The cerebrum – Latin for “brain” – is the coordinating center of sensation, intellectual and nervous activity. A cavernous malformation is also called a cavernoma or a cerebral cavernous malformation (CCM), but these names are all interchangeable and refer to the same diagnosis.
Synonyms that refer to the same diagnosis include:
- Cavernous angioma
- Cavernous hemangioma
- Cavernous malformation (“Cav Mal”)
- Cerebral cavernous malformation (CCM)
For simplicity we will just use the term “cavernoma.” Cavernomas are a vascular mass made up of abnormal dilated blood vessels characterized by distended blood-filled “caverns.” Vessels of a cavernoma mass have a tendency to leak and bleed because they lack the proper junctions between neighboring cells as well as the necessary structural support from smooth muscle and the stretchable material (elastin) that normally supports regular blood vessels.
Cavernomas are present in up to 0.5% of the general population, and they account for a large proportion (8-15%) of all brain and spinal vascular malformations. While more common in adults, people of all ages may be affected by them, and approximately 25% of all diagnosed cavernomas are found in children.
Cavernomas are dynamic structures, changing in size and number over time and can range in size from a few millimeters to several centimeters. The majority of cavernomas that are diagnosed occur in individuals with only a single lesion and no family history of the disease (these cases are termed “sporadic” meaning they are not caused by an inherited genetic mutation). Individuals with the familial (genetic) form of cavernomas are likely to have multiple lesions and may be more likely to experience symptoms associated with the disorder. Cavernomas are commonly associated with developmental venous anomalies (DVA) also called, venous malformations or venous angiomas. A DVA is a type of vascular malformation that, on its own, does not cause any clinical symptoms. At least 40% of sporadic cavernomas may develop within the vicinity of a DVA. The significance of DVA association with sporadic lesions is currently under investigation; perhaps this observation may suggest a difference in developmental mechanisms between sporadic and familial cavernomas.
In many cases, a cavernoma causes no symptoms and can go unnoticed. Leakage and bleeding from cavernomas is the underlying cause of clinical symptoms. Depending on the size and location of the cavernoma, this bleeding can cause brain damage and even in rare cases death, however, bleeding from cavernomas is often less severe than bleeding from aneurysms or AVMs because they do not contain high-pressure arterial blood flow. Symptoms of a ruptured cavernoma often come on suddenly and include a sudden, severe headache that is different from past headaches, nausea and vomiting, sensitivity to light, fainting or loss of consciousness and seizures.