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Table 7.2 (continued)

18 Song etal.

(2019) [29]

57/M B1 NM NM C6 Bilateral

CCT

VVP Y Complete

TVE with

coil

NM NM NM

19 Goyal etal.

(2020) [30]

55/M B1 1year;

quadriparesis

NM C4 Bilateral

TCT;

unilateral

VA and

CCT

VVP Y Complete

TAE with

NBCA

N 1month Complete

recovery

20 Shetty etal.

(2021) [1]

37/M B1 NM;

paraparesis

and urinary

retention

3/2 C Unilateral

TCT and

upper

intercostal

artery

VVP Y Complete

TAE with

NBCA and

onyx

N 3months Signicant

improvement

21 Shetty etal.

(2021) [1]

24/F B1 NM;

paraparesis

and pain

NM C Unilateral

VA, TCT,

and CCT

VVP Y Complete

TAE with

coiling and

onyx

Increased

symptoms

3months Signicant

improvement

22 Park etal.

(2022) [31]

33/F B1 1month;

trapezius pain

0/0 C5–C7 Unilateral

VA, TCT

VVP Y VA trapping;

complete

TAE with

NBCA

N 12months Complete

recovery

Abbreviations: CCT costocervical trunk, ECA external carotid artery, F female, IMV intramedullary vein, M male, N

no, NA not applicable, NBCA N-butyl cyanoacrylate, NM

not mentioned, SAH subarachnoid hemorrhage, TAE transarterial embolization, TCT thyrocervical trunk, TVE transvenous embolization, VA vertebral artery, VVP vertebral

venous plexus, Y yes

No. Author (year)

Age/

sex Type

Length of

history;

symptoms

Motor/

urinary Location

Feeding

artery Draining vein

Venous

pouch Treatment Complications

Follow-up

period Prognosis

J. Yu

https://t.me/med1917

Fig. 7.4 Preoperative images of an illustrative case. The

patient was a 42-year-old man who presented with pro-

gressive weakness of the limbs. (a) T2-weighted MRI

shows dilated, serpentine veins behind the cervical spinal

cord (asterisks) and high-signal edema (arrow) in the

medulla oblongata. (b) Post-contrast MRI shows enhance-

ment of the edema (arrow). (c) CTA shows the dilated

veins (asterisks) in the cervical spinal canal. (d) CTA

shows the location of the lesion (frame) by the left side of

the C4 vertebra. Abbreviations: CTA computed tomogra-

phy angiography, L left, MRI magnetic resonance

imaging

Fig. 7.5 Images showing the therapeutic process in an

illustrative case. (a) Arterial-phase DSA of the right VA

shows that the CEAVF is located epidurally at the C4 level

beside the VA; the single triangle indicates the entrance of

the drainage vein into the intradural space; the double tri-

angles indicate the drainage of the vertebral venous

plexus; the asterisks indicate the intramedullary vein

drainage. (b) Late arterial-phase DSA shows intramedul-

lary vein drainage into the brainstem venous system (tri-

angle). (c) DSA showed that the left VA was normal. (d)

After the right VA beyond the AVF was occluded with

coiling (frame), the microcatheter went into the CEAVF

via the feeding artery from the VA (arrow), and a superse-

lective angiogram was performed. Then, Onyx was cast.

(e) An unsubtracted image taken for DSA shows coils in

the VA and Onyx in the CEAVF. (f) A superselective

angiogram of the TCT (arrow) shows the incompletely

embolized CEAVF, and the triangle indicates the intradu-

ral drainage. Then, Onyx was cast via the TCT. (g and h)

DSA of the right subclavian artery shows the completely

embolized CEAVF. (h) An unsubtracted image taken for

DSA, which shows coils and Onyx. Abbreviations:

CEAVF cervical epidural arteriovenous stula, DSA digi-

tal subtraction angiography, L left, R right, TCT thyrocer-

vical trunk, VA vertebral artery

7 Endovascular Therapeutic Considerations forCervical Epidural Arteriovenous Fistula

https://t.me/med1917

Table 7.3

Summarized patient data

Characteristic Data

Age (years)

Mean±SD 42.3±17.6

Range 4–68

Sex

Female 42.9% (9/21)

Male 57.1% (12/21)

Classication

Type A 18.2% (4/22)

Type B1 81.8% (18/22)

Presentation

Sudden onset 26.7% (4/15)

