Initial Management of Brain Cavernous Malformations

Diagnostic Approach

• Brain MRI is the recommended imaging modality for diagnosis and clinical follow-up of suspected or known CCMs, with class I, level B evidence, according to the American Association of Neurological Surgeons 1
• MRI should include gradient echo or susceptibility-weighted sequences to determine if there are single or multiple CCMs, with class I, level B evidence, as recommended by the American Association of Neurological Surgeons 1
• Catheter angiography is not recommended unless arteriovenous malformation is being considered in the differential diagnosis, with class III, level C evidence, as stated by the American Association of Neurological Surgeons 1
• Follow-up imaging should be performed to guide treatment decisions or investigate new symptoms, with brain imaging performed as soon as possible after onset of clinical symptoms suspicious of hemorrhage, as recommended by the American Association of Neurological Surgeons 1

Management Algorithm Based on Clinical Presentation

Asymptomatic CCMs

• Conservative management is recommended for asymptomatic CCMs, particularly those in eloquent or deep locations, as suggested by the American Association of Neurological Surgeons 2
• The natural history risk of death or nonfatal stroke for an asymptomatic CCM is approximately 2.4% over 5 years, according to the American Association of Neurological Surgeons 3
• Surgical resection is not generally recommended for asymptomatic CCMs, especially those in eloquent, deep, or brainstem regions, as stated by the American Association of Neurological Surgeons 4
• Radiosurgery is not recommended for asymptomatic CCMs, with class III, level C evidence, as recommended by the American Association of Neurological Surgeons 5, 2

Symptomatic CCMs

• Antiepileptic therapy is reasonable for first seizure thought to be due to a CCM, with class I, level B evidence, as recommended by the American Association of Neurological Surgeons 6
• Approximately 50-60% of patients become seizure-free on medication after first diagnosis of CCM-related epilepsy, according to the American Association of Neurological Surgeons 2
• Surgery may be considered early if seizures were associated with hemorrhagic CCM or in patients who may not be compliant with medications, as suggested by the American Association of Neurological Surgeons 2
• For easily accessible, symptomatic CCMs, surgical resection may be considered, with mortality and morbidity equivalent to living with the CCM for about 2 years, with class IIb, level B evidence, as recommended by the American Association of Neurological Surgeons 2
• For deep CCMs, surgical resection may be considered if symptomatic or after prior hemorrhage, with mortality and morbidity equivalent to living with the CCM for 5-10 years, with class IIb, level B evidence, as recommended by the American Association of Neurological Surgeons 2
• For brainstem CCMs, surgical resection may be offered after a second symptomatic bleed, with class IIb, level B evidence, as recommended by the American Association of Neurological Surgeons 2
• Radiosurgery may be considered for solitary CCMs with previous symptomatic hemorrhage if located in eloquent areas with unacceptably high surgical risk, with class IIb, level B evidence, as recommended by the American Association of Neurological Surgeons 5, 2
• The recommended prescription dose for stereotactic radiosurgery is between 11-13 Gy to reduce radiation-induced adverse effects, according to the American Association of Neurological Surgeons 3

Special Considerations

Familial/Multiple CCMs

• Patients with familial or multifocal CCM may consider genetic counseling prior to pregnancy, with class I, level C evidence, as recommended by the American Association of Neurological Surgeons 6
• Radiosurgery is not recommended in familial CCM because of concern about de novo CCM genesis, with class III, level C evidence, as stated by the American Association of Neurological Surgeons 5

Pregnancy

• Patients may be counseled that the risk of neurological symptoms during pregnancy is likely not different than the non-pregnant state, with class IIa, level B evidence, as recommended by the American Association of Neurological Surgeons 6
• MRI should be considered in patients with CCM who develop new neurological symptoms during pregnancy, with class IIa, level C evidence, as recommended by the American Association of Neurological Surgeons 6

Common Pitfalls and Caveats

• Radiosurgery has no immediate effect on the CCM and may take 2-3 years to reduce hemorrhage risk, according to the American Association of Neurological Surgeons 3
• Higher radiosurgery doses (>13 Gy) are associated with increased risk of radiation-induced adverse effects, as stated by the American Association of Neurological Surgeons 3