Brain Arteriovenous Malformations

Abstract

HomeStrokeVol. 36, No. 10Brain Arteriovenous Malformations Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessEditorialPDF/EPUBBrain Arteriovenous MalformationsChildren and Adults J.P. Mohr, MS, MD J.P. MohrJ.P. Mohr From the Doris & Stanley Tananbaum Stroke Center, Neurological Institute, Columbia University Medical Center, New York, NY Search for more papers by this author Originally published1 Oct 2005https://doi.org/10.1161/01.STR.0000181775.06537.d9Stroke. 2005;36:2060–2061Although intracranial hemorrhages,1 interest in brain arteriovenous malformations (BAVMs) is expanding from its origins as a neuropathologic curiosity to the treatment-oriented specialties of neurological surgery, interventional neuroradiology, radiosurgery/therapy, and clinical neurology, and is even emerging as a subject in neuroepidemiology.Recent insights from uncontrolled series: argue for some genetic derangements in the origin of some of the lesions2,3; have revised some issues of pathophysiology4–7; track the risk of hemorrhage over time8; suggest the risk for hemorrhage can be predicted by vascular factors discoverable by noninvasive or minimally invasive imaging9,10; indicate many initial hemorrhage syndromes are mild,11 more so than that caused by non-BAVM parenchymal or aneurysmal subarachnoid hemorrhages12; have reassessed the outcomes from intervention in a variety of forms after initial hemorrhage13–15; raise questions concerning the safety and value of intervention before hemorrhage16,17; and support a proposal for randomized trials for BAVMs discovered before rupture,18,19 to name but a few of the directions in which work is proceeding in this field.The prevalence of BAVMs is difficult to estimate, given the lack of a population subject to uniform brain imaging.20 Some insights are provided from rare studies from the likes of uniform magnetic resonance screening of clinically normal recruitees for the German Air Force,21 showing a relative frequency of <0.7% of asymptomatic brain lesions, including among them BAVMs. However, most information comes from nonuniform imaging done in recently reported large prospective population settings, in which 0.5 of 100 000 cases of BAVM discovered after hemorrhage compare with up to 0.9 of 100 000 discovered before hemorrhage.22,23But what of children? In most studies, the focus is on adults,19 with some more recent studies also including children, most of them either small cohorts of children24,25 or containing reference to them as part of a larger survey. Among the latter, population-based data from the ongoing New York Islands AVM study26 suggest 20% of all AVMs are detected in patients <21 years of age, including 8% in children ≤10 years of age. Among the latter, the relative proportion of incident hemorrhage appears to be significantly higher (65%) when compared with the entire cohort (38%; P<0.01).In this issue of Stroke, the world-renowned authors have made recourse to the large clinical databases from the University of California San Francisco and Kaiser-Permanente. In size, this cohort eclipses previous efforts and seems likely to set the new reference point. Some statisticians may be uncomfortable with the lack of precise population-based data represented here, but this is a small field, with limited data from other sources. Until even better data sources are created, findings from this study will surely attract interest. Leading the list is the observation that “… children do not appear to be at increased risk for a subsequent ICH (intracerebral hemorrhage) compared with adults and may even be relatively protected.” Differing as it does from