Radiology Case Reports 15 (2020) 1849–1852
Available online at www.sciencedirect.com
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Case Report
A rare case of a cervical dural arteriovenous fistula
presenting in a younger patient with vertex
subarachnoid hemorrhage: Case report and
literature review ✩
Saba Hamid, MBBS, Woongsoon Choi, MD, Karthik Raghuram, MD,
Umar S. Chaudhry, MD∗
Department of Neuroradiology, University of Texas Medical Branch, 301 University Blvd, Galveston, TX 77555
a r t i c l e i n f o a b s t r a c t
Article history: Spinal dural arteriovenous fistulas (SDAVF) are rare entities, which mainly occur in the tho-
Received 12 July 2020 racic and lumbar regions of older adults with a significant male predominance. The clini-
Revised 16 July 2020 cal manifestations are nonspecific such as myelopathy and this can make it challenging to
Accepted 17 July 2020 diagnose. Rarely these have been described in the cervical region with even rare manifes-
Available online 10 August 2020 tations including subarachnoid hemorrhage. In the few reports of hemorrhage, it is usually
infratentorial. We present a case of 40-year-old female (uncommon gender and younger
Key Words: age) who presented with headaches and was found to have supra tentorial subarachnoid
Spinal arteriovenous dural fistulas hemorrhage and a suspicious lesion in the spinal canal. This proved to be a type 1 AVM of
Subarachnoid hemorrhage the spinal canal (per the American British and French classification). This was successfully
Cervical spine vascular treated endovascularly. With the discussion of the relevant literature we hope that this case
malformations can add to our medical knowledge as another presentation of this uncommon condition
and ultimately help in diagnosing this illusive and rare entity.
© 2020 Published by Elsevier Inc. on behalf of University of Washington.
This is an open access article under the CC BY-NC-ND license.
(http://creativecommons.org/licenses/by-nc-nd/4.0/)
classical manifestation of these entities is progressive
Introduction myelopathy [1]. There have been rare cases of these enti-
ties presenting as subarachnoid hemorrhage (SAH) where the
Spinal vascular shunts are rare entities (5%-9% of all cen- blood is usually present either over the spinal cord, in the
tral nervous system vascular malformations) [1] and comprise basal cisterns or posterior fossa [3]. We present a rare case of
of fistulas and arteriovenous malformations [2]. The mean a cervical level dural arteriovenous fistula (dAVF) in a female
age is 50-60 years, with a male to female ratio of 5:1 and who was below the mean age, presented with headaches as
the common location being the thoracolumbar region [1]. The the main symptoms and was found to have non cisternal pat-
tern of SAH.
✩
Conflicts of Interest: None.
∗
Corresponding author.
E-mail address: [email protected] (U.S. Chaudhry).
https://doi.org/10.1016/j.radcr.2020.07.043
1930-0433/© 2020 Published by Elsevier Inc. on behalf of University of Washington. This is an open access article under the CC
BY-NC-ND license. (http://creativecommons.org/licenses/by-nc-nd/4.0/)
1850 Radiology Case Reports 15 (2020) 1849–1852
Fig. 1 – (A) Coronal noncontrast head computed Fig. 3 – (A, B) Axial and sagittal T2 weighted magnetic
tomography reveals trace subarachnoid hemorrhage over resonance imaging serpiginous flow voids at the right
the left parietal lobe (white arrow). (B) Axial reconstructed dorsal aspect of dorsal thecal sac at the level of C2-C3.
image shows intraventricular hemorrhage in the occipital
horns of lateral ventricles (arrow heads).
Fig. 4 – (A, B) Diagnostic digital subtraction angiography
and 3D reconstruction of the right vertebral artery
demonstrating an arteriovenous fistula between radicular
branch of the vertebral artery (arrow) and peri-medullary
Fig. 2 – CT angiogram (A and B) shows early filling of
vein (arrow heads).
serpiginous structures in the dorsal thecal sac (arrow)
concerning for underlying spinal shunt. Compare to Fig. 6,
post procedural exam.
