Borden type I intracranial dural arteriovenous fistula

Borden type I intracranial dural arteriovenous fistula

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

Equivalent to Cognard type I and IIa, with a favorable natural history 1) 2).

Type Ia – simple dural arteriovenous fistulas have a single meningeal arterial supply

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


A small number of Type I DAVFs will convert to more aggressive DAVFs with CVD over time. This conversion to a higher-grade DAVF is typically heralded by a change in patient symptoms. Follow-up vascular imaging is important, particularly in the setting of recurrent or new symptoms. 3).


A comparative meta-analysis was completed to evaluate the outcomes of intervention versus observation of Borden type I intracranial dural arteriovenous fistula. Outcome measures included: grade progression, worsening symptoms, death due to dural arteriovenous fistula, permanent complications other than death, functional independence (mRS 0-2), and rate of death combined with permanent complication, were evaluated. Risk differences (RD) were determined using a random effects model.

Three comparative studies combined with the authors’ institutional experience resulted in a total of 469 patients, with 279 patients who underwent intervention and 190 who were observed. There was no significant difference in dAVF grade progression between the intervention and observation arms, 1.8% vs. 0.7%, respectively (RD: 0.01, 95% CI: -0.02 to 0.04, P = 0.49), or in symptom progression occurring in 31/279 (11.1%) intervention patients and 11/190 (5.8%) observation patients (RD: 0.03, CI: -0.02 to 0.09, P = 0.28). There was also no significant difference in functional independence on follow-up. However, there was a significantly higher risk of dAVF-related death, permanent complications from either intervention or dAVF-related ICH or stroke in the intervention group (11/279, 3.9%) compared to the observation group (0/190, 0%) (RD: 0.04, CI: 0.1 to 0.06, P = 0.007).

CoIntervention of Borden Type I dAVF results in a higher risk of death or permanent complication, which should be strongly considered when deciding on the management of these lesions 4).


From April 2013 to March 2016, consecutive patients with DAVF were screened at 13 study institutions. We collected data on baseline characteristics, clinical symptoms, angiography, and neuroimaging. Patients with Borden type I DAVF received conservative care while palliative intervention was considered when the neurological symptoms were intolerable, and were followed at 6, 12, 24, and 36 months after inclusion.

Results: During the study period, 110 patients with intracranial DAVF were screened and 28 patients with Borden type I DAVF were prospectively followed. None of the patients had conversion to higher type of Borden classification or intracranial hemorrhage during follow-up. Five patients showed spontaneous improvement or disappearance of neurological symptoms (5/28, 17.9%), and 5 patients showed a spontaneous decrease or disappearance of shunt flow on imaging during follow-up (5/28, 17.9%). Stenosis or occlusion of the draining sinuses on initial angiography was significantly associated with shunt flow reduction during follow-up (80.0% vs 21.7%, p = 0.02).

Conclusion: In this 3-year prospective study, patients with Borden type I DAVF showed benign clinical course; none of these patients experienced conversion to higher type of Borden classification or intracranial hemorrhage. The restrictive changes of the draining sinuses at initial diagnosis might be an imaging biomarker for future shunt flow reduction 5)


1)

Davies MA, TerBrugge K, Willinsky R, Coyne T, Saleh J, Wallace MC. The validity of classification for the clinical presentation of intracranial dural arteriovenous fistulas. J Neurosurg. 1996 Nov;85(5):830-7. doi: 10.3171/jns.1996.85.5.0830. PMID: 8893721.
2)

Strom RG, Botros JA, Refai D, Moran CJ, Cross DT 3rd, Chicoine MR, Grubb RL Jr, Rich KM, Dacey RG Jr, Derdeyn CP, Zipfel GJ. Cranial dural arteriovenous fistulae: asymptomatic cortical venous drainage portends less aggressive clinical course. Neurosurgery. 2009 Feb;64(2):241-7; discussion 247-8. doi: 10.1227/01.NEU.0000338066.30665.B2. PMID: 19190453.
3)

Shah MN, Botros JA, Pilgram TK, Moran CJ, Cross DT 3rd, Chicoine MR, Rich KM, Dacey RG Jr, Derdeyn CP, Zipfel GJ. Borden-Shucart Type I dural arteriovenous fistulas: clinical course including risk of conversion to higher-grade fistulas. J Neurosurg. 2012 Sep;117(3):539-45. doi: 10.3171/2012.5.JNS111257. Epub 2012 Jun 22. PMID: 22725983.
4)

