Vascular Malformations: Brain

Vascular Malformations
Arteriovenous malformation (AVM) Shunt Mixed Malformation Developmental venous anomaly = Combo (DVA) No Cavernous malformation Shunt Capillary telangiectasia
Classic Dural arteriovenous fistula (dAVF) Vein of Galen Malformation (VOG)

Alice Boyd Smith, MD Chief, Neuroradiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC & Assistant Professor of Radiology & Radiological Sciences Uniformed Services University of the Health Sciences Bethesda, MD

Classic AVM
Arteriovenous shunting & no intervening capillary bed
Enlarged feeding artery Nidus Early draining vein/ varix

AVM
T2

Congenital
Usually neural tissue in between

Occur anywhere in brain or spinal cord 98% solitary

Multiple AVMS usually syndromic:
Hereditary hemorrhagic telangiectasia (HHT) Cerebrofacial arteriovenous metameric syndromes (CAMS)

Nidus = Conglomeration of numerous AV shunts & dysplastic vessels

Hereditary Hemorrhagic Telangiectasia

>3 concurrent cerebral AVMS Rare!

AVM
Dysregulated angiogenesis continued vascular remodeling Peak age: 20-40 year old Risk of hemorrhage: 24%/year
~50% present with symptoms of hemorrhage NECT

An angiodyplastic disorder with AD inheritance

AVM Grading: Spetzler -Martin Scale

Size Small (<3cm) = 1 Medium (3-6 cm) = 2 Large (>6 cm) = 3 Location Noneloquent = 0 Eloquent = 1 Venous drainage Superficial = 0 Deep = 1

AVM Imaging: CT
Variable Hemorrhage Calcification: 25-30% Enhance postcontrast CTA: Enlarged arteries & draining veins
CECT

AVM
NECT NECT

AVM Imaging: MRI

Flow Voids: Bag of worms Variable hemorrhage
Blooming on T2* GRE
FLAIR T2

Calcification

Hemorrhage

T2: Increased signal gliosis Contrast: Strong enhancement MRA/MRV

AVM

AVM Imaging: Conventional Angiography

Best method of imaging Must image ICA, ECA & vertebral circulations
27-32% of AVMs have dual arterial supply

Cerebrofacial Arteriovenous Metameric Syndromes

Orbit & Sinus: What are you looking for?

Objectives
Recognize imaging findings in orbits & paranasal sinuses that will change patient management. Be able to develop checklist for imaging findings within orbits & paranasal sinuses that decreases likelihood of overlooking pertinent associated findings.

Alice Boyd Smith, Lt. Col., USAF MC Chief, Neuroradiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC & Assistant Professor of Radiology & Radiological Sciences Uniformed Services University of the Health Sciences Bethesda, MD

Orbits & Sinuses

Infection Trauma Neoplasm

Orbit: Infectious
Pre - or post-septal
Most often secondary to underlying paranasal sinusitis
Maxillary & ethmoid most common

Other etiologies:
Trauma Bacteremia Skin infections Dental infections CECT

Panophthalmitis

Orbit: Infectious
Subperiosteal abscess
Spread from sinus to orbit
Transmission via valveless venous plexus

Subperiosteal Abscess: Orbit

CT:
CECT Medial orbital wall with adjacent sinusitis Lentiform, rim enhancing Medial rectus displacement Lamina papyracea dehiscence
CECT

Direct extension:
Lamina papyracea dehiscence

MR
Post contrast: Rim enhancement; intra- & periorbital enhancement Fat suppression optimal

Visual disturbance:15-30%
Optic neuritis Ischemia: intraorbital pressure Retinal ischemia: Central artery occlusion or thrombophlebitis

Requires immediate attention!

