SPINE Volume 36, Number 25, pp E1641E1647
2011, Lippincott Williams & Wilkins
CLINICAL CASE SERIES
The Clinical and Radiological Presentation
of Spinal Dural Arteriovenous Fistula
Rajanandini Muralidharan, MD,* Andrea Saladino, MD, Giuseppe Lanzino, MD,
John L. Atkinson, MD, and Alejandro A. Rabinstein, MD*
Study Design. Retrospective consecutive case series.
Objective. To assess the symptoms, neurologic signs, and radiologic
findings in a large series of patients with myelopathy due to spinal
dural arteriovenous fistula (SDAVF).
Summary of Background. The clinical diagnosis of SDAVF is
difficult because presenting symptoms and signs can be similar to those
seen with spinal canal stenosis or peripheral nerve or root disorders.
Methods. We reviewed 153 consecutive patients with SDAVF
treated surgically at our institution between 1985 and 2008. Before
surgery, all patients had detailed neurologic examination, 147
patients had spinal magnetic resonance imaging (MRI) and all but
one, had spinal angiography. We evaluated associations between
symptoms, physical signs, spinal cord T2 signal abnormality on MRI,
and fistula level on angiogram.
Results. Mean age was 63.5 years and 119 (77.8%) were men.
Weakness and sensory changes are usually symmetric and ascend
from the lower extremities. Presenting symptoms included leg
weakness (74 patients, 48.4%), leg sensory disturbances (41
patients, 26.8%), pain involving back or legs (31 patients, 20.3%),
and sphincter disturbances (6 patients, 3.9%). Worsening weakness
with exertion was present in 66 (43.1%) patients and correlated with
thoracic fistula location (P 0.04). Pinprick level was identified
in 57 (37.3%) patients; L1 level (22.8%) was the most common,
followed by T10 (19.3%). Fistula level (2 levels) corresponded to
pinprick level in only 40% of these patients. T2 signal abnormality
involved the conus in 95% of our patients. Highest cord level of T2
signal hyperintensity (2 levels) corresponded to pinprick level in
25% of cases.
Conclusion. Leg weakness exacerbated by exercise, likely due to
worsening hypertension in the arterialized draining vein, is a common
From the *Department of Neurology, Mayo Clinic, Rochester, MN; Department
of Neurologic Surgery, Fondazione IRCCS Istituto Neurologico C. Besta
Milano, Italy; and Department of Neurosurgery, Mayo Clinic, Rochester, MN.
Acknowledgment date: August 18, 2010. Revised date: December 6, 2010.
Accepted date: January 18, 2011.
The manuscript submitted does not contain information about medical device(s)/
drug(s).
No funds were received in support of this work. No benefits in any form have
been or will be received from a commercial party related directly or indirectly to
the subject of this manuscript.
Address correspondence and reprint requests to Alejandro A. Rabinstein, MD,
Mayo Clinic, Department of Neurology, W8B, 200 First Street SW, Rochester,
MN 55905, USA; E-mail: rabinstein.alejandro@mayo.edu
DOI: 10.1097/BRS.0b013e31821352dd
Spine
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BRS204442.indd E1641
manifestation of thoracic SDAVF. Although a sensory level is often
found, it cannot reliably guide the level of imaging. Thus, the entire
spine should be examined with MRI when an SDAVF is suspected.
Key words: spinal dural arteriovenous fistula, symptoms, physical
signs, diagnosis, MRI. Spine 2011;36:E1641E1647
S
pinal dural arteriovenous fistulae (SDAVF) are rare but re-
main the most common type of spinal vascular malforma-
tions.14 The pathologic arteriovenous shunt leads to ar-
terialization of the valveless perimedullary venous plexus with
resultant reduction in arteriovenous gradient leading to reduced
outflow from the radicular vein, retrograde venous drainage,
and intramedullary edema due to venous hypertension.410 Sur-
gical or endovascular interruption of the arteriovenous shunt
often leads to halting or reversing of clinical progression.6,11,12
Although unexplained progressive myelopathy should raise
suspicion for SDAVF, the diagnosis remains difficult because
presenting clinical features are often nonspecific and can mim-
ic disorders such as spinal stenosis, demyelinating disease, and
medullary tumors.5,8,13,14 In this study, we assessed the value of
presenting symptoms, neurologic examination, and magnetic
resonance imaging (MRI) for fistula localization in a large se-
ries of patients with SDAVF.
MATERIALS AND METHODS
Patient Population and Neurologic Examination
The study was approved by the Mayo Foundation insti-
tutional review board. We performed a search of medical
records by diagnosis and operative procedure. We identified
153 consecutive patients, 34 women (22.2%, mean age
62, range 2791) and 119 men (77.8%, mean age 64.3,
range 2788) with SDAVF surgically treated at our institu-
tion between June 1985 and March 2008. Every patient was
evaluated and examined by a neurologist. Severe neurogenic
bladder and bowel were defined as incontinence and urinary
retention requiring catheterization or constipation requiring
disimpaction. Clinical findings were retrospectively adjusted
to the Aminoff-Logue disability scale15 (Table 1) and further
stratified into three classes of disability. A total score (gait and
micturition, G M) of 1 to 3 indicates mild, 4 to 5 moderate,
and 6 to 8 severe disability. The time interval between onset
of initial symptoms and diagnosis of SDAVF was calculated
as time of diagnosis.
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 CLINICAL CASE SERIES Diagnosis of SDAVF Muralidharan et al

