What Is a Brain AVM?
Why Does an AVM Matter?
Symptoms of a Brain AVM
How Is a Brain AVM Diagnosed?
How Do We Assess the Risk of a Brain AVM?
One of the most important tools used for evaluating a brain AVM is The Spetzler-Martin grading system. This scale helps neurosurgeons like myself to explore the surgical risk of removing the AVM and have conversations with patients about the risks and benefits of treatment. The grading system is based on three things: Size of the AVM, Location in the Brain, Type of Venous Drainage. Depending on the individual features of an AVM, the patient case will be given a score point total that will identify the AVM on a scale of Grade 1(lowest risk) to Grade 5(highest risk). Grade I–II: Typically safe and excellent candidates for surgical resection; Grade III: Borderline; surgery may still be reasonable, especially in experienced hands; Grade IV–V: High-risk surgical candidates; often better managed with embolization, radiosurgery, or observation. Lower grade AVM is more suitable and safer to be removed surgically, but higher grade AVMs are better suitable for non surgical approaches such as embolizations or radiosurgery. The point distribution depending on the features is listed below:
- Size of the AVM
- Small (<3 cm): 1 point
- Medium (3–6 cm): 2 points
- Large (>6 cm): 3 points
- Location in the Brain (Eloquence)
- If the AVM is located in a brain region that controls essential functions like speech, movement, or vision (called “eloquent cortex”), it adds 1 point
- If in a non-eloquent area: 0 points
- Type of Venous Drainage
- Deep venous drainage (draining into veins deep within the brain): 1 point
- Only superficial venous drainage: 0 points
Modified Spetzler-Martin Grading (Lawton-Young Supplement)
When there are borderline or complex cases of AVMs, a supplementary grading system known as The Lawton-Young Supplementary Scale is used. It specifically adds patient-specific factors to narrow down the Grade Level classification of the AVM. These scale point totals are added to the original Spetzler-Martin grade to create a total score that helps guide personalized treatment decisions. The point distribution depending on the features is listed below:
- Age of the Patient
- Younger than 20 years: 1 point
- Age 20–40: 2 points
- Over 40 years: 3 points
- History of Bleeding
- AVM has bled previously: 0 points
- AVM has not bled: 1 point
- AVM Structure
- Compact AVMs (clearly defined): 0 points
- Diffuse AVMs (poorly defined): 1 point
References
- Spetzler RF, Martin NA. A proposed grading system for arteriovenous malformations. J Neurosurg. 1986 Oct;65(4):476–83.
DOI: 10.3171/jns.1986.65.4.0476 - Lawton MT, Kim H, McCulloch CE, Mikhak B, Young WL. A supplementary grading scale for selecting patients with brain arteriovenous malformations for surgery. Neurosurgery. 2010 Jan;66(4):702–713.
DOI: 10.1227/01.NEU.0000367555.06344.C8 - Kim H, Al-Shahi Salman R, McCulloch CE, Stapf C, Young WL. Untreated brain arteriovenous malformation: patient-level meta-analysis of hemorrhage predictors. Neurology. 2014 Aug 26;83(9):590–7.
DOI: 10.1212/WNL.0000000000000688
How Are the Spetzler-Martin and Modified SM Scales Used in AVM Treatment Planning?
Treatment Options
1. Endovascular Embolization
Endovascular embolization is a minimally invasive treatment option for brain arteriovenous malformations. It involves inserting a small catheter through the wrist or the groin to reach the abnormal blood vessels in the brain. Once the AVM is accessed by the catheter, a liquid or mechanical substance is injected to block the abnormal blood flow arteries that feed into the AVM. The goal of the endovascular embolization is to completely shut down the blood supply flow to the AVM or reduce its size. This can be further supported with surgical resection or radiation treatment. Endovascular embolization is preferred to control high risk features present such as intranidal aneurysms and increased chances of rupture.
Types of Embolic Materials
- n-Butyl Cyanoacrylate (n-BCA): A surgical glue that hardens quickly to block the vessel.