The patient underwent endovascular treatment with
n-Butyl cyanoacrylate glue embolization 2 days after the
diagnostic angiography. The immediate post-embolization
angiography demonstrated near-complete occlusion of the
Case Description fistula with residual sluggish flow to the fistula (Fig. 5). A
follow-up CT angiogram of the neck showed reduction in
The patient, a 40-year-old female without significant medi- size of the feeding radicular artery without filling of peri-
cal history, presented with 2-day history of acute onset severe medullary vein, indicating successful treatment of the AVF
headache, accompanied by neck tightness, episodes of nausea (Fig. 6). The post-treatment course was uneventful, and the
and vomiting. On examination, the patient was in moderate patient was discharged after 10-days of admission.
distress and drowsy, but arousable and oriented. Vitals were
within normal limits. Comprehensive neurologic exam was
unremarkable. Computed tomography (CT) scan of the head
showed trace acute SAH near the vertex as well as intraven- Discussion
tricular hemorrhage in the occipital horns of lateral ventricles
(Fig. 1). CT angiogram of the head and neck did not demon- The vascular supply of the spinal cord is in the form of a single
strate an intracranial malformation. However, a subtle right anterior spinal artery (ASA); which supplies the anterior two
sided arteriovenous shunt at the level of C2-C3 was seen, rais- thirds and paired posterior spinal arteries (PSA) which supply
ing a suspicion for a spinal dAVF (Fig. 2). Magnetic resonance the posterior cord [2]. The ASA originates from the 2 branches
imaging of cervical spine revealed serpiginous flow voids in of the vertebral arteries and travels along the anterior median
the right dorsal aspect of the thecal sac at the level of C2- fissure [2]. The PSA originate from the intradural portion of the
C3, also supporting a spinal dAVF (Fig. 3). Digital subtraction vertebral artery or the posterior inferior cerebellar artery, with
angiography identified a fistulous communication between contributions from 10 to 23 radiculopial branches [2].
right C2 radicular branch of right vertebral artery and peri Spinal shunts (dAVF and arteriovenous malformations) are
medullary vein, consistent with a Dural AVF, a type 1 AVM as type of vascular malformation which comprise of anomalous
per the American, British and French classification discussed connections between the high-pressure arterial blood flow en-
below (Fig. 4). tering into the valve less venous system without the presence
Radiology Case Reports 15 (2020) 1849–1852 1851
The dAVF is thought to originate from venous outflow ob-
struction (eg, spontaneous thrombosis, trauma) and subse-
quent arterialization of the coronal venous plexus [2]. In ma-
jority of the case the exact etiology cannot be found with some
rare cases of postoperative fistulas also described [6].
The spinal dAVFs commonly occur in the thoracolumbar
region and are more common in the 6th decade and in males
[1]. These generally have a progressive course with clinical de-
terioration usually taking months to years (19% disability in 6
months and 50% in 3 years as per 1 study) [7]. The underlying
mechanism is venous hypertension with broad clinical pre-
sentations including lower-extremity weakness and sensory
disturbance that may manifest in gait abnormalities, sensory
loss, ill-defined lower-back pain, loss of bowel and/or bladder
control, as well as sexual dysfunction [4]. The rarer incidence
of cervical dAVF vs the thoracolumbar variants has been as-
cribed to anatomical differences in venous drainage patterns,
with small caliber radiculospinal veins [8]. When they do occur
in cervical region the dAVF, they have the same demographics
in general with presenting symptoms including myelopathy,
radiculopathy, and cranial nerve dysfunction [9]. There are a
few cases with SAH as the manifestation with a comprehen-
Fig. 5 – Endovascular treatment was performed with sive review by Aviv et al. demonstrating five such cases at the
n-Butyl cyanoacrylate embolization (arrow), achieving C3-C6 levels [9].
near-complete occlusion. Detection on imaging depends on the secondary changes
produced by the dAVF and the malformation itself [10]. MRI
is the modality of choice as both of these can be detected to-
gether. This should ideally be performed with contrast [10].
The various changes that the dAVF can produce are hemor-
rhage, arterial steal, mass effect, or venous hypertension [11].
The most common manifestation of these secondary changes
are best visualized on T2 weighted images, manifesting as
cord edema and with cord atrophy with long standing dis-
ease [10]. Another important imaging hallmark is dilated and
coiled perimedullary vessels, which can also be observed on
the T2-weighted images as flow voids, which are often more
pronounced on the dorsal surface compared with the ven-
tral surface [10]. If the shunt volume is low these may be less
Fig. 6 – (A, B) Axial and sagittal postcontrast neck computed conspicuous and are best seen on post contrast imaging and
tomography angiography demonstrates successful hence the usefulness of adding contrast to these studies [10].
