Downloaded 1,277 times
Uploaded byRoshan Valentine
Vascular Malformations Of CNS Radiology
The document provides an extensive overview of vascular malformations of the central nervous system, detailing classifications, clinical presentations, imaging techniques, and treatment options. It discusses various types of malformations, including arteriovenous malformations, dural arteriovenous fistulas, vein of Galen aneurysmal malformations, developmental venous anomalies, cavernous malformations, capillary telangiectasia, and sinus pericranii. Each category is explored concerning its pathology, imaging characteristics, and management strategies.
More Related Content
Similar to Vascular Malformations Of CNS Radiology
More from Roshan Valentine
Vascular Malformations Of CNS Radiology
- 1. VASCULAR MALFORMATIONS OFCNS Dr Roshan Valentine PG Resident-Radiology St Johns Medical College Bangalore
- 2. INTRODUCTION • Heterogenous groupof disorders • Morphogenetic errors affecting arteries , veins or various combinations of vessels
- 3.
- 4.
- 5. AV MALFORMATION • MC •Usually congenital • Tightly packed thin walled vessels (NIDUS) • Direct artery to vein shunting • No intervening capillary bed • Most AVMs are parenchymal lesions – aka PIAL AVMs
- 6. AV MALFORMATION PATHOLOGY • LOCATION:supratentorial(85%) • SIZE : 2-6cms in diameter • NUMBER : solitary (<2%multiple) • Multiple when a/w syndromes HHT , Wyburn-Mason syndrome
- 7. AV MALFORMATION PATHOLOGY GROSS :Wedge shaped tangled irregulary dilated vessels with varying wall thickness and luminal size Surrounding brain parenchyma : H’age residue , gliosis,ischemic changes HPE: well defined elastic lamina(usually absent in venous channels ) with focal areas of wall thinning
- 8. AV MALFORMATION CLINICAL FEATURES •Presentation: 2-4th decade • Headache with H’age – MC • Seizures > hemorrhage(adults ) • Seizures < hemorrhage(children ) Nidus – MC site for hemorrhage Location – peri/interventricular /basal ganglia • Focal neurological deficits(20-25%) STEAL from adjacent normal area Mass effect
- 9.
- 10. AV MALFORMATION PROGNOSIS • Allare potentially hazardous • Lifelong risk of H’age- 2-4% every year cumulative • Spontaneous regression – rare and unpredictable
- 11. AV MALFORMATION IMAGING GENERAL FEATURE •3 components – Feeding arteries – Central Nidus – Draining veins
- 12. AV MALFORMATION IMAGING CT • NECT –Normal : Very small AVM – Iso/hyperdense serpentine vessels(BAG OF WORMS) – Calcification(25-30%) – AVM Bleed – parenchymal , IVH >> SAH – Post embolization: hyperdense embolics in nidus CECT • Enhanced arterial feeders , nidus and draining veins
- 13. AV MALFORMATION MR • T1W1– tightly packed mass or honeycomb of flow voids • T2W1 – serpiginous honeycomb of flow voids adjacent high signal tissue – gliosis • FLAIR- flow voids +/- surrounding high signal • GRE - blooming (H’agic residua)
- 14. AV MALFORMATION ANGIOGRAPHY • Internalangioarchitecture best depiction • Depicts 3 components of AVM – Enlarged arteries+/- aneurysm – Nidus – Early draining veins
- 15. AV MALFORMATION ANGIOGRAPHY Feeding arteries •Dilated and tortuous • Flow related angiopathy – dilatation , stenosis or thrombosis • Pedicle aneurysm(10-15%cases)
- 16. AV MALFORMATION ANGIOGRAPHY Nidus • Tightlypacked tangle of abnormal arteries and veins with no intervening capillary bed/brain parenchym • Intranidal aneurysm(50% cases)
- 17. AV MALFORMATION ANGIOGRAPHY Draining Veins •Opacify in mid-late arterial phase(Early draining vein) • Enlarged , tortuous and may form varices exerting local mass effect • Stenosis can cause AVM H’age by ↑ intranidal pressure