diagnosis was not significantly associated with sex, preopera-

TABLE 1. Aminoff-Logue Disability Scales for
Gait and Micturition
Gait
Onset of leg weakness, abnormal stance, or gait,
without restriction of local motor activity
Restricted exercise tolerance
Need for a cane or some support for walking
Need for 2 canes or crutches for walking
Unable to stand, confined to bed or wheelchair
Micturition
Normal
Hesitancy, urgency, frequency, altered sensation
Occasional urinary incontinence or retention
Total incontinence or persistent retention
tive severity of disability, weakness on presentation or with
exertion, sensory disturbance, presence of pinprick level, neu-
rogenic bowel, or extent of T2 signal abnormalities. However,
severe neurogenic bladder was associated with delayed time of
1 diagnosis (P 0.04).
2
Clinical Presentation
3 Mostly, the course was characterized by slow progression or
4 stepwise worsening deficits. Three patients developed acute
symmetric leg weakness. Symptoms on initial presentation
5 were weakness in 48.4%, paresthesias in 26.8%, pain in
20.3%, and sphincter disturbances in 3.9% of patients (Table 3).
Average Aminoff-Logue disability score was 3.2 and average
0
micturition score 1.76. Forty-nine patients presented with
1 mild, 38 with moderate, and 66 severe disability. Severity of
2 disability at presentation did not correlate with fistula level,
extent of T2 signal changes, or clinical presentation.
3

Diagnostic Imaging
One hundred forty-one patients underwent preoperative TABLE 2. Treatment Before Referral to Mayo Clinic
spinal MRI, 11 patients had computed tomography myelog-
No. of Patients
raphy and one patient had angiography as their initial diag-
nostic examination. MRI was eventually performed in 147 Epidural surgical approaches for SDAVF 2
(96%) patients and magnetic resonance angiography (MRA)
in 44 (29%). Our MRA technique uses IV gadolinium bolus Failed acrylic glue or N-butyl 2-cyanoacrylate
7*
embolization
contrast-enhanced elliptic centric-ordered, 3D MRA, using a
phased-array spine coil. Voxel size is approximately 1.1 mm Single level diskectomy 1
1.25 mm 1.4 mm. Maximum intensity projection images Spinal decompressive laminectomy with or
8,
and 2D multiplanar reformatted images were created and re- without fusion
viewed. Spinal angiography was performed in all patients but Tethered spinal cord releasing 2
one for definitive fistula localization.
Posterior cervical decompression 1
Treatment Anterior cervical fusion 1
All patients underwent surgical obliteration of the fistula, consist-
ing of target laminectomy, dura opening, coagulation, and section Sacral lipoma/teratoma removal 1
or disconnection of the draining vein with an aneurysm clip. Spinal steroid injections 3

