Experts at the Cerebrovascular Center at Mount Sinai specialize in cavernomas (also called cavernous angiomas or cavernous malformations). Cavernomas are abnormal clusters of vessels with small bubbles (or caverns) filled with blood that make them look like a berry. These can range from microscopic up to several inches in diameter. Unlike arteriovenous malformations or AVMs, blood flow in cavernomas is low. Since the walls of cavernomas are weak, blood can slowly leak out. Cavernomas can occur in the brain and on the spinal cord. While a cavernous angioma may not affect function, it can cause seizures, stroke symptoms, hemorrhages, and headache.
Cavernoma Risk Factors
Approximately one in 200 people have a cavernous angioma. Anyone may have a cavernoma, and the majority diagnosed report no family history. However, those with more than one cavernoma are suspected to have an inherited factor.
Each child of someone with the inherited form of cavernomas has a 50 percent chance of inheriting the condition. In inherited cavernomas, current research points to a mutation in any one of three particular genes. The Cerebrovascular Center can coordinate genetic counseling for families who would like to evaluate their risk.
While many uninherited cavernomas are present at birth, and some develop later in life, often associated with other endovascular abnormalities, such as a venous malformation.
Cavernomas are usually only diagnosed after a seizure, a loss of function, or from a surprise finding when an MRI is performed for another reason. Cavernomas may have no symptoms; however, more than 30 percent of those with cavernous angiomas eventually will develop symptoms. Often these symptoms occur when patients are 20- to 40-years-old.
The type, frequency, and severity of symptoms often depend on the location of the cavernoma. Typical symptoms include:
- Epileptic seizure
- Neurological loss, such as limb weakness, vision or balance problems, or problems with memory and attention
- Brain hemorrhage, which can be small, but sometimes massive, leading to stroke-like symptoms
- Spinal cord injury
Due to their low blood flow, cavernomas are not visible on angiograms. However, cavernomas very clearly appear on MRIs and are easily distinguished from tumors. Imaging experts at the Cerebrovascular Center specialize in diagnosing cavernomas.
Treatment Options for Cavernomas
Physicians at the Cerebrovascular Center evaluate how to treat cavernomas depending on:
- How the cavernomas are bleeding: Cavernous angiomas might bleed slowly, requiring little intervention since the body will reabsorb it. However, cavernomas that are bleeding more profusely can put dangerous pressure on the surrounding brain tissue and/or cause an obvious hemorrhage. Cavernous angiomas that have already bled are more likely to bleed again, especially within two years of the first bleed. Frequency is evaluated, too; even if the cavernoma is only causing very small hemorrhages, continuous bleeding can affect function over time.
- If there are multiple cavernomas: The higher the number of cavernomas, the greater the chance of one or more hemorrhages occurring.
- Where the cavernomas are located: For instance, surgery on cavernomas in the brainstem (where the brain connects to the spinal cord), in the cerebral cortex (the outer layer of the brain responsible for speech, motor and visual abilities), or the spinal cord is considered risky. On the other hand, cavernomas in these areas can cause more damage when they do bleed, than cavernomas located elsewhere. The risk versus benefit is weighed carefully when deciding on treatment.
- The presence of other endovascular abnormalities: Up to 40 percent of cavernomas occur near a venous malformation, which can make surgical treatment more difficult.
Depending on thorough assessment of the above factors, the Cerebrovascular Center can offer:
- Watching and waiting: Many cavernomas are observed for changes, recent hemorrhage, or worsening symptoms.
- Medications: These cannot directly treat the cavernomas, but they can be effective in treating symptoms, such as seizures and headaches.
- Surgery: This is the only curative approach to cavernomas and is advocated for cavernous angiomas with recent hemorrhage, those that are expanding in size, and those that are causing seizures.
Surgeons at the Cerebrovascular Center remove cavernomas by making a small opening in the skull (a craniotomy). This is usually performed under general anesthesia, except in rare cases when mapping of the brain while awake is needed. Most patients leave the hospital within two days. Patients who experience neurological loss caused by the cavernomas may require post-surgical rehabilitation, which is coordinated by the Cerebrovascular Center.
Our neurosurgeons use the latest, safest, and most effective techniques available for removing cavernomas with as little disruption to the brain as possible. These techniques include microsurgery and computer image-guided surgical navigation.
Klingenstein Clinical Center, 1-North
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New York, NY 10029