Abstract
Cerebral vascular malformations comprise a diverse set of pathologies ranging from high-flow arteriovenous malformation and fistula to cavernous malformations and capillary telangiectasia. While rare, vascular malformations occurring during pregnancy present a unique challenge for diagnosis and management due to the need to balance both maternal and fetal well-being. Historically, concern regarding increased risk of rupture and hemorrhage of cerebral vascular malformations has led to the counseling of women with such lesions against pregnancy and vaginal delivery. However, this has been refuted by the results of more recent studies. The presence of a cerebral vascular malformation should not be a contraindication to pregnancy; however, preconception treatment should be performed for high-risk lesions. Identification of a lesion during pregnancy should be treated based on neurosurgical indications. Finally, in women with unruptured, lower risk malformations, vaginal delivery with precautions to minimize undue stress appears to be safe. However, in untreated, higher risk lesions and those that have ruptured, we recommend delivery via cesarean section. Decision making in this setting should follow that of all medical decisions: minimizing risks of harm while maximizing the benefit for the patient.
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References
Flemming KD, Robert D, Brown J. 401: Epidemiology and natural history of intracranial Vascular Malformations. In: Winn HR, editor. Youmans & Winn neurological surgery. Philadelphia, PA: Elsevier; 2017.
Gavin CG, Kitchen ND. 400: Pathobiology of true arteriovenous malformations. In: Winn HR, editor. Youmans & Winn neurological surgery. 7th ed. Philadelphia, PA: Elsevier; 2017.
Nikolaev SI, Vetiska S, Bonilla X, Boudreau E, Jauhiainen S, Rezai Jahromi B, et al. Somatic activating KRAS mutations in arteriovenous malformations of the brain. N Engl J Med. 2018;378(3):250–61.
Al-Olabi L, Polubothu S, Dowsett K, Andrews KA, Stadnik P, Joseph AP, et al. Mosaic RAS/MAPK variants cause sporadic vascular malformations which respond to targeted therapy. J Clin Invest. 2018;128(4):1496–508.
Pawlikowska L, Tran MN, Achrol AS, Ha C, Burchard E, Choudhry S, et al. Polymorphisms in transforming growth factor-beta-related genes ALK1 and ENG are associated with sporadic brain arteriovenous malformations. Stroke. 2005;36(10):2278–80.
Ge M, Du C, Li Z, Liu Y, Xu S, Zhang L, et al. Association of ACVRL1 genetic polymorphisms with arteriovenous malformations: a case-control study and meta-analysis. World Neurosurg. 2017;108:690–7.
Pawlikowska L, Tran MN, Achrol AS, McCulloch CE, Ha C, Lind DL, et al. Polymorphisms in genes involved in inflammatory and angiogenic pathways and the risk of hemorrhagic presentation of brain arteriovenous malformations. Stroke. 2004;35(10):2294–300.
van Beijnum J, van der Worp HB, Schippers HM, van Nieuwenhuizen O, Kappelle LJ, Rinkel GJ, et al. Familial occurrence of brain arteriovenous malformations: a systematic review. J Neurol Neurosurg Psychiatry. 2007;78(11):1213–7.
van Beijnum J, van der Worp HB, Algra A, Vandertop WP, van den Berg R, Brouwer PA, et al. Prevalence of brain arteriovenous malformations in first-degree relatives of patients with a brain arteriovenous malformation. Stroke. 2014;45(11):3231–5.
Santos R, Aguilar-Salinas P, Entwistle JJ, Aldana PR, Beier AD, Hanel RA. De novo arteriovenous malformation in a pediatric patient: case report and review of the literature. World Neurosurg. 2018;111:341–5.
Lv X, Wang G. Review of de novo cerebral arteriovenous malformation: haemorrhage risk, treatment approaches and outcomes. Neuroradiol J. 2018;31(3):224–9.
Barbosa Do Prado L, Han C, Oh SP, Su H. Recent advances in basic research for brain arteriovenous malformation. Int J Mol Sci. 2019;20(21):5324.
