Craniocervical arteriovenous fistula

Case contributed by Dr Andrei Tsoriev

Presentation

Slowly progressive central spastic tetraparesis, one week before MRI, bulbar symptoms appeared with dysphagia, dysarthria. No previous trauma reported.

Patient Data

Age: 59 years
Gender: Female
MRI

MRI

Magnetic resonance imaging using T1-, T2-, T2*-weighted, FLAIR, DWI with ADC sequences.

Sagittal FLAIR shows high signal in brainstem due to vasogenic edema and tortuous vessels in subarachnoid space. Axial T2 shows increased intensity and slight enlargement of the brainstem due to vasogenic edema with tortuous vessels in the basal cisterns.

Sagittal T1 shows low signal intensity in the brainstem due to vasogenic edema. On axial gradient echo images, there are round foci of absent signal in brainstem as a result of magnetic susceptibility artefacts because of microbleeds due to venous congestion.

Sagittal T2 demonstrates that the brainstem hypersignal continues down to the spinal cord up to segment C6. Tortuous draining vessels are once again seen in both anterior and posterior subarachnoid space, as well as incidental, common in this age, degenerative disk disease.

On DWI and ADC maps, there is no diffusion restriction and therefore no acute ischemia.

DSA (angiography)

Selected projections from 4-vessel brain digital subtraction angiography (DSA).

There is an arteriovenous fistula with filling and enlargement of superficial veins around the brainstem and spinal cord from a branches of the internal carotid artery.

Case Discussion

Dural arteriovenous fistula (DAVF) is mostly an acquired vascular lesion, either spontaneous, as in this case, or traumatic in origin. Dural arteriovenous fistulae at the craniocervical junction are very unusual, especially spontaneous ones without history of previous recent or old trauma.

Symptoms of DAVF of such localization may vary:

  • most frequent course is gradually developing neurologic focal deficit depending on localization, which is related to venous congestion and subsequent development of subacute necrotizing encephalomyelopathy
  • sometimes, signs of intracranial hypertension
  • radiculopathy
  • cranial nervу palsies
  • subarachnoid hemorrhage is also a complication of DAVF, may be seen in approximately 45% cases
  • occipitalgia
  • transient ischemic attack

Median age of presentation of cervical DAVFs is believed to be 58 years and there is male predominance from 2:1 to 3:1 3.

Magnetic resonance imaging is strongly indicated in cases of upper cervical myelopathy, either alone or with bulbar symptoms. Visual signs of edema of involved segments of the spinal cord and brainstem, high ADC, indicating vasogenic edema and excluding ischemic lesion, absence of contrast enhancement, excluding mass lesions, enlarged and tortuous subarachnoid vessels are strongly indicative of DAVF and selective DSA should be performed to confirm DAVF and consider its percutaneous embolization.

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Case information

rID: 33636
Published: 20th Jan 2015
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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