Spinal vascular malformations

Last revised by Francis Deng on 12 Sep 2022

Spinal vascular malformations are rare but knowledge of them is important as if undiagnosed and untreated they can lead to serious complications. The main types are arteriovenous fistulaearteriovenous malformations, cavernous malformations, and capillary telangiectasia.

Pathology

Classification

Many classifications for spinal vascular malformations have been proposed without a clear consensus nomenclature. Notably, much of the older literature described all arteriovenous shunting lesions as arteriovenous malformations (AVM), but newer classifications have distinguished arteriovenous malformations from arteriovenous fistulas (AVF) 13.

Spinal arteriovenous shunts ("malformations") have classically been categorised in a numbered system, although over time the names for each have changed and additional categories and subcategories have been proposed 9-13:

  • type I: dural AVF
  • type II: intramedullary glomus AVM
  • type III: intramedullary juvenile AVM
  • type IV: perimedullary AVF
  • type V: extradural AVF

Spetzler et al. proposed the following categories of spinal cord vascular malformations 3,5:

  • arteriovenous fistulas (AVFs)
    • intradural
      • dorsal (formerly "type I")
        • single feeder (type A)
        • multiple feeders (type B)
      • ventral (formerly "type IV")
        • small shunt
        • medium shunt
        • large shunt
    • extradural
  • arteriovenous malformations (AVMs)
    • intradural
      • intramedullary (formerly "type II")
        • compact
        • diffuse
    • extradural–intradural (formerly "type III")
    • conus medullaris
  • neoplastic vascular lesions
  • spinal aneurysms

The Bicêtre group classified spinal cord arteriovenous shunts into 3 aetiologic categories 4:

Krings et al. noted that spinal vascular malformations can be distinguished by their vascular anatomy into dural and pial arteriovenous shunting lesions and cavernous malformations, analogous to brain vascular malformations 2,6. The main types are as follows:

Patsalides et al. classified spinal vascular lesions topographically 1:

  • arteriovenous lesions
    • arteriovenous fistula (AVF) (direct shunt between artery and vein)
      • pial AVF (type IV, spinal cord AVF, ventral intradural AVF, or perimedullary AVF)
        • small
        • large
        • giant
      • dural AVF (type I, dural intradural AVF)
      • epidural AVF (type V, extradural AVF)
    • arteriovenous malformation (AVM) (nidus between artery and vein)
      • intramedullary AVM (type II, glomus-type AVM)
      • pial AVM
      • epidural AVM
      • intra- and extramedullary AVM (type III, intradural-extradural, juvenile AVM, or metameric AVM)
  • lesions without arteriovenous shunting

Several spinal vascular malformative lesions have not been featured in the above classifications:

See also

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