Download - Avm - Smith (Rsna 2011)
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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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