0914

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0914

  1. 1. Intracranial Vascular Malformations
  2. 2. Classification of intracranial vascular malformations <ul><li>AVMs (AVFs) </li></ul><ul><li>Capillary telangiectasias </li></ul><ul><li>Cavernous angiomas </li></ul><ul><li>Venous malformations -- Venous angioma -- Vein of Galen malformations -- Venous varix </li></ul>
  3. 4. <ul><li>Enlarged arterial feeders </li></ul><ul><li>Nidus </li></ul><ul><li>Dilated draining vein </li></ul>Parenchyma AVM <ul><li>Enlarged arterial feeders - high flow angiopathy with focal stenosis - flow related aneurysm </li></ul><ul><li>Nidus - tightly packed mass of abnormal vascular channels - no intervening capillary bed - no intervening normal brain paenchyma - intranidal aneurysms </li></ul><ul><li>Dilated draining vein - may form varix - vasculopathy </li></ul>
  4. 5. Pathology <ul><li>Absent or only gliotic brain in nidus. </li></ul><ul><li>Adjacent brain. </li></ul><ul><ul><li>Hemorrhagic residua. </li></ul></ul><ul><ul><li>Dystrophic calcification. </li></ul></ul><ul><ul><li>Atrophy, ischemic changes caused by vascular steal phenomenon . </li></ul></ul>
  5. 6. Location <ul><li>Cerebral hemisphere (85%) posterior fossa (15%) </li></ul><ul><li>Superficial (convexity) and deep (2:1 to 3:1) </li></ul>
  6. 7. Incidence <ul><li>About 0.02% - 0.14% in general population. </li></ul><ul><li>Solitary (98%) </li></ul><ul><li>Multiple (2%) </li></ul><ul><ul><li>Osler-Weber-Rendu disease </li></ul></ul><ul><ul><li>Wyburn-Mason syndrome </li></ul></ul>
  7. 8. Osler-Weber-Rendu Disease ( H ereditary H emorrhagic T elangiectasia ) <ul><li>AD neurocutaneous syndrome. </li></ul><ul><li>Epistaxis in 85% of patients. </li></ul><ul><li>Multiple mucocutaneous and visceral vascular abnormalities including: (1) capillary telangiectasia (skin and mucosa). (2) AVM or AVF in liver, lungs, brain and spine. </li></ul>
  8. 9. 15-year-old woman with a history of recurrent epistaxis, and a family history of HHT. She was also subsequently diagnosed with a pulmonary AVM. AVF left frontal lobe cortex right parietal cortex
  9. 10. Wyburn-Mason Syndrome (Unilateral Retinocephalic Vascular Malformations) <ul><li>Cutaneous vascular nevi. </li></ul><ul><li>Retinal and optic nerve vascular malformations. </li></ul><ul><li>Ipsilateral cerebral AVM involving the visual pathways and midbrain. </li></ul><ul><li>Typically unilateral. </li></ul>
  10. 11. Basal ganglionic and midbrain AVMs Retinal AVM
  11. 12. Clinical Presentation of AVM <ul><li>Peak age at initial presentation: 20 - 40 y/o. </li></ul><ul><li>AVMs are the most common symptomatic CVM. </li></ul><ul><li>Clinical presentation: </li></ul><ul><ul><li>Hemorrhage (50%). </li></ul></ul><ul><ul><li>Seizure (25%). </li></ul></ul><ul><ul><li>Miscellaneous (25%). </li></ul></ul>
  12. 13. Hemorrhagic Risk <ul><li>50% with 2% to 3% per year, cumulative. </li></ul><ul><li>Increased risk of hemorrhage: </li></ul><ul><ul><li>Periventricular or intraventricular location. </li></ul></ul><ul><ul><li>Associated aneurysm. </li></ul></ul><ul><ul><li>Central or deep venous drainage. </li></ul></ul><ul><ul><li>Stenosis or occlusion of draining vein. </li></ul></ul>
  13. 14. Computed Tomography <ul><li>Iso- to slightly hyperdense on NECT. </li></ul><ul><li>Calcification in 30%. </li></ul><ul><li>Strong, uniform enhancement of serpentine feeding and draining vessels, nidus. </li></ul><ul><li>Secondary changes. </li></ul><ul><ul><li>Hematoma, mass effect. </li></ul></ul><ul><ul><li>Atrophy. </li></ul></ul>
  14. 15. 40 year old female with a long history of seizures that are increasing in frequency, despite adequate anti-convulsant therapy
  15. 16. Magnetic Resonance Imaging <ul><li>Signal varies depending on : </li></ul><ul><ul><li>Pulse sequence. </li></ul></ul><ul><ul><li>Flow rate. </li></ul></ul><ul><ul><li>Presence and age of hemorrhage. </li></ul></ul><ul><li>Typical uncomplicated AVM. </li></ul><ul><ul><li>“ Honeycomb” of “flow voids”. </li></ul></ul><ul><ul><li>Little or no mass effect. </li></ul></ul><ul><ul><li>No normal brain inside AVM. </li></ul></ul><ul><ul><li>Adjacent gliosis, atrophy. </li></ul></ul>
