Living With Arteriovenous Malformation

The diagnosis of a brain arteriovenous malformation (AVM) can be a lot to take in. This is especially true if the AVM has ruptured and caused serious neurological consequences.

You may be wondering what your treatment options are and how you will live with this condition. Alternatively, an incidental finding of an AVM can prompt a lengthy discussion about the risks of rupture and generate fear and anxiety.

Fortunately, treatment options and rehabilitation programs are available to manage the AVM and help you to adapt to your new life. Below, we will discuss living with an AVM and the different types of therapies available.

An AVM is a tangle of abnormal blood vessels in the brain connecting arteries and veins. AVMs lack normal fine branching blood vessels called capillaries between the artery and vein, which are essential for transfer of oxygen and nutrients to surrounding tissues. 

Without capillaries, blood rushes from the high-pressure artery to the low-pressure vein, causing the draining vein to become engorged with blood. Over time, this turbulent blood flow can weaken the vessel walls leading to AVM rupture.

Most patients do not experience symptoms until the AVM ruptures. During the rupture, a tear in an AVM vessel causes blood to leak into the surrounding brain tissue under significant pressure. This condition is called intracerebral hemorrhage or brain bleed. Symptoms of a ruptured AVM include the following:

• Loss of consciousness
• Confusion and difficulty concentrating
• Seizures
• Nausea and vomiting
• Difficulty speaking
• Blurred or double vision
• Weakness or paralysis in the limbs

Unruptured AVMs may present with headaches or seizures. Since most do not produce symptoms until rupture, AVMs may be incidentally found on imaging of the head for an unrelated reason.

AVMs are relatively rare, occurring in less than 1% of the population. The exact cause of AVMs is unknown, but they are thought to be congenital, meaning that they are present at birth, although they also may form during the life of an adult. In some cases, AVMs may be associated with other conditions, such as Ehlers-Danlos syndrome or Hereditary Hemorrhagic Telangiectasia (HHT). 

As the name suggests, a ruptured AVM bursts and bleeds into the surrounding brain tissues. A ruptured AVM is a life-threatening condition that requires immediate medical attention. When an AVM ruptures, it can cause severe sudden headaches, seizures, paralysis and even death has been reported up to 10% of cases, with 10 – 20% of patients sustaining severe neurological impairments that interfere with everyday life. With treatment, most individuals will survive an AVM rupture.

On the other hand, an unruptured AVM has not yet burst. An unruptured AVM does not usually cause symptoms (may rarely present with seizures) but may be discovered during an imaging test for an unrelated reason. While an unruptured AVM is not as immediately dangerous as a ruptured AVM, it still carries a risk of rupture. The annual risk of rupture is 2 – 4%, but this can be elevated in AVMs with associated aneurysms, deep location, or only a single draining vein.

Once an AVM ruptures, the risk of subsequent rupture is increased to approximately 6 – 15% with a peak risk in the first year before declining back to a 2 – 4% risk of hemorrhage after 3 – 5 years. The decision to treat an AVM is made by weighing the risk of AVM rupture against the risks of treatment.

Surgical removal, endovascular embolization, and radiosurgery are the three main types of treatment for AVMs. The best mode of treatment for a particular AVM depends on its size and location and the patient’s age and health.

Surgery is the most successful at curing an AVM, but can be associated with risks. The risk of sustaining neurological complications after surgery is increased for AVMs that are large or located in deep areas near critical brain structures.

Endovascular embolization is typically used in combination with surgery to facilitate removal of the AVM. Embolization and radiosurgery can be used alone or in combination to treat an AVM if surgery is deemed too risky.

However, when using embolization or radiosurgery alone, the likelihood of obtaining a cure is lower than that of surgery. Endovascular embolization may also be used with palliative intent to alleviate AVM symptoms.

If you have been diagnosed with an AVM, the thought of its possible rupture into the brain can be frightening.

Rest assured, however, as currently there are treatment options available to prevent the rupture and cure the AVM in many cases. While living with an AVM, these are some things you can do to manage your condition:

• Stay on top of your medical care. This means attending all appointments and taking any medications prescribed by your doctor.
• Learn as much as you can about your condition. This will help you to be an active participant in your care and make informed decisions about your treatment.
• Take care of yourself physically and emotionally. It can be surprising how much a healthy diet and regular exercise can help you manage stress.

Fortunately, most patients living with an AVM can live long and full lives. Some individuals live with an AVM for life without it ever being detected. Cure rates after surgery are as high as 100% in patients with low-grade AVMs, and a combination of treatment options are available for more complex cases.

