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Posterior fossa arterio-venous malformations: current multimodal treatment strategies and results

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Abstract

The present study aimed to determine the clinical presentation, the multimodal interdisciplinary treatment strategies and outcome of posterior fossa arterio-venous malformations (AVMs) in our neurovascular centre. Fifty-three patients with a posterior fossa AVM were seen between 1998 and 2012 and analysed retrospectively. Patients were either managed conservatively or treated with endovascular, microsurgical or radiosurgical procedures or in combination. Thirty-nine patients (74 %) presented with intracranial haemorrhage and 14 patients (26 %) with unspecific symptoms. In 22 cases with haemorrhage (56 %), an intracerebellar haematoma was found, whereas 17 patients (44 %) suffered from subarachnoid haemorrhage. AVMs were located in the cerebellum in 44 patients (83 %), in the brainstem in four patients (7.5 %) and the cerebello-pontine angle in another four individuals (7.5 %). Forty-two patients (79 %) were treated either by emboliziation (n = 12, 29 %), surgical resection (n = 16, 38 %), surgical resection with preoperative embolization (n = 12, 29 %) or radiotherapy alone (n = 2, 4 %). A total of eleven patients did not receive any treatment (21 %). Both, morbidity and mortality related to treatment were 12 %, whereas overall morbidity and mortality was 26 and 15 %, respectively. Complete AVM elimination was achieved in 81 % of the treated lesions. A multimodal treatment sequence nowadays represents the gold standard for posterior fossa AVMs. Patients are at high risk for morbidity and mortality, due to the impact of haemorrhage and treatment. Therefore, treatment has to be thoroughly indicated, especially for those patients without bleeding. The initial neurological condition seems to be crucial in terms of clinical outcome.

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References

  1. Al-Shahi R, Warlow C (2001) A systematic review of the frequency and prognosis of arteriovenous malformations of the brain in adults. Brain: J Neurol 124(Pt 10):1900–1926

    Article  CAS  Google Scholar 

  2. Batjer H, Samson D (1986) Arteriovenous malformations of the posterior fossa. Clinical presentation, diagnostic evaluation, and surgical treatment. J Neurosurg 64(6):849–856. doi:10.3171/jns.1986.64.6.0849

    Article  PubMed  CAS  Google Scholar 

  3. da Costa L, Thines L, Dehdashti AR, Wallace MC, Willinsky RA, Tymianski M, Schwartz ML, ter Brugge KG (2009) Management and clinical outcome of posterior fossa arteriovenous malformations: report on a single-centre 15-year experience. J Neurol, Neurosurg, Psychiatry 80(4):376–379. doi:10.1136/jnnp.2008.152710

    Article  Google Scholar 

  4. Dammann P, Asgari S, Bassiouni H, Gasser T, Panagiotopoulos V, Gizewski ER, Stolke D, Sure U, Sandalcioglu IE (2011) Spontaneous cerebellar hemorrhage–experience with 57 surgically treated patients and review of the literature. Neurosurg Rev 34(1):77–86. doi:10.1007/s10143-010-0279-0

    Article  PubMed  Google Scholar 

  5. Dolderer S, Kallenberg K, Aschoff A, Schwab S, Schwarz S (2004) Long-term outcome after spontaneous cerebellar haemorrhage. Eur Neurol 52(2):112–119. doi:10.1159/000080268

    Article  PubMed  Google Scholar 

  6. Donauer E, Loew F, Faubert C, Alesch F, Schaan M (1994) Prognostic factors in the treatment of cerebellar haemorrhage. Acta Neurochir 131(1–2):59–66

    Article  PubMed  CAS  Google Scholar 

  7. Drake CG, Friedman AH, Peerless SJ (1986) Posterior fossa arteriovenous malformations. J Neurosurg 64(1):1–10. doi:10.3171/jns.1986.64.1.0001

    Article  PubMed  CAS  Google Scholar 

  8. Fleetwood IG, Steinberg GK (2002) Arteriovenous malformations. Lancet 359(9309):863–873. doi:10.1016/S0140-6736(02)07946-1

