Cavernoma

Cavernoma Overview

A cavernoma is a collection of small blood vessels in the brain that are enlarged and irregular in structure. Because they lack fibers that normally make them stretch, the blood vessels are prone to leakage (bleeding), which can cause a variety of symptoms including headaches and seizures. Some people have no symptoms but others with the disease may experience a severe brain hemorrhage.

Cavernomas are known by several other names including cavernous malformations, cerebral cavernous malformations (CCM), cavernous angioma, and cavernous hemangioma. The lesions are described as looking like raspberries or like popcorn. While cavernomas can occur anywhere in the body, they are typically of concern when they are located in the brain and spinal cord.

The lesions are localized collections that are not cancerous and can develop throughout one’s lifetime. Unlike other types of brain lesions, cavernomas do not contain brain tissue within the malformation. They can range in size from microscopic to several inches in diameter. Although these malformations are rare, they account for 8-15 % of all brain and spinal vascular malformations. As many as 40% of people who are affected will not experience symptoms or may never be diagnosed.

There are several types of cavernomas:

• Familial cavernous angioma is a hereditary form of the condition that typically shows up in one’s 20s or 30s. About a third of patients with this type will develop symptoms.
• Sporadic cavernous angioma is not inherited and may be present from birth or may develop later in life.
• Associated venous angioma is a type of cavernoma that develops near another lesion known as a venous angioma, which is a small tangle of abnormal veins.

Wellstar neurologists and neurosurgeons can diagnose cavernomas and, depending on their size and location, help patients determine the need for and type of treatment.

Symptoms

• At least 30% of people with a cavernoma will develop symptoms, while some experience no symptoms at all. When symptoms do occur they depend on the location of the lesion and on the strength or weakness of the walls and their propensity for bleeding.
• Seizure: (epileptic seizure) is the most common symptom. There are many types of seizures, ranging from mild to dramatic. The type of seizure depends on the location of the cavernoma.
• Neurological problems: Examples are weakness in the arms or legs and problems relating to vision, balance and/or memory and attention. The symptoms may come and go as the cavernoma changes.
• Bleeding: Cavernomas can bleed slowly within the walls of the lesion and remain quite small. If a small hemorrhage (bleed) occurs it may be reabsorbed into the body and not require treatment. Continued small hemorrhages in the same cavernoma can lead to deterioration in the function of the vessels. In a type of bleed known as an overt hemorrhage, the bleed may move through a weak spot in the wall and into surrounding brain tissue. The more cavernomas a patient has, the greater the chance of a bleed at some point.
• Headache: Headache typically occurs following a recent bleed

Risk Factors

There are few known risk factors for cavernoma. The sporadic (non-hereditary) form of the disease occurs for no apparent reason. About 20% of cases are a familial (hereditary) form of the condition. Researchers have identified three different genes associated with cavernoma and are working to understand the relationship between these gene mutations and symptoms of the disease.

Some experts believe that prior radiation (for conditions including cancer) can be a risk factor for developing cavernoma. There is a higher rate of familial cavernoma among Hispanic-American families who originally came from New Mexico. And the condition is more likely to develop in the vicinity of the blood vessel malformation known as venous angioma.