- Onyx: A non-adhesive liquid that solidifies slower so it can be a more controlled deeper injection into the AVM source.
- PHIL (Precipitating Hydrophobic Injectable Liquid): A newer embolic agent with radiopacity and slower solidification.
How the Procedure Works
Risks and Considerations
Advantages of Embolization
Is Embolization Right for Me?
References
- Natarajan SK, Ghodke B, Britz GW, Hallam DK, Sekhar LN. Multimodality treatment of brain arteriovenous malformations with microsurgery after embolization with Onyx: single-center experience and technical nuances. Neurosurgery. 2008;62(6 Suppl 3):1213–1226.
- Abud DG, Riva R, Nakiri GS, et al. Treatment of brain AVMs by double arterial catheterization with simultaneous injection of Onyx: technical note. J Neuroradiol. 2007;34(4):248–253.
- Saatci I, Geyik S, Yavuz K, Cekirge HS. Endovascular treatment of brain AVMs with prolonged intranidal Onyx injection technique: long-term results in 350 consecutive patients with completed endovascular treatment course. J Neurosurg. 2011;115(1):78–88.
- Lawton MT, Rutledge WC, Kim H, et al. Brain arteriovenous malformations. Nat Rev Dis Primers. 2015;1:15008.
2. Microsurgical Resection
When Is Surgery Recommended?
How the Procedure Works
Recovery and Postoperative Care
Benefits of Surgery
Risks and Considerations
References
- Spetzler RF, Martin NA. A proposed grading system for arteriovenous malformations. J Neurosurg. 1986;65(4):476–83.
DOI: 10.3171/jns.1986.65.4.0476 - Lawton MT, Kim H, McCulloch CE, et al. A supplementary grading scale for selecting patients with brain arteriovenous malformations for surgery. Neurosurgery. 2010;66(4):702–713.
DOI: 10.1227/01.NEU.0000367555.06344.C8 - Morgan MK, Davidson AS, Assaad NNA, Stoodley MA. Critical review of brain AVM surgery, surgical results, and natural history in 2017. World Neurosurg. 2017;106:948–959.
DOI: 10.1016/j.wneu.2017.07.093 - Lawton MT. Seven AVMs: Tenets and Techniques for Resection. Thieme Medical Publishers; 2014.
3. Stereotactic Radiosurgery (e.g., Gamma Knife)
How Does It Work?
Why CyberKnife?
CyberKnife has multiple advantages over traditional the SRS systems:
- With CyberKnife there is no rigid frame for the patient’s head that is needed. Instead, a custom soft mask is utilized for increased comfort.
- CyberKnife will also ensure submillimeter accuracy with tracking of motion of the head in real-time throughout the treatment.
- With CyberKnife, delivery can be delivered over multiple sessions, if required depending on the location and size of the AVM,
- This treatment system is particularly beneficial for eloquent AVMs in deep-seated locations adjacent to critical areas of the brain such as the motor cortex, brainstem, and visual pathways.
Who Is a Good Candidate for CyberKnife?
Stereotactic radiosurgery is usually ideal for patients:
- When the AVMs are small to medium size, which is generally less than 3 cm.
- It is also preferred that the patient has an AVM located in deep or eloquent brain areas where surgery would be high risk.
- It is recommended if the patient had a previous AVM rupture and if the AVM is no longer amenable to be surgically removed.
Your AVM will be evaluated by imaging, size, location, and your clinical history to determine whether SRS is a safe and effective treatment.
What to Expect During Treatment
- Planning Imaging
- The patient is going to get imaging between an MRI and occasionally a CT angiogram to map the AVM in 3D.
- In some cases, we will get a stereotactic catheter angiogram to visualize the Vascular anatomy of the AVM.
- Treatment Planning
- We organize a patient-specific radiation plan involving a collaborative team of the neurosurgeon, radiation oncologist, and a medical physicist.
- Our goal is to give the patient the best chance of getting all the radiation possible to the AVM and minimizing the radiation to the normal brain tissue.
