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Vascular Malformations: BrainVascular Malformations: Brain

Alice Boyd Smith, Lt. Col., USAF MCAlice Boyd Smith, Lt. Col., USAF MCChief, NeuroradiologyChief, Neuroradiology

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

Bethesda, MDBethesda, MD

Vascular MalformationsVascular 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 angiomaCavernous angioma Developmental venous anomaly (DVA)Developmental venous anomaly (DVA) Capillary telangiectasiaCapillary telangiectasia Sinus pericraniiSinus pericranii

AVMAVM

Nonneoplastic vascular Nonneoplastic vascular abnormalitiesabnormalities

Arteriovenous shunting & Arteriovenous shunting & no intervening capillary bedno intervening capillary bed

congenitalcongenital–– congenital congenital –– Usually neural tissue in Usually neural tissue in

betweenbetween Occur anywhere in brain or Occur anywhere in brain or

spinal cordspinal cord 98% solitary98% solitary

–– Multiple AVMS usually Multiple AVMS usually syndromic:syndromic: Hereditary hemorrhagic Hereditary hemorrhagic

telangiectasia (HHT)telangiectasia (HHT)

AVM

HHT AVMAVM

Peak age: 20Peak age: 20--40 year old40 year old Risk of hemorrhage: 2Risk of hemorrhage: 2--4%/year4%/year

–– ~~50% present with symptoms of 50% present with symptoms of hemorrhagehemorrhagehemorrhagehemorrhage

Spontaneous obliteration rare: < 1%Spontaneous obliteration rare: < 1% Dysregulated angiogenesis Dysregulated angiogenesis

continued vascular remodelingcontinued vascular remodeling

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AVM Grading: Spetzler AVM Grading: Spetzler --Martin ScaleMartin Scale SizeSize

–– Small (<3cm) = 1Small (<3cm) = 1–– Medium (3Medium (3--6 cm) = 26 cm) = 2–– Large (>6 cm) = 3Large (>6 cm) = 3g ( )g ( )

LocationLocation–– Noneloquent = 0Noneloquent = 0–– Eloquent = 1Eloquent = 1

Venous drainageVenous drainage–– Superficial = 0Superficial = 0–– Deep = 1Deep = 1

AVM Imaging: CTAVM Imaging: CT

Variable HemorrhageVariable Hemorrhage Calcification: 25Calcification: 25--30%30% Enhance postEnhance post contrastcontrast Enhance postEnhance post--contrastcontrast CTA: Enlarged arteries & draining CTA: Enlarged arteries & draining

veinsveins

AVM Imaging: MRIAVM Imaging: MRI

Flow Voids: “Bag of Flow Voids: “Bag of worms”worms”

Variable hemorrhageVariable hemorrhagegg–– “Blooming” on T2* GRE“Blooming” on T2* GRE

T2: Increased signal T2: Increased signal gliosisgliosis

Contrast: Strong Contrast: Strong enhancementenhancement

MRA/MRVMRA/MRV

AVM Imaging: Conventional AVM Imaging: Conventional AngiographyAngiography

Best method of imagingBest method of imaging Must image ICA, ECA & vertebral Must image ICA, ECA & vertebral

circulationscirculations–– 2727--32% of AVMs have dual arterial supply32% of AVMs have dual arterial supply

Dural arterial supply via leptomeningeal Dural arterial supply via leptomeningeal anastomoses or transdural anastomosesanastomoses or transdural anastomoses–– Transdural anastomoses affects treatment Transdural anastomoses affects treatment

decisionsdecisions

AVM: Associated AbnormalitiesAVM: Associated Abnormalities

FlowFlow--related related aneurysm on feeding aneurysm on feeding artery: 10artery: 10--15%15%yy

Intranidal Intranidal “aneurysm”: >50%“aneurysm”: >50%

Vascular “steal”: Vascular “steal”: Ischemia in adjacent Ischemia in adjacent brainbrain

Increased Risk of HemorrhageIncreased Risk of Hemorrhage

Central venous drainageCentral venous drainage Intranidal aneurysmIntranidal aneurysm

–– Difficult to detect by MRDifficult to detect by MR–– Difficult to detect by MRDifficult to detect by MR

PeriPeri-- or intraventricular locationor intraventricular location +/+/-- obstruction of venous outflowobstruction of venous outflow

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AVM: TreatmentAVM: Treatment

EmbolizationEmbolization Radiation: Stereotaxic radiosurgery Radiation: Stereotaxic radiosurgery Microvascular surgeryMicrovascular surgery Microvascular surgeryMicrovascular surgery

dAVFdAVF

Arteriovenous shunts within duraArteriovenous shunts within dura 1010--15% of intracranial vascular 15% of intracranial vascular

malformationsmalformations2 t2 t 2 types:2 types:–– Adult: Tiny vessels in wall of thrombosed dural Adult: Tiny vessels in wall of thrombosed dural

venous sinus venous sinus typically middle aged & older typically middle aged & older patientspatients Usually acquired Usually acquired -- traumatrauma

–– Infantile: Multiple highInfantile: Multiple high--flow AVflow AV--shunts shunts involving several thrombosed dural sinusesinvolving several thrombosed dural sinuses

dAVF Grading: Cognard ClassificationdAVF Grading: Cognard Classification

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

Type V: Spinal perimedullary venous drainageType V: Spinal perimedullary venous drainage–– Progressive myelopathyProgressive myelopathy

dAVFdAVF

Most common near skull baseMost common near skull base–– Transverse sinus most commonTransverse sinus most common

Hemorrhage incidence: 2Hemorrhage incidence: 2--4% per year4% per yearll Spontaneous closure rareSpontaneous closure rare

