From the Guidelines
Cavernomas carry a significant risk of bleeding, seizures, and neurological deficits, with an annual bleeding risk of approximately 3.3% to 4.5%, and management should prioritize minimizing morbidity and mortality. The risk of bleeding from cavernomas is a major concern, with higher risks for those with prior hemorrhage or brainstem location 1.
Key Considerations
- The annual bleeding risk is approximately 3.3% to 4.5% per year, with higher risks for those with prior hemorrhage or brainstem location 1.
- Management depends on symptoms, location, and bleeding history.
- For asymptomatic cavernomas, observation with regular MRI monitoring is typically recommended.
- Symptomatic patients with accessible lesions, especially those with recurrent hemorrhages or medically refractory seizures, may benefit from surgical removal.
- Stereotactic radiosurgery is an alternative for deep-seated lesions where surgery carries high risk, though its efficacy remains controversial 1.
Treatment Options
- Patients with seizures require anticonvulsant medication, typically levetiracetam (500-1500 mg twice daily) or lamotrigine (25-200 mg twice daily).
- Anticoagulants and antiplatelet drugs should generally be avoided due to increased bleeding risk.
- Pregnancy may increase hemorrhage risk due to hemodynamic changes.
Prognosis and Follow-up
- Most patients with asymptomatic cavernomas have a good prognosis, while those with brainstem lesions or multiple hemorrhages face higher morbidity.
- Regular follow-up with a neurosurgeon or neurologist is essential, with MRI surveillance typically performed annually for stable lesions and more frequently after bleeding events.
Genetic Considerations
- Multiple cavernous malformations have been identified in 13% of sporadic cases and 50% of familial cases, with genetic analysis identifying mutations of least 3 genes: CCM1 (KRIT1) at 7q21-q22, CCM2 (MGC4607) at 7p15-p13, and CCM3 (PDCD10) at 3q25.2-q27 1.
From the Research
Cavernoma Risk
- Cavernomas can lead to intracerebral hemorrhage, seizures, focal neurological deficits, or headaches if left untreated 2
- The risk of hemorrhage varies depending on the location of the lesion, with brainstem cavernomas having a higher risk of bleeding 3
- Hemispheric lesions appear to be more benign with lower annual bleed rate and risk of persisting disability, whereas those found in the thalamus, basal ganglia and brainstem typically have higher rebleed risk resulting in higher cumulative morbidity following subsequent hemorrhages 4
Prognosis
- The clinical presentation and the location of the lesion are the most important factors involved in determining the optimal course of treatment of cavernomas 2
- Cavernomas can exist as fairly benign lesions, frequently remaining clinically silent for life, which argues against the active treatment of all cavernoma patients 5
- The prognosis for patients with cavernomas is generally good, with microsurgical removal resulting in excellent results and an acceptable morbidity 3
Management
- The management of brain and spinal cavernomas includes two main options: conservative treatment or surgical removal 5
- Microsurgical resection, stereotactic radiosurgery, and conservative management are the three available methods of treatment, each with its advantages and limitations 2
- Stereotactic radiosurgery (SRS) is an effective treatment alternative for deep-seated cavernomas with multiple hemorrhages, reducing pretreatment annual rebleed rates and stabilizing lesions with no more than one bleed 4
- SRS can also be effective for cavernomas causing therapy-resistant epilepsy, especially if applied within 3 years after presentation 4
- The dose schedule efficacy, safety and late adverse effects of SRS were evaluated for patients with symptomatic cavernomas, showing that SRS can be an effective and reliable method in terms of symptom control and prevention of rebleeding, especially in patients with inoperable cavernomas 6