- 19. AV MALFORMATION TREATMENT • Completeobliteration of nidus for cure • Traditonal Rx – Surgical excision for nidus • Acute and emergent surgical intervention – in life threatening ICH
- 20. AV MALFORMATION STEREOTACTIC RADIOSURGERY •Focussed irradiation to nidus • Indication – Unresectable because of location – Size < 3.5cms • Adv : Non invasive • Disadv :Effect takes years Risk of h’age till it disappears completely
- 21. AV MALFORMATION ENDOVASCULAR RX •Adjunct to Sx/ RadioSx • Used in small AVMs or 1-2 feeding arteries • Embolisation – Precedes surgery /radiosx reduce size of nidus • Complete cure if : small AVM , few feeders , single draining vein
- 22. DURAL AV FISTULA •2nd MC CVM with AV shunting • Tiny crack like vessels that shunt blood b/w meningeal arteries and small venules within dural sinus wall • ETIOLOGY : Acquired – ↑ angiogenesis within dural sinus wall after thrombosis
- 23. DURAL AV FISTULA PATHOLOGY •LOCATION: Trans Sinus>Sig sinus>Cav sinus(adults) Sup Sigmoid sinus (Children) • SIZE : Tiny single vessel shunts to massive complex lesions with multiple feeders • NUMBER : Multiple lesions are uncommon
- 24. DURAL AV FISTULA PATHOLOGY GROSS: Multiple enlarged dural feeders on the wall of thrombosed dural sinus : Fistulas connecting feeders with arterialized draining veins HPE: irregular intimal thickening with variable loss of internal elastic lamina
- 25. DURAL AV FISTULA CLINICALFEATURE • Mostly in adults(40-60yrs) • C/F varies wht location and drainage pattern TS-SigS - Bruit and tinnitus Cav S – Pulsatile proptosis , chemsois , retroorbital pain • Lesions with cortical venous drainage(Malignant dAVF) : seizures, dementia , progressive FND
- 26. DURAL AV FISTULA CLINICALFEATURE PROGNOSIS • 98% w/o cortical venous drainage - benign course • Malignant dAVF – aggressive clinical course with H’age and ND • Multiple dAVF – poor clinical prognosis
- 27. DURAL AV FISTULA TREATMENT •Conservative – Observation +/- carotid compression technique If rsk of H’age • Endovascular – Embolisation of arterial feeders with particulate or liquid agents , coil embolization of venous sinus • Surgical resection of involved dural venous sinus • Stereotactic RadioSx- 2-3 years for obliteration
- 28. DURAL AV FISTULA-IMAGING GENERALFEATURES • Mostly in posterior fossa and skull base • MC – TS-SigS junction
- 29. DURAL AV FISTULA-IMAGING CT •Normal to striking • Enlarged dural sinus or draining vein/transosseous venous channels • Car-Cav fistula : Enlarged SOV • CECT – Enlarged feeding arteries and draining veins Dural sinus may be thrombosed or stenotic
- 30.
- 31. DURAL AV FISTULA-IMAGING MRI •Dilated cortical vein without nidus adjacent to normal appearing brain • MC finding – thrombosed dural venous sinus with flow voids • Thrombus – T1 and T2 isointense typically T2* - blooming
- 32.
- 33. DURAL AV FISTULA-IMAGING ANGIOGRAPHY •Best imaging tool • DSA with superselective catheterization of dural and transosseous feeders required • Dural branches – arise from ECA , ICA and vertebral arteries • Presence of dural sinus thrombosis, flow reversal with drainage into cortical veins and engorged tortuous pial veins
- 34. DURAL AV FISTULA-IMAGING ANGIOGRAPHY •High flow venopathy can cause stenosis . Occlusion or hemorrhage • Dysplastic venous pouches may cause H’age • Increased H’age with cortical venous drainage and dysplastic venous dilatation
- 35.
