AVM Flashcards

1
Q

What is the avg age at presentation for arteriovenous malformations (AVMs)?

A

30 yrs (10–40 yrs).

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1
Q

What is the nidus of an AVM?

A

The nidus is a tangle of abnormal arteries/veins connected by at least 1 fistula.

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2
Q

What is the main histologic abnormality in the vasculature of an AVM?

A

Absence of smooth muscle layer; ↑ venous pressure (fibromuscular thickening with incomplete elastic lamina)

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3
Q

What is the morbidity and mortality per bleed for AVMs?

A

Morbidity: 30%–50%/bleed
Mortality: 5%–10%/bleed (1%/yr)

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4
Q

What is the rate of hemorrhage per yr for AVMs?

A

AVMs have a 2%–4% chance of hemorrhage/yr.

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5
Q

Are most AVM cases familial or sporadic?

A

Most AVMs are sporadic.

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6
Q

What familial/genetic syndromes are associated with AVMs?

A

Osler-Weber-Rendu (hereditary hemorrhagic telangiectasia; HHT) and Sturge-Weber syndromes are associated with AVMs.

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7
Q

What characteristics portend an increased risk of hemorrhage from AVMs?

A

Previous hemorrhage,
increased age,
aneurysm,
deep venous sinus drainage,
deep location,
single draining vein,
and venous stenosis

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8
Q

Aneurysms are found in what % of pts with AVMs?

A

6%–8% of AVM pts harbor aneurysms.

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9
Q

What are the common presenting signs of AVMs?

A

Intracerebral hemorrhage (42%–72%)
> seizures (11%–33%)
> HA
> focal neurologic deficit.

Children are more likely to present with hemorrhage than adults.

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10
Q

What imaging modality is ideal to r/o a bleed?

A

CT is ideal to r/o cerebral bleeds.

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11
Q

What is the gold standard imaging modality for AVMs?

A

Angiography is the gold standard modality for imaging AVMs.

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12
Q

What other imaging modalities can be used for AVMs? What are their advantages?

A

CT angiography (good vascular detail),
MR angiography (good anatomy detail),
functional MRI (eloquent areas),
Diffusion tensor imaging (for white matter tracts)

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13
Q

What scale is used to evaluate AVM pts for surgery?

A

Spetzler-Martin scale/grading system (totals possible: I–V).

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14
Q

What 3 AVM characteristics in the Spetzler-Martin scale are predictive of surgical outcomes?

A

AVM characteristics that predict surgical outcome:
1. Diameter (<3 cm = 1, 3–6 cm = 2, >6 cm = 3)

  1. Location (noneloquent area = 0, eloquent area = 1)
  2. Pattern of venous drainage (superficial = 0, deep = 1)

Smaller score = better surgical outcome

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15
Q

How does AVM diameter/size scoring correlate with surgical outcomes?

A

The smaller the AVM diameter/size (<3 cm), the better the outcomes.

16
Q

What brain areas are considered eloquent?

A

Eloquent areas include:
sensorimotor,
language,
visual,
thalamus, hypothalamus,
internal capsule,
brainstem, cerebellar peduncles,
and deep cerebellar nuclei.

17
Q

What are the 4 Tx options for AVMs?

A

Observation, Surgery, radiosurgery, and endovascular embolization

18
Q

What is the goal of Tx with AVMs? Why?

A

Complete obliteration is the goal, since there is no benefit or ↑ risk of bleed if the obliteration is partial.

19
Q

Is Tx of unruptured AVMs beneficial?

A

Controversial but likely not. Recent studies suggest tx if unruptured led to increased risk of hemorrhage, clinical impairment, and death (Wedderburn CJ et al., Lancet Oncol 2008; van Beijnum J et al., JAMA 2011)

20
Q

Which lesions are most amenable to surgery?

A

Those with low (I–III) Spetzler-Martin scores are most amenable to surgery.

21
Q

What is frequently done for grade III lesions before surgery?

A

Embolization can be performed for grade III lesions before surgery.

22
Q

What is the main advantage of surgery?

A

Immediate cure and reduction in the risk of hemorrhage.

23
Q

For what AVM lesions is SRS preferred?

A

Radiosurgery is preferred for lesions <3 cm that are located in deep or eloquent regions of the brain.

24
Q

What is the main disadvantage of SRS for AVMs?

A

The main disadvantage of SRS is the lag time of 1–3 yrs to complete obliteration (i.e., continued bleeding risk).

25
Q

How does RT lead to AVM obliteration?

A

Vascular wall thickening (fibrointimal hyperplasia) and luminal thrombosis from RT effect result in obliteration of the AVM.

26
Q

Is the bleeding risk completely eliminated after SRS?

A

No. It is reduced by ∼54% during latency period and 88% after obliteration but not eliminated. (Maruyama K et al., NEJM 2005; Yen CP et al., Stroke 2011)

27
Q

On what do SRS cure rates for AVMs primarily depend?

A

Size of AVM: 81%–91% if <3 cm, lower if >3 cm (Maruyama K et al., NEJM 2005)

Radiation dose

28
Q

What can be done for high-grade AVMs (IV–V) not amenable for surgery?

A

Staged SRS (different components targeted at separate sessions) (Sirin S et al., Neurosurg 2006)

29
Q

For which AVMs can embolization be curative?

A

AVMs <1 cm that are fed by a single artery can be cured by embolization alone.

30
Q

How are AVMs with feeding artery aneurysms managed?

A

If the aneurysm is >7 mm in diameter, clip or coil the aneurysm 1st, then treat the AVM. The aneurysm is at greater risk for rupture if the AVM is treated 1st.

31
Q

What SRS doses are commonly used for AVMs?

A

Lesions <3 cm: 21–22 Gy to 50% IDL. If the lesion is in the brainstem, lower the dose to ≤16 Gy.

Lesions >3 cm: 16–18 Gy to 50% IDL

32
Q

What are the reported rates of permanent weakness or paralysis, aphasia, and hemianopsia for grades I–III AVM pts treated with surgery?

A

The rate of serious postsurgical complications is 0%–15%.

33
Q

What are common early and delayed complications after SRS for AVMs?

A

Early: seizures (up to 10%), n/v, HA

Late: seizures, neurological deficit, hemorrhage, radionecrosis (1%–3% risk), edema, venous congestion, cyst formation

34
Q

What is the incidence of transient vs. permanent neurologic complications after SRS for AVMs?

A

Complications after SRS for AVMs are as follows: transient (5%) vs. permanent (1.4%).

35
Q

On what 2 factors do complication rates after SRS for AVMs primarily depend?

A

Size of AVM and RT dose.

36
Q

What does the follow-up entail after Tx for AVMs?

A

Adequate follow-up includes routine H&P + MRI q6mos for 1–3 yrs, then annually.

37
Q

What study needs to be performed once the MRI shows evidence of AVM obliteration?

A

An angiogram needs to be performed (in addition to MRI) to confirm complete AVM obliteration.

38
Q

Compare and contrast advantages and disadvantages of different management options for AVM