So JM, Holman RE. Wyburn-Mason syndrome. Treasure Island, FL: StatPearls; 2020.
Sanghavi M, Rutherford JD. Cardiovascular physiology of pregnancy. Circulation. 2014;130(12):1003–8.
Johnson AC, Cipolla MJ. The cerebral circulation during pregnancy: adapting to preserve normalcy. Physiology (Bethesda). 2015;30(2):139–47.
Krause DN, Duckles SP, Pelligrino DA. Influence of sex steroid hormones on cerebrovascular function. J Appl Physiol. 2006;101(4):1252–61.
Liu XJ, Wang S, Zhao YL, Teo M, Guo P, Zhang D, et al. Risk of cerebral arteriovenous malformation rupture during pregnancy and puerperium. Neurology. 2014;82(20):1798–803.
Ceylan D, Tatarli N, Avsar T, Arslanhan A, Bozkurt SU, Bagci P, et al. In vivo effect of pregnancy on angiogenesis potential of arteriovenous malformation tissue samples: an experimental study. J Neurosurg Sci. 2017;61(2):151–6.
Jellinger K. Vascular malformations of the central nervous system: a morphological overview. Neurosurg Rev. 1986;9(3):177–216.
Aoki R, Srivatanakul K. Developmental venous anomaly: benign or not benign. Neurol Med Chir (Tokyo). 2016;56(9):534–43.
NORD. Arteriovenous malformation: national organization for rare diseases; 2019. https://rarediseases.org/rare-diseases/arteriovenous-malformation/.
Kim T, Kwon OK, Bang JS, Lee H, Kim JE, Kang HS, et al. Epidemiology of ruptured brain arteriovenous malformation: a National Cohort Study in Korea. J Neurosurg. 2018:1–6.
Mohr JP, Kejda-Scharler J, Pile-Spellman J. Diagnosis and treatment of arteriovenous malformations. Curr Neurol Neurosci Rep. 2013;13(2):324.
Hofmeister C, Stapf C, Hartmann A, Sciacca RR, Mansmann U, terBrugge K, et al. Demographic, morphological, and clinical characteristics of 1289 patients with brain arteriovenous malformation. Stroke. 2000;31(6):1307–10.
Kim H, Al-Shahi Salman R, McCulloch CE, Stapf C, Young WL, Coinvestigators M. Untreated brain arteriovenous malformation: patient-level meta-analysis of hemorrhage predictors. Neurology. 2014;83(7):590–7.
Shaligram SS, Winkler E, Cooke D, Su H. Risk factors for hemorrhage of brain arteriovenous malformation. CNS Neurosci Ther. 2019;25(10):1085–95.
Al-Shahi R, Warlow C. A systematic review of the frequency and prognosis of arteriovenous malformations of the brain in adults. Brain. 2001;124(Pt 10):1900–26.
Laakso A, Hernesniemi J. Arteriovenous malformations: epidemiology and clinical presentation. Neurosurg Clin N Am. 2012;23(1):1–6.
Stapf C, Khaw AV, Sciacca RR, Hofmeister C, Schumacher HC, Pile-Spellman J, et al. Effect of age on clinical and morphological characteristics in patients with brain arteriovenous malformation. Stroke. 2003;34(11):2664–9.
Gross BA, Du R. Natural history of cerebral arteriovenous malformations: a meta-analysis. J Neurosurg. 2013;118(2):437–43.
Camargo EC, Feske SK, Singhal AB. Stroke in pregnancy: an update. Neurol Clin. 2019;37(1):131–48.
Fairhall JM, Stoodley MA. Intracranial haemorrhage in pregnancy. Obstet Med. 2009;2(4):142–8.
Porras JL, Yang W, Philadelphia E, Law J, Garzon-Muvdi T, Caplan JM, et al. Hemorrhage risk of brain arteriovenous malformations during pregnancy and puerperium in a North American Cohort. Stroke. 2017;48(6):1507–13.
