Vascular Malformations of the Brain: A Review of Imaging Features and Risks Comprehensive Neuroradiology: Best Practices October 27-30, 2016 Sudhakar R. Satti, MD Associate Director Neurointerventional Surgery Christiana Care Health System Newark, DE
Cerebrovascular Malformations: Developmental Venous Anomaly Cavernous Malformation Arteriovenous Malformation (AVM) Telangectasia
Developmental Venous Anomaly:
Developmental Venous Anomaly: Most common CVM: 60% of all CVM are DVA 2.5% (consecutive autopsies) Normal brain parenchyma Normal histiologic vessel wall
DVA: Location Frontal lobe: (36% - 56%) Frontal Horn lateral ventricle Cerebellum: (14% to 29%) 4 th Ventricle Parietal (12% to 24%) Occipital (4%) Temporal (2% to 19%) Basal ganglia (6%) thalamus, ventricles (11%) Brainstem (< 5%)
DVA: Presentation Usually asymptomatic Hemorrhage: Look for cavernoma Infarction: DVST Seizure: Look for cavernoma
DVA: Associations 75% solitary multiple think: blue rubber nevus syndrome ~20% (range 8-33%) associated with cavernous malformations (Mixed vascular malformation) Associated with venous head and neck malformations
DVA: CT imaging Can be seen on unenhanced Curvilinear structure draining to ventricle Ca+: associated cavernoma
DVA: MR imaging FLAIR/T2: CSF signal Post contrast T1 sequences curvilinear structure T2*/Gradient (most sensitive): associated cavernous hemangioma SWI: better detectability ; not compromised by venous flow
DVA: Catheter Angiography No AV shunting No enlarged arterial feeders Caput medusae; inverse umbrella
DVA: Clinical DO NOT TOUCH LESIONS Important to note presence prior to resection of cavernoma venous infarction Rarely thrombose: venous infarction
Cavernous Malformations
Cavernous Malformations 8-10% of CVM Prevalence = 0.1-0.5% 15% Symptomatic
Hemorrhage Cavernous Malformations: Presentation Seizures response to XRT? Focal Neurologic Deficits.25 0.5% /yr Headaches
Cavernoma: Hemorrhage 1 st bleed:.25-.75%/yr Hemorrhage recurrence 4.5%/yr Patterns Hemorrhage: 1. Slow ooze 2. Intra-lesional hemorrhage 3. Extra- lesional hemorrhage
Cavernoma: CT imaging Difficult to see on NCCT unless hemorrhage Generally do not enhance Associate punctate Ca+
Cavernoma : MR imaging (Zabramski Classification) Type I: Subacute hemorrhage T1 hyperintense (T2: hyper/hypo intense) Type II: Classic Popcorn Lesion T1 and T2 mixed intensity Low signal rim/blooming on GRE/T2* Type III: Chronic hemorrhage complete hemosiderin ring T1 hypo/iso intense and T2 hypointense Low signal rim/blooming on GRE/T2* Type IV: Multiple punctate microhemorrhages T1/T2 poorly visualized Punctate black dots: lgre/t2*
Cavernoma: Catheter Angiography Generally angiographically occult
Cavernoma: Treatment and Prognosis Usually asymptomatic conservative. Symptoms: mass effect, seizure, repeated hemorrhage Symptomatic lesions: Complete resection curative
Arteriovenous Malformations
Arteriovenous Malformations: Hemorrhage common < 20 yo Mean age diagnosis: 31 yo 4% of population; only 12% symptomatic M=F
Arteriovenous Malformations: Solitary >95% Multiple HHT (Osler-Weber-Rendu) or Wyburn-Mason syndrome Dysregulation of vascular endothelium growth factor (VEGF)
Arteriovenous Malformations: Presentation Incidental : 15% Seizures: 20% Headaches Ischemic events: Vascular steal Hemorrhage: parenchymal subarachnoid intraventricular
AVM: Hemorrhage Risk Unruptured Hemorrhage rate= 2 4%/yr Re-hemorrhage rates= 6 to 18%/yr; highest in the first 6 12 mos Fatality rate= 1-1.5%/yr (50-70% from hem)
AVM: Hemorrhage Risk Presentation with Hemorrhage Deep Drainage Nidus Size Associated Aneurysm Hypertension Age Location- Infra/Supratentorial; Borderzone; Deep Patient Risk Factors: Cocaine/Amphetamine
Cavernoma: Hemorrhage 1 st bleed:.25-.75%/yr Hemorrhage recurrence 4.5%/yr Patterns Hemorrhage: 1. Slow ooze 2. Intra-lesional hemorrhage 3. Extra- lesional hemorrhage
AVM: CT imaging Hyper/iso dense serpentine structures (i.e. enlarged, tortuous vessels) c enhancement No mass effect (if unruptured) +/- Hemorrhage Hydrocephalus Ca++
AVM: Angiography ALWAYS AV SHUNT. Necessary to evaluate : Angioarchitecture Arteries/Nidus/Veins Flow speed High (fistula), Normal (nidus) Associated. lesions (Feeding artery aneurysm, Intra-nidal aneurysm, Outflow venous stenosis)
*Best Screening AVM: MR imaging Flow Structures: (Art/Nidus/Veins/AV shunt) Hemorrhage- asah vs IPH Parenchyma- Infarct, edema, gliosis fmri surgical planning
AVM: Angiography ALWAYS AV SHUNT. Necessary to evaluate : Angioarchitecture Arteries/Nidus/Veins Flow speed High (fistula), Normal (nidus) Associated. lesions (Feeding artery aneurysm, Intra-nidal aneurysm, Outflow venous stenosis)
Martin Spetzler Surgical Grading: Size: V. Drainage: Eloquent?: <3cm=1 3-6=2 >6=3 Deep=1 Superficial=0 Yes=1 No=0 Grade Est. Deficit (major or minor) 1 or 2 0% 3 4% 4 7% 5 12-22% Σ Scores = Grade
A Randomized Trial of Unruptured Brain Arteriovenous Malformations ARUBA was a prospective, multicentre, parallel design, nonblinded, RCT 39 active clinical sites in 9 countries Medical management superior to medical management with interventional therapy for the prevention of death or stroke in patients with UNRUPTURED brain AVM 33 months follow-up High exclusion rate: 1740 patients screened 226 enrolled
Capillary Telangectasia
Capillary Telangectasia: 2 nd most common CVM Location: Pons, Cerebellum, Spinal Cord Associated with Osler-Weber-Rendu
Capillary Telangectasia: MR Imaging *Occult on CT and Angiography MRI: No mass effect T1: typically iso to low signal compared with brain parenchyma T2: slightly increased signal intensity T1 C+ (GAD): may demonstrate ill-defined focal enhancement Gradient echo (GE)/T2*: typically low signal
Capillary Telangectasia: Management DO NOT TOUCH LESIONS DIFFERENTIAL CONSIDERATIONS: Enhancing mass: (usually mass effect present) -Glioma vs MET Evolving Infarction Demyelination vs Cerebritis Vascular malformation
Thank you. Questions/Feedback ssatti@christianacare.org