  16. 17. 50 year old female with a lifelong history of severe headaches. Now with intermittent but severe trigeminal neuralgia
  17. 18. Capillary Telangiectasia
  18. 19. Capillary Telangiectasia <ul><li>Nests or racemose collections of dilated capillaries with abnormal walls (lack smooth muscle, elastic fibers). </li></ul><ul><li>Contain normal brain. </li></ul><ul><li>May contain blood storage products from previous hemorrhage. </li></ul>
  19. 20. Location <ul><li>Anywhere in brain, spinal cord. </li></ul><ul><li>Pons, medulla, spinal cord are favored sites. </li></ul>
  20. 21. Incidence <ul><li>Second most common intracranial vascular malformation identified at autopsy (venous angioma are the most common type). </li></ul><ul><li>Multiple lesions are the rule. </li></ul>
  21. 22. Clinical Presentation <ul><li>Most are clinically silent. </li></ul><ul><li>May hemorrhage, especially if associated with cavernous angioma. </li></ul>
  22. 23. Imaging <ul><li>Cerebral angiography is usually normal. (angiographically occult vascular malformation) </li></ul><ul><li>NECT usually normal; CECT may show faint areas of high density. </li></ul>
  23. 24. Imaging <ul><li>MR. </li></ul><ul><li>Multiple hypointense foci on T2WI and gradient refocused scans. </li></ul><ul><li>Poorly delineated foci of increased signal on contrast-enhanced studies. </li></ul>
  24. 27. Cavernous Angioma
  25. 28. Cavernous Angioma <ul><li>Numerous large endothelial-lined sinusoidal spaces. </li></ul><ul><li>Fibrous septae separate vascular spaces. </li></ul><ul><li>Hemorrhage in different stages of evolution. </li></ul><ul><li>Calcification common. </li></ul><ul><li>No normal brain within lesion. </li></ul><ul><li>Adjacent brain surrounding lesion is often hemosiderin-stained, gliotic. </li></ul>
  26. 29. Inheritance <ul><li>Sporadic form. </li></ul><ul><ul><li>Characterized by isolated lesions. </li></ul></ul><ul><ul><li>10% to 15% multiple. </li></ul></ul><ul><li>Familial form. </li></ul><ul><ul><li>Characterized by multiple lesions (75%). </li></ul></ul><ul><ul><li>AD, variable penetrance. </li></ul></ul>
  27. 30. <ul><li>Location. </li></ul><ul><ul><li>80% supratentorial , with the frontal and temporal lobes the most frequent sites. </li></ul></ul><ul><ul><li>Approximately 50~80% of all cases are multiple. </li></ul></ul><ul><li>Age at presentation. </li></ul><ul><ul><li>Between 20 to 40 years without gender predilection. </li></ul></ul><ul><li>Symptoms. </li></ul><ul><ul><li>Seizure, focal neurologic deficit, headache. </li></ul></ul>
  28. 31. Computed Tomography <ul><li>Iso- or slightly hyperdense on NECT. </li></ul><ul><li>Calcification common. </li></ul><ul><li>Variable enhancement. </li></ul>
  29. 32. Magnetic Resonance Imaging <ul><li>Reticulated “popcorn-like” lesion with mixed-signal core, low-signal rim. </li></ul><ul><li>Gradient-refocused scans may detect multiple lesions. </li></ul>
  30. 33. T2WI PDI T1WI T1WI+C
  31. 35. Associated Abnormalities <ul><li>Most common vascular malformation coexisting with other vascular malformations. </li></ul><ul><ul><li>Venous angioma. </li></ul></ul><ul><ul><li>Capillary telangiectasia. </li></ul></ul><ul><ul><li>AVM. </li></ul></ul>
  32. 36. Venous Angioma
  33. 37. Venous Angioma (developmental venous anomaly, DVA) <ul><li>Dilated medullary (white matter) veins form “ caput medusae.” </li></ul><ul><li>Enlarged transcortical draining “collector vein” </li></ul><ul><li>Normal brain is present between the enlarged tributary veins. </li></ul>
  34. 38. Incidence <ul><li>Most common brain vascular malformation. </li></ul><ul><li>Usually solitary (exception: blue rubber bleb nevus syndrome) </li></ul><ul><li>Blue rubber bleb nevus syndrome: </li></ul><ul><ul><li>Bluish discolored skin and mucocutaneous lesions. </li></ul></ul><ul><ul><li>Multiple intracranial venous angiomas . </li></ul></ul><ul><ul><li>Sinus pericranii. </li></ul></ul><ul><ul><ul><li>Abnormally large communication between intracranial and extracranial venous circulations may be congenital or acquired. </li></ul></ul></ul>
