Cerebral Cavernous Malformation

A cerebral cavernous malformation (CCM), sometimes called a cavernous angioma or cavernoma, is a collection of capillaries (very small blood vessels) in the brain or spinal cord. The walls of the capillaries are very thin and prone to leaking. When they fill with blood, the capillaries stretch out and create “caverns.”

CCMs can cause neurological problems and can even be life-threatening. However, most people never develop symptoms.

CCMs affect about 1 in 500 people. CCMs are one of the more common cerebrovascular disorders in children and adults.

Some types of CCMs are genetic, while the underlying cause of the majority of CCMs are not well understood. There are two common types of CCMs:

• Familial CCMs are inherited from a parent and occur in at least 20% of all cases of CCMs. Those with this form of a CCM typically develop more than one cerebral cavernous malformation. The hereditary form is caused by one of three genes, CCM1, CCM2 and CCM3. People with familial CCMs have 50% chance of passing the gene to offspring.
• Sporadic CCMs occur in people with no family history of the disorder. Those with a sporadic CCM typically have only one malformation.

Signs and symptoms of CCMs depend on their location in the brain or spinal cord. Common symptoms may include:

• Seizures (most common first symptom)
• Hemorrhage (bleeding) in the brain
• Headaches  
• Weakness or numbness in the arms or legs
• Paralysis
• Vision problems
• Hearing loss or tinnitus (ringing or buzzing in the ear)
• Dizziness or nausea
• Central pain syndrome

CCMs are typically diagnosed by magnetic resonance imaging (MRI) of the brain or computed tomography (CT or CAT scan) of the brain. Many people do not have symptoms from their CCM, so the condition may be detected on an MRI or CT performed for another purpose.

Treatment will depend on your child’s symptoms and the location of the malformation. Treatment options include:

• Observation may be recommended if the CCM is not causing medical problems or if the CCM is in a location that would be difficult to access. In some cases, periodic imaging with MRI may be recommended. In other cases, imaging is only performed if new or worsening symptoms develop.
• Medications may be prescribed by a neurologist to address seizures, headaches or pain.
• Surgical removal of the malformation by a neurosurgeon can be a safe and effective option if the CCM is in a location that can be easily accessed without causing injury to important brain or spinal cord structures.