Cavernous Malformations, Venous Malformations, and Capillary Telangiectasias

Richard E. Clatterbuck, M.D., Ph.D.

Department of Neurological Surgery

The Johns Hopkins University School of Medicine

Cavernous Malformations

• Abnormal vascular channels lined by endothelial cells (cells that line all blood vessels) but lacking other components of typical blood vessel walls

• Have been described as having an appearance similar to mulberries

AKA

• Cavernous angioma

• Cavernous hemangioma

• Cerebral angioma

• Cavernoma

• Cavmals

• CCMs (cerebral cavernous malformations)

Radiographic Appearance

• Classically described as a “popcorn” lesion with a reticulated appearance on MRI scans

Epidemiology

• Occur in 0.5% of the population (1 in 200 people) and may represent 10% of vascular malformations

• Perhaps slightly more prevalent in females, 1.8:1 in our series

Presentation

• Mean age 34.6 years (7.8-78.5)

• Average of 3.4 lesions per patient, solitary in 63% and multiple in 25%

• 19% had venous malformations

• 4% had capillary telangiectasias

• Headache (65%), seizures (49%), focal deficits (46%), hemorrhage (13%), asymptomatic (1.5%)

Hemorrhage rates

• 3.1% per patient year hemorrhage rate

• 0.9% in males

• 4.2% in females

• No difference in lesions in the cerebrum or brainstem

Seizure rates

• 4.8% per patient year

• 2.4% new onset seizure rate per patient year

• 5.5% recurrent seizure rate per patient year

Venous malformation

• Persistence of a developmentally expressed venous drainage pattern

• Classically described as a caput medusae appearance

AKA

• Developmental venous anomaly (DVA)

• Venous anomaly

• Venous angioma

• Venous malformation

Presentation

• Mean age 39.1 years (18.7-73)

• 19% had another cerebrovascular malformations

• Headache (50.8%), seizures (30.2%)

Hemorrhage rate

• 0.15% per lesion year

Capillary Telangiectasia

• Normal capillary structurally at the cellular level but abnormal in size, dramatically dilated

AKA

• Capillary malformations

• Captels

Presentation

• Much rarer lesions with unclear although certainly low hemorrhage rate

• Present throughout life but typically in 3rd or 4th decade

• Symptoms include headache, numbness, dizziness, visual disturbance

How are these related?

Cerebral Vascular Malformations

• Cavernous malformation

• Venous malformation

• Capillary telangiectasia