Gross BA, Du R. Hemorrhage from arteriovenous malformations during pregnancy. Neurosurgery. 2012;71(2):349–55; discussion 55–6.
van Beijnum J, Wilkinson T, Whitaker HJ, van der Bom JG, Algra A, Vandertop WP, et al. Relative risk of hemorrhage during pregnancy in patients with brain arteriovenous malformations. Int J Stroke. 2017;12(7):741–7.
Zhu D, Zhao P, Lv N, Li Q, Fang Y, Li Z, et al. Rupture risk of cerebral arteriovenous malformations during pregnancy and puerperium: a single-center experience and pooled data analysis. World Neurosurg. 2018;111:e308–e15.
Lee S, Kim Y, Navi BB, Abdelkhaleq R, Salazar-Marioni S, Blackburn SL, et al. Risk of intracranial hemorrhage associated with pregnancy in women with cerebral arteriovenous malformations. J Neurointerv Surg. 2021;13(8):707–10.
Davidoff CL, Lo Presti A, Rogers JM, Simons M, Assaad NNA, Stoodley MA, et al. Risk of first hemorrhage of brain arteriovenous malformations during pregnancy: a systematic review of the literature. Neurosurgery. 2019;85(5):E806–E14.
Flemming KD, Lanzino G. Management of unruptured intracranial aneurysms and cerebrovascular malformations. Continuum. 2017;23(1, Cerebrovascular Disease):181–210.
Willinsky RA, Lasjaunias P, Terbrugge K, Burrows P. Multiple cerebral arteriovenous malformations (AVMs). Review of our experience from 203 patients with cerebral vascular lesions. Neuroradiology. 1990;32(3):207–10.
Bari O, Cohen PR. Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse events and pregnancy outcomes. Int J Women’s Health. 2017;9:373–8.
Kanekar S, Bennett S. Imaging of neurologic conditions in pregnant patients. Radiographics. 2016;36(7):2102–22.
Tranvinh E, Heit JJ, Hacein-Bey L, Provenzale J, Wintermark M. Contemporary imaging of cerebral arteriovenous malformations. AJR Am J Roentgenol. 2017;208(6):1320–30.
Josephson CB, White PM, Krishan A, Al-Shahi Salman R. Computed tomography angiography or magnetic resonance angiography for detection of intracranial vascular malformations in patients with intracerebral haemorrhage. Cochrane Database Syst Rev. 2014;9:CD009372.
Derdeyn CP, Zipfel GJ, Albuquerque FC, Cooke DL, Feldmann E, Sheehan JP, et al. Management of brain arteriovenous malformations: a scientific statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2017;48(8):e200–e24.
Robinson JL, Hall CS, Sedzimir CB. Arteriovenous malformations, aneurysms, and pregnancy. J Neurosurg. 1974;41(1):63–70.
Yamada S, Nakase H, Nakagawa I, Nishimura F, Motoyama Y, Park YS. Cavernous malformations in pregnancy. Neurol Med Chir (Tokyo). 2013;53(8):555–60.
Pollock BE, Garces YI, Stafford SL, Foote RL, Schomberg PJ, Link MJ. Stereotactic radiosurgery for cavernous malformations. J Neurosurg. 2000;93(6):987–91.
Tonetti D, Kano H, Bowden G, Flickinger JC, Lunsford LD. Hemorrhage during pregnancy in the latency interval after stereotactic radiosurgery for arteriovenous malformations. J Neurosurg. 2014;121(Suppl):226–31.
Nishida T, Faughnan ME, Krings T, Chakinala M, Gossage JR, Young WL, et al. Brain arteriovenous malformations associated with hereditary hemorrhagic telangiectasia: gene-phenotype correlations. Am J Med Genet A. 2012;158A(11):2829–34.
McDonald J, Pyeritz RE. Hereditary hemorrhagic telangiectasia. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, et al., editors. GeneReviews®. Seattle, WA: University of Washington; 1993.
