A cavernous malformation (CM) is also referred to as a cavernous angioma, cavernous hemangioma or simply cavernoma. A CM physically resembles a mulberry. They are composed of microscopic, dilated, blood-filled channels and caverns lined by capillary-like vessel walls. Their size can vary greatly. They may be found in the brain and spinal cord.
With the increasing use of brain imaging, up to 50% of cavernomas are found by chance. The rest come to attention because of symptoms or a family history of CM.
Cavernomas located in the brainstem are fortunately uncommon. Their course is more unique and management is more complicated.
Signs and Symptoms
When a CM comes to attention clinically, it is most often due to a seizure, hemorrhage or new neurological problem. The specific symptoms are often related to its location.
Hemorrhage from a CM is not typically life-threatening. The risk for hemorrhage from a CM discovered by chance is very low—just a fraction of a percent yearly. However, the risk for hemorrhage in the inherited form is higher, due to the increased number of cavernomas.
About 80% of cavernomas occur randomly (sporadic form). They are often associated with an architecturally distinctive vein (called a venous angioma or developmental venous anomaly). Another 20% of patients have the familial form. Most of these patients have a mutation in one of three genes which can then be passed on to their children. In the familial form, patients often have numerous cavernomas.
Cavernomas may also develop following radiation therapy to the brain.
There are a few primary treatment options for cavernomas:
- Observation If you have no symptoms, observation is very reasonable. Surgery for an asymptomatic cavernoma would only be considered under certain special circumstances.
- Anti-seizure medication About one-half of symptomatic cavernomas present with a seizure. Medication is the first line of treatment when a CM causes a seizure.
- Surgery Surgical removal is done through a craniotomy (open or minimally invasive), usually with the aid of computerized image guidance and a microscope. Surgery is generally considered when a cavernoma has bled or the seizures are not being well-controlled by medication.
- Stereotactic radiosurgery (SRS) may be cautiously considered for a single CM that has previously hemorrhaged and is located in a high-risk surgical area. It is not used for treating a CM that is familial, asymptomatic or located in a surgically accessible area.