- Treatment Day
- The patient will be on the treatment table with a mask to keep the head in a still position comfortably.
- During the treatment, the Cyberknife robot will deliver hundreds of narrow beams directed at the AVM location.
- This is a completely painless procedure, and no anesthesia is required; it usually takes about 45–90 minutes.
- Post-Treatment
- The patient will go home the same day.
- There is no need for recovery time, and most patients are back at normal routines immediately.
- The patient will have periodic follow-up imaging to monitor the progress of the AVM.
How Effective Is CyberKnife?
- AVMs that are less than 3 cm usually have obliteration rates of 70-90% over 2-3 years of the treatment.
- Larger AVMs may require staged or combined therapy, such as embolization then radiosurgery.
- Once fully occluded, the chance of bleeding is essentially zero.
Risks and Side Effects
There are risks associated with radiosurgery even though it avoids open surgery:
- Before the AVM closes within the 1-3 years from radiosurgery, the bleeding risk is still present.
- Radiation-induced edema could be a risk as well. This may be associated with headaches, fatigue or sometimes temporary neurological symptoms.
- Rare radiation necrosis could be a risk, and is especially seen in larger AVMs.
The entire treatment group will closely monitor the patient with serial imaging as well as neurological assessment.
References
- Starke RM, Kano H, Ding D, et al. Stereotactic radiosurgery for brain arteriovenous malformations: evaluation of long-term outcomes in a multicenter cohort. J Neurosurg. 2017;126(1):36–44.
DOI: 10.3171/2015.12.JNS151575 - Colombo F, Cavedon C, Casentini L, et al. CyberKnife radiosurgery for arteriovenous malformations: a single-center experience. J Neurosurg. 2009;111(3):431–438.
DOI: 10.3171/2009.2.JNS081333 - Paddick I, Lippitz B. AVM radiosurgery and the role of the radiosurgery-based grading system. Prog Neurol Surg. 2013;27:119–128.
DOI: 10.1159/000341767 - Sheehan JP, Williams BJ, Yen CP. Radiosurgery for arteriovenous malformations. Neurosurg Focus. 2014;37(3):E12.
DOI: 10.3171/2014.7.FOCUS14303
What Are the Risks of Not Treating a Brain AVM?
Key Studies Supporting These Risks
- Kim H et al., Neurology, 2014: Meta-analysis of untreated AVMs showed 2–4% annual rupture risk, with higher rates in ruptured, deep, or high-flow lesions.
DOI: 10.1212/WNL.0000000000000688 - Stapf C et al., Lancet Neurology, 2006: Found significant morbidity and mortality following AVM rupture, even in first-time presentations.
DOI: 10.1016/S1474-4422(06)70542-3 - Mohr JP et al., ARUBA Trial, Lancet, 2014: Compared conservative management vs. interventional therapy for unruptured AVMs; controversial results emphasized the need for personalized care.
DOI: 10.1016/S0140-6736(13)62302-8
What Are Intranidal and Prenidal Aneurysms and Why Do They Matter?
Some brain AVMs are associated with small, weak spots in the blood vessels in the brain called aneurysms. The aneurysms could form either inside the AVM itself or along the feeder arteries to the AVM. Intranidal aneurysms refer to the ones within the AVM and Prenidal aneurysms refer to the ones that are prior to the AVM, in the feeding artery.
Aneurysms are important to identify and be precautious about, since they significantly increase the risk of bleeding. Several cases have suggested that AVMs with associated aneurysms have higher hemorrhage rates than the AVMs without any associated aneurysms. When rupturing and bleeding occurs, the aneurysm will primarily burst compared to the AVM.
How Are These Aneurysms Detected?
How Are They Treated?
References
- da Costa L, Wallace MC, ter Brugge KG, et al. The natural history and predictive features of hemorrhage from brain arteriovenous malformations. Stroke. 2009;40(1):100–105.
DOI: 10.1161/STROKEAHA.108.520759 - Redekop G, TerBrugge K, Montanera W, Willinsky R. Arterial aneurysms associated with cerebral arteriovenous malformations: classification, incidence, and risk of hemorrhage. J Neurosurg. 1998;89(4):539–546.
