Vascular Malformations: Brain Vascular Malformations: Brain Alice Boyd Smith, Lt. Col., USAF MC Alice Boyd Smith, Lt. Col., USAF MC Chief, Neuroradiology Chief, Neuroradiology American Institute for American Institute for Radiologic Pathology Radiologic Pathology & Assistant Professor of Radiology & Radiological Sciences Assistant Professor of Radiology & Radiological Sciences Uniformed Services University of the Health Sciences Uniformed Services University of the Health Sciences Bethesda, MD Bethesda, MD Vascular Malformations Vascular Malformations Arteriovenous malformation (AVM) Arteriovenous malformation (AVM) Dural arteriovenous fistula (dAVF) Dural arteriovenous fistula (dAVF) Vein of Galen Malformation (VOG) Vein of Galen Malformation (VOG) Vein of Galen Malformation (VOG) Vein of Galen Malformation (VOG) Cavernous angioma Cavernous angioma Developmental venous anomaly (DVA) Developmental venous anomaly (DVA) Capillary telangiectasia Capillary telangiectasia Sinus pericranii Sinus pericranii AVM AVM Nonneoplastic vascular Nonneoplastic vascular abnormalities abnormalities Arteriovenous shunting & Arteriovenous shunting & no intervening capillary bed no intervening capillary bed congenital congenital – congenital congenital – Usually neural tissue in Usually neural tissue in between between Occur anywhere in brain or Occur anywhere in brain or spinal cord spinal cord 98% solitary 98% solitary – Multiple AVMS usually Multiple AVMS usually syndromic: syndromic: Hereditary hemorrhagic Hereditary hemorrhagic telangiectasia (HHT) telangiectasia (HHT) AVM HHT AVM AVM Peak age: 20 Peak age: 20-40 year old 40 year old Risk of hemorrhage: 2 Risk of hemorrhage: 2-4%/year 4%/year – ~50% present with symptoms of 50% present with symptoms of hemorrhage hemorrhage hemorrhage hemorrhage Spontaneous obliteration rare: < 1% Spontaneous obliteration rare: < 1% Dysregulated angiogenesis Dysregulated angiogenesis continued vascular remodeling continued vascular remodeling
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American Institute for American Institute for Radiologic PathologyRadiologic Pathology&&
Assistant Professor of Radiology & Radiological SciencesAssistant Professor of Radiology & Radiological SciencesUniformed Services University of the Health SciencesUniformed Services University of the Health Sciences
Type I: In sinus wall, normal antegrade venous Type I: In sinus wall, normal antegrade venous drainagedrainage
Type II: In main sinusType II: In main sinus–– A: Reflux into sinusA: Reflux into sinus–– B: Reflux into cortical veins: 10B: Reflux into cortical veins: 10--20% hemorrhage20% hemorrhage
Type III: Direct cortical drainageType III: Direct cortical drainage–– 40% hemorrhage40% hemorrhage
Type IV: Direct cortical drainage + venous ectasiaType IV: Direct cortical drainage + venous ectasia–– 2/3 hemorrhage2/3 hemorrhage
Multiple arterial feedersMultiple arterial feeders–– Dural/transosseous branches from ECA: most Dural/transosseous branches from ECA: most
commoncommon–– Tentorial/dural branches from ICA or VATentorial/dural branches from ICA or VATentorial/dural branches from ICA or VATentorial/dural branches from ICA or VA
Involved dural sinus frequently thrombosedInvolved dural sinus frequently thrombosed Flow reversal in dural sinus/cortical veins Flow reversal in dural sinus/cortical veins progressive symptoms, risk of hemorrhageprogressive symptoms, risk of hemorrhage
dAVF second most common sitedAVF second most common site Abnormal communication between carotid artery & Abnormal communication between carotid artery &
cavernous sinuscavernous sinus–– Enlarges cavernous sinusEnlarges cavernous sinus–– Usually see enlarged superior ophthalmic veinUsually see enlarged superior ophthalmic vein
CCF may be contralateral to dilated SOV CCF may be contralateral to dilated SOV
Classified by arterial supply & venous drainage Classified by arterial supply & venous drainage (Barrow):(Barrow):–– A: Direct ICAA: Direct ICA--cavernous sinus highcavernous sinus high--flow shuntflow shunt–– B: Dural ICA branchesB: Dural ICA branches--cavernous shuntcavernous shunt–– C: Dural ECAC: Dural ECA--cavernous shuntcavernous shunt–– D: ECA/ICA dural branches shunt to cavernous sinusD: ECA/ICA dural branches shunt to cavernous sinus
CCF: ImagingCCF: Imaging
CT:CT:–– Marked dilation & Marked dilation &
enhancement of enhancement of cavernous sinuscavernous sinus
–– May see prominent SOVMay see prominent SOV MRI:MRI:
–– Abnormal flow voids in Abnormal flow voids in cavernous sinuscavernous sinus
–– Enlargement of Enlargement of cavernous sinuscavernous sinus
Non-Contrast
CCF dAVF: TreatmentdAVF: Treatment
EndovascularEndovascular Surgical resectionSurgical resection Stereotaxic radiosurgeryStereotaxic radiosurgery Stereotaxic radiosurgeryStereotaxic radiosurgery Observation: Type 1Observation: Type 1
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Vein of Galen Malformation Vein of Galen Malformation (VOG)(VOG)
Arteriovenous fistula involving Arteriovenous fistula involving aneurysmal dilatation of aneurysmal dilatation of median prosencephalic veinmedian prosencephalic vein
–– In brain In brain ischemia, ischemia, calcificationcalcification
DWI: Restricted DWI: Restricted diffusion if acute diffusion if acute ischemia, infarctionischemia, infarction
VOG: AngiographyVOG: Angiography
Choroidal or muralChoroidal or mural Dural venous sinus anomaliesDural venous sinus anomalies
–– Falcine sinus in 50%Falcine sinus in 50%–– Falcine sinus in 50%Falcine sinus in 50%–– +/+/-- absence or stenosis of other sinusesabsence or stenosis of other sinuses
Choroidal
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VOG: TreatmentVOG: Treatment
ChoroidalChoroidal–– Medical therapy for congestive heart Medical therapy for congestive heart
failure failure until 5 or 6 mountil 5 or 6 mo–– 55--6 mo: Transcatheter embolization6 mo: Transcatheter embolization
Arterial more effective than venousArterial more effective than venous
MuralMural–– Transcatheter embolization performed Transcatheter embolization performed
Dilated capillaries Dilated capillaries interspersed within interspersed within normal brainnormal brainU ll llU ll ll Usually small, Usually small, asymptomatic incidental asymptomatic incidental findingsfindings–– Rare reports of Rare reports of
hemorrhage existhemorrhage exist
Most located in Most located in brainstem brainstem PonsPons
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Capillary TelangiectasiaCapillary Telangiectasia T2: Increased signalT2: Increased signal T2*: Low signalT2*: Low signal Ill defined enhancement after contrast administrationIll defined enhancement after contrast administration Occult on angiographyOccult on angiography Treatment: NoneTreatment: None
Sinus PericraniiSinus Pericranii
Communication between extracranial Communication between extracranial venous system & dural venous sinusvenous system & dural venous sinus
RareRareRareRare May be congenital or acquiredMay be congenital or acquired
Sinus Pericranii
CT: Single/multiple bone defects Vascular enhancement Conventional angiogram: Seen during venous phase
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