Progressive onset 73.3% (11/15)

Aminoff-Logue scale score

Mean±SD 2.3±2.9

Range 0–8

Location

C1–C3 26.3% (5/19)

C4–C7 73.7% (14/19)

Feeding artery/arteries

Single 27.2% (6/22)

Multiple 72.8% (16/22)

Vertebral artery 68.2% (15/22)

Costocervical trunk 59.1% (13/22)

Thyrocervical trunk 54.5% (12/22)

Draining vein

Vertebral venous plexus 100% (22/22)

Intramedullary vein 18.2% (4/22)

Venous pouch 86.4% (19/22)

Treatment

Complete transarterial embolization 50% (11/22)

Complete transvenous embolization 18.2% (4/22)

Incomplete endovascular embolization 31.8% (7/22)

Complications 9.5% (2/21)

Prognosis

Complete recovery 42.1% (8/19)

Signicant improvement 36.8% (7/19)

Moderate improvement 21.1% (4/19)

Fig. 7.5 (continued)

J. Yu

https://t.me/med1917

7.4 Discussion

A spinal epidural AVF is relatively uncommon

and can produce neurological deterioration

because a large stula can lead to mass effects

with subsequent radiculopathy/myelopathy or

reux into the intradural perimedullary veins,

leading to congestion myelopathy [32]. They are

often located in the ventral/ventrolateral wide

epidural space outside the dura mater, with rich

venous anastomoses (9). Lesions tend to occur in

middle-aged people. In Huang etal.’s review of

spinal epidural AVFs at all levels of the spine in

101 patients, the mean age was 46 years, and

there was no signicant difference in prevalence

according to sex; in this review, CEAVFs

accounted for more than 50% [4]. Therefore, for

CEAVFs, the age and sex distributions may be

similar. In the present review, the mean age was

42.3 ± 17.6 years, and 42.9% of patients were

female; the results were similar.

The precise mechanism of CEAVF is still

unclear, and most CEAVFs are considered

acquired [33–36]. We present a male patient

whose case supports this hypothesis; he had both

CEAVF and dural AVF (DAVF), and thrombosis

continuously progressed near the DAVF over a

3-month period (Fig. 7.6). As with DAVF,

Fig. 7.6 A patient with multiple AVFs, including CEAVF

and anterior fossa DAVF.The images are of the cases from

our institute. (a) CT shows subarachnoid hemorrhage

before the medulla oblongata (frame). (b) DSA of the right

VA shows the location of the CEAVF (circle) at the high

cervical level. (c) The left and right images show the DAVF

of the anterior fossa. The frame in the left image shows that

the SSS was not initially blocked, the frame in the right

image shows that the SSS was blocked after 3months of

follow-up, and the long arrow emphasizes the change.

Abbreviations: CEAVF cervical epidural arteriovenous s-

tula, CT computed tomography, D AV F dural arteriovenous

stula, DSA digital subtraction angiography, R right, SSS

superior sagittal sinus, VA vertebral artery

7 Endovascular Therapeutic Considerations forCervical Epidural Arteriovenous Fistula

https://t.me/med1917

Fig. 7.7 Anastomosis between VA and TCT.The images

are of the cases from our institute, and the anastomosis

between VA and TCT became apparent after VA occlu-

sion. (a) DSA shows anastomosis between the VA and

TCA at the high cervical level (asterisk). (b) DSA shows

the anastomosis between the VA and TCA at the middle

cervical level (asterisk). Abbreviations: DSA digital sub-

traction angiography, TCT thyrocervical trunk, VA verte-

bral artery

CEAVFs may be initiated by thrombosis in the

epidural space; after epidural thrombosis, intra-

venous stasis and/or acute hydrostatic distur-

bances within epidural compartments might

reinforce the development of venous thrombosis

[37]. Then, the epidural arteries around the

thrombus were recruited, including the numerous

osseous and epidural branches of the VA, the

TCT, and the CCT, and they were often anasto-

mosed (Fig. 7.7) [21, 38]. Because of the rela-

tively rich supply of feeding arteries and abundant

epidural space, CEAVF tends to occur at the

C4–7 spinal canal, and even the bone can be

involved [29]. In our review, C4–7 levels

accounted for 73.7% of CEAVFs.