  35. 39. Location <ul><li>Supratentorial 65%. </li></ul><ul><ul><li>Frontal lobe is most common site. </li></ul></ul><ul><ul><li>Typically near frontal horn of lateral ventricle. </li></ul></ul><ul><li>Infratentorial 35%. </li></ul><ul><ul><li>Cerebellar hemispheres. </li></ul></ul><ul><ul><li>Typically near 4 th ventricle. </li></ul></ul>
  36. 40. Clinical Presentation <ul><li>Most are asymptomatic (60%). Symptoms are often caused by coexisting cavernous angioma. </li></ul><ul><li>Symptoms. </li></ul><ul><ul><li>Headache. </li></ul></ul><ul><ul><li>Seizure. </li></ul></ul><ul><ul><li>Hemorrhage, focal neurologic deficit. </li></ul></ul>
  37. 41. Computed Tomography <ul><li>NECT scans usually normal. </li></ul><ul><li>CECT scans: </li></ul><ul><ul><li>Enhancing tuft near angle of ventricle. </li></ul></ul><ul><ul><li>Dilated collector vein. </li></ul></ul>
  38. 42. Magnetic Resonance Imaging <ul><li>T1-, T2WI may be normal. </li></ul><ul><li>Postcontrast studies show radially arranged dilated tributaries, enlarged collector vein. </li></ul><ul><li>Hemorrhage suggests associated cavernous angioma or venous restrictive disease. </li></ul>
  39. 44. 46-year-old female who presented with a first seizure.
  40. 45. Vein of Galen Malformation
  41. 46. Vein of Galen Malformation <ul><li>Not a real aneurysm, rather this rare lesion reflects arteriovenous fistulae draining into the Galenic system </li></ul><ul><li>Causes: pial AVM with deep venous drainage or direct AV fistula or both </li></ul><ul><li>Clinically: </li></ul><ul><ul><li>Neonate with intratable high output failure </li></ul></ul><ul><ul><li>Infant with hydrocephalus and seizure </li></ul></ul><ul><ul><li>Older children/adults with SAH </li></ul></ul>
  42. 48. CT or MRI <ul><li>Round mass posterior to 3 rd ventricle. </li></ul><ul><li>Strong, uniform enhancement (unless thrombosed). </li></ul><ul><li>Hydrocephalus is common. </li></ul>
  43. 49. The patient is a 3-day-old male with intractable congestive heart failure. Cardiac catheterization revealed no structural abnormalities
  44. 51. Summary <ul><li>CVMs without symptom: </li></ul><ul><ul><li>Venous angioma. </li></ul></ul><ul><ul><li>Capillary telangiectasia. </li></ul></ul><ul><li>Most common CVM: venous angioma. </li></ul><ul><li>Most common symptomatic CVM: AVM </li></ul>
  45. 53. Cerebral Venous Thrombosis <ul><li>Risk factors </li></ul><ul><ul><li>Infections: sinusitis, meningitis, abscess </li></ul></ul><ul><ul><li>Trauma: head trauma, neurosurgery </li></ul></ul><ul><ul><li>Medial conditions: dehydration, pregnancy, coagulopathy, polycythemia, sickle cell anemia, malignancies, CVD(SLE, Wegner’s) </li></ul></ul><ul><ul><li>Medicine: OCP, HRT, steroids </li></ul></ul>
  46. 54. Clinical Presentation <ul><li>Symptoms: headache, double vision, blurred vision, altered consciousness, nausea, vomiting, seizures </li></ul><ul><li>Signs: pailledema, focal neurologic deficit, cranial nerve palsies, nystagmus </li></ul>
  47. 55. Cerebral Venous Thrombosis <ul><li>Cord Sign: direct visualization of thromubs, elongated, high-attenuation lesion on unenhanced CT in a dural sinus or cortical V </li></ul><ul><li>Empty Delta Sign: if the thrombus is located in the superior sagittal sinus, a triangular filling defect can be indentified on post contrast images </li></ul>
  48. 56. MRI <ul><li>Acute CVT may be difficult to see because clot is isodense to brain on T1W and hypointense on T2W </li></ul><ul><li>3-7 days, clot becomes hyperintense on T1W </li></ul><ul><li>Cortical & subcortical in a nonarterial territory hyperintensity on FLAIR </li></ul><ul><li>Restriction on DWI </li></ul>
  49. 57. <ul><li>24 year old female is 10 days postpartum and presents with a 2 day history of increasingly severe headache, meningismus and diplopia. </li></ul>
  50. 59. 28 year old female is 6 weeks pregnant and complains of headache, left-sided sensory disturbance and blurred vision.
  51. 60. Thanks for Your Attention !

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