Statistical Analysis Spinal cord biopsy 4||,**

Data were summarized using descriptive statistics, including
percentages and counts for categorical data and means and
standard deviations for continuous data. The 2 was used to
assess for associations between pairs of categorical variables,
while analysis of variance methods were used to evaluate a dif-
ference in a particular continuous parameter between multiple
categories. All analyses were carried out using the JMP statisti-
cal software package (version 8, SAS Institute Inc., Cary, NC).
A P value less than 0.05 was considered statistically significant.
RESULTS
Time of Diagnosis and Previous Treatment
Mean interval from symptom onset to diagnosis was
24.5 31.5 months (median 12 months, range 3 days to
276 months). Before referral to our institution, 33 patients
had undergone invasive intervention for symptoms that in
retrospect could be attributed to SDAVF (Table 2). Time of
Immunosuppression 9
Other 3
*One patient underwent failed acrylic embolization followed by failed epi-
durally directed fistula obliteration.
One patient had steroids and underwent acrylic embolization prior to
surgery.
One patient had SDAVF resection when discovered during thoracolumbar
laminectomy.
Later unsuccessfully treated with embolization followed by surgery after
SDAVF obliteration.
One patient received intravenous immunoglobulin (IVIG).
||One patient had two biopsies.
**One patient had open spinal cord biopsy after 4 years of immunosuppres-
sive therapy.
Treated with IVIG, Copaxone, Betaseron, or steroids. One patient was
suspected of having Guillain Barr.
Hemorrhoidectomy, prosthetic knee replacement, and hip arthroplasty.
One patient also received IVIG postoperatively.
SDAVF indicates spinal dural arteriovenous fistula.

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 CLINICAL CASE SERIES Diagnosis of SDAVF Muralidharan et al

TABLE 3. Initial Symptoms Among 153 Patients With Spinal Dural Arteriovenous Fistul
Symptom Total N (%) Symptom n (%)
Bilateral lower extremity weakness 61 (82)

Motor 74 (48.4) Unilateral lower extremity weakness 12 (16)

Bilateral upper extremity weakness 1 (2)

Bilateral lower extremity numbness 16 (39)

Unilateral lower extremity numbness 7 (17)

Sensory 41 (26.8)
Bilateral lower extremity dysesthesias/paresthesias 12 (29)

Unilateral lower extremity dysesthesias/paresthesias 6 (15)

Back pain radiation to lower extremities 24 (77)
Pain 31 (20.3)
Pain confined to one or both extremities 7 (23)
Sphincter dysfunction 6 (3.9) Neurogenic bowel or bladder 6 (100)
Respiratory 1 (0.6) Dyspnea 1 (100)

Eighty-two percent (N 61) of patients presented with
bilateral leg weakness, 16% (N 12) had unilateral leg weak-
ness and one had bilateral arm weakness. Leg weakness was ex-
acerbated by climbing stairs, exercise, bending, or standing for
prolonged time, and was relieved with rest or recumbence. In
fact, 43% (N 66) reported worsening of weakness with exer-
tion on initial presentation and 71% (N 47) continued to do so
throughout their disease course. Holocord T2 signal abnormal-
ity (P 0.02) and presence of thoracic fistula (P 0.04) were
significantly associated with worsening weakness with exertion.
Thirty-nine percent of patients (N 16) had bilateral and
17% (N 7) had unilateral leg numbness on initial presenta-
tion, either patchy or involving the whole limb. Twenty-nine
percent (N 12) reported bilateral dysesthesias or paresthe-
sias; sensory symptoms were unilateral in 15% (N 6). In
76% of patients (N 102), the numbness became symmetric
over time. Twenty-four patients had back pain with or with-
out leg radiation while seven patients had pain confined to
the extremities.
Sphincter disturbances were reported by 133 patients.
Of these, 77% had a combination of neurogenic bowel and
bladder, but isolated neurogenic bowel was rare (N 3).
Thirty-six patients had severe sphincter disturbance. Weak-
ness at presentation was more frequent in patients with severe
neurogenic bladder (P 0.04). The presence of neurogenic
sphincter disturbance did not correlate with fistula location
or extent of T2 signal change.
Pinprick level was identified in 57 patients (37%) and
ranged from T5 to S4. Most common level identified was
L1 (N 13, 23%), followed by T10 (N 11, 19%).
Pinprick level corresponded to the highest level of T2 signal
change ( two levels) in 25% of patients, and to fistula level
( two levels) in 40% (N 23). Pinprick level was more fre-
quently found in cases with holocord T2 signal abnormality
(P 0.03).
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On examination, 62 patients (41%) had upper motor neu-
ron (UMN) signs, 51 (33%) had lower motor neuron (LMN)
signs, and 37 (24%) patients had a combination; three pa-
tients had normal motor examinations. Pattern of weakness
did not correlate with the presence of pinprick level or severity
of disability.
Electromyography
Electromyography was performed in 49% of patients (N
75). Fifty-six percent (N 42) had electromyogram (EMG)
findings consistent with a LMN process and 33% UMN pro-
cess. Eight patients had normal EMG studies. Patients with
severe disability at presentation were more likely to show
UMN involvement (54% of patients with severe disability
vs. 12% mild and 28% moderate; P 0.01). There was no
association between weakness on exertion and EMG results.
Although there was no correlation between EMG UMN find-
ings and cord T2 signal abnormality, our small number of
cases may account for the lack of statistical significance.
Magnetic Resonance Imaging and Spinal Angiography
Only 10 patients had no T2 cord signal changes (in one pa-
tient probably due to artifactual image degradation); of these
patients three presented with lower extremity weakness and
two had worsening of their symptoms with exertion (Table 4).
T2 fast spin echo and T2 gradient echo pulse sequences were
used in three of these patients, T2 fast spin echo in two, and
we could not retrieve the MRI scans to determine the type of
T2 sequence in the remainder five patients. Figures 1 and 2
illustrate two of these cases.
When present, MRI T2 signal hyperintensity involved the
conus in all but seven cases (95%). These patients had T2
signal changes between levels T2 and T12, and all had tho-
racic fistulas. Nine patients had isolated conus/lumbar T2
signal abnormality, and all but one had the lumbar fistulas.
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 CLINICAL CASE SERIES Diagnosis of SDAVF Muralidharan et al

