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14.10.2019
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Diagnostic Approach to Intracranial AVM
Luisa BiscoitoNeuroradiology Department
University Hospital Santa Maria
Lisboa‐Portugal
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
No disclosures
Learning Objectives
• Definition
• Angioarquitecture
• Clinical Presentation
• Imaging approach• CT• MR• DSA
• How do we classify
• Treatment Options
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
• CT and TC‐Angio
• MR andMR‐Angio
• Digital Angiography
Choice of the examen depends on the availability andquality of the equipments
GOAL
Correct and Fast Diagnosis with less risk
Best Treatment Available
Brain AVM
─ Vascular malformations of the capillary bed, which is replaced by dysplasticvascular channels (nidus) connected to afferent arterial feeders anddraining veins, with shunts within. No capillary bed.
─ Considered congenital lesions or adquired lesions during early months afterbirth
─ Rare, 1/10 less frequent than brain aneurysms
─ The most common cause of intracerebral hemorrhage in young patientsassociated with significant disability and mortality
─ No gender predilection
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
TOPOGRAPHY
SIZE
NATURAL HISTORY
AGE
CLINICAL PRESENTATION
ANGIOARQUITECTUREArteriesNidusVeins
Arterial Feeders
Drainage Veins
Nidus
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Brain AVM
Location
Morello e Borghi 1973, Krayenbuhl 1972, Berry 1966, Perret e Nishioka,1966
Cortical, subcortical 76,6 – 90%
Deep 2 – 8 %
Brainstem 2,6 – 7 %
Cerebellum 5 – 9,6 %
Supratentorially
Infratentorially
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
ANGIOARCHITECTURE
Arterial Feeders
Nidus
Drainage Veins
Different Patterns
Vascular response to chronic shunts and anatomic variations
Angulations, stenosis, dilatations, aneurysms, thrombosis …
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
ARTERIAL FEEDERS
Direct (terminal arteries)
Indirect (“en Passage’’ arteries)”
Aneurysms
Flow angiodisplasia
Stenosis
Dilatations
Association with AVM 2 - 23 %
Flow-related ProximalDistalIntranidal
Unique or Multiple
Cortical arteries or perforators
Angulations
Aneurysms
No Flow-related15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
‘En passage’ Artery
Terminal Artery
Stenosis
Dilatation
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
20 years LATER
T-O AVM
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
FLOW related Aneurysm
Intranidal Aneurysms?
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
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Brain AVM
Dilatation of arterial feeder
Normal diameter after closing the shunt
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
Brain AVMNIDUS
Dysplastic vascular channels
No arteriolar or capillary vessels
Low resistance and INCREASE FLOW (shunts)
May have intranidal aneurysms
SIZE
Micro < 1 cmSmall 1-2 cmModerate 2-4 cmLarge 4-6 cmGiant > 6 cm
MORPHOLOGYCompact, plexiform
Diffuse
Modified from Yarsagil,1987
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
SIZE
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM Brain AVM
JUNCTIONAL TERRITORY
DIFFUSE
DEEP
COMPACT, PLEXIFORM
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Brain AVM
INTRANIDAL ANEURYSM
DISTAL ANEURYSM
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
Differential Diagnosis: hypervascular tumors, cavernomas, proliferative angiopathy
Hypoxico‐Ischemic birthAt 3 y F‐Up no lesion
4 years laterSeizures and focal neurologic deficit
Brain AVM
Vascular malformationDilated arteries and veinsNo individualized nidus
Brain AVMPROLIFERATIVE ANGIOPATHY
Brain AVM
TOPOGRAPHY
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
Cortical (sulcal)
Lesions that reach the CORTEX
Cortical Arteries Superficial Veins
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Brain AVM
Cortico-subcortical (gyral)
Lesions that reach the CORTEX
Cortical Arteries Superficial Veins
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
Cortico-ventricular (pyramidal)
Lesions that reach the CORTEX
Superficial Veins
Cortical Arteries
Perforators Arteries
Deep Veins
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVMLesions that reach the CORPUS CALLOSUM
Superficial Veins
Cortical Arteries
Perforators Arteries
Deep Veins
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVMDEEP Lesions
Supra and Infratentorial
Deep grey nuclei, Diencephalon, Brainstem, Deep Cerebellar Nuclei
Perforator Arteries
Deep Veins
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
CHOROIDAL Lesions (ventricular)
DEEP Lesions
Choroidal Arteries
Choroidal Veins
Deep Veins
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVMVENOUS DRAINAGE
SINGLE OR MULTIPLE
Morphological/hemodynamic changes inducedby high blood flow in the nidus
Thrombosis, Tortuosities, Stenosis, Ectasias
SUPERFICIAL , DEEP
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Brain AVM
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
• Hemorrhage 30‐82%
• Seizures 16‐53%• Cortical location and large AVM • About 58% of patients with seizures will develop epilepsy in the next 5 years
• Headaches 7‐48%
• Focal Neurologic Deficits 1‐40%• Venous Hyperpressure and ischemi