- 36.
- 37. CAROTID CAVERNOUS FISTULA •AV shunting developing within cavernous sinus • Direct – High Flow – Rupture of cavernous ICA into CS • Indirect – Slow flow , low pressure – Fistula b/w dural br of ICA and the CS
- 38. CAROTID CAVERNOUS FISTULA ETIOLOGY •Almost always acquired • Direct – Traumatic: central skull base # – Non-Traumatic: Preexisting cavernous ICA aneurysm • Indirect – Degenerative – sequelae of dural sinus thrombosis
- 39. CAROTID CAVERNOUS FISTULA PATHOLOGY •GROSS – Dilated CS (Direct CCF) – Enlarged crack like vessels(Indirect)
- 40. CAROTID CAVERNOUS FISTULA CLINICALFEATURES DIRECT INDIRECT EPIDEMIOLOGY Less common More common DEMOGRAPHICS M=F Any age Women 40-60yrs PRESENTATION Bruit Pulsatile xophthalmos Orbital edema ↓vision Glaucoma Painless proptosis Vision changes TREATMENT Fistula closure by transarterial transfistula detachable balloon embolisation Conservative Superselectiv eembolisation
- 41. CAROTID CAVERNOUS FISTULA-IMAGING GENERALFEATURES • Presence of AV shunting within cavernous sinus • Based on degree of shunt – subtle to striking
- 42. CAROTID CAVERNOUS FISTULA-IMAGING CTFINDINGS • Mild or striking proptosis • Prominent CS • Enlarged SOV • Enlarged EOM CECT • Enalrged SOV and CS
- 43. CAROTID CAVERNOUS FISTULA-IMAGING MRI •T1 – Bulging SOV and CS with flow voids • T2 – Asymmetric flow related signal loss in the affected veins
- 44. CAROTID CAVERNOUS FISTULA-IMAGING ANGIOGRAPHY DIRECTCCF • Rapid flow with early opacification of CS • Fistula may be noted in ICA segment INDIRECT CCF • Multiple dural feeders from Cavernous br of ICA and deep br of ECA(mid meningeal and dist max A) • Anastomoses b/w ICA and ECA feeders are common
- 45.
- 46. VEIN OF GALENANEURYSMAL MALFORMATION • Direct AV fistula b/w deep choroidal arteries and persistent embryonic precursor of VOG • Large midline venous pouch behind the 3rd ventricle • MC extracardiac cause of high-output cardiac failure in newborn
- 47. VEIN OF GALENANEURYSMAL MALFORMATION • In normal fetal dvpt : arterial supply of choroid plexus drains via single transient midline vein – median prosencephalic vein • Internal cerebral vein drains fetal chorid plexus as MPV regresses • Persistent high flow fistula prevents regression
- 48. VEIN OF GALENANEURYSMAL MALFORMATION CLINICAL FEATURES • >30% of symptomatic VM in children • Rare in adults • Neonates – high output CCF with cranial bruit • Older infants – macrocrania + hydrocephalus +/- CCF • Older Children – Developmental delay and seizures • Young adults - Headache • Large VGAMS – cerebral ischemia and dystrophic changes • Left untreated – Die of progressive brain damage and intracatable CCF
- 49. VEIN OF GALENANEURYSMAL MALFORMATION TREATMENT • Goal – not anatomic cure of VGAM but control malformation to allow normal brain dvpt • Staged arterial embolization at 4/5m
- 50. VGAM – IMAGING CT •NECT – Enlarge dwell delineated hyperdense mass at tentorial apex – Obstructive hydrocephalus – H’age and calcification may be present. • CECT – Strong uniform enhancement
- 51. VGAM – IMAGING MRI •Enlarged serpentine arterial feeders adjacent to the lesion
- 52. VGAM – IMAGING ANGIOGRAPHY 2forms based on angioarchitecture • Choroidal – Multiple br from pericallosal choroidal and thalamoperforate arteries drain into dilated midline venous sac • Mural – single or few enlarged collicular or post choridal arteries drain into sinus wall • Venous drainage into persistent embryonic FALCINE SINUS
- 53.
