cavernous malformations, venous malformations, and capillary telangiectasias richard e. clatterbuck,...

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