DOI: 10.3171/jns.1998.89.4.0539 - Lv X, Li Y, Jiang C, et al. Endovascular treatment of AVM-associated aneurysms: a review of 50 cases. AJNR Am J Neuroradiol. 2009;30(3):701–706.
DOI: 10.3174/ajnr.A1429
Understanding the ARUBA Trial and Its Controversy
What Was the ARUBA Trial?
The ARUBA trial is a Randomized Trial of Unruptured Brain Arteriovenous Malformations. This trial was a multicenter international study aimed to answer: Should unruptured brain AVMs be treated, or is it safer to observe?
Published in The Lancet in 2014, the trial included 223 adult patients with unruptured brain AVMs, and randomized those patients to receive either medical management alone or medical management plus interventional treatment which could include surgery or embolizations.
Key Findings
- After a median follow-up of 33 months, the trial reported that the medical management group had significantly fewer strokes and deaths than the intervention group.
- In the observation group, the risk of stroke or death was 10.1%, and in the intervention group it was 30.7%.
- Authors concluded that the outcome in the intervention group was significantly worse than the medical management group.
Why Was the ARUBA Trial Controversial?
While there are important issues emerging from the ARUBA trial about risks of treatment, the neurovascular community has widely criticized the trial mainly for four reasons:
1. Short Follow-Up Duration
- Most of the natural history of an AVM will not have developed with a median follow-up of just over 33 months so this is not long enough to have a complete development of an AVM.
- AVMs deteriorate, nearly invariably, later in life and the cumulative lifetime risk of rupture in young individuals is great.
- While acute complications of an intervention were examined, transitional benefit of AVM obliteration for long term was not examined.
2. Heterogeneous and Non-Standardized Treatment Group
- The category “intervention” may have involved any combination of surgery, embolization or radiosurgery with none having a standardized protocol.
- Some patients only received embolization, which is rarely curative and usually used as a complementary treatment.
- Many AVMs may have also had poor surgical candidacy or inappropriately undertreated thus introducing bias into the trial.
3. Selection Bias and Limited Enrollment
- The trial terminated prematurely at 223 patients of the predicted 800 patients as a difference in the observed outcome was noticed sooner than expected.
- Many AVM centers withdrew or only enrolled patients with low-grade AVMs limiting how the results can be generalized.
4. Underrepresentation of Surgical Expertise
- Much of the treating centers were without experienced cerebrovascular neurosurgeons.
- Follow-up retrospective studies conducted at high-volume centers found much better outcomes using surgery, especially with low-grade AVMs (Spetzler-Martin I–II). This is directly inconsistent with the ARUBA results.
Current Interpretation in Practice
In spite of the flaws of the study, ARUBA brought to attention a real issue: treating unruptured AVMs is not a risk-free endeavor and treatment should be individualized. That said, the majority of experts do not interpret ARUBA to say that you should avoid all intervention. Instead it showcased and solidified that:
- Low-grade, surgically accessible AVMs should still be strong candidates for curative resection and have low risk.
- Deep or eloquent AVMs should still be considered for staged, multimodal therapy, especially in younger patients.
- For select patients with high-grade, asymptomatic lesions, observation may be reasonable.
In conclusion, the ARUBA trial reinforced the value of experienced, multidisciplinary evaluation, which we provide in our AVM program.
References
- Mohr JP 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):614–621.
DOI: 10.1016/S0140-6736(13)62302-8 - Lawton MT, Rutledge WC, Kim H, et al. Brain AVMs and ARUBA: the US perspective. J Neurosurg. 2015;123(2): 643–648.
DOI: 10.3171/2014.10.JNS132693 - Potts MB, Zumofen DW, Raz E, et al. The ARUBA study: a planned end to a controversy? World Neurosurg. 2014;82(6):e859–e861.
DOI: 10.1016/j.wneu.2014.08.005
Why Choose Dr. Yim for AVM Treatment?
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