CEAVFs with a single feeding artery were

relatively rare; multiple feeding arteries were

more common, and the VA was also the most

common feeding artery [4, 12, 18, 19, 26]. In our

review, the model of multiple feeding arteries

accounted for 72.8% of patients, and the VA was

also the most common feeding artery in 68.2% of

cases. Anatomically, the cervical vertebral

venous plexus is composed of vertical, valveless

channels that extend along the entire course of

the spinal canal; thus, the drainage of a CEAVF

may be extensive [7, 39].

Normally, an antireux-impeding mechanism

between both the epidural and perimedullary

venous systems works well, resulting from nar-

rowing and zigzagging of the vein walls while

crossing the dura [9]. Therefore, the drainage of

CEAVF can be limited to the epidural venous

plexus. In previous reports, type A is less com-

mon than type B, accounting for 3% of spinal

epidural AVFs in all locations, which illustrates

that it is uncommon for an epidural AVF to shunt

arterialized blood into the spinal venous system

[4, 40]. In this review, type A accounted for

18.2% of CEAVFs. CEAVFs are classied as

type B (B1 or B2). Type B2 CEAVFs are rarely

reported because they are asymptomatic

(Fig.7.1a) [4].

The natural history of type B2 CEAVFs is

unknown: they may remain silent or develop a

mass effect due to thrombosis and transform into

type B1. Type B1 CEAVF appears difcult to sta-

bilize, although, rarely, CEAVF can have sponta-

neous regression [41]. In this review, most defects

of type B1 had a progressive history. During pro-

gression, if type B CEAVFs induce the acute dis-

ruption of dural antireux mechanisms, the

radicular vein can become involved, and type B

CEAVFs evolve into type A [1, 42].

Here, it should be noted that type A CEAVF

may be disguised as type B1 on digital subtraction

angiography (DSA) by the occlusion of intradural

reux due to compression or thrombosis. For

instance, in a report by Miyamoto et al., the

patient suffered sudden tetraparesis; magnetic

resonance imaging showed cervical cord edema,

but DSA failed to show that the CEAVF had intra-

J. Yu

https://t.me/med1917

dural reux. A reasonable explanation was that a

type A CEAVF took on the appearance of type B

after occlusion of the intramedullary drainage

[24]. Additionally, the patient in Fig.7.6 suffered

subarachnoid hemorrhage, and DSA showed

CEAVF without intramedullary vein drainage,

which further supports the hypothesis [43].

Type B1 and A CEAVFs can lead to severe

spinal cord dysfunction through compression,

venous hypertension, or hemorrhage, presenting

with radiculopathy and myelopathy [2].

Therefore, these patients should undergo surgery

or EVT. Type A CEAVFs have a signicantly

higher deterioration rate than type B CEAVFs,

which makes early intervention more urgent [13,

40, 44, 45]. The goal of CEAVF treatment is to

disconnect the arteriovenous shunt; in particular,

intradural intramedullary drainage must be com-

pletely interrupted [46]. Precise localization of

the stula point is essential, and the stulous

zone is often the arterialized epidural pouch [9].

Fortunately, venous pouches were observed in

86.4% of CEAVFs in this review. Treatment

should be aimed at the venous pouch.

Currently, EVT, including TAE, TVE, and

combinations of the two, is becoming a popular

method to treat CEAVFs [8, 46, 47]. EVT is

effective alone or in combination. The rates of

complete or near-complete occlusion of the spi-

nal epidural AVF with EVT ranged from 73.3%

to 94.4%, and the rates of symptom improvement

ranged from 62.5% to 91% [29]. In this review,

complete obliteration via TAE or TVE was

achieved in 68.2% of CEAVFs, while emboliza-

tion was incomplete in 31.8%, and most of the

incompletely embolized CEAVFs were cured

after follow-up or subsequent surgery. In a review

by Huang etal., the initial rate of complete AVF

obliteration by EVT was 55%, and the rate

increased to 92% during the follow-up [4]. To

improve the rate of complete occlusion, the new

technique was helpful, such as the pressure

cooker technique using the Scepter Mini dual-

lumen balloon (MicroVention, Aliso Viejo,

California, USA) or coiling to prevent collateral

inow [47, 48].

In general, EVT is safe; in this review, only

9.5% of CEAVFs developed complications due to

the increased mass effect, and these complica-

tions were resolved by surgery and conservative

treatment with observation. However, the resolu-

tion of all pathological signs on imaging did not

guarantee complete recovery from clinical symp-

toms because the spinal cord was irreversibly

injured in some cases. In this review, complete or

signicant recovery was observed in 78.9% of

CEAVFs, and moderate improvement was

observed in 21.1%.