previous reports focusing mainly on adults27,28 and from other sources claiming high risks of hemorrhage recurrence in children,29,30 this inference is sure to have an impact on current views of pathophysiology and is bound to prompt controversy.Hopefully, this publication will spur a larger effort. Among the many things lacking in the field is insight into whether and to what extent BAVM in children is a different disease than that in adults. The current publication suggests they compare favorably with adults for the first hemorrhage and follow a different course for recurrence. Given the middle-aged emergence of clinical syndromes in some patients and the stable course followed by some whose lesions are deemed too daunting for therapy at any age, those interested in treatment need better insight into whose BAVM is a static anomaly versus one best dealt with before it becomes a threat to life.Closer study of children might help change or expand the simile for BAVMs. Although some may be time bombs, others seem like meningiomas, and some even like arachnoid cysts, more dangerous when misdiagnosed as needing treatment than they are when left undiscovered.The opinions in this editorial are not necessarily those of the editors or the American Heart Association.See related article, pages 2099–2104FootnotesCorrespondence to J.P. Mohr, MS, MD, Doris & Stanley Tananbaum Stroke Center, Neurological Institute, Columbia University Medical Center, 710 West 168th Street, New York, NY 10032. E-mail [email protected] References 1 Stapf C, Labovitz DL, Sciacca RR, Mast H, Mohr JP, Sacco RL. Incidence of adult brain arteriovenous malformation hemorrhage in a prospective population-based stroke survey. Cerebrovasc Dis. 2002; 13: 43–46.CrossrefMedlineGoogle Scholar2 Pawlikowska L, Tran MN, Achrol AS, McCulloch CE, Ha C, Lind DL, Hashimoto T, Zaroff J, Lawton MT, Marchuk DA, Kwok PY, Young WL; UCSF BAVM Study Project. Polymorphisms in genes involved in inflammatory and angiogenic pathways and the risk of hemorrhagic presentation of brain arteriovenous malformations. Stroke. 2004; 35: 2294–2300.LinkGoogle Scholar3 Kilic T, Pamir MN, Kullu S, Eren F, Ozek MM, Black PM. Expression of structural proteins and angiogenic factors in cerebrovascular anomalies. Neurosurgery. 2000; 46: 1179–1191.CrossrefMedlineGoogle Scholar4 Young WL, Kader A, Ornstein E, Baker KZ, Ostapkovich N, Pile-Spellman J, Fogarty-Mack P, Stein BM. Cerebral hyperemia after arteriovenous malformation resection is related to “breakthrough” complications but not to feeding artery pressure. The Columbia University Arteriovenous Malformation Study Project. Neurosurgery. 1996; 38: 1085–1089.MedlineGoogle Scholar5 Mast H, Mohr JP, Osipov A, Pile-Spellman J, Marshall RS, Lazar RM, Stein BM, Young WL. “Steal” is an unestablished mechanism for the clinical presentation of cerebral arteriovenous malformations. Stroke. 1995; 26: 1215–1220.CrossrefMedlineGoogle Scholar6 Sure U, Battenberg E, Dempfle A, Tirakotai W, Bien S, Bertalanffy H. Hypoxia-inducible factor and vascular endothelial growth factor are expressed more frequently in embolized than in nonembolized cerebral arteriovenous malformations. Neurosurgery. 2004; 55: 663–669.CrossrefMedlineGoogle Scholar7 Montanera W, Marotta TR, terBrugge KG, Lasjaunias P, Willinsky R, Wallace MC. Cerebral arteriovenous malformations associated with moyamoya phenomenon. AJNR Am J Neuroradiol. 1990; 11: 1153–1156.MedlineGoogle Scholar8 Halim AX, Johnston SC, Singh V, McCulloch CE, Bennett JP, Achrol AS, Sidney S, Young WL. Longitudinal risk of intracranial hemorrhage in patients with arteriovenous malformation of the brain within a defined population. Stroke. 