- 54. VGAM – IMAGING ULTRASOUND •Antenatally • Hypoechoic to mildle echogenic midline mass behinf the third ventricle • CDFI : Bidirectional turbulent flow
- 55. CVMS WITHOUT AVSHUNTING
- 56. DEVELOPMENTAL VENOUS ANOMALY •VENOUS ANGIOMA/VENOUS MALFORMATION • Umbrella shaped CVM with mature venous part. • No arterial component • May represent anatomic variant of otherwise normal venous drainage
- 57. DEVELOPMENTAL VENOUS ANOMALY CLINICALFEATURES • Usually asymptomatic • Headache/seizures • H’age with FND ( if a/w cavernous malformation) • MC vascular malformation at autopsy
- 58. DEVELOPMENTAL VENOUS ANOMALY TREATMENT •Solitary DVA – no Rx • Histologically mixed – based on coexisting lesion
- 59. DEVELOPMENTAL VENOUS ANOMALY IMAGING •Location : MC near the frontal horn of ventricle • Size < 3cm • Usually solitary
- 60. DEVELOPMENTAL VENOUS ANOMALY NECT •Normal , enlarged draining vein may appear hyperdense CECT • Numerous linear or punctate enhancing foci and converge on single enlarged tubular draining vein
- 61.
- 62. DEVELOPMENTAL VENOUS ANOMALY MR •T1 – Normal if DVA is small H’age if mixed malformations • T1 C+ - stellate collection of linear enhancement structures joining subependymal collector vein. • GRE – if H;age in coexisting cavernous malformation - Occasionally hypo – Not H’age but deoxyHb within venous blood
- 63.
- 64. DEVELOPMENTAL VENOUS ANOMALY •MRA – Arterial phase usually normal • MRV – MEDUSA HEAD drainage pattern
- 65. DEVELOPMENTAL VENOUS ANOMALY ANGIOGRAPHIC FINDINGS •Normal arterial and capillary phase • Venous phase – Medusa head appearance
- 66. CAVERNOUS MALFORMATION • CAVERNOUS ANGIOMA/CAVERNOMA •Characterised by intralesional hemorrhages into thin walled angiogenically immature blood filled locules called CAVERNS • Well marginated lesion with no normal brain parrenchyma • Inherited or acquired
- 67. CAVERNOUS MALFORMATION PATHOLOGY • Location: Mostly infratentorial (PONS) • Dark blue well circumscribed lobulated lesion with mulberry like config • CCMs do not contain brain parenchyma • Adjacent parenchyma: reactive changes , hemosiderin deposition • Dystrophic calcification+ • HPE – collagenised sinusoids with NO MUSCULARIS/ELASTICA(avm has it )
- 68.
