INTRACEREBRAL HEMORRHAGE / VASCULAR MALFORMATIONS

Dural arteriovenous fistula (DAVF)

Created 21/04/2021, last revision 03/06/2023

  • dural arteriovenous fistula (DAVF) is characterized by abnormal connections (shunts) between an artery and a vein; DAVF usually presents with tinnitus, hemorrhage, or venous hypertension
  • DAVF accounts for ∼ 10-15% of vascular malformations; it most commonly affects patients aged 40-60 years
  • most DAVFs are idiopathic; some patients have a history of previous craniotomy, head trauma, or dural sinus thrombosis  [Gandhi, 2012]

Pathology

Classification

Type I – anterograde drainage directly into dural venous sinuses/meningeal veins
Ia – 1 feeding artery
Ib – >1 feeding arteries
Type II – anterograde drainage into dural sinuses/meningeal veins + retrograde drainage into subarachnoid veins
Type III – predominantly retrograde drainage into cortical veins with their dilatation, no dural sinus or meningeal venous drainage
Borden classification of DAVF
Cognard [Cognard, 1995]
This classification provides valuable data for the determination of the risk with each dural AV fistula and enables decision-making about the appropriate therapy
Type I – only anterograde drain into dural sinuses, benign course
Type II 

  • IIa – simultaneous retrograde drainage into the dural sinus  (intracranial hypertension in 20% of cases)
  • IIb – simultaneous retrograde drainage into cortical veins  (bleeding in 10%)
  • IIa+b – simultaneous retrograde drainage into dural sinus + cortical veins
Type III, IV, V – no dural sinus drainage (high risk of bleeding – 40-65%)

  • III – direct cortical venous drainage without venous ectasia
  • IV – direct cortical venous drainage with venous ectasia
  • V – spinal venous drainage (⇒ high risk of progressive myelopathy)
Cognard classification of DAVFs

Clinical presentation

Clinical presentation is variable and depends on the fistula location and venous drainage

 

  • pulsatile tinnitus (typically with sigmoid and transverse sinus drainage)
  • symptoms of venous hypertension/congestion
    • headache
    • hemorrhagic venous infarction
    • spinal myelomalacia
    • cranial nerve palsies
    • ocular (orbital) symptoms – conjunctival chemosis and swelling (carotid-cavernous fistula) – DDx of cavernous sinus thrombosis!
    • intracerebral or subarachnoid hemorrhage (high risk, especially in type II and III) [Li, 2015]
  • DAVF can be high-flow and consume a significant portion of EF => exertional dyspnea, left ventricular hypertrophy (LVH)

Diagnostic evaluation

  • CT/MRI
    • more useful for detecting complications (hemorrhage, venous infarction, edema)
    • insufficient for the DAVF diagnosis – vascular imaging must be added (however, enlarged arterial feeders or dilated pial vessels in the subarachnoid space may sometimes be seen)
  • vascular imaging (CTA/MRA or DSA)
    • often, multiple feeders are present with no intervening nidus
    • dural sinuses are filled with contrast during the arterial phase
    • dilated and tortuous cortical veins in the subarachnoid space
    • DSA remains the best method to accurately assess feeding vessels and the presence and extent of retrograde venous drainage
  • neurosonology
    • Doppler shows decreased resistance (RI< 0.45) + increased flow velocity
DAVF fed from occipital artery (ECA branch)
DAVF - accelerated flow and decreased peripheral resistance in ECA
DAVF in a patient with pulsatile tinnitus. Ultrasound showed increased flow in the ECA branch with aliasing and low pulsatility. MRA confirmed suspected DAVF.
Dural arteriovenous fistula (DAVF) on DSA

Dural arteriovenous fistula (DAVF) on MRA

DSA showing fistula between superficial temporal artery and superior sagittal sinus. White arrow show reflux from sinus to cortical veins

Management

  • treatment decision is based on:
    • type of the fistula
    • patient’s age and comorbidities
    • presence of symptoms attributable to the fistula

Conservative treatment

  • usually in Borden I, Cognard I, and IIa
  • in mild cases, intermittent external compression of the feeding artery may be helpful (retroauricular compression in DAVF fed by the occipital artery)
ECA monitoring during retroauricular compression of the feeding occipital artery

Endovascular and surgical treatment

  • an indisputable indication is DAVF Borden types II and III or Cognard IIb-V because of the high risk of bleeding (an annual risk of ~8%)
  • type I with severe tinnitus may also be indicated for endovascular treatment
Endovascular treatment
  • transarterial approach (TAE) – super-selective distal catheterization
    • e.g., Onyx [Lv, 2009]
  • transvenous approach (TVE) – mainly in multiple small feeding arteries, unsuitable for embolization
  • combined approach (TAE + TVE)
Surgery
  • if endovascular treatment is not possible or has been unsuccessful/only partially successful
Stereotactic radiosurgery (SRS)
  • 20-30 Gy ⇒ irradiated vessels become thrombosed
  • obliteration is gradual (within 2-3 years)
  • unsuitable as primary treatment if reflux into cortical veins is present
Embolization of direct arteriovenous fistula

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Dural arteriovenous fistula (DAVF)
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