Dural arteriovenous fistula explained

Dural arteriovenous fistula
Field:
Symptoms:
Complications:Hemorrhagic stroke, cardiomegaly
Diagnosis:Medical imaging:
Treatment:Arteriovenous fistula obliteration, including:

A dural arteriovenous fistula (DAVF) or malformation is an abnormal direct connection (fistula) between a meningeal artery and a meningeal vein or dural venous sinus.

Signs and symptoms

The most common signs/symptoms of DAVFs are:[1]

  1. Pulsatile tinnitus
  2. Occipital bruit
  3. Headache
  4. Visual impairment
  5. Papilledema

Pulsatile tinnitus is the most common symptom in patients, and it is associated with transverse-sigmoid sinus DAVFs. Carotid-cavernous DAVFs, on the other hand, are more closely associated with pulsatile exophthalmos. DAVFs may also be asymptomatic (e.g. cavernous sinus DAVFs).[2]

Location

Most commonly found adjacent to dural sinuses in the following locations:

  1. Transverse (lateral) sinus,[3] left-sided slightly more common than right[4]
  2. Intratentorial
  3. From the posterior cavernous sinus, usually draining to the transverse or sigmoid sinuses
  4. Vertebral artery (posterior meningeal branch)

Causes

It is still unclear whether DAVFs are congenital or acquired.[5] Current evidence supports transverse-sigmoid sinus junction dural malformations are acquired defects, occurring in response to thrombosis and collateral revascularization of a venous sinus.[6] [7]

Diagnosis

Cerebral angiography is the diagnostic standard. MRIs are typically normal but can identify venous hypertension as a result of arterial-venous shunting.

Classification

Borden Classification

The Borden Classification of dural arteriovenous malformations or fistulas, groups into three types based upon their venous drainage:[8]

  1. Type I: dural arterial supply drains anterograde into venous sinus.
  2. Type II: dural arterial supply drains into venous sinus. High pressure in sinus results in both anterograde drainage and retrograde drainage via subarachnoid veins.
  3. Type III: dural arterial supply drains retrograde into subarachnoid veins.
Type I

Type I dural arteriovenous fistulas are supplied by meningeal arteries and drain into a meningeal vein or dural venous sinus. The flow within the draining vein or venous sinus is anterograde.

  1. Type Ia – simple dural arteriovenous fistulas have a single meningeal arterial supply
  2. Type Ib – more complex arteriovenous fistulas are supplied by multiple meningeal arteries

The distinction between Types Ia and Ib is somewhat specious as there is a rich system of meningeal arterial collaterals. Type I dural fistulas are often asymptomatic, do not have a high risk of bleeding and do not necessarily need to be treated.

Type II

The high pressure within a Type II dural AV fistula causes blood to flow in a retrograde fashion into subarachnoid veins which normally drain into the sinus. Typically this is because the sinus has outflow obstruction. Such draining veins form venous varices or aneurysms which can bleed. Type II fistulas need to be treated to prevent hemorrhage. The treatment may involve embolization of the draining sinus as well as clipping or embolization of the draining veins.

Type III

Type III dural AV fistulas drain directly into subarachnoid veins.[9] These veins can form aneurysms and bleed. Type III dural fistulas need to be treated to prevent hemorrhage. Treatment can be as simple as clipping the draining vein at the site of the dural sinus. If treatment involves embolization, it will only typically be effective if the glue traverses the actual fistula and enters, at least slightly, the draining vein.

The Cognard et al. Classification[10] correlates venous drainage patterns with increasingly aggressive neurological clinical course.

ClassificationLocation and clinical course
Type IConfined to sinus wall, typically after thrombosis.
Type IIIIa - confined to sinus with reflux (retrograde) into sinus but not cortical veins.IIb - drains into sinus with reflux (retrograde) into cortical veins (10-20% hemorrhage).
Type IIIDrains direct into cortical veins (not into sinus) drainage (40% hemorrhage).
Type IVDrains direct into cortical veins (not into sinus) drainage with venous ectasia (65% hemorrhage).
Type VSpinal perimedullary venous drainage, associated with progressive myelopathy.

To simplify the above systems of DAVF classification, the two main factors that should be considered to determine aggressiveness of these lesions are:

Treatment decisions are more complicated and require consultation with a neurosurgeon and team familiar with these lesions.

Treatment

Indications

Interventions

Embolization

One approach used for treatment is embolization.[11] A six-vessel angiogram is employed to determine the vascular supply to the fistula. Detachable coils, liquid embolic agents like NBCA, and onyx, or combinations of both are injected into the blood vessel to occlude the DAVF. Preoperative embolization can also be used to supplement surgery.[12]

Surgery

DAVFs are also managed surgically. The operative approach varies depending on the location of the lesion.

