Cavernoma

What is a Cavernoma?

A cavernoma (also known as a cavernous malformation or cavernous angioma) is a cluster of delicate, irregularly shaped, enlarged blood vessels (capillaries) located in the brain, spinal cord, or brainstem.

How Common Are Cavernomas?

Cavernomas occur in approximately 1 in 100–200 people and account for about 10–15% of all cerebrovascular diseases affecting the brain and spinal cord.

About 30% of patients with cavernomas experience their first symptoms between ages 20 and 30.

• 70–80% of cavernomas are located in the cerebral hemispheres.
• Annual hemorrhage risk: 0.7–1.7% per cavernoma (some patients may have multiple cavernomas).

Approximately 20% of cases are hereditary (familial cavernomas), where patients frequently develop multiple cavernomas. If one parent has the hereditary form, the probability of passing it to a child is 50%.

Age Distribution of Diagnosis:

• 25–30% of cases are diagnosed before age 20.
• 60% of cases occur between ages 20–40.
• 10–15% of cases occur in patients older than 40.

Symptoms of Cavernous Angiomas

In about 50% of cases, cavernomas cause no symptoms and are discovered incidentally during CT or MRI scans performed for other reasons.

Symptoms may occur when a cavernoma enlarges, its thin walls rupture, leading to hemorrhage, or if the cavernoma is located in functionally important areas of the brain, brainstem, or spinal cord.

Most Common Symptoms Include:

• Epileptic seizures (50%)
• Hemorrhages and associated deficits (25%)
• Neurological problems (depending on location) without visible hemorrhage (25%)

How Is a Cavernoma Diagnosed?

A cavernous angioma is often referred to as an "angiographically invisible" vascular lesion. Despite being composed of blood vessels, cavernomas cannot be detected using CT angiography (CTA), MRI angiography (MRA), or digital subtraction angiography (DSA) due to extremely slow blood flow within the lesion.

Imaging Methods:

• Computed Tomography (CT/DT) – widely available and useful for detecting hemorrhage, but less sensitive for small cavernomas or complex structures like the brainstem and spinal cord.
• Magnetic Resonance Imaging (MRI) – the most accurate method for diagnosing cavernomas, especially when using contrast enhancement.

MRI scans should be performed at institutions where neuroradiologists (specialists in brain and spinal imaging) interpret the results. Follow-up MRIs may be required to monitor changes in cavernoma size, hemorrhages (even asymptomatic ones), or the development of new cavernomas.

What Should You Do If Diagnosed with a Cavernoma?

If you or a loved one has confirmed or suspected cavernous angioma, a neurosurgical consultation is recommended.

We offer in-person and remote consultations via video calls. Appointments can be scheduled online, via phone, or email. Contact us to arrange a remote consultation.

Treatment Options for Cavernous Angiomas

For Asymptomatic Cavernomas

Cavernomas found incidentally and causing no symptoms are typically monitored with MRI scans:

• Annual MRI scans for the first 2 years
• Subsequent MRIs every 3–5 years

If new symptoms arise or hemorrhage is suspected, an immediate MRI is required.

Despite decades of surgical experience treating cavernomas, the surgical management of asymptomatic cavernomas remains controversial. The risks of surgery must be carefully weighed against the natural course of the disease, depending on cavernoma location.

For example:

• Stroke and mortality risk after cavernoma surgery: 6%
• Natural risk of an unruptured cavernoma: 2.4% over 5 years
• Risk of rebleeding after first hemorrhage: 29.5% over 5 years

Thus, surgical risk is high for asymptomatic cavernomas but significantly lower compared to repeated hemorrhages after an initial bleed.

For Symptomatic Cavernomas

Surgery may be considered for epilepsy if:

• Seizures cannot be controlled with medications.
• The cavernoma is in a "low-risk" accessible brain area.
• A neurologist confirms that the cavernoma is highly likely to be the seizure source.

Surgery may also be recommended for patients who have had one hemorrhage causing neurological deficits in an easily accessible location.

For cavernomas in high-risk functional brain areas, surgery and rebleeding risks must be carefully evaluated. Surgical intervention may be considered if multiple hemorrhages occur.

Stereotactic Radiosurgery (SRS)

Stereotactic radiosurgery may be considered for single cavernomas with previous symptomatic hemorrhage if the cavernoma is deep within a functionally critical area and surgery poses an unacceptably high risk.

However, after stereotactic radiosurgery, the hemorrhage risk temporarily increases, making MRI monitoring crucial:

• Brainstem cavernomas → Post-radiation hemorrhage risk: 11–15% per year for the first 2 years, then 1–2.4% thereafter.
• Radiosurgery should NOT be performed for asymptomatic cavernomas, surgically accessible cavernomas, or familial cavernomas.
• Radiation therapy may induce the formation of new cavernomas.

What to Expect After Surgery?

Most patients stay in the hospital for only a few days and can return to normal activities within a few weeks. A longer recovery may be needed for patients with neurological deficits.

Rebleeding Risk After Cavernoma Surgery

The risk of rebleeding varies significantly:

• Some patients never experience another hemorrhage.
• Others have frequent episodes of bleeding.