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Cavernous Malformations, Venous Malformations, and Capillary Telangiectasias

Department of Neurological Surgery. Cavernous Malformations, Venous Malformations, and Capillary Telangiectasias. Richard E. Clatterbuck, M.D., Ph.D. The Johns Hopkins University School of Medicine. Cavernous Malformations.

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Cavernous Malformations, Venous Malformations, and Capillary Telangiectasias

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  1. Department of Neurological Surgery Cavernous Malformations, Venous Malformations, and Capillary Telangiectasias Richard E. Clatterbuck, M.D., Ph.D. The Johns Hopkins University School of Medicine

  2. 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

  3. AKA • Cavernous angioma • Cavernous hemangioma • Cerebral angioma • Cavernoma • Cavmals • CCMs (cerebral cavernous malformations)

  4. Radiographic Appearance • Classically described as a “popcorn” lesion with a reticulated appearance on MRI scans

  5. 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

  6. 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%)

  7. 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

  8. Seizure rates • 4.8% per patient year • 2.4% new onset seizure rate per patient year • 5.5% recurrent seizure rate per patient year

  9. Venous malformation • Persistence of a developmentally expressed venous drainage pattern • Classically described as a caput medusae appearance

  10. AKA • Developmental venous anomaly (DVA) • Venous anomaly • Venous angioma • Venous malformation

  11. Presentation • Mean age 39.1 years (18.7-73) • 19% had another cerebrovascular malformations • Headache (50.8%), seizures (30.2%)

  12. Hemorrhage rate • 0.15% per lesion year

  13. Capillary Telangiectasia • Normal capillary structurally at the cellular level but abnormal in size, dramatically dilated

  14. AKA • Capillary malformations • Captels

  15. 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

  16. How are these related?

  17. Cerebral Vascular Malformations • Cavernous malformation • Venous malformation • Capillary telangiectasia

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