TABLE 4. Characteristics of Patients Without Magnetic Resonance Imaging T2 Signal Abnormalities

Severe
Worsening Neurogen- Aminoff Presenting
TTD Presenting Symptoms ic Bladder and Logue Abnormal Examination
Sex Age (Months) Symptoms with Exertion or Blowel* Fistula Scale Vessels Findings
Lower extrem-
M 69 276 T5 3 T4 to conus UMN
ity paresthesias
Lower extrem-
F 56 54 + + T11 5 T10T12 UMN
ity paresthesias
Acute unilateral
F 52 1 + T7 1 T5 to conus Normal
leg weakness
Upper extrem- Jugular fora-
M 72 10 + C4 2 UMN
ity weakness men to C5

M 55 120 Low back pain T6 2 T6 to conus UMN

Fecal inconti-
M 73 60 + T5 5 UMN
nence
Lower extrem-
F 53 24 T9 1 None UMN
ity paresthesias

M 41 7 Low back pain T7 5 Both

Lower extrem-
M 65 24 + T8 2 UMN
ity weakness
Lower extrem-
M 68 18 S1 2 Both
ity weakness
*Defined as overt urinary incontinence or requiring indwelling or daily urinary catheterization and overt fecal incontinence or constipation requiring digital
stimulation or disimpaction.
Missing information.
TDT indicates time of diagnosis; M, male; F, female; UMN, upper motor neuron.

