Special Disease Management Considerations

  • Nabeel A. HerialEmail author
  • Magdy Selim


In this chapter, we present clinical scenarios of patients with spontaneous intracerebral hemorrhage, intraventricular hemorrhage, and hemorrhages secondary to underlying brain arteriovenous malformation and dural arteriovenous fistulas. Based on current available evidence, we also discuss management for each of the above conditions.


Intracerebral hemorrhage Intraventricular hemorrhage External ventricular drain Recombinant factor VII Arteriovenous malformation Dural arteriovenous fistula Embolization Stereotactic radio surgery 



Authors would like to thank Dr. Reid Gooch from the Department of Neurosurgery, Thomas Jefferson University for sharing a case of brain arteriovenous malformation for presentation in this chapter.


  1. 1.
    Bhattathiri PS, Gregson B, Prasad KS, Mendelow AD, STICH Investigators. Intraventricular hemorrhage and hydrocephalus after spontaneous intracerebral hemorrhage: results from the STICH trial. Acta Neurochir Suppl. 2006;96:65–8.CrossRefPubMedGoogle Scholar
  2. 2.
    Witsch J, Bruce E, Meyers E, Velazquez A, Schmidt JM, Suwatcharangkoon S, et al. Intraventricular hemorrhage expansion in patients with spontaneous intracerebral hemorrhage. Neurology. 2015;84(10):989–94.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Passero S, Ulivelli M, Reale F. Primary intraventricular haemorrhage in adults. Acta Neurol Scand. 2002;105:115–9.CrossRefPubMedGoogle Scholar
  4. 4.
    Zhu XL, Chan MS, Poon WS. Spontaneous intracranial hemorrhage: which patients need diagnostic cerebral angiography? A prospective study of 206 cases and review of the literature. Stroke. 1997;28:1406–9.CrossRefPubMedGoogle Scholar
  5. 5.
    Hemphill JC 3rd, Greenberg SM, Anderson CS, Becker K, Bendok BR, Cushman M, et al. Guidelines for the management of spontaneous intracerebral hemorrhage: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2015;46:2032–60.CrossRefPubMedGoogle Scholar
  6. 6.
    Wang YC, Lin CW, Shen CC, Lai SC, Kuo JS. Tissue plasminogen activator for the treatment of intraventricular hematoma: the dose-effect relationship. J Neurol Sci. 2002;202(1–2):35–41.CrossRefPubMedGoogle Scholar
  7. 7.
    Ávila C, Lozano C, De La Cueva VA, Moliner J, López M, San Miguel F, et al. Intraventricular hemorrhage treated with intraventricular fibrinolysis: a 10-year experience. Med Intensiva. 2013;37:61–6.CrossRefGoogle Scholar
  8. 8.
    Dunatov S, Antoncic I, Bralic M, Jurjevic A. Intraventricular thrombolysis with rt-PA in patients with intraventricular hemorrhage. Acta Neurol Scand. 2011;124:343–8.CrossRefPubMedGoogle Scholar
  9. 9.
    Fountas KN, Kapsalaki EZ, Parish DC, Smith B, Smisson HF, Johnston KW, et al. Intraventricular administration of rt-PA in patients with intraventricular hemorrhage. South Med J. 2005;98:767–73.CrossRefPubMedGoogle Scholar
  10. 10.
    Naff N, Williams MA, Keyl PM, Tuhrim S, Bullock MR, Mayer SA, et al. Low-dose recombinant tissue-type plasminogen activator enhances clot resolution in brain hemorrhage: the intraventricular hemorrhage thrombolysis trial. Stroke. 2011;42:3009–16.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Lapointe M, Haines S. Fibrinolytic therapy for intraventricular hemorrhage in adults. Cochrane Database Syst Rev. 2002:CD003692.Google Scholar
  12. 12.
    Khan NR, Tsivgoulis G, Lee SL, Jones GM, Green CS, Katsanos AH, et al. Fibrinolysis for intraventricular hemorrhage: an updated meta-analysis and systematic review of the literature. Stroke. 2014;45:2662–9.CrossRefPubMedGoogle Scholar
  13. 13.
