Arteriovenous Malformations
Arteriovenous malformations are blood vessel defects that occur before birth when the fetus is growing in the uterus (prenatal development). The blood vessels appear as a tangled mass of arteries and veins.
They do not possess the capillary (very fine blood vessels) bed which normally exists in the common area where the arteries and veins lie in close proximity (artery-vein interface). An arteriovenous malformation (AVM) may hemorrhage, or bleed, leading to serious complications that can be life-threatening.
AVMs represent an abnormal interface between arteries and veins. Normally, arteries carry oxygenated blood to the body’s tissues through progressively smaller blood vessels. The smallest are capillaries, which form a web of blood vessels (the capillary bed) through the body’s tissues.
The arterial blood moves through tissues by these tiny pathways, exchanging its load of oxygen and nutrients for carbon dioxide and other waste products produced by the body cells (cellular wastes). The blood is carried away by progressively larger blood vessels, the veins.
AVMs lack a capillary bed and arterial blood is moved (shunted) directly from the arteries into the veins. AVMs can occur anywhere in the body and have been found in the arms, hands, legs, feet, lungs, heart, liver, and kidneys. However, 50% of these malformations are located in the brain, brainstem, and spinal cord.
Owing to the possibility of hemorrhaging, such AVMs carry the risk of stroke, paralysis, and the loss of speech, memory, or vision. An AVM that hemorrhages can be fatal. Approximately three of every 100,000 people have a cerebral AVM and roughly 40-80% of them will experience some bleeding from the abnormal blood vessels at some point.
The annual risk of an AVM bleeding is estimated at about 1-4%. After age 55, the risk of bleeding decreases. Pre-existing high blood pressure or intense physical activity do not seem to be associated with AVM hemorrhage, but pregnancy and labor could cause a rupture or breaking open of a blood vessel.
An AVM hemorrhage is not as dangerous as an aneurysmal rupture. (An aneurysm is a swollen, blood filled vessel where the pressure of the blood causes the wall to bulge outward.) There is an approximate 10% fatality rate associated with AVM hemorrhage, compared to a 50% fatality rate for ruptured aneurysms.
Although AVMs are congenital defects, meaning a person is born with them, they are rarely discovered before age 20. A genetic link has been proposed for some AVMs, but studies are only suggestive, not positive. The majority of AVMs are discovered in people age 20-40.
Medical researchers estimate that the malformations are created during days 45-60 of fetal development. A second theory suggests that AVMs are primitive structures that are left over from the period when fetal blood circulating systems began to develop. However they form, AVMs have blood vessels that are abnormally fragile.
The arteries that feed into the malformation are unusually swollen and thin walled. They lack the usual amount of smooth muscle tissue and elastin, a fibrous connective tissue. These blood vessels commonly accumulate deposits of calcium salts and hyalin. The venous part of the malformation receives blood directly from the artery.
Without the intervening capillary bed, the veins receive blood at a higher pressure than they were designed to handle. This part of the malformation is also swollen (dilated) and thin walled.
There is a measurable risk of an aneurysm forming near an AVM, increasing the threat of hemorrhage, brain damage, and death. Approximately 10-15% of AVMs are accompanied by saccular aneurysms, a type of aneurysm that looks like a small sac attached to the outer wall of the blood vessel.
Although the malformation itself lacks capillaries, there is often an abnormal proliferation of capillaries next to the defect. These blood vessels feed into the malformation, causing it to grow larger in some cases. As the AVM receives more blood through this “steal,” adjacent brain tissue does not receive enough.
These areas show abnormal nerve cell growth, cell death, and deposits of calcium in that area (calcification). Nerve cells within the malformation may demonstrate abnormal growth and are believed to be nonfunctional.
Causes and Symptoms
Most people do not realize that they have an AVM unless it hemorrhages enough to produce symptoms. Small AVMs are more likely to hemorrhage. If a hemorrhage occurs, it produces a sudden, severe headache. The headache may be focused in one specific area or it may be more general.
It can be mistaken for a migraine in some cases. The headache is accompanied by other symptoms, such as vomiting, a stiff neck, sleepiness, lethargy, confusion, irritability, or weakness anywhere in the body. Seizures occur in about a quarter of AVM cases. A person may experience decreased, double, or blurred vision.
Hemorrhaging from an AVM is generally less dangerous than hemorrhaging from an aneurysm, with a survival rate of 80-90%. Other symptoms occur less frequently, but sometimes appear alongside major symptoms such as the sudden severe headache.
Additional warning signs of a bleeding AVM are impaired speech or smell, fainting, facial paralysis, a drooping eyelid, dizziness, and ringing or buzzing in the ears. Although large AVMs are less likely to hemorrhage, they can induce symptoms based on their mass alone.
Large AVMs exert pressure against brain tissue, cause abnormal development in the surrounding brain tissue, and slow down or block blood flow. Hydrocephalus, a swelling of brain tissue caused by accumulated fluids, may develop.
The warning signs associated with a large non-bleeding AVM are similar to the symptoms of a small malformation that is bleeding. Unexplained headaches, seizures, dizziness, and neurological symptoms, such as sensory changes, are signals that demand medical attention.
Based on the clinical symptoms such as severe headache and neurological problems, and after a complete neurologic exam, a computed tomography scan (CT) of the head will be done. In some cases, a whooshing sound from arteries in the neck or over the eye or jaw (called a bruit), can be heard with a stethoscope.
The CT scan will reveal whether there has been bleeding in the brain and can identify AVMs larger than 1 inch (2.5 cm). Magnetic resonance imaging (MRI) is also used to identify an AVM. A lumbar puncture, or spinal tap, may follow the MRI or CT scan.
A lumbar puncture involves removing a small amount of cerebrospinal fluid from the lower part of the spine. Blood cells or blood breakdown products in the cerebrospinal fluid indicate bleeding. To pinpoint where the blood is coming from, a cerebral angiography is done.
This procedure uses x rays to map out the blood vessels in the brain, including the vessels that feed into the malformation. The information gained from angiography complements the MRI and helps distinguish the precise location of the AVM.
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About AVM