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.
Jermakowicz WJ, Tomycz LD, Ghiassi M, Singer RJ. Use of endovascular embolization to treat a ruptured arteriovenous malformation in a pregnant woman: a case report. J Med Case Rep. 2012;6:113.
Lv X, Liu P, Li Y. Pre-existing, incidental and hemorrhagic AVMs in pregnancy and postpartum: gestational age, morbidity and mortality, management and risk to the fetus. Interv Neuroradiol. 2016;22(2):206–11.
Solomon RA, Connolly ES Jr. Arteriovenous malformations of the brain. N Engl J Med. 2017;376(19):1859–66.
Tayebi Meybodi A, Lawton MT. Modern radiosurgical and endovascular classification schemes for brain arteriovenous malformations. Neurosurg Rev. 2018;43:49.
Sadasivan B, Malik GM, Lee C, Ausman JI. Vascular malformations and pregnancy. Surg Neurol. 1990;33(5):305–13.
Agarwal N, Guerra JC, Gala NB, Agarwal P, Zouzias A, Gandhi CD, et al. Current treatment options for cerebral arteriovenous malformations in pregnancy: a review of the literature. World Neurosurg. 2014;81(1):83–90.
Lv X, Li Y. The clinical characteristics and treatment of cerebral AVM in pregnancy. Neuroradiol J. 2015;28(4):385–8.
Beach RL, Kaplan PW. Seizures in pregnancy: diagnosis and management. Int Rev Neurobiol. 2008;83:259–71.
Hernandez-Diaz S, Smith CR, Shen A, Mittendorf R, Hauser WA, Yerby M, et al. Comparative safety of antiepileptic drugs during pregnancy. Neurology. 2012;78(21):1692–9.
Hemphill JC III, 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(7):2032–60.
Bernstein PS, Martin JN Jr, Barton JR, Shields LE, Druzin ML, Scavone BM, et al. Consensus bundle on severe hypertension during pregnancy and the postpartum period. J Midwif Womens Health. 2017;62(4):493–501.
Gross BA, Du R. Rate of re-bleeding of arteriovenous malformations in the first year after rupture. J Clin Neurosci. 2012;19(8):1087–8.
Beecher JS, Lyon K, Ban VS, Vance A, McDougall CM, Whitworth LA, et al. Delayed treatment of ruptured brain AVMs: is it ok to wait? J Neurosurg. 2018;128(4):999–1005.
Pollock BE, Flickinger JC, Lunsford LD, Maitz A, Kondziolka D. Factors associated with successful arteriovenous malformation radiosurgery. Neurosurgery. 1998;42(6):1239–44; discussion 44–7.
Trivedi RA, Kirkpatrick PJ. Arteriovenous malformations of the cerebral circulation that rupture in pregnancy. J Obstet Gynaecol. 2003;23(5):484–9.
Salvati A, Ferrari C, Chiumarulo L, Medicamento N, Dicuonzo F, De Blasi R. Endovascular treatment of brain arteriovenous malformations ruptured during pregnancy--a report of two cases. J Neurol Sci. 2011;308(1–2):158–61.
Nagayama K, Kurita H, Tonari A, Takayama M, Shiokawa Y. Radiosurgery for cerebral arteriovenous malformation during pregnancy: a case report focusing on fetal exposure to radiation. Asian J Neurosurg. 2010;5(2):73–7.
Bervini D, Morgan MK, Ritson EA, Heller G. Surgery for unruptured arteriovenous malformations of the brain is better than conservative management for selected cases: a prospective cohort study. J Neurosurg. 2014;121(4):878–90.
Kazemi P, Villar G, Flexman AM. Anesthetic management of neurosurgical procedures during pregnancy: a case series. J Neurosurg Anesthesiol. 2014;26(3):234–40.