There was no evidence of cord microhemorrhages on the T2 Follow-up

gradient echo sequences in none of the cases in which this
sequence was performed.
We were able to retrieve and review MRI scans of
93 patients to determine the extent of vessel abnormal-
ity. Seventy-eight patients had vessel abnormalities; 59%
extending from thoracic to caudal spinal cord, 14% cervi-
cal to conus/caudal spinal cord, and 15.4% isolated tho-
racic involvement. Fifty-five percent showed a correlation
between the highest level of T2 cord signal abnormality and
vessel abnormality 2 levels.
Diagnosis of SDVAF was confirmed by spinal angiography.
Fistula was most commonly located in the thoracic region
(N 90, 59%) (Figure 3). T2 signal changes were operation-
ally divided into three groups, C1T6, T7T12, and L1 to
sacrum and compared to angiographic fistula location. T2
signal changes correlated poorly with the presence of SDAVF
within the respective levels (17% in C1T6, 41% in T7T12
and 42% in L1 to sacrum). Five patients had T2 abnormali-
ties below the level of the fistula by a range of 3 to 11 levels.
MRA was performed before spinal angiography in 44 pa-
tients. MRA localized fistula exactly in 18 patients (42.9%)
and within 2 levels in 25 patients (59.5%).
E1644 www.spinejournal.com
Mean follow-up was 31 36.2 months, range 1 month to
15.8 years. Fifty-nine, 85, 62, and 124 patients were seen at
1, 3, 6, and last-month follow-up. Average Aminoff-Logue
disability scores at discharge and last follow-up were 3.29
1.4 and 2.53 1.43, respectively, while average mictu-
rition scores were 2.0 1.1 and 1.64 1.17, respective-
ly. Success rate with surgery was 94%. Gait claudication
was associated with better postoperative outcomes. MRI
examinations performed on 104 patients postoperatively
(mean 19.13 months, range 1116) showed complete res-
olution of the T2-weighted abnormalities in 42.3%, im-
provement in 41.3%, stability in 11.5%, and worsening
in 4.9%. Changes in postoperative MRI did not correlate
with functional outcomes (P 0.05 for all comparisons).
A more detailed analysis of postoperative outcomes will be
discussed in a separate manuscript.
DISCUSSION
SDAVFs are relatively rare and their diagnosis is often missed
due to nonspecific presenting clinical characteristics.4,14,16 In
our series, 22% of patients underwent unnecessary invasive
treatments elsewhere for symptoms attributable to SDAVF.
December 2011