    Hanley DF, Lane K, McBee N, Ziai W, Tuhrim S, Lees KR, et al. Thrombolytic removal of intraventricular haemorrhage in treatment of severe stroke: results of the randomised, multicentre, multiregion, placebo-controlled clear iii trial. Lancet. 2017;389(10069):603–11.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Wang D, Liu J, Norton C, Liu M, Selim M. Local fibrinolytic therapy for intraventricular hemorrhage: a meta-analysis of randomized controlled trials. 6th World Intracranial Hemorrhage Conference, Baltimore, May 2017 (abstract).Google Scholar
  15. 15.
    Dowlatshahi D, Demchuk AM, Flaherty ML, Ali M, Lyden PL, Smith EE, et al. Defining hematoma expansion in intracerebral hemorrhage: relationship with patient outcomes. Neurology. 2011;76(14):1238–44.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Brott T, Broderick J, Kothari R, Barsan W, Tomsick T, Sauerbeck L, et al. Early hemorrhage growth in patients with intracerebral hemorrhage. Stroke. 1997;28:1–5.CrossRefPubMedGoogle Scholar
  17. 17.
    Diringer MN, Skolnick BE, Mayer SA, Steiner T, Davis SM, Brun NC, et al. Thromboembolic events with recombinant activated factor VII in spontaneous intracerebral hemorrhage: results from the Factor Seven for Acute Hemorrhagic Stroke (FAST) trial. Stroke. 2010;41:48–53.CrossRefPubMedGoogle Scholar
  18. 18.
    Mayer SA, Brun NC, Begtrup K, Broderick J, Davis S, Diringer MN, et al. Efficacy and safety of recombinant activated factor VII for acute intracerebral hemorrhage. N Engl J Med. 2008;358(20):2127–37.CrossRefPubMedGoogle Scholar
  19. 19.
    Mayer SA, Davis SM, Skolnick BE, Brun NC, Begtrup K, Broderick JP, et al. Can a subset of intracerebral hemorrhage patients benefit from hemostatic therapy with recombinant activated factor VII? Stroke. 2009;40:833–40.CrossRefPubMedGoogle Scholar
  20. 20.
    Gladstone D, Aviv R, Demchuk A, Hill MD, Thorpe K, Khoury J, Sucharew H, De Masi S, Ka L, Mamdani M, Al-Ajlan F, Carrozzella J, Jauch E, Broderick JP, Flaherty M; for the SPOTLIGHT and STOP-IT Study Steering Committee and Investigators. Randomized trial of hemostatic therapy for ‘Spot Sign’ positive intracerebral hemorrhage: primary results from the SPOTLIGHT/STOP-IT Collaboration. AHA International Stroke Conference, Houston, February 2017 (abstract).Google Scholar
  21. 21.
    Brown RD Jr, Wiebers DO, Torner JC, O’Fallon WM. Incidence and prevalence of intracranial vascular malformations in Olmsted County, Minnesota, 1965 to 1992. Neurology. 1996;46(4):949–52.CrossRefPubMedGoogle Scholar
  22. 22.
    Berman MF, Sciacca RR, Pile-Spellman J, Stapf C, Connolly ES Jr, Mohr JP, et al. The epidemiology of brain arteriovenous malformations. Neurosurgery. 2000;47(2):389–96; discussion 397.CrossRefPubMedGoogle Scholar
  23. 23.
    ApSimon HT, Reef H, Phadke RV, Popovic EA. A population-based study of brain arteriovenous malformation: long-term treatment outcomes. Stroke. 33(12):2794–800.Google Scholar
  24. 24.
    Choi JH, Mohr JP. Brain arteriovenous malformations in adults. Lancet Neurol. 2005;4(5):299–308.CrossRefPubMedGoogle Scholar
  25. 25.
    Brown RD Jr, Wiebers DO, Forbes G, O'Fallon WM, Piepgras DG, Marsh WR, et al. The natural history of unruptured intracranial arteriovenous malformations. J Neurosurg. 1998;68(3):352–7.CrossRefGoogle Scholar
  26. 26.
