INTRACEREBRAL HEMORRHAGE / VASCULAR MALFORMATIONS
Cerebral cavernous malformation
Created 20/04/2021, last revision 29/04/2023
- cerebral cavernous malformation (CCM) is a well-circumscribed accumulation of dilated, thin-walled vessels affecting the brain
- only a layer of endothelium and subendothelial stroma is present; smooth muscle cells and elastic fibers are absent
- the pathogenesis of CM remains unclear
- only a layer of endothelium and subendothelial stroma is present; smooth muscle cells and elastic fibers are absent
- forms:
- sporadic
- incidence in the population 0.4-0.9% [Sage, 1993]
- accounts for 8-15% of all vascular malformations
- usually one lesion (in about 70% of cases)
- familial
- sporadic
- can be localized anywhere (approximately 80% are supratentorial)
- CM-associated anomalies:
- café au lait spots
- DVA (venous angioma)
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Pathophysiology
- erythrocyte diapedesis through the pathological cavernoma wall
- hemoglobin degradation products lead to pathological excitability, oxidative damage, and gliosis ⇒ epilepsy
- bleeding into the cavernoma ⇒ ↑ malformation volume
- bleeding into the surrounding area
Clinical presentation
- asymptomatic in up to 44% of cases
- epileptic seizures (45-70%) [Májovský, 2014] [Sage, 1993]
- mainly in lesions localized in the frontal and temporal lobes
- cavernous malformations are the cause of approximately 4% of refractory epilepsies
- bleeding
- low risk (0.25-0.7% per year), increased with previous bleeding (⇒ 4.5% / year)
- higher risk with infratentorial lesions
- hemorrhages are not fatal due to low pressure within the malformation; repeated hemorrhages, however, may ultimately lead to severe deficit
- headache (20-30%)
- focal neurological deficit (e.g., in case of cerebellar or brainstem lesion)
- risk factors for adverse outcome
- multiple lesions
- CCM3 genotype
- early clinical presentation
- infratentorial localization
- diameter >1 cm
- associated venous anomaly (DVA)
Diagnostic evaluation
- CT
- MRI (sensitivity 100%)
- popcorn appearance
- heterogeneous lesion with hemosiderin rim (T2-hypointense)
- prominent finding on GRE – “blooming artifact”
- slight enhancement is possible [Pinker, 2006]
- popcorn appearance
- DSA is normal
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- the Zabramski MRI classification has been proposed as a way to classify cerebral cavernous malformations
- useful rather for scientific purposes
- type I: subacute hemorrhage
- T1: hyperintense
- T2: hypo-/hyperintense
- type II: classic “popcorn” lesion
- T1 and T2: mixed-signal intensity centrally
- T2*/GRE/SWI: hypointense rim with blooming
- type III: chronic hemorrhage
- T1: hypointense/isointense centrally
- T2: hypointense centrally
- T2*/GRE/SWI: hypointense rim with blooming
- type IV: multiple punctate microhemorrhages
- GRE/SWI: “black dots” with blooming
- difficult to distinguish from small capillary telangiectasias
Management
Surgery and radiotherapy
- microsurgical resection
- relatively safe surgical procedure with low morbidity and mortality [Májovský, 2014]
- proven indications:
- refractory epilepsy (up to 90% of patients are seizure free after the surgery)
- recurrent bleeding
- in infratentorial CMs, surgery is preferred in cerebellar lesions and hemorrhagic lesions localized near the ventricle or cistern [Amato, 2013]
- stereotactic radiosurgery (SRS) may be considered for inoperable lesions [Liščák, 2013] [Liščák, 2000]
- indications and therapy results are controversial
- in contrast to AVMs, the direct effect of therapy cannot be observed; the long-term clinical course may change compared to untreated lesions
Conservative therapy and follow-up
- follow-up MRI every 1-2 years in asymptomatic lesions
- keep blood pressure in the normal range
- beta-blockers may reduce the risk of intracranial hemorrhage or persistent/progressive focal neurological deficit in patients with CCM (Zuurbier, 2022)