As an AVM survivor, you can adopt self-care strategies at home to complement treatments prescribed by your surgeon. Here are some ways to care for yourself at home while living with an AVM:

Familiarize Yourself with your AVM Symptoms: Identify your specific AVM symptoms, such as headaches, seizures, or balance issues. This will help you get medical help earlier and prevent your symptoms from worsening.

Avoid Triggering Factors:  It is essential to be aware of your triggers and take necessary precautions to prevent complications. An AVM survivor should avoid high-impact sports and activities that may increase the risk of head injury.

Monitor Your Blood Pressure: Maintaining healthy blood pressure is crucial for an AVM survivor. Regularly monitoring your blood pressure at home, following recommendations from your healthcare provider, and adjusting your lifestyle are essential to maintaining your health.

Create a Safe Home Environment: It is crucial to take measures to prevent falls and injuries. This includes removing hazards such as clutter and loose carpets and installing handrails in areas such as the bathroom and stairs.

Ensure Emotional and Mental Well-being: Living with an AVM is a challenging experience, both physically and emotionally. It can cause significant stress and anxiety. Therefore, you must seek support from your loved ones, medical professionals, and other social support groups to help you cope with the difficult times.

Those living with AVM must understand the potential risks associated with their condition and take steps to manage and prevent them. Although the chances are slim, the following are the possible complications of the condition:

Hemorrhage: People living with AVM are at risk of hemorrhage. This is because the high-pressure arterial blood flow directly into the veins can make the walls of the affected blood vessels weaker and more prone to rupture. Hemorrhages can have severe consequences, including stroke, neurological damage or even death.

Seizures: AVM can lead to seizures as the altered blood flow and pressure caused by the malformation can irritate the surrounding brain tissue.

Headache: Headaches are another common complication of an AVM survivor. They can range from mild to severe and may be due to the increased pressure on the surrounding blood vessels or nerve tissues in the brain.

Neurological Deficits: AVM sometimes leads to neurological deficits or impairments. These may include muscle weakness, numbness, vision problems or difficulty speaking. Neurological deficits may occur suddenly due to bleeding or gradually as the malformation changes blood flow and pressure over time.

Hydrocephalus: In rare cases, AVM can cause hydrocephalus, a condition where fluid accumulates in the brain. This complication occurs when blood flow disruption caused by AVM prevents the proper circulation and absorption of cerebrospinal fluid.

Although the outlook for patients with AVMs is generally positive, AVM rupture can be fatal in up to 10% of individuals. For many people, the thought of living with this time bomb is too much to bear, and they will prefer to have their AVM surgically removed.

AVM surgery is one of the most challenging operations in neurosurgery. The operation comes with potential risks, and it is not always possible to remove the AVM completely.

This can be very difficult for patients and their families, who may feel like they are facing this rare condition all alone. However, there are support networks available to help.

Organizations like the Brain Aneurysm Foundation and The Aneurysm and AVM Foundation offer information and support to patients and their families and can help connect individuals with others who are going through similar experiences.

There are also online forums where people can share their stories and offer advice and support to others. 

While living with an AVM can be very difficult, knowing that you are not alone is important. Finding individuals who understand what you are going through because they have gone through similar experiences themselves can be reassuring and extremely helpful. These individuals can provide different perspectives on treatment options and information about how they were able to manage their condition.

Yes, you can survive an AVM, and in fact, most people do. Some individuals with AVMs live their entire lives without ever being diagnosed. AVM rupture can be life-threatening, but early evaluation and proper treatment promote the best possible outcomes. With prompt medical care, most people with AVMs can live full, active lives.

Not all AVMs eventually bleed. AVM rupture depends on several factors, though generally there is an estimated rupture risk of 2 – 4% each year.

The cumulative lifetime risk of rupture, assuming an average life expectancy of 70 years and a 2 – 4% annual bleeding risk can be estimated using the formula 105 – age.

This formula is simplified for convenience and does not consider the fact that the risk of rupture may change over time. The risk of AVM rupture is also increased if there are associated aneurysms.

Although there is no guarantee that an AVM will eventually bleed, it is important to be aware of the risks so that you can seek treatment if necessary.

AVM patients face many challenges after diagnosis. Some patients have a ruptured AVM and require rehabilitation therapy. Other patients may only need to make small changes to their lifestyle.

Each treatment and outcome are unique to every patient, but a skilled neurosurgeon and medical team can make a world of difference. Seek an experienced neurosurgeon for a comprehensive evaluation and discussion of how to best manage your AVM. 

• The Aneurysm and AVM Foundation 
• AVM Research Foundation
• Brain Aneurysm Foundation 
• Child Neurology Foundation 
• National Institute of Neurological Disorders and Stroke