- 69. CAVERNOUS MALFORMATION CLINICAL FEATURES •2/3 are soliary • Peak presentn : 40-60yrs • MC presentn : Seizures(50%)>Headache and FND • H’age risk – 0.25-0.75% per year
- 70. CAVERNOUS MALFORMATION TREATMENT OPTIONS •Total surgical removal via microsurgical resexn – for symptomatic lesions with recurrent H’age • Stereotactic Radiosx = for inaccessible lesions
- 71. CAVERNOUS MALFORMATION IMAGING CT –Hyperdense lesion with scatteres intralesional calcification
- 72. CAVERNOUS MALFORMATION-IMAGING MRI MR –DIAGNOSTIC Focal central heterogeneity(varying hemorrhage within caverns)- POPCORN appearance on T2WI Circumferential hypointense ring of hemosiderin form around high intense central areas
- 73. CAVERNOUS MALFORMATION-IMAGING ANGIOGRAPHY • Noidentifiable feeding arteries/veins • Negative unless mixed with other lesions
- 74. CAPILLARY TELANGIECTASIA • CAPILLARYANGIOMA • Collection of enlarged thin walled vessels resembling capillaries. • Vessels surrounded by normal brain parenchyma • Probably congenital lesions • MC sites : Pons , cerebellum(can occur anywhere)
- 75. CAPILLARY TELANGIECTASIA CLINICAL FEATURES •Peak Presentation : 30-40 years • Usually silent, discovered incicdentally at imaging
- 76. CAPILLARY TELANGIECTASIA IMAGING • Usually<1cm CT – Usually Normal MR • T1W1 –usually normal • T2 – 50% normal - 50 % show stippled foci of hyperintensity • T2* - Best sequence for demonstrating the lesion(poorly delineated greyish hypointensity) • T1+C – BRUSH LIKE
- 77.
- 78.
- 79. SINUS PERICRANII • Largetranscalvarial communication between intra and extra-cranial venous drainage system • Mostly congenital • May be a/w other dva
- 80. SINUS PERICRANII CLINICAL FEATURES •Rare • Children and young adults • NON TENDER NON PULSATILE BLUISH COMORESSIBLE SCALP MASS • Increase in size with Valsalva • Reduce on upright position
- 81. SINUS PERICRANII TREATMENT • Surgicalremoval of extracranial component- cosmetic purpose
- 82. SINUS PERICRANII- IMAGING CT NECT •Iso to hyperdense • Typically well demarcated calvarial defect CECT • Strong uniform enhancement
- 83. SINUS PERICRANII-IMAGING MRI • T1WI– iso • T2WI- Hyperintense ANGIOGRAM • Arterial and capillary phase normal • Venous phase – Visualised on late venous phase mostly • Contrast accumulating within the lesion and adjacent to the skull defect with pericranial scalp vein
- 84. ENDOVASCULAR MANAGEMENT ofCVM EMBOLIC AGENTS USED Liquid agents Acrylic glue • Chemically N-butyl cyanoacrylate • Polymerises on contact with hydroxyl ions in blood • Induces vasculitis and thereby thrombosis of AVM
- 85. ENDOVASCULAR MANAGEMENT ofCVM ONYX • Ethylene vinyl alcohol polymer • Mixed with dimethyl sulfoxide (DMSO) • On mixing it forms a soft spongy polymer • Its more effective than acrylic glue
- 86. ENDOVASCULAR MANAGEMENT ofCVM PARTICULATE AGENTS SILICONE SPHERES • First agents ever used • Mixed with Ba to make it radiopaque • Increased rebleeding rate
- 87. ENDOVASCULAR MANAGEMENT ofCVM BALLOONS • Silicone or latex made • Occlude large AVM feeding arteries/high flow fistula • Replaced by coils POLY VINYL ALCOHOL • Delivered via guide wire dependent catheters(~500mics) • Flow dependent catheters (<350mics) • Clumping of particles causing slowing and then thrombosis
- 88. ENDOVASCULAR MANAGEMENT ofCVM MICROCOILS • Used along with embolic agents • They reduce the flow in rapid av shunts • Also in treatment of intracranial aneurysm a/w AVMs
Editor's Notes
- #6 Nidus – high flow low resistance vascular bed rerplacing normal arterioles
- #8 Thin wall cause hemorrhage Apex – ventricle , base near cortex
- #10 Eloquent area – area with identifiable neuro funxn Grade 4 n above – unresectable and untreatbale
- #13 Left) NECT shows serpentine hyperdensities . (Right) CECT shows strong uniform enhancement . Wedge-shaped configuration is typical for AVM. Roughly 85% of AVMs are supratentorial.