    Article  PubMed  Google Scholar 

  9. Fults D, Kelly DL Jr (1984) Natural history of arteriovenous malformations of the brain: a clinical study. Neurosurgery 15(5):658–662

    Article  PubMed  CAS  Google Scholar 

  10. Hamilton MG, Spetzler RF (1994) The prospective application of a grading system for arteriovenous malformations. Neurosurgery 34(1):2–6, discussion 6–7

    Article  PubMed  CAS  Google Scholar 

  11. Kelly D. Flemming RDBJ (2011) Youmans Neurological Surgery, vol 4. 6th edn. Saunders

  12. Kelly ME, Guzman R, Sinclair J, Bell-Stephens TE, Bower R, Hamilton S, Marks MP, Do HM, Chang SD, Adler JR, Levy RP, Steinberg GK (2008) Multimodality treatment of posterior fossa arteriovenous malformations. J Neurosurg 108(6):1152–1161. doi:10.3171/JNS/2008/108/6/1152

    Article  PubMed  Google Scholar 

  13. Khaw AV, Mohr JP, Sciacca RR, Schumacher HC, Hartmann A, Pile-Spellman J, Mast H, Stapf C (2004) Association of infratentorial brain arteriovenous malformations with hemorrhage at initial presentation. Stroke: J Cerebral Circulation 35(3):660–663. doi:10.1161/01.STR.0000117093.59726.F9

    Article  CAS  Google Scholar 

  14. Kobayashi S, Sato A, Kageyama Y, Nakamura H, Watanabe Y, Yamaura A (1994) Treatment of hypertensive cerebellar hemorrhage—surgical or conservative management? Neurosurgery 34(2):246–250, discussion 250–241

    Article  PubMed  CAS  Google Scholar 

  15. Langer DJ, Lasner TM, Hurst RW, Flamm ES, Zager EL, King JT Jr (1998) Hypertension, small size, and deep venous drainage are associated with risk of hemorrhagic presentation of cerebral arteriovenous malformations. Neurosurgery 42(3):481–486, discussion 487–489

    Article  PubMed  CAS  Google Scholar 

  16. Mohr JP, Moskowitz AJ, Parides M, Stapf C, Young WL (2012) Hull down on the horizon: a randomized trial of unruptured brain arteriovenous malformations (ARUBA) trial. Stroke: J Cerebral Circulation 43(7):1744–1745. doi:10.1161/STROKEAHA.112.653584

    Article  CAS  Google Scholar 

  17. Mohr JP, Parides MK, Stapf C, Moquete E, Moy CS, Overbey JR, Salman RA-S, Vicaut E, Young WL, Houdart E, Cordonnier C, Stefani MA, Hartmann A, von Kummer Rd, Biondi A, Berkefeld J, Klijn CJM, Harkness K, Libman R, Barreau X, Moskowitz AJ (2013) Medical management with or without interventional therapy for unruptured brain arteriovenous malformations (ARUBA): a multicentre, non-blinded, randomised trial. The Lancet

  18. Mpotsaris A, Loehr C, Harati A, Lohmann F, Puchner M, Weber W (2010) Interdisciplinary clinical management of high grade arteriovenous malformations and ruptured flow-related aneurysms in the posterior fossa. Interv Neuroradiol: J of Peritherapeutic neuroradiology, Surgical Procedures and related Neurosciences 16(4):400–408

    CAS  Google Scholar 

  19. Nagy G, Major O, Rowe JG, Radatz MW, Hodgson TJ, Coley SC, Kemeny AA (2012) Stereotactic radiosurgery for arteriovenous malformations located in deep critical regions. Neurosurgery 70(6):1458–1469. doi:10.1227/NEU.0b013e318246a4d0, discussion 1469–1471

    Article  PubMed  Google Scholar 

  20. Pollock BE, Gorman DA, Brown PD (2004) Radiosurgery for arteriovenous malformations of the basal ganglia, thalamus, and brainstem. J Neurosurg 100(2):210–214. doi:10.3171/jns.2004.100.2.0210

    Article  PubMed  Google Scholar 

  21. Schmidt NO, Reitz M, Raimund F, Treszl A, Grzyska U, Westphal M, Regelsberger J (2011) Clinical relevance of associated aneurysms with arteriovenous malformations of the posterior fossa. Acta Neurochir Suppl 112:131–135. doi:10.1007/978-3-7091-0661-7_23