• Asymptomatic
CLINICAL PRESENTATION
Mast et al, 1995
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
Hemorrhage 30-82% (Mast e al,1995)
Overall Hemorrhagic risk 2- 4 % year
After first hemorrhageMortality 12-19%Morbidity 32-48%
After re-hemorrhageMortality 36-49%Morbidity 63-76%
Hemorrhagic Risk in Ruptured AVM 6-15,8 % 1st year(Graff e al,1983) 1,7-6,2 % in the next years
Parenchyma haematoma 63%Hematoma and SAH 32%Intraventricular 6%
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVMHemorrhage
Overall Hemorrhagic risk 2- 4 % year. Increases with previous hemorrhage
Previous Hemorrhage is the only strong predictor of risk of hemorrhage
Deep venous drainage, venous constriction, ectasia may have some influence on bleeding risk
Flow related aneurysms the risk is not quantifiable but may have influence
Size of AVM, age, pregnancy, female gender are not associated with increased risk
NATURAL HISTORY (Olivecrona)
25 % patients without treatment will die from hemorrhage
33 % patients will have high morbidity
25 % asymptomatic
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Morgan et al, Act Neur, 2017
Brain AVM
IMAGING
• CT and TC‐Angio
• MR and RM‐Angio
• Digital Angiography
Choice of the examen depends on the availability andquality of the equipments
OBJECTIVE
Correct and Fast Diagnosis with less risk
Best Treatment Available
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Brain AVMPROTOCOL
CT
MR
DSA
• NCCT • CTA (from skull base to convexity) • CECT (may be needed)
T1WI Flair DWI T2WI T2*/SWI MRA, ASL T1 3D GD
Selective Internal carotid arteries, Vertebral arteries, External Carotid arteries (if cortical lesions)
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
• Parenchyma• Hemorrhage (acute, old)• Atrophy, Calcifications, Edema, Gliosis• Location
• Vessels• Nidus (size, location, aneurysms..)• Feeding arteries• Draining Veins
IMAGING
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
• DIGITAL ANGIOGRAPHY • Still the exam with more sensivity/specificity to characterize AVM angioarchitecture. Dynamic.
• New equipments (better resolution, 3D reformat, vaso‐CT)
• Permit the endovascular treatment of AVM
• Invasive Technique (complication neurologic rate 0,07‐1,3%)
• Radiation
• Expensive, time‐consuming
• Specialized centers (INR)
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019 15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019 15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
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15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019 15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
• Acute Neurologic Symptons
• First examen most of the time – CT –• Hemorrhage• Edema • Mass effect
• Hydrocephalus• Vascular Pattern if contrast i.v.• Easily available and fast
• Disavant: allergic reaction; radiation exposure
• MR• Less frequently the first examen• More time (difficult in non collaborative
patients ). New faster sequences are better.• Sensivity for acute hemorrhage = or better
than CT (DWI, T2*; SWI)• Some contraindications (metalls, pacemaker…)
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
Time from onset Hemo T1 T2 T2* DWI CT
Hyperacute Oxy Hb iso/hypo hyper hypo Hyper(ADC ↓)
hyper
Acute (3h‐3d) Desoxy Hb iso/hypo hypo hypo hypo hyper
Subacute early4d‐8d
MetHbintracell
hyper hypo hypo hypo Hyper andafter iso
Subacute late1‐5 w
MetHbextracell
Hyper centralHypoperipher
Hyper central hypoperipher
Hyper central hypoperipher
Hyper central hypoperipher
Iso andafter hypo
Chronic HemossidIntramacrophage
hypo hypo hypo hypo hypo
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
HEMORRHAGE
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Brain AVM
CT-ANGIO (multidetectors; 4D)reformat, MIP, VR
• Fast, easy access and well tolerated
• Minimal risks
• High spacial‐resolution
• No resolution of parenchyma
• Direct and indirect signs of BAVM
• Dilated serpiginous vessels
• Strongly enhanced ovoid/round lesionwith multiple tubular/round morphology– nidus‐
• Assimetry in density (HU) of the veins –shunt‐
• Calcifications15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
• Non‐acute Neurologic SymptomsCT
• NCCT has low sensivity detecting vascular lesion (normal CT does not mean absenceof AVM)
• CECT has sensivity/specificity inferior to MR. Except the CT‐Angio
• Spontaneous slightly hyperdense tubular structures or ovoid lesion, no mass effect
• Atrophy
• Calcifications (DD with CM)
• Usually no mass effect (unless if venousdilatations)
• Exclusion of other lesions (tumors…)15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
• Non‐acute Neurologic Symptoms
• MR (++)
• Higher sensivity
• Allow better charaterization of parenchyma/vessels
• Nidus. Size. Location
• Old Hemorrhage
• Edema/gliosis
• Exclusion of other lesions (tumors…)
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
After 1 month
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Brain AVM
• 125 AVM
• CT‐Angio, MRI, TOF 1,5T , DSA
• CT‐Angio sensivity of 90% in detectionAVM compared to 89% in T2WI and74% in TOF MR
• CT‐Angio superior detectinganeurysms in arterial feeders andintranidal
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
• 4D‐CTA• Advantage: high temporal resolution.
