Cerebral Cavernous Malformation (Cavernoma)

You’ve been diagnosed with a cavernous malformation — what does it mean?

A CCM diagnosis means that there is a low-flow vascular lesion in the brain (or spinal cord), made up of multiple dilated, blood-filled vascular caverns separated by thin walls.

It is not a malignant tumor. However, a CCM can:

• remain asymptomatic and stable for years, or
• bleed, enlarge, or irritate the cortex and trigger epileptic seizures.

What exactly are cerebral cavernous malformations (CCM)?

CCMs are clusters of dilated, thin-walled capillary-type vessels lacking a normal muscular layer. They often contain both old and recent microhemorrhages, with hemosiderin deposition in the surrounding tissue.

Typical features include:

• “Popcorn”/mulberry appearance on MRI with mixed signal intensities
• A dark hemosiderin rim on T2 and especially on SWI sequences
• Usually no intense contrast enhancement
• Often associated with a developmental venous anomaly (DVA), which should be preserved during surgery

Causes, sporadic vs familial forms

CCMs can be:

• Sporadic — typically a single lesion, often near a DVA or occasionally in an area exposed to prior radiation.
• Familial (hereditary) — autosomal dominant inheritance, with multiple lesions throughout the brain/spinal cord, due to pathogenic variants in CCM1 (KRIT1), CCM2, or CCM3 (PDCD10).

Familial disease often presents with multiple CCMs on SWI/gradient echo sequences, even if routine sequences show only a few. Certain genotypes (e.g., CCM3) may be more aggressive and can be associated with hemorrhagic events at a younger age.

What symptoms can occur, and what are the key risks?

A CCM may present with:

• Sudden neurological symptoms due to acute hemorrhage (weakness, speech disturbance, imbalance, etc.)
• Seizures (focal or generalized), especially with cortical/supratentorial lesions
• Focal neurological deficits (e.g., sensory changes, weakness, visual disturbance)
• Headache, sometimes localized—particularly with recent hemorrhagic expansion
• Incidental discovery on MRI performed for another reason

The risk of symptomatic hemorrhage is higher in:

• Deep lesions (thalamus, basal ganglia) and especially brainstem CCMs
• Lesions with a recent symptomatic bleed (higher rebleed risk over the following years)

How is it diagnosed and followed?

Diagnosis is primarily MRI-based:

• Brain MRI with T1, T2, FLAIR — defines the lesion, recent blood products, and any associated edema.
• Gradient echo or SWI — the most sensitive sequences to detect small CCMs and the true disease burden.
• Contrast-enhanced MRI — CCMs typically do not enhance strongly; marked enhancement raises concern for tumor or AVM.
• Catheter angiography (DSA) — CCMs are usually angiographically occult; DSA is used when AVM needs to be excluded.

When lesions are near eloquent areas, functional MRI and diffusion tensor imaging (DTI) can help map critical networks and white-matter tracts to plan the safest surgical corridor.

When is surgery recommended, and when is observation safer?

The decision is individualized. In general, surgery may be considered for lesions with:

• Documented symptomatic hemorrhage causing neurological deficit
• Recurrent hemorrhages or clear hemorrhagic growth
• Medically refractory seizures clearly linked to a single, surgically accessible CCM
• Progressive symptoms from mass effect in a surgically favorable location

Non-operative surveillance is often preferred when:

• The CCM is incidental and asymptomatic
• It is in a high-risk surgical location (deep or brainstem) without compelling indications
• There is no hemorrhage history and seizures are well controlled with medication

What treatment options are available?

Conservative management

• Regular clinical follow-up and interval MRI
• Anti-seizure medication for patients with seizures
• Careful, individualized decisions regarding anticoagulants/antiplatelets when truly necessary

Microsurgical removal (lesionectomy)

• Goal: complete removal of the CCM and, when safe, the hemosiderin rim
• Use of neuronavigation, functional MRI planning, intraoperative mapping and monitoring
• Strict emphasis on preserving any associated DVA to avoid venous infarction

Epilepsy-directed surgery

• CCM removal combined with resection of adjacent epileptogenic cortex (“lesionectomy plus”) in non-eloquent regions
• Aim: seizure freedom and, when possible, gradual medication reduction under specialist guidance

Stereotactic radiosurgery (SRS)

• An option for deep/inoperable lesions with recurrent symptomatic hemorrhage
• Outcomes remain debated; not considered first-line therapy in most cases
• Potential risks include edema and, in some contexts, formation of new lesions—particularly in familial disease

What should I expect in the hospital?

For an elective CCM resection:

• Preoperative evaluation (high-resolution MRI; sometimes fMRI/DTI)
• Discussion of the surgical plan and location-specific risks
• Microsurgery with neuronavigation and neurophysiological monitoring
• Postoperative monitoring in a high-dependency unit or ward during the first 24–48 hours
• Early mobilization and gradual return to light activities

In an acute hemorrhage scenario, admission may be urgent, with stabilization and a decision for immediate vs delayed surgery based on clinical and radiological factors.

Recovery and long-term outlook

Prognosis depends mainly on:

• CCM location (superficial vs deep/brainstem)
• The presence and severity of prior hemorrhages
• Whether permanent neurological deficits existed before surgery
• Seizure response to medical and/or surgical therapy

In many patients—particularly with superficial CCMs—complete removal provides a very low likelihood of future bleeding from that specific lesion, and often meaningful seizure improvement or remission.

Daily life, exercise, and restrictions

After stabilization or treatment:

• Most patients can gradually return to work and physical activity
• With active epilepsy, standard safety restrictions apply (driving regulations, supervised swimming, etc.)
• Avoid head trauma and high-impact activities for a period, based on individualized guidance
• There are typically no strict restrictions on air travel or altitude, except in special circumstances

Our team provides individualized recommendations based on the lesion, treatment plan, and neurological status.

When should I seek urgent medical help?

Urgent evaluation (ER/emergency services) is needed if you develop:

• A sudden, severe, or clearly “different” headache
• New weakness, numbness, or speech difficulty
• Sudden visual loss or double vision
• A new seizure—especially prolonged or recurrent seizures
• Confusion, marked drowsiness, or behavioral change