Cavernoma, also known as cavernous angioma, cavernous hemangioma and cerebral cavernous malformation (CCM), is a condition consisting of clusters of abnormally dilated thin-walled blood vessels. The clumped blood vessels are most often found in the brain and spinal cord; far fewer are found in other parts of the body including the skin and the retina of the eye. Lurie Children's Division of Neurosurgery treats cavernoma. Learn more.
Sometimes patients have only one of these clumps but sometimes they may have several. The size of the lesions can vary, ranging from being barely visible to the naked eye to a few inches in diameter.
Cavernomas are often described as looking like a raspberry because they are made up of many bubble-like structures called caverns that are filled with blood. The bubble-like caverns are grossly dilated thin walled vessels that leak due to defects in their walls.
Sometimes cavernomas are hereditary; this form of the condition is often associated with multiple cavernomas. Although this kind can happen in any family, it occurs at a higher rate among Mexican-American families because of a specific genetic mutation that has been found in this group. Each child of someone with the familial form has a 50% chance of inheriting the problem.
Up to 40% of solitary cavernomas may develop in the vicinity of another blood vessel abnormality called a venous angioma. Venous angiomas, also known as venous malformations or deep venous developmental anomalies (DVAs), usually don’t create problems unless they are close to cavernomas and consequently make it difficult to surgically remove the nearby cavernoma. Cavernomas are not visible on an angiogram because blood flows through the lesion slowly. This is one quality that makes cavernous angiomas different from arteriovenous malformations in which the blood flow is strong and fast and so are readily visible in an angiogram.
Estimates suggest that cavernomas occur in approximately one out of every 100-200 people, that is, roughly in 0.5 to 1% of the general population.
- Children under 20 years of age: 25 to 30%
- Adults ages 20 to 40: 60%
- Adults over 40: 10 to 15%
It is thought that more than 30% of people with cavernomas eventually develop symptoms.
Cavernomas are diagnosed most often when they become symptomatic and can be detected by:
- An MRI scan (the best way to diagnose the condition and may be repeated to assess change in the cavernoma’s size, any recent bleeding or the appearance of new lesions)
- Genetic testing for familial cases
- CT scans that can be used to demonstrate recent bleeding (but are not as sensitive as MRI scans)
Symptoms can vary widely and sometimes come and go, but they may include the following:
- Weakness in the arms or legs
- Vision problems
- Balance problems
- Memory and attention problems
- Medications to treat seizures and headaches
- Surgery may be recommended for cavernomas with recent hemorrhage, those that are expanding in size, and in some cases, those which are causing seizures
- Radiosurgery, by gamma knife, linear accelerator or new shaped beam techniques (this is a controversial treatment that has been used on symptomatic cavernous angiomas that are too difficult or dangerous to reach through traditional surgical approaches)
However, watchful waiting is sometimes the best course of action. Often people can go their entire lives without any problems, so a “wait and see” course is sometimes the wisest choice.
Learn about the care available in Lurie Children's Division of Neurosurgery.
Risk of Hemorrhage
The risk of hemorrhage is related to the location and number of cavernomas that are present. The higher the number, the greater the chance of one or more hemorrhages occurring sometime over a lifetime. On average, cavernous angiomas that have bled in the past are those that are the most likely to bleed again, particularly in the first two years after their initial bleed. It is also important to note that a hemorrhage in a cavernoma in the brain stem can be life-threatening, as the brainstem is responsible for regulating critical life processes including breathing and heartbeat.