Vascular Malformations Of CNS Radiology
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Vascular malformations of cns including highand low flow shunts - AVM , dural av fistula , vein of galen malformation , venous malformation , sinus pericranii , cavernous malformation , capillary telangiectasia
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Vascular Malformations Of CNS Radiology
- 1. VASCULAR MALFORMATIONS OF CNS Dr Roshan Valentine PG Resident-Radiology St Johns Medical College Bangalore
- 2. INTRODUCTION • Heterogenous group of disorders • Morphogenetic errors affecting arteries , veins or various combinations of vessels
- 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
- 10. AV MALFORMATION PROGNOSIS • All are 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 • Internal angioarchitecture 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 • Tightly packed 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 • Complete obliteration 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 CLINICAL FEATURE • 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 CLINICAL FEATURE 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 GENERAL FEATURES • 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
- 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
- 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. DURAL AV FISTULA
- 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 CLINICAL FEATURES 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 GENERAL FEATURES • Presence of AV shunting within cavernous sinus • Based on degree of shunt – subtle to striking
- 42. CAROTID CAVERNOUS FISTULA-IMAGING CT FINDINGS • 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 DIRECT CCF • 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
- 46. VEIN OF GALEN ANEURYSMAL 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 GALEN ANEURYSMAL 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 GALEN ANEURYSMAL 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 GALEN ANEURYSMAL 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 2 forms 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. VGAM – IMAGING
- 54. VGAM – IMAGING ULTRASOUND • Antenatally • Hypoechoic to mildle echogenic midline mass behinf the third ventricle • CDFI : Bidirectional turbulent flow
- 55. CVMS WITHOUT AV SHUNTING
- 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 CLINICAL FEATURES • 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
- 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
- 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 )
- 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 • No identifiable feeding arteries/veins • Negative unless mixed with other lesions
- 74. CAPILLARY TELANGIECTASIA • CAPILLARY ANGIOMA • 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
- 79. SINUS PERICRANII • Large transcalvarial 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 • Surgical removal 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 of CVM 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 of CVM 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 of CVM PARTICULATE AGENTS SILICONE SPHERES • First agents ever used • Mixed with Ba to make it radiopaque • Increased rebleeding rate
- 87. ENDOVASCULAR MANAGEMENT of CVM 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 of CVM MICROCOILS • Used along with embolic agents • They reduce the flow in rapid av shunts • Also in treatment of intracranial aneurysm a/w AVMs
Hinweis der Redaktion
- Nidus – high flow low resistance vascular bed rerplacing normal arterioles
- Thin wall cause hemorrhage Apex – ventricle , base near cortex
- Eloquent area – area with identifiable neuro funxn Grade 4 n above – unresectable and untreatbale
- Left) NECT shows serpentine hyperdensities . (Right) CECT shows strong uniform enhancement . Wedge-shaped configuration is typical for AVM. Roughly 85% of AVMs are supratentorial.
- 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.
- FND – focal neurological deficits
- FND – focal neurological deficits Benign course – h’age is are
- Sov – superior ophthalmic vein
- 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
- 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)
- Stratify risk of dAVf rupture
- 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.
- The right cavernous sinus is enlarged by numerous dilated arterial and venous channels.
- Skull bas e# - stretch injury of ica /puncture from bony segment
- 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 .
- T2WI enlarged right cavernous sinus containing numerous abnormal “flow voids”
- Narrowed ICA before terminating in a large venous pouch.Venous reflux into SOV and IOV is present
- Enlarged choroidal veins(staright arrow) draining directly to dilated MPV and to torcu via falcine culcus. Torcular heterophili(venous sinus confluence) enlarged.
- CECT scan massive VGAM(bold) draining into an enlarged falcine sinus (staright), causing obstructive hydrocephalus.
- Sagittal T2WI shows prominent arteries supplying an enlarged median prosencephalic vein . Note enlarged falcine sinus(curved ) .
- DSA in the same patient shows that the VGAM(bold arrow) is supplied by multiple direct arterial fistulas .
- Neonatal transcranial US shows a large VGAM posterior to the 3rd ventricle. Prominent vessels with arterial flow(staright) supply the lesion.
- enlarged medullary veins(BOLD) draining into a single transmantle collector vein .
- CECT , CTA classic DVA in left cerebellar hemisphere .
- classic DVA with enlarged WM veins(bold) and a collector vein draining into the anterior aspect of the superior sagittal sinus.
- Subacute(curved) , classic “popcorn ball” appearances of CCMs. Microhemorrhages are seen as multifocal “blooming black dots” .
- Based on imaging
- Irritative effect of hemosiderin , gliosis , compression on cortex
- Irritative effect of hemosiderin , gliosis , compression on cortex
- T2WI shows classic “popcorn ball” appearance with locules of blood in different stages of evolution surrounded by hemosiderin rim
- Graphic depicts pontine capillary telangiectasia with tiny dilated capillaries interspersed with normal brain
- FLAIR scan shows faint patchy hyperintensity in the pons. 7-47D. T2* GRE scan shows susceptibility effect with grayish hypointensity in the mid pons.
- T1 C+ scan shows the brush-like faint enhancement
- 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
- CT venogram – SP communicating with sup sagittal sinus
- 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