Materials that can be used for embolization

include N-butyl-2-cyanoacrylate glue, Onyx

(Medtronic, Irvine, California, USA), and coils

[36, 46, 47]. If the lesion has extremely high ow

and expanded drainage channels, coils may be

used to reduce blood ow, which can cause the

liquid embolic agent to form a plug [49].

Intramedullary vein drainage must be obliterated,

but care must be taken to prevent embolic agents

from occluding the intramedullary arteries [50].

7.5 Conclusions

CEAVFs create a complex connection between

feeding and drainage vessels in the epidural

space. They can be classied into type A and type

B. EVT is an effective treatment for CEAVFs.

Complete obliteration of nearly 70% of CEAVFs

can be achieved via EVT. In this study, nearly

80% of patients made a complete or signicant

recovery during follow-up.

Acknowledgments We thank Dr. Lai Qu (Intensive Care

Unit, The First Hospital of Jilin University) for drawing

Fig.7.3.

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7 Endovascular Therapeutic Considerations forCervical Epidural Arteriovenous Fistula

https://t.me/med1917

X. Lv (ed.), Endovascular and Neurovascular Surgery for Spinal Vascular Malformations,

https://doi.org/10.1007/978-981-97-4661-3_8

Current Status oftheTreatment

forSpinal Dural Arteriovenous

Fistulas

JinluYu

Abstract

Spinal dural arteriovenous stula (SDAVF) is

a rare disease. Currently, the understanding of

SDAVFs is not complete. Therefore, we per-

formed a review of this condition. The follow-

ing characteristics of SDAVF were discussed:

natural history and current diagnosed state,

anatomy and angioarchitecture, therapeutic

goals and options, therapeutic techniques,

complications and recurrence, and prognosis.

We found that SDAVF is a rare disabling dis-

ease and is often misdiagnosed. SDAVF has a

poor natural history, and spontaneous disap-

pearance is uncommon. SDAVF has a com-

plex angioarchitecture, which is the

consequence of the arteriovenous shunt and

venous medullary outow impairment or

obstruction. SDAVF is a potentially curable

cause and thus should be treated aggressively.

Surgical and endovascular interventions can

be used. Microsurgery appeared to be an ef-

cient method in terms of the clinical effect,

radicality, and lower recurrence in comparison

with endovascular embolization. However,

endovascular embolization is becoming popu-

lar due to advances in the techniques. Although

there are complications and unsatisfactory

prognoses, SDAVF treatments are still recom-

mended; moreover, the sooner the treatment is

administered, the better the prognosis.

Keywords

Spinal dural arteriovenous stula ·

Microsurgery · Embolization · Complication ·

Prognosis

8.1 Introduction

A spinal dural arteriovenous stula (SDAVF) is a

type I spinal vascular malformation in the dura,

consisting of an arteriovenous shunt without an

intervening capillary network (Fig. 8.1) [1].

SDAVF is often supplied by one or more radicu-

lomeningeal arteries and ends in a single draining

vein; therefore, the arterial ow is transferred to

engorged spinal venous collaterals in a retrograde

manner [2]. SDAVF is rare, with an annual inci-

dence of 5–10 cases/one million persons,

accounting for 80–85% of all spinal vascular

malformations [3–5]. Patients with SDAVF are

typically middle-aged men, and men are affected

5 times more often than women [6]. SDAVF is

exceedingly rare in children [7].

The etiology of SDAVF is unknown, although

it is most likely an acquired disease after throm-

J. Yu (*)

Department of Neurosurgery, First Hospital of Jilin

University, Changchun, China

e-mail: jlyu@jlu.edu.cn

https://t.me/med1917

Fig. 8.1 Angiographic

features of SDAVF.

(a–c) Case A was a

lumber SDAVF.Cases in

B and C were thoracic

SDAVFs. The left

images in a–c are from

the arterial phase, and

the right images are

from the venous phase.

Arrows in a–c indicate

the feeding artery.

Asterisks in a–c indicate

the stula point.

Arrowheads in a–c

indicate the draining

vein. Abbreviation:

SDAVF spinal dural

arteriovenous stula

J. Yu

https://t.me/med1917

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