–– Most are type IMost are type I

dAVF Imaging: CTdAVF Imaging: CT

NECT: May be normalNECT: May be normal CECT: May see tortuous dural feeders & CECT: May see tortuous dural feeders &

enlarged dural sinusenlarged dural sinusgg

dAVF Imaging: MRIdAVF Imaging: MRI

Flow voids around dural venous sinusFlow voids around dural venous sinus Thrombosed sinusThrombosed sinus Dilated cortical veins without parenchymal nidusDilated cortical veins without parenchymal nidusp yp y T2: Focal hyperintensity in adjacent brain T2: Focal hyperintensity in adjacent brain

retrograde leptomeningeal venous drainage/ retrograde leptomeningeal venous drainage/ venous perfusion abnormalitiesvenous perfusion abnormalities

MRA: May be negativeMRA: May be negative MRV: Occluded sinus, collateral flowMRV: Occluded sinus, collateral flow

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dAVF: Conventional AngiographydAVF: Conventional Angiography

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

Tortuous engorged pial veins Tortuous engorged pial veins ”pseudophlebitic pattern””pseudophlebitic pattern”

dAVF

Carotid Cavernous Fistula (CCF)Carotid Cavernous Fistula (CCF)

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

Neonatal > infant Neonatal > infant presentationpresentation–– Rare adult presentationRare adult presentation

Classification:Classification:–– Choroidal: Multiple feeders from Choroidal: Multiple feeders from

pericallosal, choroidal, & pericallosal, choroidal, & thalmoperforating arteriesthalmoperforating arteries

–– Mural: Few feeders from Mural: Few feeders from collicular or posterior choroidal collicular or posterior choroidal arteriesarteries

VOGVOG Newborns: Most common extracardiac cause of Newborns: Most common extracardiac cause of

highhigh--output congestive heart failureoutput congestive heart failure < 1% of cerebral vascular malformations< 1% of cerebral vascular malformations

VOG: CT FindingsVOG: CT Findings

Venous pouchVenous pouch May have hydrocephalusMay have hydrocephalus AtrophyAtrophy

P h l t hP h l t h Parenchymal atrophyParenchymal atrophy Intraventricular hemorrhage: Intraventricular hemorrhage:

RareRare Post contrast: Avid Post contrast: Avid

enhancement of feeding enhancement of feeding arteries and veinarteries and vein

VOG: MR ImagingVOG: MR Imaging

Flow voidsFlow voids T1 hyperintensity T1 hyperintensity

–– In pouch In pouch ppthrombusthrombus

–– 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

laterlater

Cavernous MalformationCavernous Malformation

AKA: Angioma, cavernoma, cavernous AKA: Angioma, cavernoma, cavernous hemangiomahemangioma

Variable size intercapillary vascular Variable size intercapillary vascular spaces sinusoids & larger cavernousspaces sinusoids & larger cavernousspaces, sinusoids, & larger cavernous spaces, sinusoids, & larger cavernous spacesspaces–– No intervening brainNo intervening brain–– “Blood sponge”“Blood sponge”

2 types:2 types:–– Inherited: Multiple & bilateralInherited: Multiple & bilateral–– SporadicSporadic

Cavernous Malformation: Cavernous Malformation: ImagingImaging

Little or no mass effectLittle or no mass effect–– Unless complicated by Unless complicated by

hemorrhagehemorrhage May have internal areas May have internal areas

of thrombosis or of thrombosis or hemorrhagehemorrhage–– Peripheral hemosiderin Peripheral hemosiderin

causes T2 shortening causes T2 shortening resulting in a black resulting in a black “halo” around the lesion“halo” around the lesion

Cavernous Malformation

Cavernous MalformationCavernous Malformation Risk of hemorrhage Risk of hemorrhage

0.250.25--0.7%/year0.7%/year–– More common in posterior More common in posterior

fossa lesionsfossa lesions–– In patients with prior In patients with prior

hemorrhage annual ratehemorrhage annual ratehemorrhage annual rate hemorrhage annual rate of rehemorrhage of rehemorrhage 4.5%4.5%

Treatment:Treatment:–– Observation: Observation:

Asymptomatic or Asymptomatic or inaccessible lesionsinaccessible lesions

–– Surgical excisionSurgical excision–– Radiosurgery: Radiosurgery:

Progressively symptomatic Progressively symptomatic but surgically inaccessiblebut surgically inaccessible

Cavernous Malformation

MPGR

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Developmental Venous Developmental Venous Anomaly (DVA)Anomaly (DVA) May represent anatomic variantMay represent anatomic variant

–– Seen in up to 3% of autopsiesSeen in up to 3% of autopsies

Enlarged medullary veinsEnlarged medullary veins Drain into dural sinus or deep Drain into dural sinus or deep

ependymal veinependymal vein Usually solitaryUsually solitary “Medusa head” or “palm tree”“Medusa head” or “palm tree”

Developmental Venous AnomalyDevelopmental Venous Anomaly Isolated or associated with cavernous Isolated or associated with cavernous

angiomaangioma Hemorrhage unusualHemorrhage unusual

DVA Imaging: CTDVA Imaging: CT Calcification & ischemia may occur in the region Calcification & ischemia may occur in the region

drained drained most likely due to chronic venous most likely due to chronic venous obstructive diseaseobstructive disease–– RareRare

DVA

DVA: TreatmentDVA: Treatment

NONE!NONE!–– Removal may cause Removal may cause

venous infarctionvenous infarctionvenous infarctionvenous infarction

Capillary TelangiectasiaCapillary Telangiectasia

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

Sinus PericraniiSinus Pericranii

Spontaneous Spontaneous regression rareregression rare

Risk of hemorrhageRisk of hemorrhage Risk of hemorrhageRisk of hemorrhage TreatmentTreatment

–– SurgerySurgery–– EndovascularEndovascular

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