May result in blindness

AVM: Associated Abnormalities

Flow-related aneurysm on feeding artery: 10-15% Intranidal aneurysm: >50% Vascular steal: Ischemia in adjacent brain

Increased Risk of Hemorrhage

Location
Periventricular Basal ganglia Thalamus NECT

Arterial
Pedicle aneurysm Intranidal aneurysm
Difficult to detect by MR

NECT

Venous
Central venous drainage Obstruction of venous outflow Varix

Small nidus

AVM: Treatment
Embolization Radiation: Stereotaxic radiosurgery
Eloquent

Arteriovenous Fistulas
Distinguished from AVMs by presence of direct, high flow fistula between artery & vein
Dural AVF (dAVF) Cavernous carotid fistula (CCF) Vein of Galen malformation

Combination

Surgery

dAVF
Arteriovenous shunts within dura 10-15% of intracranial vascular malformations 2 types:
Adult: Tiny vessels in wall of thrombosed dural venous sinus typically middle aged & older patients
Usually acquired - trauma T1 SSFSE SSFSE

dAVF
T1

Infantile: Multiple high-flow AVshunts involving several thrombosed dural sinuses

Fetal MRI

dAVF Grading: Cognard Classification

Type I: In sinus wall, normal antegrade venous drainage Type II: In main sinus
A: Reflux into sinus B: Reflux into cortical veins: 10-20% hemorrhage

dAVF Grading: Lalwani et al

Type III: Direct cortical drainage

40% hemorrhage

Type IV: Direct cortical drainage + venous ectasia

2/3 hemorrhage

Type V: Spinal perimedullary venous drainage

Progressive myelopathy

Type I

Type II

Type III

Type IV

dAVF
Most common near skull base
Transverse sinus most common

dAVF Imaging: CT
NECT: May be normal CECT: May see tortuous dural feeders & enlarged dural sinus
CECT

Hemorrhage incidence: 2-4% per year Spontaneous closure rare

Most are type I

dAVF Imaging: MRI

Flow voids around dural venous sinus Thrombosed sinus Dilated cortical veins without parenchymal nidus T2: Focal hyperintensity in adjacent brain MRA: May be negative MRV: Occluded sinus, collateral flow
T1+Gd T2 T2

dAVF
T1 T1

!!!

dAVF: Conventional Angiography

Multiple arterial feeders
Dural/transosseous branches from ECA: most common Tentorial/dural branches from ICA or VA CECT

dAVF

Involved dural sinus frequently thrombosed Flow reversal in dural sinus/cortical veins progressive symptoms, risk of hemorrhage Tortuous engorged pial veins pseudophlebitic pattern

Pseudophlebitic pattern

Carotid Cavernous Fistula (CCF)

dAVF second most common site Abnormal communication between internal carotid artery & cavernous sinus
Enlarges cavernous sinus Usually see enlarged superior ophthalmic vein
CCF may be contralateral to dilated SOV

Venous Drainage
SOV
Superficial Middle Cerebral V.

Uncal v. Cerebellar
Ca Sin verno us us

Classified by arterial supply & venous drainage (Barrow):

A: Direct ICA-cavernous sinus high-flow shunt B: Dural ICA branches-cavernous shunt C: Dural ECA-cavernous shunt D: ECA/ICA dural branches shunt to cavernous sinus

SPS IPS

Pterygoid & basilar plexus

CCF: Imaging
T1

CCF

CT:
Marked dilation & enhancement of cavernous sinus May see prominent SOV

MRI:
Abnormal flow voids in cavernous sinus Enlargement of cavernous sinus

Non-Contrast

CCF
T1+Gd T2

dAVF: Treatment
Endovascular Surgical resection Stereotaxic radiosurgery Observation:
Indirect CCF

Vein of Galen Malformation (VOGM)

Arteriovenous fistula involving aneurysmal dilatation of median prosencephalic vein (MPV) Neonatal > infant presentation
Rare adult presentation

VOGM
Newborns: Most common extracardiac cause of high-output congestive heart failure < 1% of cerebral vascular malformations
CECT

Drains MPV in 50%

Classification:
Choroidal: Multiple feeders from pericallosal, choroidal, & thalmoperforating arteries Mural: Few feeders from collicular or posterior choroidal arteries