    Article  PubMed  Google Scholar 

  22. Solomon RA, Stein BM (1986) Management of arteriovenous malformations of the brain stem. J Neurosurg 64(6):857–864. doi:10.3171/jns.1986.64.6.0857

    Article  PubMed  CAS  Google Scholar 

  23. Spetzler RF, Hargraves RW, McCormick PW, Zabramski JM, Flom RA, Zimmerman RS (1992) Relationship of perfusion pressure and size to risk of hemorrhage from arteriovenous malformations. J Neurosurg 76(6):918–923. doi:10.3171/jns.1992.76.6.0918

    Article  PubMed  CAS  Google Scholar 

  24. Spetzler RF, Martin NA (1986) A proposed grading system for arteriovenous malformations. J Neurosurg 65(4):476–483. doi:10.3171/jns.1986.65.4.0476

    Article  PubMed  CAS  Google Scholar 

  25. Spetzler RF, Ponce FA (2011) A 3-tier classification of cerebral arteriovenous malformations. Clinical Article J Neurosurg 114(3):842–849. doi:10.3171/2010.8.JNS10663

    Article  Google Scholar 

  26. Symon L, Tacconi L, Mendoza N, Nakaji P (1995) Arteriovenous malformations of the posterior fossa: a report on 28 cases and review of the literature. Br J Neurosurg 9(6):721–732

    Article  PubMed  CAS  Google Scholar 

  27. van Loon J, Van Calenbergh F, Goffin J, Plets C (1993) Controversies in the management of spontaneous cerebellar haemorrhage. A consecutive series of 49 cases and review of the literature. Acta Neurochir 122(3–4):187–193

    Article  PubMed  Google Scholar 

  28. Weisberg LA (1986) Acute cerebellar hemorrhage and CT evidence of tight posterior fossa. Neurology 36(6):858–860

    Article  PubMed  CAS  Google Scholar 

  29. Yilmaz A, Musluman AM, Kanat A, Cavusoglu H, Terzi Y, Aydin Y (2011) The correlation between hematoma volume and outcome in ruptured posterior fossa arteriovenous malformations indicates the importance of surgical evacuation of hematomas. Turk Neurosurg 21(2):152–159. doi:10.5137/1019-5149.JTN.3401-10.0

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, University Hospital Essen, University Duisburg-Essen, Hufelandstrasse 55, 45122, Essen, Germany

    Klaus-Peter Stein, Philipp Dammann, Alexia-Sabine Moldovan, Yuan Zhu, Ulrich Sure & I. Erol Sandalcioglu

  2. Department of Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen, University Duisburg-Essen, Hufelandstrasse 55, 45122, Essen, Germany

    Isabel Wanke & Marc Schlamann

  3. Clinic Hirslanden, Neuroradiology, Witellikerstrasse 40, 8032, Zürich, Switzerland

    Isabel Wanke

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  1. Klaus-Peter Stein
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  2. Isabel Wanke
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  4. Philipp Dammann
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  5. Alexia-Sabine Moldovan
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  6. Yuan Zhu
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  7. Ulrich Sure
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  8. I. Erol Sandalcioglu
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Correspondence to Klaus-Peter Stein.

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Comments

Isabel Cuervo-Arango Herreros, Asturias, Spain and Álvaro Campero, Tucumán, Argentina

In this interesting and well-written article, Klaus-Peter Stein et al. report a retrospective study about clinical presentation, multimodal interdisciplinary treatment strategies and outcome of posterior fossa arterio-venous malformations in 53 patients in the University Hospital Essen, Germany, between 1998 and 2012, as well as a thorough review of the related literature. They make the difference in terms of clinical presentation between haemorrhagic and non-haemorrhagic groups, as well as neurological symptoms, which will determine the treatment planning. In addition, the authors evaluate the treatment options in an interdisciplinary approach, what we believe is the first step that must be taken.

An important finding in their series is that 56 % of patients had intracranial haematoma in the haemorrhagic group, against the literature where 60–90 % had subarachnoid haemorrhage. We think that further research should support this finding.