Krywko DM, King KC. Aortocaval compression syndrome. Treasure Island, FL: StatPearls; 2020.
van Rooij WJ, Sluzewski M, Beute GN. Brain AVM embolization with Onyx. AJNR Am J Neuroradiol. 2007;28(1):172–7; discussion 8.
Bokhari MR, Al-Dhahir MA. Cavernous brain angiomas. Treasure Island, FL: StatPearls; 2020.
Petersen TA, Morrison LA, Schrader RM, Hart BL. Familial versus sporadic cavernous malformations: differences in developmental venous anomaly association and lesion phenotype. AJNR Am J Neuroradiol. 2010;31(2):377–82.
Zafar A, Quadri SA, Farooqui M, Ikram A, Robinson M, Hart BL, et al. Familial cerebral cavernous malformations. Stroke. 2019;50(5):1294–301.
Joseph NK, Kumar S, Brown RD Jr, Lanzino G, Flemming KD. Influence of pregnancy on hemorrhage risk in women with cerebral and spinal cavernous malformations. Stroke. 2021;52(2):434–41.
Otten P, Pizzolato GP, Rilliet B, Berney J. [131 cases of cavernous angioma (cavernomas) of the CNS, discovered by retrospective analysis of 24,535 autopsies]. Neurochirurgie. 1989;35(2):82–3, 128–31.
Del Curling O Jr, Kelly DL Jr, Elster AD, Craven TE. An analysis of the natural history of cavernous angiomas. J Neurosurg. 1991;75(5):702–8.
Robinson JR, Awad IA, Little JR. Natural history of the cavernous angioma. J Neurosurg. 1991;75(5):709–14.
Kalani MY, Zabramski JM. Risk for symptomatic hemorrhage of cerebral cavernous malformations during pregnancy. J Neurosurg. 2013;118(1):50–5.
Gross BA, Lin N, Du R, Day AL. The natural history of intracranial cavernous malformations. Neurosurg Focus. 2011;30(6):E24.
Gross BA, Du R. Hemorrhage from cerebral cavernous malformations: a systematic pooled analysis. J Neurosurg. 2017;126(4):1079–87.
Zabramski JM, Kalani MYS. 409: Natural history of cavernous malformations. In: Winn HR, editor. Youmans & Winn Neurological Surgery. Philadelphia, PA: Elsevier; 2017.
Safavi-Abbasi S, Feiz-Erfan I, Spetzler RF, Kim L, Dogan S, Porter RW, et al. Hemorrhage of cavernous malformations during pregnancy and in the peripartum period: causal or coincidence? Case report and review of the literature. Neurosurg Focus. 2006;21(1):e12.
Witiw CD, Abou-Hamden A, Kulkarni AV, Silvaggio JA, Schneider C, Wallace MC. Cerebral cavernous malformations and pregnancy: hemorrhage risk and influence on obstetrical management. Neurosurgery. 2012;71(3):626–30; discussion 31.
Zyck S, Gould GC. Cavernous venous malformation. Treasure Island, FL: StatPearls; 2020.
Morrison L, Akers A. Cerebral cavernous malformation, familial. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, et al., editors. GeneReviews®. Seattle, WA: University of Washington; 1993.
Simonazzi G, Curti A, Rapacchia G, Gabrielli S, Pilu G, Rizzo N, et al. Symptomatic cerebral cavernomas in pregnancy: a series of 6 cases and review of the literature. J Matern Fetal Neonatal Med. 2014;27(3):261–4.
Flemming KD, Goodman BP, Meyer FB. Successful brainstem cavernous malformation resection after repeated hemorrhages during pregnancy. Surg Neurol. 2003;60(6):545–7; discussion 7–8.
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Rallo, M.S., Majmundar, N., Sreenivasan, S., Roychowdhury, S., Nanda, A., Gupta, G. (2023). Cerebral Vascular Malformations in Pregnancy: Considerations for Diagnosis and Management. In: Gupta, G., et al. Neurological Disorders in Pregnancy. Springer, Cham. https://doi.org/10.1007/978-3-031-36490-7_9
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