    Mast H, Young WL, Koennecke HC, Sciacca RR, Osipov A, Pile-Spellman J, et al. Risk of spontaneous haemorrhage after diagnosis of cerebral arteriovenous malformation. Lancet. 1997;350(9084):1065–8.CrossRefPubMedGoogle Scholar
  27. 27.
    Wilkins RH. Natural history of intracranial vascular malformations: a review. Neurosurgery. 1985;16(3):421–30.CrossRefPubMedGoogle Scholar
  28. 28.
    Ondra SL, Troupp H, George ED, Schwab K. The natural history of symptomatic arteriovenous malformations of the brain: a 24-year follow-up assessment. J Neurosurg. 1990;73(3):387–91.CrossRefPubMedGoogle Scholar
  29. 29.
    Stapf C, Mast H, Sciacca RR, Choi JH, Khaw AV, Connolly ES, et al. Predictors of hemorrhage in patients with untreated brain arteriovenous malformation. Neurology. 2006;66(9):1350–5.CrossRefPubMedGoogle Scholar
  30. 30.
    Hetts SW, Cooke DL, Nelson J, Gupta N, Fullerton H, Amans MR, et al. Influence of patient age on angioarchitecture of brain arteriovenous malformations. AJNR Am J Neuroradiol. 2014;35(7):1376–80.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Mohr JP, Parides MK, Stapf C, Moquete E, Moy CS, Overbey JR, et al. Medical management with or without interventional therapy for unruptured brain arteriovenous malformations (ARUBA): a multicentre, non-blinded, randomised trial. Lancet. 2014;383(9917):614–21.CrossRefPubMedGoogle Scholar
  32. 32.
    Hartmann A, Mast H, Mohr JP, Pile-Spellman J, Connolly ES, Sciacca RR, et al. Determinants of staged endovascular and surgical treatment outcome of brain arteriovenous malformations. Stroke. 2005;36(11):2431–5.CrossRefPubMedGoogle Scholar
  33. 33.
    Cunha e Sa MJ, Stein BM, Solomon RA, McCormick PC. The treatment of associated intracranial aneurysms and arteriovenous malformations. J Neurosurg. 1992;77(6):853–9.CrossRefPubMedGoogle Scholar
  34. 34.
    Hamilton MG, Spetzler RF. The prospective application of a grading system for arteriovenous malformations. Neurosurgery. 1994;34(1):2–6.PubMedGoogle Scholar
  35. 35.
    Sure U, Butz N, Siegel AM, Mennel HD, Bien S, Bertalanffy H. Treatment-induced neoangiogenesis in cerebral arteriovenous malformations. Clin Neurol Neurosurg. 2001;103(1):29–32.CrossRefPubMedGoogle Scholar
  36. 36.
    Ding D, Starke RM, Kano H, Mathieu D, Huang PP, Kondziolka D, et al. Stereotactic radiosurgery for ARUBA (a randomized trial of unruptured brain arteriovenous malformations)-eligible Spetzler-Martin grade I and II arteriovenous malformations: a multicenter study. World Neurosurg. 2017;102:507–17.CrossRefPubMedGoogle Scholar
  37. 37.
    Morgan MK, Hermann Wiedmann MK, Stoodley MA, Heller GZ. Microsurgery for Spetzler-Ponce class A and B arteriovenous malformations utilizing an outcome score adopted from gamma knife radiosurgery: a prospective cohort study. J Neurosurg. 2016;23:1–12.Google Scholar
  38. 38.
    Ding D, Yen CP, Starke RM, Xu Z, Sun X, Sheehan JP. Radiosurgery for Spetzler-Martin grade III arteriovenous malformations. J Neurosurg. 2014;120(4):959–69.CrossRefPubMedGoogle Scholar
  39. 39.
    Ding D, Yen CP, Xu Z, Starke RM, Sheehan JP. Radiosurgery for patients with unruptured intracranial arteriovenous malformations. J Neurosurg. 2013;118(5):958–66.CrossRefPubMedGoogle Scholar
  40. 40.
