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0914
 

0914

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    0914 0914 Presentation Transcript

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