Schartz D, Rahmani R, Gunthri A, Kohli GS, Akkipeddi SMK, Ellens NR, Romiyo P, Kessler A, Bhalla T, Mattingly TK, Bender MT. Observation versus intervention for Borden type I intracranial dural arteriovenous fistula: A pooled analysis of 469 patients. Interv Neuroradiol. 2022 Sep 13:15910199221127070. doi: 10.1177/15910199221127070. Epub ahead of print. PMID: 36113111.
5)

Nishi H, Ikeda H, Ishii A, Kikuchi T, Nakahara I, Ohta T, Sakai N, Imamura H, Takahashi JC, Satow T, Okada T, Miyamoto S. A multicenter prospective registry of Borden type I dural arteriovenous fistula: results of a 3-year follow-up study. Neuroradiology. 2022 Apr;64(4):795-805. doi: 10.1007/s00234-021-02752-5. Epub 2021 Oct 10. PMID: 34628528; PMCID: PMC8907088.

Dural arteriovenous fistula

Dural arteriovenous fistula

Dural arteriovenous fistulas (DAVFs) are pathologic vascular connections that shunt dural arterial flow directly to dural venous drainage.

DAVFs comprise 10–15% of all intracranial AVMs 1). 61–66% occur in females, and patients are usually in their 40 s or 50 s. They occur rarely in children, and when they do they tend to be complex, bilateral dural sinus malformations 2)

Dural arteriovenous fistulas can occur at any dural sinus but are found most frequently at the cavernous or transverse sinus.

Intracranial dural arteriovenous fistula.

Spinal dural arteriovenous fistula.

The etiology and pathophysiology of DAVFs is not fully understood. Several hypotheses for development of DAVF and classifications for predicting risk of hemorrhage and neurological deficit have been proposed to help clinical decision making according to its natural history 3).

Radical treatment is to obliterate the draining veins in any treatment modalities including endovascular treatment or surgical treatment. Radiosurgery is the last choice. Transvenous embolization plays the main role in the DAVF of the cavernous sinus and anterior condylar confluence. Transarterial embolization with Onyx has dramatically improved the obliteration rate of the transverse-sigmoid, superior sagittal sinuses, and other non-sinus lesions. Transarterial NBCA injection is still the gold standard in the endovascular treatment of the spinal dural and epidural AVFs. Understanding of the functional microvascular anatomy is mandatory, especially in the transarterial liquid injection (Onyx and NBCA). Surgical treatment in the DAVF of the anterior cranial base, craniocervical junction, tentorial region, and spine is a safe and radical treatment. Postoperative follow-up is necessary from the viewpoint of chronological and spacial multi-occurrence of this disease 4).


1)

Arnautovic KI, Krisht AF. Transverse-Sigmoid Sinus Dural Arteriovenous Malformations. Contemp Neurosurg. 2000; 21:1–6
2)

Ashour R, Aziz-Sultan MA, Soltanolkotabi M, et al. Safety and efficacy of onyx embolization for pediatric cranial and spinal vascular lesions and tumors. Neurosurgery. 2012; 71:773–784
3)

Sim SY. Pathophysiology and classification of intracranial and spinal duraAVF. J Cerebrovasc Endovasc Neurosurg. 2022 Apr 21. doi: 10.7461/jcen.2022.E2021.04.001. Epub ahead of print. PMID: 35443276.
4)

Kuwayama N. Management of Dural Arteriovenous Fistulas. Adv Tech Stand Neurosurg. 2022;44:251-264. doi: 10.1007/978-3-030-87649-4_14. PMID: 35107684.

Intracranial dural arteriovenous fistula clinical features

Intracranial dural arteriovenous fistula clinical features

Clinical features of DAVF vary depending on their location, arterial supply, degree of arteriovenousshunting, and most importantly, their venous drainage pattern 1) 2) 3) 4)

DAVF lacking cortical vein drainage (CVD) may be asymptomatic, or present with symptoms related to increased dural sinus blood flow, such as pulsatile tinnitus, the latter particularly common for transverse sinus and sigmoid sinuses lesions.