2004; 35: 1697–1702.LinkGoogle Scholar9 Nataf F, Meder JF, Roux FX, Blustajn J, Merienne L, Merland JJ, Schlienger M, Chodkiewicz JP. Angioarchitecture associated with haemorrhage in cerebral arteriovenous malformations: a prognostic statistical model. Neuroradiology. 1997; 39: 52–58.CrossrefMedlineGoogle Scholar10 Kubalek R, Moghtaderi A, Klisch J, Berlis A, Quiske A, Schumacher M. Cerebral arteriovenous malformations: influence of angioarchitecture on bleeding risk. Acta Neurochir (Wien). 2003; 145: 1045–1052.CrossrefMedlineGoogle Scholar11 Hartmann A, Mast H, Mohr JP, Koennecke HC, Osipov A, Pile-Spellman J, Duong DH, Young WL. Morbidity of intracranial hemorrhage in patients with cerebral arteriovenous malformation. Stroke. 1998; 29: 931–934.CrossrefMedlineGoogle Scholar12 Choi J, Mohr JP, Sciacca RR, Hartmann A, Khaw AV, Mast H, Sacco RL, Stapf C. Clinical outcome after first and subsequent hemorrhage in patients with untreated brain arteriovenous malformations. Abstract 663 presented at the 30th International Conference on Stroke, New Orleans, February 2, 2005.Google Scholar13 Hartmann A, Stapf C, Hofmeister C, Mohr JP, Sciacca RR, Stein BM, Faulstich A, Mast H. Determinants of neurological outcome after surgery for brain arteriovenous malformation. Stroke. 2000; 31: 2361–2364.CrossrefMedlineGoogle Scholar14 Hartmann A Pile-Spellman J, Stapf C, Sciacca RR, Faulstich A, Mohr JP, Schumacher HC, Mast H. Risk of endovascular treatment of brain arteriovenous malformations. Stroke. 2002; 33: 1816–1820.LinkGoogle Scholar15 Hartmann A. Marx P, Schilling A, Pietilä T, Mohr JP, Mast H. Neurologic complications following radiosurgical treatment of brain arteriovenous malformations. European Stroke Meeting. 2003.Google Scholar16 Mohr JP, Stapf C, Sciacca RR, Khaw AV, Mast H, Connolly ES, Pile-Spellman J. Natural history versus treatment outcome in patients with unruptured brain arteriovenous malformation (AVM). Stroke. 2004; 35: 328(Abstract).Google Scholar17 Lawton MT, Du R, Tran MN, Achrol AS, McCulloch CE, Johnston SC, Quinnine NJ, Young WL. Effect of presenting hemorrhage on outcome after microsurgical resection of brain arteriovenous malformations. Neurosurgery. 2005; 56: 485–493.CrossrefMedlineGoogle Scholar18 Choi JH, Mohr JP. Brain arteriovenous malformations in adults. Lancet Neurol. 2005; 4: 299–308.CrossrefMedlineGoogle Scholar19 ARUBA—A Randomized Trial of Unruptured Brain AVMs. www.arubastudy.org; accessed July 26, 2005.Google Scholar20 Al-Shahi R, Fang JS, Lewis SC, Warlow CP. Prevalence of adults with brain arteriovenous malformations: a community based study in Scotland using capture-recapture analysis. J Neurol Neurosurg Psychiatry. 2002; 73: 547–551.CrossrefMedlineGoogle Scholar21 Weber F, Knopf H. Cranial MRI as a screening tool: findings in 1,772 military pilot applicants. Aviat Space Environ Med. 2004; 75: 158–161.MedlineGoogle Scholar22 Stapf C, Mast H, Sciacca RR, Berenstein A, Nelson PK, Gobin YP, Pile-Spellman J, Mohr JP; New York Islands AVM Study Collaborators. The New York Islands AVM Study: design, study progress, and initial results. Stroke. 2003; 34: E29–33.LinkGoogle Scholar23 Al-Shahi R, Bhattacharya JJ, Currie DG, Papanastassiou V, Ritchie V, Roberts RC, Sellar RJ, Warlow CP; Scottish Intracranial Vascular Malformation Study Collaborators. Prospective, population-based detection of intracranial vascular malformations in adults: the Scottish Intracranial Vascular Malformation Study (SIVMS). Stroke. 