About Arteriovenous Malformation (AVM)

Basic Blood Vessel Anatomy of the Brain

Overview of an AVM

Symptoms

AVMs in Children

AVMs and Pregnancy

Quick Facts

References

Basic Blood Vessel Anatomy in the Brain

An artery is a blood vessel that carries blood that is high in oxygen and nutrients from the heart to nourish other
parts of the body. The walls of an artery are very elastic as they are meant to withstand high pressure as blood
is pumped out of the heart. The arteries gradually become smaller and smaller as they get out to the tissues
until reaching the capillary bed, which consists of tiny, thin-walled vessels, where oxygen and nutrients
exchange with carbon dioxide and waste products.

The blood then continues into the veins, blood vessels that carry blood back to the heart. Normally, as the high-
pressure arterial blood is pumped through a capillary bed there is a gradual decrease in blood pressure. The
walls of veins are therefore not as elastic as arteries as they are not meant to carry blood under high pressure.

In the brain, the veins drain blood into venous dural sinuses (not to be confused with the air-filled sinuses in the
facial bones that are often associated with sinus infections, etc.). Venous dural sinuses are folds in the dura
mater (lining of the brain) that collect blood and then drain into the internal jugular veins that bring blood back
to the heart.

Back to top

Overview of an AVM

An arteriovenous malformation (AVM) is a complex

tangle of abnormal arteries and veins linked by one
or more direct connections called fistulas or
shunts.

This tangle of abnormal arteries and veins is

referred to as a nidus. Normally, as the high-
pressure arterial blood is pumped through a
capillary bed there is a gradual decrease in blood
pressure before reaching the venous system. With
an AVM, the capillary bed is absent and the high-pressure arterial blood bypasses normal brain tissue and is
pumped directly into the normally low-pressure venous system.

There is typically high blood flow through the nidus of the AVM, but it is not known whether the flow is a cause
or effect of the abnormal blood vessels, or both. One thought is that the high-pressure blood from the arterial
system gravitates towards the path of least resistance. Another thought is that the AVM itself recruits blood
vessels.

Ultimately, the arterial blood rushes through the AVM, instead of working through available capillary beds, which
feed the surrounding brain tissue, increasing blood flow through the nidus. This re-direction of the arterial blood
away from the brain tissue and through the AVM is referred to as shunting.

Over time, the high blood flow and shunting of high-pressure arterial blood through the AVM causes the feeder
arteries and veins making up the AVM to dilate (or expand). This dilation weakens veins making
them susceptible to hemorrhage; feeder arteries become susceptible to aneurysms, a weakened spot in the
blood vessel wall that expands and can eventually hemorrhage. A hemorrhage in the brain is a type of stroke in
which a blood vessel ruptures and bleeds into the surrounding brain. For more information on strokes, please
visit the American Stroke Association
at www.strokeassociation.org.

There is a 10-15 percent risk of death, and a 20-30 percent

chance of permanent brain damage, related to each bleed.
Each time blood leaks into the brain, normal brain tissue is
damaged. This results in loss of normal function, which may
be temporary or permanent. Some possible symptoms
include arm or leg weakness/paralysis, or difficulty with
speech, vision, or memory. The amount of brain damage
depends upon how much blood has leaked from the AVM
(Higashida).

Smaller AVMs present with hemorrhage more often than

large ones. In addition, the size of the hematoma (a localized
swelling filled with blood resulting from a break in a blood
vessel) is larger from the small AVM, compared with the
medium or large AVM. There appears to be no difference in
the frequency of hemorrhage between large and medium
AVMs. However, whether size of the AVM is a true risk factor
is unclear.

Treatment is primarily aimed at preventing new or future hemorrhage of the AVM. For more information on
treatment, please visit the Treatment of AVMs section.
(CT Scan of Hemorrhage)

The structure of the abnormal vessels varies between what is usually found in arteries and that of veins. The
tissue in and around the abnormal vessels is usually a kind of scar or fibrotic tissue (gliotic), but sometimes
brain tissue is also found in some regions of the AVM.

AVMs may arise in the brain, spine, lungs, kidneys, and skin. Brain AVMs are the most common and can occur
anywhere in the brain. When an AVM occurs in the dura mater of the brain (the outermost lining of the brain),
this is called a dural arteriovenous fistula (DAVF).
AVMs are thought to be congenital (that is, present at birth), arising from developmental derangements at the
embryonic stage of vessel formation, at the fetal stage. However, this has never been clearly established and
they may arise after birth. AVMs are usually single, except when associated with hereditary hemorrhagic
telangiectasia (HHT). For more information on causes of AVMs, please visit the Causes of AVMs section.

Back to top

Symptoms

In about 50% of patients the presentation is a sudden hemorrhage, or bleeding into the brain, a form of stroke.
Other potential complications include seizures, headaches, and stroke-like symptoms (difficulty with movement,
speech, and vision). These complications may occur in conjunction with, or independently of, hemorrhage.

Hemorrhage

The risk of hemorrhage is ~2-4% per year. There is a 10-15% risk of death related to each bleed and a 20-30%
chance of permanent brain damage. The risk of bleeding is higher in the first years after the first bleed. After a
first hemorrhage, if the AVM is not treated, the risk of another hemorrhage happening is about 3 times higher.
However, for unknown reasons, this increased risk appears to decrease over time, so that eventually, the risk of
a new bleed returns to the risk of rupture before the AVM presented.