    Maruyama K, Kawahara N, Shin M, Tago M, Kishimoto J, Kurita H, et al. The risk of hemorrhage after radiosurgery for cerebral arteriovenous malformations. N Engl J Med. 2005;352(2):146–53.CrossRefPubMedGoogle Scholar
  41. 41.
    Chaudhary MY, Sachdev VP, Cho SH, Weitzner I Jr, Puljic S, Huang YP. Dural arteriovenous malformation of the major venous sinuses: an acquired lesion. AJNR Am J Neuroradiol. 1982;3(1):13–9.PubMedGoogle Scholar
  42. 42.
    Watanabe A, Takahara Y, Ibuchi Y, Mizukami K. Two cases of dural arteriovenous malformation occurring after intracranial surgery. Neuroradiology. 1984;26(5):375–80.CrossRefPubMedGoogle Scholar
  43. 43.
    Cognard C, Gobin YP, Pierot L, Bailly AL, Houdart E, Casasco A, et al. Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage. Radiology. 1995;194(3):671–80.CrossRefPubMedGoogle Scholar
  44. 44.
    Borden JA, Wu JK, Shucart WA. A proposed classification for spinal and cranial dural arteriovenous fistulous malformations and implications for treatment. J Neurosurg. 1995;82(2):166–79. Erratum in: J Neurosurg. 1995 Apr;82(4):705–6.CrossRefPubMedGoogle Scholar
  45. 45.
    Davies MA, TerBrugge K, Willinsky R, Coyne T, Saleh J, Wallace MC. The validity of classification for the clinical presentation of intracranial dural arteriovenous fistulas. J Neurosurg. 1996;85(5):830–7.CrossRefPubMedGoogle Scholar
  46. 46.
    Davies MA, Saleh J, Ter Brugge K, Willinsky R, Wallace MC. The natural history and management of intracranial dural arteriovenous fistulae. Part 1: benig lesions. Interv Neuroradiol. 1997;3(4):295–302.CrossRefPubMedGoogle Scholar
  47. 47.
    Davies MA, Ter Brugge K, Willinsky R, Wallace MC. The natural history and management of intracranial dural arteriovenous fistulae. Part 2: aggressive lesions. Interv Neuroradiol. 1997;3(4):303–11.CrossRefPubMedGoogle Scholar
  48. 48.
    Duffau H, Lopes M, Janosevic V, Sichez JP, Faillot T, Capelle L, et al. Early rebleeding from intracranial dural arteriovenous fistulas: report of 20 cases and review of the literature. J Neurosurg. 1999;90(1):78–84.CrossRefPubMedGoogle Scholar
  49. 49.
    Willinsky R, Terbrugge K, Montanera W, Mikulis D, Wallace MC. Venous congestion: an MR finding in dural arteriovenous malformations with cortical venous drainage. AJNR Am J Neuroradiol. 1994;15(8):1501–7.PubMedGoogle Scholar
  50. 50.
    van Dijk JM, terBrugge KG, Willinsky RA, Wallace MC. Clinical course of cranial dural arteriovenous fistulas with long-term persistent cortical venous reflux. Stroke. 2002;33(5):1233–6.CrossRefPubMedGoogle Scholar
  51. 51.
    van Dijk JM, TerBrugge KG, Willinsky RA, Wallace MC. Multiplicity of dural arteriovenous fistulas. J Neurosurg. 2002;96(1):76–8.CrossRefPubMedGoogle Scholar
  52. 52.
    Roy D, Raymond J. The role of transvenous embolization in the treatment of intracranial dural arteriovenous fistulas. Neurosurgery. 1997;40(6):1133–41.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Departments of Neurology and Neurosurgery, Sidney Kimmel Medical CollegeThomas Jefferson UniversityPhiladelphiaUSA
  2. 2.Department of Neurology, Stroke DivisionBeth Israel Deaconess Medical CenterBostonUSA

Personalised recommendations