Furthermore, the description of radiologic findings as size, feeding artery aneurysms, high-flow/fistulous nidus and venous drainage, will help many neurovascular radiologist and surgeons to take the best therapeutic option either surgical, endovascular or combined in each case. However, only the AVM size (<3 cm) was associated with a statistical higher risk for haemorrhage in their series.

In the present study, there is no clear relationship between clinical-radiological findings and the treatment adopted, probably because of the wide variability of both, so we think that a classification and therapeutic protocol to posterior fossa AVMs needs further to be clarified, as the authors concluded.

Finally, we would like to remark, once again, the relevant role played by a multidisciplinary team for this pathology, and we congratulate the authors for the article and also their results.

Karl Schaller, Geneva, Switzerland

This is a comprehensive retrospective report by a group of experienced neurovascular surgeons and interventional neuroradiologists who are reporting on a large series (53 patients) who had been diagnosed for posterior fossa AVM. Three quarters of all patients were admitted following AVM rupture. Twenty percent of patients were not treated, and the vast majority underwent a combination of endovascular treatment and microsurgical removal, or embolization, or microsurgery alone, respectively. Two patients had radiosurgery. Among those 11 patients who were not treated, in four of them the AVM was judged untreatable.

In my opinion, this is an important clinical series of this distinct group of cranial AVMs—those located in the posterior fossa. I had wished the authors had highlighted a little more the potential relevance of intranidal aneurysms, which might be amenable to selective treatment in otherwise intractably complex AVMS. It is, however, always difficult to look into all these subtle angiographical details, especially when analysing a series of such a long study period.

Some of the conclusions do not come as surprise, notably that the initial neurological condition is predictive for outcome. It is clearly not advised to be more conservative in one’s attitude when it comes to the clinical judgement of posterior fossa AVMs: Especially in view of the ARUBA trial, neurologists who are seeing AVM-patients more and more frequently and even the fewer and fewer fully trained neurovascular surgeons may develop a more conservative attitude towards intracranial AVMs. With all the respect for the group who has contributed to the ARUBA trial, it is too early to derive to the conclusion that conservative therapy and surveillance would be less harmful than treatment in all cranial AVMs. There are by far too many substantial methodological flaws in the ARUBA study, and the observation period was far from being long enough to observe AVM ruptures further down the timeline.

With the present study regarding posterior fossa AVS the authors have demonstrated in my opinion, that due to their particular localization, these AVMs pose an important and maybe so far underrated threat to the patients. This is illustrated for example by the fact that three quarters of the patient presented with AVM rupture, which is a far higher rate than the nowadays presumed 30–50 % of supra-tentorial AVMs. In addition, posterior fossa haemorrhages frequently require extremely rapid surgical removal. I would thus distinguish posterior fossa AVMs from those localized supra-tentorally for very practical and logical reasons, and I would be very hesitant to apply a conservative attitude based on easy grounds such as the ARUBA study. As the authors have clearly pointed out: It is the initial clinical state, which is the most important determinant for long-term outcome. Should thus unruptured posterior fossa AVMS come to the attention of a dedicated neurovascular team, then elaborate high-resolution and selective angiography will become instrumental in decision-making, as additional venous anomalies, such as stenosis, or intranidal aneurysms may render certain AVMs of a particular risk for rupture. Then the indication for surgery should be made more deliberately than it seems presently to be the case within the somewhat unsettled neurovascular community following the insufficient ARUBA study.

The authors are to be commended for having highlighted with a diligently worked up series of posterior fossa AVMs, that such AVMs are a treatable disease, ideally when they come to our attention unruptured, and the surgeons, together with their closest partner from interventional radiology, should lead the discussion and not just following it, and we should guide the patients rather than leaving them in the hands of neurologists and epidemiologists who are at safe distance when it comes to taking concrete responsibilities in case of AVM rupture of a previously diagnosed unruptured one for which an observational attitude was taken.

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Stein, KP., Wanke, I., Schlamann, M. et al. Posterior fossa arterio-venous malformations: current multimodal treatment strategies and results. Neurosurg Rev 37, 619–628 (2014). https://doi.org/10.1007/s10143-014-0551-9

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