Cavernous Malformations of the Brain and Spinal Cord

The neurosurgeons at Houston Methodist diagnose and treat many kinds of cerebrovascular conditions, including cavernous malformations of the brain and spinal cord, which are a type of vascular malformation composed of clusters of abnormal, fragile blood vessels. These dilated blood vessels have multiple pockets (caverns), through which blood slowly passes. They often look like small blackberries. Cavernous malformations are also called cavernomas, cavernous angiomas, cavernous hemangiomas and intracranial vascular malformations.

Cavernous malformations account for 8 to 15 percent of all brain and spinal vascular malformations. An associated venous malformation can be found in up to 40 percent of patients with cavernous malformations. Most people (60 percent) are between the ages of 20 and 40 when they are diagnosed.

About 20 percent of people affected with cavernomas have a genetic form. If a parent has this genetic form, their child may have a 50 percent chance of developing it. If a person has the nongenetic (called sporadic) form of this condition, their children have no greater risk of developing a cavernoma than the general population.

Cerebral cavernous malformations occur in the brain’s cortex, the thin covering of the brain often referred to as “gray matter.” There is no brain tissue within the malformation and no defined borders. They can change in size and number over time, and tend to leak blood.

Symptoms of Cavernous Malformations of the Brain and Spinal Cord
Many people with this abnormality will not have any symptoms, but approximately 30 percent will have one or more of a diverse list of symptoms.  Specific symptoms often depend upon where the cavernoma is located and whether it is leaking blood:
  • Seizures
  • Headaches
  • Trouble speaking or swallowing
  • Weakness or numbness in the face, arms or legs
  • Problems with vision, balance, memory and attention
  • Bleeding (hemorrhage) into surrounding tissue, which can cause damage

Diagnosis of  Cavernous Malformations of the Brain and Spinal Cord

Most cavernous malformations are not diagnosed until the patient exhibits symptoms. If symptoms indicate a cavernous malformation, your doctor may order imaging scans to detect dilated blood vessels.

A specialized variation of magnetic resonance imaging (MRI) called gradient echo sequencing is the most reliable way to diagnose cavernomas. Sometimes a computed tomography (CT) scan with contrast (dye) is also used. Angiograms do not detect cavernous malformations because of slow blood flow.

Treatment Options for Cavernous Malformations of the Brain and Spinal Cord
If you are not exhibiting any symptoms, your doctor may recommend monitoring your condition, with periodic testing to determine any changes — a “wait-and-watch” approach. If the cavernoma is bleeding, or if symptoms progress, surgery may be needed to remove the cavernoma. Surgery for cavernous malformations is much safer today than in the past because of advances in microsurgery and computer-assisted image- guided navigation. The two most common surgical procedures for cavernous malformations of the brain and spinal cord are craniotomy and laminectomy (or decompression surgery). 

Unlike other vascular malformations, such as arteriovenous malformations (AVM), cavernous malformations do not respond to radiation treatment. In fact, radiation therapy, including Gamma Knife® or Cyberknife® surgery, or linear accelerator (LINAC) high-energy X-ray treatment, often results in complications in patients with cavernous malformations.

Cerebral cavernous malformations are removed in a surgery called a craniotomy. During a craniotomy, part of the skull is removed so the surgeon can access the brain. This section of skull (called the bone flap) is replaced at the end of surgery. A craniotomy is usually performed under general anesthesia, unless the doctor needs to map brain function while you are awake. In that case, your responses help the surgeon treat the precise area of your brain that is affected and lowers the risk of damage to functional areas of your brain.

Cavernous malformations of the spine can be removed during a laminectomy. In this procedure, the back part of the vertebra that covers the spinal canal (the lamina) is removed; this relieves pressure on the spinal cord or nerves and potentially eliminates symptoms.

Recovery and Rehabilitation From Treatment for Cavernous Malformations of the Brain and Spinal Cord
After surgery, you will spend a few days in the hospital recovering. After you return home, you can resume normal activities within a few weeks. Many patients can be cured and do not experience neurological deficits. With physical therapy (rehabilitation), patients who do not have neurological deficits are often able to return to their baseline (their condition at the time of surgery), and may even show continued improvement. Patients who have neurological deficits may need a longer period of rehabilitation.

The rebleeding rate of cavernous malformations varies. Some patients’ symptoms never recur, while others experience frequent recurrence.

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