- #23 Local hypoeperfusion in thrombosed dural venous sinus – increased intrasinsu pressure thrombosed transverse sinus with multiple tiny arteriovenous in the dural wall(thickarrow) . Lesion is mostly supplied by transosseous feeders(curved arrow) from the external carotid artery.
- #26 FND – focal neurological deficits
- #27 FND – focal neurological deficits Benign course – h’age is are
- #30 Sov – superior ophthalmic vein
- #31 CTA source ,right-sided tinnitus shows no obvious abnormality, although the right sigmoid sinus looks peculiar. 7-11B. Bone CT in the same patient shows multiple enlarged transosseous vascular channels
- #33 Contrast-enhanced MRA dural sinus thrombosis , multiple enhancing vascular channels 7-11D. MRA innumerable tiny feeding arteries(bold arrow) supplying a dAVF at the transverse-sigmoid sinus junction. The sinus has partially recanalized , and the distal sigmoid sinus and jugular bulb are partially opacified.(curved arrow)
- #36 Stratify risk of dAVf rupture
- #37 DSA of the external carotid artery in a patient with tinnitus, dAVF in the occluded transverse sinus(bold arrow) supplied by the middle meningeal artery (curved arrow), transosseous branches(straight) from the ECA.
- #38 The right cavernous sinus is enlarged by numerous dilated arterial and venous channels.
- #39 Skull bas e# - stretch injury of ica /puncture from bony segment
- #43 CECT The right cavernous sinus is enlarged , and the ipsilateral superior ophthalmic vein is more than 4 times the size of the left superior ophthalmic vein .
- #44 T2WI enlarged right cavernous sinus containing numerous abnormal “flow voids”
- #46 Narrowed ICA before terminating in a large venous pouch.Venous reflux into SOV and IOV is present
- #47 Enlarged choroidal veins(staright arrow) draining directly to dilated MPV and to torcu via falcine culcus. Torcular heterophili(venous sinus confluence) enlarged.
- #51 CECT scan massive VGAM(bold) draining into an enlarged falcine sinus (staright), causing obstructive hydrocephalus.
- #52 Sagittal T2WI shows prominent arteries supplying an enlarged median prosencephalic vein . Note enlarged falcine sinus(curved ) .
- #54 DSA in the same patient shows that the VGAM(bold arrow) is supplied by multiple direct arterial fistulas .
- #55 Neonatal transcranial US shows a large VGAM posterior to the 3rd ventricle. Prominent vessels with arterial flow(staright) supply the lesion.
- #57 enlarged medullary veins(BOLD) draining into a single transmantle collector vein .
- #62 CECT , CTA classic DVA in left cerebellar hemisphere .
- #64 classic DVA with enlarged WM veins(bold) and a collector vein draining into the anterior aspect of the superior sagittal sinus.
- #67 Subacute(curved) , classic “popcorn ball” appearances of CCMs. Microhemorrhages are seen as multifocal “blooming black dots” .
- #69 Based on imaging
- #70 Irritative effect of hemosiderin , gliosis , compression on cortex
- #71 Irritative effect of hemosiderin , gliosis , compression on cortex
- #73 T2WI shows classic “popcorn ball” appearance with locules of blood in different stages of evolution surrounded by hemosiderin rim
- #75 Graphic depicts pontine capillary telangiectasia with tiny dilated capillaries interspersed with normal brain
- #78 FLAIR scan shows faint patchy hyperintensity in the pons. 7-47D. T2* GRE scan shows susceptibility effect with grayish hypointensity in the mid pons.
- #79 T1 C+ scan shows the brush-like faint enhancement
- #80 Coronal graphic depicts a classic sinus pericranii (SP BOLD ARROW) with an expanded venous pouch under the scalp connecting to the intracranial venous system through a transcalvarial channel. a/w wd DVA
- #83 CT venogram – SP communicating with sup sagittal sinus
- #84 Late venous phase DSA shows angiographic findings of sinus pericranii with enlarged venous pouches connecting directly to the superior sagittal sinus through a transcalvarial channel