Stereotactic radiosurgery

Stereotactic radiosurgery is used for obliterating DAVFs sometimes in conjunction with embolization or surgery, and is considered an important adjunct and sometimes a primary treatment method for non-aggressive DAVFs.[13] Use of this method, however, is limited as obliteration occurs over the course of up to 2–3 years after the delivery of radiation.

Epidemiology

10–15% of intracranial AV malformations are DAVFs. There is a higher preponderance in females (61–66%), and typically patients are in their fourth or fifth decade of life. DAVFs are rarer in children.

Research

External Manual Carotid Compression is Effective in Patients with Cavernous Sinus Dural Arteriovenous Fistulaetreatment. The patients were instructed to compress the carotid artery and jugular vein with the contralateral hand for ten seconds several times each hour (about 6 to 15 times per day).https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3345454/

See also

Notes and References

  1. Sundt. Thoralf M.. Piepgras. David G.. 1983-07-01. The surgical approach to arteriovenous malformations of the lateral and sigmoid dural sinuses. Journal of Neurosurgery. 59. 1. 32–39. 10.3171/jns.1983.59.1.0032. 6864280. 0022-3085.
  2. Book: Greenberg, Mark S.. Handbook of neurosurgery. 2010-01-01. Greenberg Graphics. 978-1604063264. 892183792.
  3. Graeb. D. A.. Dolman. C. L.. 1986-06-01. Radiological and pathological aspects of dural arteriovenous fistulas. Case report. Journal of Neurosurgery. 64. 6. 962–967. 10.3171/jns.1986.64.6.0962. 0022-3085. 3701446.
  4. Transverse-Sigmoid Sinus Dural Arteriovenous Malformations. Contemporary Neurosurgery. 21. 15. 1–6. en-US. July 1999. Arnautovic. Kenan I.. Krisht. Ali F.. 10.1097/00029679-199907020-00001.
  5. Book: 100 case reviews in neurosurgery. Rahul. Jandial. R.. Aizenberg, Michele. Y.. Chen, Mike. 9780323356374. 966563367. 2016-10-27. Elsevier .
  6. Polster. Sean P.. Zeineddine. Hussein A.. Baron. Joseph. Lee. Seon-Kyu. Awad. Issam A.. October 2018. Patients with cranial dural arteriovenous fistulas may benefit from expanded hypercoagulability and cancer screening. Journal of Neurosurgery. 129. 4. 954–960. 10.3171/2017.5.JNS17788. 1933-0693. 29148899. free.
  7. LaHue. Sara C.. Kim. Helen. Pawlikowska. Ludmila. Nelson. Jeffrey. Cooke. Daniel L.. Hetts. Steven W.. Singh. Vineeta. 2018-04-01. Frequency and characteristics associated with inherited thrombophilia in patients with intracranial dural arteriovenous fistula. Journal of Neurosurgery. 130. 4. 1346–1350. 10.3171/2017.10.JNS171987. 1933-0693. 6173991. 29624150.
  8. 1995. A proposed classification for spinal and cranial dural arteriovenous fistulous malformations and implications for treatment. Journal of Neurosurgery. 82. 2. 166–79. 10.3171/jns.1995.82.2.0166. 7815143. Borden JA, Wu JK, Shucart WA. 358930.
  9. Web site: jonathanborden-md.com. 2007-12-22. 2022-03-31. https://web.archive.org/web/20220331124104/http://www.jonathanborden-md.com/DAVF/Type-III. dead.
  10. 1995 . Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage . Radiology . 194 . 3. 671–80 . 10.1148/radiology.194.3.7862961. 7862961 . Cognard . C. . Gobin . Y. P. . Pierot . L. . Bailly . A. L. . Houdart . E. . Casasco . A. . Chiras . J. . Merland . J. J. .
  11. 2007. Treatment of dural arteriovenous fistula using ethylene vinyl alcohol (onyx) arterial embolization as the primary modality: short-term results. Journal of Neurosurgery. 107. 6. 1120–5. 10.3171/JNS-07/12/1120. 18077948. Carlson AP, Taylor CL, Yonas H. 27522333.
  12. Barnwell. Stanley L.. Halbach. Van V.. Higashida. Randall T.. Hieshima. Grant. Wilson. Charles B.. 11425407. 1989-09-01. Complex dural arteriovenous fistulas. Journal of Neurosurgery. 71. 3. 352–358. 10.3171/jns.1989.71.3.0352. 2769386. 0022-3085.
  13. Lewis. Adam I.. Tomsick. Thomas A.. Tew. John M.. 2009-05-28. Management of tentorial dural arteriovenous malformations: transarterial embolization combined with stereotactic radiation or surgery. Journal of Neurosurgery. 15. 4. 851–859. 10.3171/jns.1994.81.6.0851. 7965115.