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Research ArticleNeurointervention

Spinal Epidural Arteriovenous Fistula with Perimedullary Venous Reflux: Clinical and Neuroradiologic Features of an Underestimated Vascular Disorder

M. Mull, A. Othman, M. Dafotakis, F.-J. Hans, G.A. Schubert and F. Jablawi
American Journal of Neuroradiology October 2018, DOI: https://doi.org/10.3174/ajnr.A5854
M. Mull
aFrom the Departments of Diagnostic and Interventional Neuroradiology (M.M., A.O., F.J.)
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A. Othman
aFrom the Departments of Diagnostic and Interventional Neuroradiology (M.M., A.O., F.J.)
eDepartment of Diagnostic and Interventional Radiology (A.O.), University Hospital Tübingen, Tübingen, Germany
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M. Dafotakis
bNeurology (M.D.)
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F.-J. Hans
fDepartment of Neurosurgery (F.-J.H.), Paracelsus Kliniken, Osnabrück, Germany.
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G.A. Schubert
cNeurosurgery (G.A.S.), University Hospital Aachen, RWTH Aachen University, Aachen, Germany
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F. Jablawi
aFrom the Departments of Diagnostic and Interventional Neuroradiology (M.M., A.O., F.J.)
dDepartment of Neurosurgery (F.J.), Justus-Liebig-University, Giessen, Giessen, Germany
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  • Fig 1.
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    Fig 1.

    A, Sagittal T2- weighted images (3T; T2-TSE; slice thickness, 3 mm) reveal extensive congestive myelopathy (white arrowhead). B–D, Spinal CE-MRA (3T; time-resolved imaging with strochastic trajectories (TWIST); sagittal MIP; coronal and axial MPR) shows arterialized pouch in the lumbar ventrolateral epidural space (white arrows) in association with arterialized perimedullary veins in the thoracic region (white arrowheads) suspicious for a SEAVF in the lumbar region. E, DSA in lateral projection shows a SEAVF (white arrow) supplied via branches of the left L2 segmental artery (black arrowhead) and drained via the respective intradural radicular vein (white arrowheads). Note the extraspinal venous outlet (asterisk).

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    Fig 2.

    A–B, Sagittal T2- and contrast-enhanced T1-weighted images (3T; T2-TSE; T1-TSE; slice thickness, 3 mm) show extensive congestive thoracic myelopathy. C, Spinal CE-MRA (sagittal MIP) reveals an abnormal arterialized epidural pouch in the lumbar region (white arrow) in addition to thoracic arterialized perimedullary veins (white arrowhead). D, DSA (posteroanterior projection) exams identify the fistula in the epidural space on the vertebral level of L4 (white arrow), supplied via the right L4 segmental artery and drained by the contralateral L4 intradural radicular vein (white arrowhead). E and F, Axial and coronal MPR of DynaCT, 2 mm, 8 seconds rotation: Note the multisegmental and bilateral extension of the arterialized epidural pouch and the left sided origin of the intradural radicular drainage vein crossing the dura at the contralateral neural foramen (white arrowhead).

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    Fig 3.

    A, Spinal CE-MRA (1.5 T, sagittal MIP) reveals an extensive pathological arterialization of a ventrolateral epidural venous pouch extending over four vertebral levels (white arrow). B–C, Further reconstructions of the source MRA images (coronal and axial MPR) demonstrate precisely the epidural pouch (white arrow) and show the filling of the intradural radicular drainage vein (white arrowhead). D–E, DSA exams (posteroanterior projections) identify the multisegmental ventrolateral epidural pouch of the SEAVF (white arrow) with multiple left-sided arterial feeders supplied by the thoracic segmental arteries T 10 and T 11. Note the distant origin of the intradural radicular drainage vein (asterisk).

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    Table 1:

    Clinical presentation of patients with spinal epidural arteriovenous fistula

    Patient No.Age (yr)/SexDuration of Symptoms (mo)Symptoms at DiagnosisStatus at Discharge
    178, M6Paraparesis, sphincter dysfunctionImproved
    263, M1Paraparesis, sphincter dysfunction hypesthesia below T10Stable
    377, M1.5Paraparesis, hypesthesia L4, ataxia, sphincter dysfunctionStable
    460, F1.5Paraplegia, hypesthesia disturbances below T10Improved
    568, F24Neurogenic claudication <100 mImproved
    677, M1.5Paraplegia, hypesthesia below L1, sphincter dysfunctionStable
    764, F1Paraparesis, sphincter dysfunctionImproved
    883, M60Paraparesis, hypesthesia, sphincter dysfunctionStable
    972, M0Paraparesis, hypesthesia, sphincter dysfunctionStable
    1080, M12Paraparesis, sphincter dysfunctionStable
    1177, M2Paresis of left foot, ataxiaStable
    1278, M3Paraparesis, hypesthesiaStable
    1359, M5AtaxiaStable
    • View popup
    Table 2:

    Angiomorphologic characteristics of spinal epidural arteriovenous fistulas

    Patient No.Arterial FeederaOrigin of Intradural Radicular Drainage VeinaExtension of Epidural PouchbExtension of Arterialized Perimedullary VeinsbLocation of Arterialized Perimedullary VeinsbNo. of DSAs until Diagnosis
    1L3 RL3 LL3–L4T8–T12D = V2
    2L3 bilateral, L4 LL3 LL2–L4T3–T12D = V2
    3L1 RL1 RL1NDD < V1
    4L3 bilateralL3 RL3T9–L1D > V1
    5Left iliolumbar arteryS1S1T7–T12D > V3
    6T10 L and T11 LL2 LT 10–L3T6–L1D = V3
    7L1 LL2 LL1–L2T3–T12D = V1
    8T12 LT12 LT12 LT11D > V3
    9L3 LL3 LL3 LT6–T12D < V1
    10L4 RL4 RL2–L4T3–T10D = V1
    11L3 LS1 bilateralL3–S1T10–L1D > V1
    12L3 RL3 RL3 RT9–T12D < V1
    13L1 RL1 RL1 RT10–L1D > V1
    • Note:—D indicates dorsal to spinal cord; V, ventral to spinal cord; L, left; R, right; ND, no data.

    • ↵a DSA.

    • ↵b MRA/DSA.

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M. Mull, A. Othman, M. Dafotakis, F.-J. Hans, G.A. Schubert, F. Jablawi
Spinal Epidural Arteriovenous Fistula with Perimedullary Venous Reflux: Clinical and Neuroradiologic Features of an Underestimated Vascular Disorder
American Journal of Neuroradiology Oct 2018, DOI: 10.3174/ajnr.A5854

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Spinal Epidural Arteriovenous Fistula with Perimedullary Venous Reflux: Clinical and Neuroradiologic Features of an Underestimated Vascular Disorder
M. Mull, A. Othman, M. Dafotakis, F.-J. Hans, G.A. Schubert, F. Jablawi
American Journal of Neuroradiology Oct 2018, DOI: 10.3174/ajnr.A5854
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