occlusion of the arteriovenous fistula with radio dense Apart from diagnosis noninvasive evaluation of the shunt lo-
embolization material (arrow). cation is extremely helpful to guide the invasive conventional
angiography [10]. Spinal contrast-enhanced MRA has greatly
contributed to localizing these lesions, with techniques such
as time resolved MRA adding to the sensitivity of these find-
of an intervening capillary bed [4]. The first clinical observa- ings as well as reducing the time for finding the appropriate ar-
tion of a spinal vascular malformation was published in Ger- terial feeders to these malformations [12]. Digital subtraction
many in 1890 by Berenbruch [1]. Whereas the first descrip- angiography remains the gold standard for this technique [12].
tion by clinical angiography was in 1967 by Di Chiro from Na- On selective angiography, stasis of contrast material in the
tional Institute of Health, USA [5]. To date there have been radiculomedullary arteries, especially the ASA, can be seen
seven classification systems for these entities [5], with the [10]. After injection into the segmental artery harboring the AV
2 leading systems in use being the American/British/French fistula, early venous filling and retrograde contrast uptake of
system and the Spetzler classification [2]. As per the Amer- the radiculomedullary veins is seen, usually with an extensive
ican/British/French system the Type I lesions, or the “single network of dilated perimedullary veins [10]. With the classic
coiled vessel” type, are dAVFs and consist of a radicular artery appearance on MRI the differential is limited. If the primary
draining into an engorged spinal vein on the dorsal aspect of malformation is small and only edema is seen, entities such
the dural sheath of a nerve root [2]. These are the most com- as glioma, ischemia, or demyelination are included in the dif-
monly encountered malformations [4] with the Spetzler clas- ferential [10].
sification dividing them into extradural and intradural types The two options in the treatment of SDAVFs are surgical oc-
(further classified into dorsal and ventral lesions) [4]. clusion of the intradural vein that received the blood from the
1852 Radiology Case Reports 15 (2020) 1849–1852
shunt or endovascular therapy using a liquid embolic agent [2] Abecassis IJ, Osbun JW, Kim L. Classification and
after super selective catheterization of the feeding radicu- pathophysiology of spinal vascular malformations. Handb
lomeningeal artery [10]. The key principle is obliteration of Clin Neurol 2017;143:135–43.
[3] Matsumoto H, Minami H, Yamaura I, Yoshida Y, Hirata Y.
the fistulous connection with restoration of normal antero-
Newly detected cervical spinal dural arteriovenous fistula on
grade arterial flow and venous drainage [4]. For some lesions,
magnetic resonance angiography causing intracranial
surgery remains the treatment of choice, particularly when subarachnoid hemorrhage. World Neurosurg.
the arterial supply is in close association with the ASA, PSA, or 2017;105:1038.e1–1038.e9.
artery of Adamkiewicz; in those cases, the risk of spinal cord [4] Brown PA, Zomorodi AR, Gonzalez LF. Endovascular
ischemia and worsening of neurological function with cura- management of spinal dural arteriovenous fistulas. Handb
tive embolization may be prohibitive [1]. The liquid embolic Clin Neurol 2017;143:199–213.
[5] Takai K. Spinal arteriovenous shunts: angioarchitecture and
agents include n-butyl cyanoacrylate and Onyx with these
historical changes in classification. Neurol Med Chir (Tokyo)
agents showing low recurrence rates (0%-25%) [4,5]. Our case 2017;57(7):356–65.
was unique because of the younger age, no preceding symp- [6] Kanematsu R, Hanakita J, Takahashi T, Tomita Y, Minami M.
toms and SAH that occurred over the convexity rather than An acquired cervical dural arteriovenous fistula after
the cisterns or posterior fossa, which has been described [3]. cervical anterior fusion: case report and literature review.
In our patient endovascular therapy yielded a very good re- World Neurosurg. 2019;128:50–4.
[7] Cho W-S, Kim K-J, Kwon O-K, Kim CH, Kim J, Han MH,
sponse.
et al. Clinical features and treatment outcomes of the spinal
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Neurosurg Spine 2013;19(2):207–16.
[8] McGurgan IJ, Lonergan R, Killeen R, McGuigan C. Cervical
Conclusion spine arteriovenous fistula associated with hereditary
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Spinal dAVF are rare malformations which can have nonspe- 2017;2017:186–200.
[9] Aviv RI, Shad A, Tomlinson G, Niemann D, Teddy PJ,
cific clinical findings. We present a case of an atypical presen-
Molyneux AJ, et al. Cervical dural arteriovenous fistulae
tation of this entity in an unusual age group and anatomical
manifesting as subarachnoid hemorrhage: report of two
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