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Cavernous Malformation - Blood Vessel Mass

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Introduction

Cavernous malformations are irregularities in the capillaries in the brain or spinal cord. They are referred to by several other names including cavernous hemangioma, cavernoma, cerebral cavernous malformation (CCM), familial hemangioma, nevus cavernosus, and cavernous angioma. Capillaries are the tiny blood vessels that connect arteries and veins. Cavernous malformations are enlarged, abnormally shaped capillaries. They are not cancerous. They may or may not cause any problems. Not all of cavernous malformations need treatment. Cavernous malformations cause symptoms if they leak which can lead to serious medical problems including seizure, stroke, paralysis, and death. Surgery is used to cure the condition.

Anatomy

Your brain is located inside of your skull. It is the control center of your body. Your brain controls the way you think, behave, feel, and move your body. Your brain communicates with the nerves in your body for functions you can control, such as talking or moving your arms and legs. Your brain also controls the life-sustaining functions that you cannot control and that happen automatically, including your heartbeat, body temperature, blood pressure, and body metabolism.
The cells in your brain and spinal cord need oxygen to function and survive. Arteries and veins carry oxygen in blood throughout your body and central nervous system in an ongoing process called circulation. Capillaries are tiny blood vessels that connect your arteries and veins. They help to deliver nutrients to cells and carry waste products away.

Causes

Cavernous malformations are an uncommon disorder that affects the capillaries in the brain, spinal cord, and rarely other parts of the body. A cavernous malformation is made of small blood-filled caverns or bubble-like areas. The bubbles make the blood filled mass look like a raspberry. Cavernous malformations can be microscopic or inches in diameter. The lining of the bubbles are irregular and can leak, regardless of size. Bleeding causes symptoms, life-threatening medical conditions, or death.
Cavernous malformations are an inherited condition in about 20% of all cases. Three genes have been identified as an inherited cause of cavernous malformations. The familial type of cavernous malformation tends to cause multiple masses. Cavernous malformations can occur sporadically in people with no family history of the condition, and be present at birth or occur later in life.

Symptoms

Cavernous malformations may not cause symptoms in about 70% of people. Symptoms usually develop when people are in their 20s or 30s. Symptoms occur when the mass leaks blood. The risk of bleeding is greater for people with more than one mass. Cavernous malformations that bleed once tend to do so again.
Cavernous malformations can cause headaches and seizures. The type of seizure that results depends on the location of the mass in the brain. Repeated seizures are termed epilepsy.
Depending on the function of the area of the brain or spinal cord where the mass is located, a variety of neurological symptoms may occur. You may experience muscle weakness or paralysis, swallowing disorders, vision changes, balance problems, or difficulty thinking and remembering things. Symptoms may come and go, or be temporary and improve. However, more neurological function is lost with each bleeding episode.
Cavernous malformations may bleed into surrounding brain tissue. The blood causes nerve injury and damage as the brain tissues compress to accommodate it. Bleeding in the brain is termed hemorrhage. Hemorrhages can cause stroke or death.

Diagnosis

A doctor can diagnose a cavernous malformation by reviewing your medical history and conducting examinations. You should tell your doctor if you have family members with the condition. Your doctor will complete a thorough neurological examination.
A magnetic resonance imaging (MRI) scan is used to create an image of your brain or spinal cord. The image allows your doctor to see the location, size, and blood supply involved with the mass or masses. MRI can also help diagnose stroke. MRIs may be repeated over time to check for recurrent bleeding, changes in mass size, or new cavernous malformations.

Treatment

Cavernous malformations that do not cause symptoms may not require treatment and are monitored over time with MRI scans. Surgery may be used to remove masses that cause seizures or symptoms. After surgical removal of the mass, the condition is cured, and people usually improve significantly. Masses that are in a delicate area of the brain may not be able to be removed, even if they cause seizures or symptoms.
The preferred time to remove a mass is after it has bled. Cavernous malformations in the brain are removed with a craniotomy. A craniotomy is a procedure that removes a piece of the skull to access the brain. Cavernous malformations in the spinal cord are removed with laminectomy. This surgery is used to remove a piece of the spine to allow access to the spinal cord and mass. Microsurgery and image guided surgical navigation have made surgeries to remove cavernous malformations safer. These advanced procedures result in less surrounding nerve tissue disruption.
People typically remain in the hospital for a few days following their surgery. Regular activities usually may be resumed in a few weeks. Patients with neurological problems may benefit from rehabilitation.

Prevention

There is no known way to prevent cavernous malformations from occurring. If you experience any symptoms, you should contact your doctor to receive prompt medical treatment.

Am I at Risk

If your family members have the inherited form of cavernous malformation, you have an increased risk of developing the condition. Mexican Americans have the highest incidence of cavernous malformations. Cavernous malformations can be present at birth or occur later in life in people that have no family history of the condition. Having a vein abnormality in the brain increases the risk of cavernous malformations, as they tend to occur near such sites in about 40% of cases.

Complications

Cavernous malformations that bleed can cause epilepsy, neurological dysfunction, stroke, and death.

Advancements

Some physicians may recommend radiosurgery with gamma knife, linear accelerator, and shaped beam radiation. However, the practice is considered controversial because of a lack of long-term research results regarding the procedures. Researchers are studying the effectiveness and safety of radiosurgery for cavernous malformations.

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