The symptoms of a hemorrhage depend on location of the AVM, as well as the severity of the bleed. These
symptoms may include:

Sudden and severe headache, nausea & vomiting

 Seizure

Loss of consciousness

"Stroke-like" symptoms: problems speaking, numbness/tingling, muscle weakness, changes in vision.

Hemorrhage is a result of blood vessels that are weakened by the shunting of high-pressure arterial blood
through the abnormal arteries and veins of the AVM. The short-term and long-term neurological effects of the
hemorrhage differ depending on how much blood has leaked into the brain and where the hemorrhage is
located. Factors for spontaneous bleeding before or in the absence of treatment include:

History of prior hemorrhage

Deep venous drainage (exclusive)

Feeding artery aneurysms and intranidal aneurysms

Venous restrictive disease: blockage in the drainage system

Seizures

Seizures that are not caused by hemorrhage are the initial symptom in 16-53% of patients. Types of seizure
include:

Generalized seizures, which involves the entire body and loss of consciousness. These types of seizures
are more common in frontal AVMs.

Focal seizures, "unusual feelings" or involuntary muscle movement, depending on the location of the
AVM in the brain. People generally don't have a loss of consciousness. These types of seizures are more
common with parietal AVMs.

A seizure happens when a brief, strong surge of electrical activity affects part or all of the brain. It is thought
that the scar tissue usually found in and around the abnormal vessels of an AVM disrupt the normal electrical
activity of the brain causing seizures. For more information on seizures, please visit the Epilepsy Foundation
at www.epilepsyfoundation.org .

Headache

Headache is the symptom that leads to diagnosis in 7-48% of patients. These headaches usually do not have
any distinctive features, such as frequency, duration, or severity. Whether AVMs cause headaches is not clear.

Stroke-like symptoms

Focal neurological deficits without signs of underlying hemorrhage have been reported in 1 to 40% of patients.
Traditionally, these symptoms were ascribed to a problem called "cerebral steal" - redirection of the blood flow
from surrounding brain tissue through the AVM disrupting the normal functioning of the surrounding brain tissue.
(Taylor et al) But there is very little evidence that "cerebral steal" is a clinically important mechanism.

Another theory is that the abnormal vessels making up the nidus pulsate and press on adjacent brain structures.
Small AVMs are less likely than larger AVMs to have stroke-like symptoms resulting from pressure on adjacent
brain. (Mast et al)

One study suggested that learning disabilities have been documented in 66% of adults with AVMs, suggesting
that functional brain deficits are present before there are other clinical signs of the lesion. (lazar et al)

Back to top

AVMs and Children

Because AVMs are most likely congenital lesions; they can be discovered in children. Although most childhood
AVMs are detected in school-aged children, they can also become symptomatic in the first few days of life.

As in adults, AVMs in children can present with a brain hemorrhage, seizures, headache, focal neurological
deficits, or incidentally. In addition, large AVMs in newborns can cause congestive heart failure. These babies
will present with respiratory distress (rapid, distressed breathing). This is particularly true of babies with a
specific type of AVM called a Vein of Galen Malformation (VOGM).

Compared to those in adults, AVMs in children are more likely to present with bleeding. The reason for this is
not known. It could represent a biological difference between the AVMs in children versus adults. On the other
hand, it could simply be due to children being less likely to get imaging studies that might detect an AVM unless
they present with very severe symptoms, such as symptoms of a hemorrhage.

Children appear to have a similar annual (yearly) risk for spontaneous bleeding compared to adults. If you
correct for the fact that AVMs in children are more likely to present with hemorrhage than those in adults,
childhood AVMs might actually be at lower risk for spontaneous bleeding. However, this has to be balanced with
the longer expected life-span of a child compared to an adult. All other things being equal, a child will have a
higher risk of bleeding from an AVM simply because he or she will be living with that AVM for longer.

Back to top

AVMs and Pregnancy

Intracerebral hemorrhage (ICH) and subarachnoid hemorrhage (SAH) of an AVM are uncommon complications of
pregnancy. The occurrence of SAH during pregnancy is ~1:1,000 pregnancies, a rate that is five times higher
then in non-pregnant women. In a review of all ICH complicating pregnancy, 77% were caused by aneurysms,
and 23% by AVM. In compiling all of the data, it is noted that the AVM hemorrhages were in younger women, but
there was no relation between hemorrhage and parity or gestational age. It is recommended that women with
AVMs considering pregnancy consult with their physician. Many women go on to have normal pregnancies and
deliveries.

Back to top

AVM Quick Facts

 AVMs arise in the brain, spine, lungs, kidneys and skin. Brain AVMs are the most common.

The overall ratio of AVMs to aneurysms is probably in the range of 1:10.

Most patients present between the ages of 20 and 60 years of age. The mean age is about 35-40.

AVMs are equally distributed between male/female.

Patients with AVMs may have additional vascular anomalies that increase the complexity of treatment.
Approximately 10-58% of patients have various kinds of aneurysms.

It is estimated that in the United States 18 in 100,000 have an AVM in the brain.

When an AVM bleeds, there is a 10-15% risk of death related to each bleed and a 20-30% chance of
permanent brain damage.

The risk of bleeding is higher in the first years after the first bleed.

In about 50% of patients the presentation is a sudden hemorrhage, or bleeding into the brain, a form of
stroke. Other potential complications include seizures, headaches, and stroke-like symptoms (difficulty
with movement, speech, and vision). These complications may occur in conjunction with, or
independently of, hemorrhage.

About 5-10% of AVMs are discovered by accident while the individual is being tested for other
unrelated medical problems.