2003; 34: 1163–1169.LinkGoogle Scholar24 Meyer-Heim AD, Boltshauser E. Spontaneous intracranial haemorrhage in children: aetiology, presentation and outcome. Brain Dev. 2003; 25: 416–421.CrossrefMedlineGoogle Scholar25 Nataf F, Schlienger M, Lefkopoulos D, Merienne L, Ghossoub M, Foulquier JN, Deniaud-Alexandre E, Mammar H, Meder JF, Turak B, Huart J, Touboul E, Roux FX. Radiosurgery of cerebral arteriovenous malformations in children: a series of 57 cases. Int J Radiat Oncol Biol Phys. 2003; 57: 184–195.CrossrefMedlineGoogle Scholar26 Stapf C, Khaw AV, Sciacca RR, Hofmeister C, Schumacher HC, Pile-Spellman J, Mast H, Mohr JP, Hartmann A. Effect of age on clinical and morphological characteristics in patients with brain arteriovenous malformation. Stroke. 2003; 34: 2664–2669.LinkGoogle Scholar27 Mast H, Young WL, Koennecke HC, Sciacca RR, Osipov A, Pile-Spellman J, Hacein-Bey L, Duong H, Stein BM, Mohr JP. Risk of spontaneous haemorrhage after diagnosis of cerebral arteriovenous malformation. Lancet. 1997; 350: 1065–1068.CrossrefMedlineGoogle Scholar28 Pollock BE, Flickinger JC, Lunsford LD, Bissonette DJ, Kondziolka D. Factors that predict the bleeding risk of cerebral arteriovenous malformations. Stroke. 1996; 27: 1–6.CrossrefMedlineGoogle Scholar29 Marzo ME, Garaizar C, Prats-Vinas JM, Zuazo E, Sousa T. Cerebral arteriovenous malformations in children under 10 years. Rev Neurol. 1996; 24: 558–561.MedlineGoogle Scholar30 Menovsy T, van Overbeeke JJ. Cerebral arteriovenous malformations in childhood: state of the art with special reference to treatment. Eur J Pediatr. 1997; 156: 741–746.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Porta M, Moreno J, Werner M, Chirife Ó and López-Rueda A (2022) Vasos intracraneales en localización anómala en adultos, Radiología, 10.1016/j.rx.2021.11.002, 64:1, (41-53), Online publication date: 1-Jan-2022. Porta M, Moreno J, Werner M, Chirife Ó and López-Rueda A (2022) Anomalous location of intracranial vessels in adults, Radiología (English Edition), 10.1016/j.rxeng.2021.11.001, 64:1, (41-53), Online publication date: 1-Jan-2022. Deng X, Wei X, Zhang Y, Wang B, Zhang D, Yu S, Jiang T and Zhao J (2020) Impact of AVM location on language cortex right-hemisphere reorganization: A voxel-based lesion-symptom mapping study, Clinical Neurology and Neurosurgery, 10.1016/j.clineuro.2019.105628, 189, (105628), Online publication date: 1-Feb-2020. Deng X, Xu L, Zhang Y, Wang B, Wang S, Zhao Y, Cao Y, Zhang D, Wang R, Ye X, Wu J and Zhao J (2015) Difference of language cortex reorganization between cerebral arteriovenous malformations, cavernous malformations, and gliomas: a functional MRI study, Neurosurgical Review, 10.1007/s10143-015-0682-7, 39:2, (241-249), Online publication date: 1-Apr-2016. Deng X, Zhang Y, Xu L, Wang B, Wang S, Wu J, Zhang D, Wang R, Wang J and Zhao J Comparison of language cortex reorganization patterns between cerebral arteriovenous malformations and gliomas: a functional MRI study, Journal of Neurosurgery, 10.3171/2014.12.JNS14629, 122:5, (996-1003) Racette S and Sauvageau A (2007) Brain Arteriovenous Malformation and Its Implication in Forensic Pathology, Journal of Forensic Sciences, 10.1111/j.1556-4029.2006.00337.x, 52:1, (189-191), Online publication date: 1-Jan-2007. Racette S and Sauvageau A (2007) Authors? Response, Journal of Forensic Sciences, 10.1111/j.1556-4029.2007.00544.x, 52:5, (1226-1226), Online publication date: 1-Sep-2007. Berman M and Young W (2007) Commentary on:, Journal of Forensic Sciences, 10.1111/j.1556-4029.2007.00543.x, 52:5, (1225-1225), Online publication date: 1-Sep-2007. October 2005Vol 36, Issue 10 Advertisement Article InformationMetrics https://doi.org/10.1161/01.STR.0000181775.06537.d9PMID: 16192458 Originally publishedOctober 1, 2005 Keywordscerebral hemorrhagecerebral arteriovenous malformationschildPDF download Advertisement