Cerebrovascular malformations are vascular malformations related to the vessels that supply the brain and other cranial structures.
Classification
Over the years, cerebral vascular malformations have been classified in a variety of ways by many authors, often on the basis of the presence or absence of arteriovenous shunting, histopathological features or demographics of affected individuals 4.
High or low flow
- high flow
- brain arteriovenous malformation (AVM) (also known as cerebral, pial, or parenchymal AVM)
- cerebral proliferative angiopathy
- dural arteriovenous fistula (DAVF)
- pial arteriovenous fistula (PAVF)
- low flow
- cerebral cavernous (venous) malformation
- capillary telangiectasia
- developmental venous anomaly (DVA) (formerly called venous angiomas)
- cerebral venous varix
- sinus pericranii (in skull vault)
- mixed vascular malformation (cavernous malformation with a developmental venous anomaly)
Presence or absence of shunting
- with shunting
- without shunting
- capillary telangiectasia
- cavernous venous malformation (cavernoma)
- sinus pericranii
- venous malformation
- developmental venous anomaly (DVA)
- venous varix
- dural sinus malformation (although usually associated with shunting lesion)
Histomorphology
- arterial lesions
- focal arterial lesions
- genetic influences (e.g. arterial aneurysms associated with neurofibromatosis type 1, Ehlers-Danlos syndrome type IV, and familial immune deficiency syndrome)
- segmental arterial lesions
- twin aneurysm and segmental aneurysm
- metameric arterial lesions
- defected mesodermal cell migration gives rise to metameric syndrome or CAMS (craniofacial arteriovenous metameric syndrome), including Wyburn-Mason Syndrome
- focal arterial lesions
- capillary lesions
- focal capillary lesions
- associated with hereditary hemorrhagic telangiectasia
- focal lesion at arteriovenous junction may lead to classic AVM, choroidal AVM or dural AV fistula
- segmental capillary lesions
- metameric capillary lesions
- focal capillary lesions
- venous lesions
- focal venous lesions
- segmental venous lesions
- metameric venous lesions (also termed as craniofacial venous metameric syndrome)
- Sturge Weber syndrome (non familial)
- blue rubber bleb nevus syndrome (familial)
Pediatric
Some lesions are only encountered in specific age-groups, usually within early childhood.
- in utero: pial high flow AVF, VGAM, DSM
- neonate: VGAM, pial high flow AVF, DSM
- infant: VGAM, pial AVMs, cavernous angiomas, aneurysm
- child: pial AVM (nidal > fistulous), aneurysm, cavernous venous malformation (cavernoma), dural AVF