalies: current concepts. Ann Neurol. 2009; 66: 271–83.
  29. Lasjaunias P, Burrows P, Planet C. Developmental venous anomalies (DVA): the so-called venous angioma. Neurosug Rev. 1986; 9: 233–44.
  30. Saito Y, Kobayashi N. Cerebral venous angiomas: clinical evaluation and possible etiology. Radiology. 1981; 139: 87–94.
  31. Wilson CB Cryptic vascular malformations. Clin Neurosurg. 1992; 38: 49–84.
  32. Gallione CJ, Pasyk KA, Boon LM, Lenno F, Johnson DW, Helmbold E,et al. A gene for familial venous malformations maps to chromosome 9p in a second large kindred. J Med Genet. 1995; 32: 197–9.
  33. Valavanis A, Wellauer J, Yasargil MG. The radiological diagnosis of cerebral venous angioma: cerebral angiography and computed tomography. Neuroradiology. 1983; 24: 193–9.
  34. Pryor J, Setton A, Berenstein A. Venous anomalies and associated lesions. Neurosurg Clin N Am. 1999; 10: 519–25.
  35. Garner TB, Del Curling O Jr, Kelly DL Jr, Laster DW. The natural history of intracranial venous angiomas. J Neurosurg. 1991; 75: 715-22.
  36. Kondziolka D, Dempsey PK, Lunsford L.D. The case of conservative management of venous angiomas. Can J Neurol Sci. 1991; 18: 295–9.
  37. Handa H, Moritake K. Venous angioma of the brain. In: Fein JM, Flamm ES. Cerebrovascular Surgery. Vol. IV. Springer, New York, 1985, pp.1139-1149.
  38. Hon JM, Bhattacharya JJ, Counsell CE, Papanastassiou V, Ritchie V, Roberts RC, et al. The presentation and clinical course of intracranial developmental venous anomalies in adults: a systematic review and prospective, population-based study. Stroke. 2009; 40: 1980–5.
  39. Topper R, Jurgens E, Reual J, Thron A. Clinical significance of intracranial developmental venous anomalies. J Neurol Neurosurg Psychiatry. 1999; 67: 234–8.
  40. Lo S, Cheung Y, Tang K, Chan C, Siu C, Kwok K, et al. Non-haemorrhagic Pontine Venous Infarct due to Thrombosed Cerebellar Venous Angioma. J HK Coll Radiol. 2009; 12: 79–84.
  41. McLaughlin MR, Kondziolka D, Flickinger JC,  Lunsford S, Lunsford LD. The prospective nature history of cerebral venous malformations. Neurosurg. 1998; 43: 195-201.
  42. Griffiths D, Newey A, Faulder K, Steinfort B, Krause M. Thrombosis of a Developmental Venous Anomaly Causing Venous Infarction and Pontine Hemorrhage. J. Stroke Cerebrovasc. Dis. 2013;  22: 653-5.
  43. Vaitkevicius H, Erdemli HE, Anderson WS, Zamani AA, Friedlander RM, Folkerth RD.  Biopsy neuropathology of cerebral ischemia associated with a developmental venous anomaly. Clin Neurol Neurosurg. 2013; 115: 621–3.
  44. Numaguchi Y, Kitamura K, Fukui M,  Ikeda J, Hasuo K, Kishikawa T, et al. Intracranial venous angiomas. Surg Neurol. 1982; 18: 193–202.
  45. Fuji M, Kitahara T, Moroi J, Kato S, Ito H. Temporal lobe epilepsy associated with cerebral venous angioma-case report. Neurol Med Chir. 1998; 38: 413–6.
  46. Agnoli AL, Hildbrandt G. Cerebral venous angiomas. Acta Neurochir. 1985; 78: 4–12.
  47. Sohail A, Xiong Z, Qureshi MH, Qureshi AI. Complex Partial Epilepsy Associated with Temporal Lobe Developmental Venous Anomaly. J Vasc Interv Neurol. 2015; 8: 24-7.
  48. Scheidegger O, Wiest R, Jann K, König T, Meyer K, Hauf M.  Epileptogenic developmental venous anomaly: insights from simulataneous EEG/fMRI. Clin EEG Neurosci. 2013; 44: 157–60.
  49. Dănăilă L. The venous malformations of the brain. Proc Rom Acad, Series B.  2013; 15: 14–33.
  50. Biller J, Toffol GJ, Shea JF, Fine M, Azar-Kia B. Cerebral venous angiomas. A continuing controversy. Arch Neurol. 1985; 42: 367-70.
  51. Bitoh S, Hasegawa H, Fujiwara M, Sakurai M. Angiographically occult vascular malformations causing intracranial hemorrhage. Surg Neurol. 1982; 17: 35-42.
  52. Kramer RA, Wing SD. Computed tomography of angiographically occult cerebral vascular malformations. Radiology. 1977; 123: 649-52.
  53. Pak H, Patel SC, Malik GM, Ausman JI. Successful evacuation of a pontine hematoma secondary to rupture of a venous angioma. Surg Neurol. 1981; 15: 164–7.
  54. Cohen JE, Boitsova S, Moscovici S, Itshayek E. Concepts and controversies in the management of cerebral developmental venous anomalies. Isr Med Assoc J. 2010; 12: 703-6.
  55. Meng G, Bai C, Yu T, Wu Z, Liu X, Zhang J, et al. The association between cerebral developmental venous anomaly and concomitant cavernous malformation: an observational study using magnetic resonance imaging. BMC Neurol. 2014; 15: 14-50.

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Int J Biomed. 2016;6(3):207-212. © 2016 International Medical Research and Development Corporation. All rights reserved.