Generalized central nervous system symptoms that may be related to venous hypertension or cerebrospinal fluid malabsorption, while resulting cranial nerve palsy, are often because of an arterial steal phenomenon or occasionally mass effect from an enlarged arterial feeder.

In addition, cavernous sinus dural arteriovenous fistula may present with orbital symptoms, including chemosisproptosisophthalmoplegia, and decreased visual acuity.

DAVF with CVD typically have more aggressive clinical presentations, including the sudden onset of severe headacheseizures, nonhemorrhagic neurological deficit (NHND), and intracranial hemorrhage, including intraparenchymal, subarachnoid, and subdural hematoma.

In a meta-analysis, Lasjaunias et al 5) reviewed 195 cases of DAVF and found that focal neurological deficits were related to the presence of associated cortical venous drainage (CVD) and venous congestion in the affected vascular territory. Less common aggressive presentations include brain stem or cerebellar dysfunction secondary to venous congestion, parkinsonism-like symptoms, extra-axial hemorrhage in the cervical spine, as well as cervical and upper thoracic myelopathy.

DAVF with extensive arteriovenous shunting, particularly in the setting of dural sinus thrombosis, can result in impaired venous drainage from the brain and the global venous hypertension. This can lead to cerebral edema, encephalopathy, and cognitive decline 6).


Pulsatile tinnitus is the most common presenting symptom of a DAVF. Cortical venous drainage with resultant venous hypertension can produce intracranial hypertension, and this is the most common cause of morbidity and mortality and thus the strongest indication for Intracranial dural arteriovenous fistula treatment.

DAVFs may also cause global cerebral edema or hydrocephalus due to poor cerebral venous drainage or by impairing the function of the arachnoid granulations, respectively. Other DAVF symptoms/signs include headaches, seizures, cranial nerve palsies, and orbital venous congestion.


Leptomeningeal venous drainage can lead to venous hypertension and intracranial hemorrhage.

The majority of patients presented with non-aggressive symptoms. 18% presented with intracranial hemorrhage: all the hemorrhages occurred in high-grade DAVFs 7).

see Dural arteriovenous fistula presenting as an acute subdural hemorrhage.


Only 4 cases of DAVF causing syncope have been reported, all in combination with other neurological symptoms. In comparison, they report a unique case of DAVF presenting solely with recurrent syncope, a previously undocumented finding in the literature. The case adds to other reports of nonspecific DAVF presentations and highlights the importance of considering this etiology 8).


1)

Gandhi D, Chen J, Pearl M, Huang J, Gemmete JJ, Kathuria S.Intracranial dural arteriovenous fistulas: classification, imaging findings, and treatment.AJNR Am J Neuroradiol. 2012; 33:1007–1013. doi: 10.3174/ajnr.A2798.
2)

Sarma D, ter Brugge K.Management of intracranial dural arteriovenous shunts in adults.Eur J Radiol. 2003; 46:206–220.
3)

Houser OW, Campbell JK, Campbell RJ, Sundt TMArteriovenous malformation affecting the transverse dural venous sinus–an acquired lesion.Mayo Clin Proc. 1979; 54:651–661.
4) , 5)

Lasjaunias P, Chiu M, ter Brugge K, Tolia A, Hurth M, Bernstein M.Neurological manifestations of intracranial dural arteriovenous malformations.J Neurosurg. 1986; 64:724–730. doi: 10.3171/jns.1986.64.5.0724.
6)

Miller TR, Gandhi D. Intracranial Dural Arteriovenous Fistulae: Clinical Presentation and Management Strategies. Stroke. 2015 Jul;46(7):2017-25. doi: 10.1161/STROKEAHA.115.008228. Epub 2015 May 21. PMID: 25999384.
7)

Signorelli, F. et al. Diagnosis and management of dural arteriovenous fistulas: A 10 years single-center experience Clinical Neurology and Neurosurgery , Volume 128 , 123 – 129
8)

Sheinberg DL, Luther E, Chen S, McCarthy D, Starke RM. Recurrent Syncope Caused by a Dural Arteriovenous Fistula: A Case Report and Review of the Literature. Neurologist. 2021 Mar 4;26(2):62-65. doi: 10.1097/NRL.0000000000000322. PMID: 33646991.
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