T1

Falcine Sinus

Venous Pouch

VOGM: CT Findings
Venous pouch May have hydrocephalus Parenchymal atrophy Intraventricular hemorrhage: Rare Post contrast: Avid enhancement of feeding arteries and vein
CECT NCCT

VOGM

VOGM: MR Imaging
T2

VOGM: Angiography
Choroidal or mural Dural venous sinus anomalies

Flow voids T1 hyperintensity

In pouch thrombus In brain ischemia, calcification

T1

Falcine sinus in 50% +/- absence or stenosis of other sinuses

DWI: Restricted diffusion if acute infarction

Choroidal VOGM

VOGM: Treatment
Choroidal
Medical therapy for congestive heart failure until 5 or 6 mo 5-6 mo: Transcatheter embolization

Mural
Transcatheter embolization performed later

VOGM
CECT

Cavernous Malformation
AKA: Angioma, cavernoma, cavernous hemangioma Variable size intercapillary vascular spaces, sinusoids, & larger cavernous spaces
No intervening brain 2 types: Inherited: Multiple & bilateral Sporadic

Cavernous Malformation: Imaging

Little or no mass effect
Unless complicated by hemorrhage
T2

Cavernous Malformation
T2

May have internal areas of thrombosis or hemorrhage

Peripheral hemosiderin causes T2 shortening resulting in a black halo around the lesion

Cavernous Malformation
NECT

Cavernous Malformation
MRI NECT
-Variable -Popcorn ball - Surrounding edema in acute hemorrhage -Post contrast: minimal/ no enhance look for DVA!
T2 T1

CT Findings T1+Gd -Negative : 30-50% -40-60% Ca++ -No mass effect -Surrounding brain normal -Little or no enhance -CTA usually negative

Angiography: Usually occult

Cavernous Malformation
Risk of hemorrhage: 0.250.7%/year
More common in posterior fossa lesions In patients with prior hemorrhage annual rate of rehemorrhage 4.5% T2

Cavernous Malformation
T2

Treatment:
Observation: Asymptomatic or inaccessible lesions Surgical excision Radiosurgery: Progressively symptomatic but surgically inaccessible

MPGR

Developmental Venous Anomaly (DVA)

May represent anatomic variant
Seen in up to 3% of autopsies

Developmental Venous Anomaly

Isolated or associated with cavernous angioma Hemorrhage unusual
T1+Gd

Enlarged medullary veins Drain into dural sinus or deep ependymal vein Usually solitary Medusa head or palm tree

DVA Imaging: CT
Calcification & ischemia may occur in the region drained most likely due to chronic venous obstructive disease
Rare NECT CECT CECT

DVA Imaging: MRI

Surrounding T2 hyperintensity
May occur in asymptomatic Acute edema from thrombosis Gliosis from chronic outflow obstruction

DVA
T1+Gd

DVA: Treatment
NONE!
Removal may cause venous infarction
T1+Gd

Capillary Telangiectasia
Dilated capillaries interspersed within normal brain Usually small, asymptomatic incidental findings
Rare reports of hemorrhage exist

Capillary Telangiectasia
T2: Increased signal T2*: Low signal Ill defined enhancement after contrast administration: Stippled/brush stroke Occult on angiography Treatment: None T1 T1+Gd T2

Most located in brainstem Pons

Capillary Telangiectasia
T1+Gd

Sinus Pericranii
Communication between extracranial venous system & dural venous sinus Rare May be congenital or acquired
E

Sinus Pericranii
CECT

Sinus Pericranii
MRV T1+Gd

CT: Single/multiple bone defects Vascular enhancement Conventional angiogram: Seen during venous phase

Sinus Pericranii
Spontaneous regression rare Risk of hemorrhage Treatment
Surgery Endovascular
T1+Gd

Vascular Malformations
Arteriovenous malformation (AVM)
Classic Dural arteriovenous fistula (dAVF) Vein of Galen Malformation (VOG)

T1+Gd

Developmental venous anomaly (DVA) Cavernous malformation Capillary telangiectasia Cavernous Malformations

The End
Thank you!