• Disadvantage: radiation doses
• 17 MAV’s
• Conclusion: 4D‐CTA is enough for diagnosis and classification of BAVM
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
• 4D‐CTA• Advantage: high temporal resolution.
• Disadvantage: radiation doses
• 17 MAV’s
• Conclusion: 4D‐CTA is enough for diagnosis and classification of BAVM
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
MR-ANGIO (3D TOF, CE-MR angio)
• 3D TOF• High spatial resolution withoutGadolinium
• Less sensivity than DSA
• If recent hemorrhage ‘tatoo’ of the hematoma can mislead vessel analysis
• Thrombosed vessels (with hypersignal) may give false images in MR Angio
• Advantage: non invasive• Disadvantage: flow dependent inducevariations in vessel signal. Orientationof vessel. More time acquisition.
• CE MR angio• Advantage compared to TOF
• Higher sensivity to slow flow• Better signal in turbulent flow areas(less artifact)
• ‘tatoo’ of brain hematoma can beeliminated
• CE RM angio is comparable to TOF in detection of nidus
• CE RM angio is superior to TOF in detection of arterial feeders anddraining veins
Tranvinh et al, AJR, June 2017
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVMSPETZLER –MARTIN CLASSIFICATION
Surgical scale that evaluate the surgic risk (morbi/mortality)
Does not evaluate the prognostic concerning the natural history of disease
Size
< 3cm 13-6 2> 6 3
Eloquent Location
Yes 1No 0
Deep Venous Drainage
Yes 1No 0
Grau I to V
Eloquent Areassensori-motor, language, visual cortex, hypothalamus and thalamus, internal capsula brain stem, cerebelar peduncles, deep cerebelar nuclei
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15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Spetzer, Martin, J Neur, 1986
Brain AVM
TREATMENT – GOAL – eliminate the risk of hemorrhage by complete exclusion of AVM
Multimodality approach (neurointerventionists, neurosurgeons, radiosurgeons)
SURGERY
ENDOVASCULARRADIOSURGERY
If possible do just one treatment
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
3 y later
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019 15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019 15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
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15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019 15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
TREATMENT
ARUBA Trial (2014) – randomized trial of unruptured BAVM
2,3% annual rupture rate in the group assigned for observation. Worst outcome in patients treated by neurosurgery, embolization or radiosurgery
Critics:Lack of external validityWide heterogeneity of treatment modalitiesScarcity of patients treated by surgery (only 5)Very short time follow‐up…
Meling, TR, Acta Neur, 2014Proust F, Neurochirurgie, 2014
Lancet, 2014
EUROPEAN CONSENSUS CONFERENCE (EANS, ESMINT, EGKS)
‘the results of a randomized trial (ARUBA) cannot be applied equally for all unruptured BAVM and for all treatment modalities
15 th Cycle, 3rd Module – Vascular Diseases – Rovinj, 13‐17 oct, 2019
Brain AVM
Clinical Suspiction of AVM
Look for hemorrhage
NO Acute Bleeding ACUTE Bleeding
Look for life savingtreatable signs:Mass effectHydrocephalus
If patient stable
CHARACTERIZE THE AVM
CT/CTA and/or MR/MRA
Look for features than will make decision in treatment choice
NIDUSSizeLocationIntranidal an/pseudoan
FEEDING ARTERIESCortical or perforatorsDural supplyMultiple or not
DRAINAGE VEINSSup or deepMult or notStenosis, ectasiaThrombosis
PARENCHYMAOld hemorrhageEdema/gliosisAtrophy
DIFFERENTIAL DIAGNOSISOther entities
CT or MR