INTRACEREBRAL HEMORRHAGE / VASCULAR MALFORMATIONS

Dural arteriovenous fistula (DAVF)

David Goldemund M.D.
Updated on 29/02/2024, published on 21/04/2021

  • dural arteriovenous fistula (DAVF) is characterized by abnormal connections (shunts) between an artery and a vein; DAVF typically presents with tinnitus, hemorrhage, or venous hypertension
  • DAVF accounts for ∼ 10-15% of vascular malformations and 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

  • DAVFs usually have multiple feeders
    • supratentorial
      • middle meningeal artery, superficial temporal artery (from ECA)
      • ethmoidal branches of the ophthalmic artery
    • cavernous sinus (ICA and/or ECA branches)  → Carotid-cavernous fistula (CCF)
    • posterior fossa
      • vertebral arteries
      • occipital artery (fom ECA)
  • most typical drainage paths:

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 location of the fistula and the pattern of venous drainage. Common symptoms include:

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

Diagnostic evaluation

  • CT/MRI
    • more useful for detecting complications (hemorrhage, venous infarction, edema)
    • insufficient for diagnosing DAVF itself – 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 without an 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 can show decreased resistance (RI< 0.45) and increased flow velocity
    • not as sensitive as CTA/MRA or DSA for diagnosing DAVFs
DAVF fed from the occipital artery (ECA branch)
DAVF - accelerated flow and decreased peripheral resistance in the ECA
In a patient presenting with pulsatile tinnitus, ultrasound revealed increased flow in the external carotid artery (ECA) branch, characterized by aliasing and reduced pulsatility. Magnetic resonance angiography (MRA) subsequently confirmed the suspected diagnosis of a dural arteriovenous fistula (DAVF)
Dural arteriovenous fistula (DAVF) on DSA

Dural arteriovenous fistula (DAVF) on MRA

DSA reveals a fistula between the superficial temporal artery and the superior sagittal sinus. The white arrow indicates reflux from the sinus to the cortical veins

Management

  • treatment decision is based on the following factors:
    • type of the fistula
    • patient’s age and comorbidities (older patients and those with comorbidities may be better candidates for conservative treatment)
    • presence of symptoms attributable to the fistula

Conservative treatment

  • regular monitoring and lifestyle modifications are usually recommended for Borden I, Cognard I-IIa DAVFs
  • in mild cases, intermittent external compression of the feeding artery may be helpful (retroauricular compression in DAVF fed by the occipital artery)
Monitoring of the external carotid artery (ECA) during retroauricular compression of the feeding occipital artery

Endovascular and surgical treatment

  • typically considered for DAVFs with a higher risk of bleeding, such as Borden II and III or Cognard IIb-V DAVFs (an annual risk ~ 8%)
  • type I with severe tinnitus may also be indicated for endovascular treatment
Endovascular treatment
  • endovascular treatment involves occluding the abnormal connection between the artery and vein using embolization techniques
  • transarterial approach (TAE) – super-selective distal catheterization with injecting embolic agents into the feeding arteries
    • e.g., using Onyx [Lv, 2009]
  • transvenous approach (TVE) – involves occluding the venous drainage pathways; mainly used in cases with multiple small feeding arteries unsuitable for embolization
  • combined approach (TAE + TVE)
Surgery
  • typically reserved for cases where endovascular treatment is not possible or has been unsuccessful/partially successful
Stereotactic radiosurgery (SRS)
  • a minimally invasive second-line treatment option if endovascular or surgical treatment is not feasible or has been unsuccessful
  • typically dose of 20-30 Gy is used ⇒ irradiated vessels become thrombosed
  • obliteration is gradual, occuring within 2-3 years
  • unsuitable as primary treatment if there is reflux into cortical veins
Embolization of the direct arteriovenous fistula

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Dural arteriovenous fistula (DAVF)
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