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 CLINICAL CASE SERIES
Figure 1. T2-weighted fast-spin echo and gradient echo sagittal mag-
netic resonance imaging showing absence of intramedullary signal in-
volving the thoracic spinal cord (A) and conus medullaris (B) in patient
1. Left subclavian artery injection early (C) and late (D) phases, dem-
onstrate shunt at the C4 level with opacification of dilated and tortuous
veins consistent with spinal dural arteriovenous fistula.
Figure 2. T2-weighted fast-spin echo sagittal magnetic resonance im-
aging showing absence of intramedullary signal involving the lower
spinal cord (A) and conus medullaris (B) in patient 2. Right T7 intercos-
tal artery injection early (C) and late (D) phases, demonstrate shunt at
the T7 level with opacification of tortuous veins consistent with spinal
dural arteriovenous fistula.
Spine
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BRS204442.indd E1645
Diagnosis of SDAVF Muralidharan et al
Figure 3. Anatomic location of the fistula. Fistula was most commonly
located in the thoracic region, followed by lumbar, sacral, and cervi-
cal levels.
The delay to correct diagnosis in our patients (mean of 24.5
months) is comparable to other series.6,17 At the time of diag-
nosis 43% of our patients were severely disabled. Though our
study did not find a correlation between preoperative severity
of disability at presentation and time of diagnosis (P 0.67),
early diagnosis and treatment is important to improve prog-
nosis as some previous studies have noted that postoperative
recovery may depend on the extent of necrotizing myelopathy
present upon diagnosis, severity of disability prior to surgery,
and duration of symptoms.4,13,15,1820 Yet, no clear consensus
exists on this matter.2,8,9,18
Our patients were predominantly men and diagnosis
was most frequent in the early sixth decade, which is in
agreement with previous series.4,5,8,13,14,21,22 Only two of our
patients were under the age of 30 at symptom onset, showing
the rarity of SDAVF in young patients when compared with
other spinal vascular malformations, such as arteriovenous
malformations (AVMs).
Presenting symptoms are usually symmetric and ascend
from the lower extremities, reflective of venous hyperten-
sion and resultant cord edema or ischemia that manifests
as T2 signal abnormality on MRI, involving the caudal
spinal cord and conus, as seen in 95% of our patients.
Sphincter dysfunction is uncommon (5%) upon pre-
sentation. These findings are similar to those reported by
other studies.8,22
Exacerbation of weakness with exertion and gait clau-
dication is a fairly common feature of SDAVFs, mimick-
ing more benign conditions, such as lumbar spinal stenosis.
This similarity may lead to diagnostic delay or inappropri-
ate surgeries, particularly in older patients. In our series, 66
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 CLINICAL CASE SERIES
patients (43%) had worsening of weakness with exertion,
and in the majority, symptoms abated with rest. Leg clau-
dication with exertion was more frequent in patients with
thoracic fistulas. These maneuvers increase cord venous
blood volume in the spinal cord and may consequently
worsen congestion and ischemia.11,16,22,23 Chronic cord
ischemia may also produce defective autoregulatory mecha-
nisms leading to lack of increased blood flow during times
of increased demand, such as with exercise, further worsen-
ing ischemia in those situations.11
Fistula was most commonly found in the thoracic region
followed by the lumbar region. Three patients had low cervi-
cal fistulas (below C2), a rare finding.3 One patient developed
bilateral arm weakness and had a C4 fistula without T2 signal
abnormality. This presentation is only encountered in cervical
SDAVF and it has localizing value.13
Although uncommon at presentation, sphincter dysfunc-
tion is frequently encountered in SDAVF patients (87%) and
is thought to occur because of involvement of sacral roots at
the level of the conus. Delayed time of diagnosis was associ-
ated with severe neurogenic bladder, which is generally a late
manifestation. This is likely due to the progression of venous
hypertension and congestion at the level of the conus, an area
vulnerable to ischemia due to the presence of relatively fewer
venous outflow channels compared with higher spinal cord re-
gions.4,14 Bladder dysfunction also correlated with weakness on
presentation (P 0.04).
Examination revealed pinprick level in 37% of patients,
most commonly at L1, but this did not correlate well with
fistula level. Therefore, physical examination alone is not
enough to localize SDAVF and the entire spine should be stud-
ied with MRI when this is suspected. There was no correla-
tion between highest level of T2 signal abnormality and pin-
prick level, but this was not unexpected as T2 signal changes
often arise caudally from the level of the conus regardless
of fistula location. Findings on electromyography reflect the
pathophysiology of the disease, showing dysfunction of vari-
ous spinal cord and/or root segments, due to the progressive
ischemia that almost invariably begins in the caudal spinal
cord, regardless of fistula level.22,24 Yet, EMG findings may be
deceiving if considered in isolation and they can be at most
supportive, but never diagnostic of SDAVF.
Our results confirm the value of MRI with and without
contrast as an excellent tool to aid in the diagnosis of SDAVF.
T2 signal hyperintensity involved the conus in 95% of cases,
regardless of the highest level of T2 signal change or fistula
level, supporting the notion that venous congestion of the
draining veins and resultant increased intramedullary venous
pressure affect the caudal end of the spinal cord first regard-
less of fistula level.4 This is further supported by the fact that
no patient had isolated cervical or cervicalthoracic T2 sig-
nal involvement, even when fistula was located in the cervical
spine. The distribution and progression of spinal cord changes
explains why patients present with symptoms ascending from
their lower extremities.
The occurrence of SDAVF without T2 signal abnormali-
ties is rare but the possibility needs to be considered, as
E1646 www.spinejournal.com
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BRS204442.indd E1646
Diagnosis of SDAVF Muralidharan et al
exemplified by 10 patients in our series. In a study by Gilb-
ertson et al,17 13 of the 16 patients with normal angiograms
in the presence of SDAVF had no T2 signal abnormalities
on MRI. These patients presented more often with milder
disability, as ours generally did (Table 2). Eighty percent of
our patients without T2 signal changes (N 8) did not have
worsening deficits with exertion. Exertion increases venous
congestion, leading to venous hypertension and diminished
arteriovenous pressure gradient. This impairs drainage of
intramedullary spinal veins, which shares a common out-
flow with the radicular vein, causing increased cord edema
and/or ischemia leading to myelopathic changes, which
manifests as increased T2 signal.4,7,8,16,25 In fact, holocord
T2 signal abnormality was associated with worsening of
symptoms with exertion (P 0.02), suggesting that the ex-
tent of T2 signal abnormality may reflect the degree of com-
promised cord that is vulnerable to further ischemia under
conditions of increased metabolic demand. Though extent
of T2 signal abnormalities did not correlate with severity
of disability upon presentation, holocord signal correlat-
ed with presence of pinprick level (P 0.027), typically
a manifestation of serious spinal cord disease. Though the
reversibility of MRI signal changes and clinical symptoms
after fistula obliteration support the theory that venous hy-
pertension is pivotal in the pathogenesis of SDVAF,16,17,26 the
relationship between the extent of these radiologic changes
and the degree of postoperative recovery is controversial.
Our preliminary outcome analysis suggests that there is no
significant correlation between changes in postoperative
MRI and functional outcome; this will be analyzed in detail
in a separate manuscript.
Ours is one of the largest series of angiographically
documented SDAVF presented in the literature. Additional
strengths of our study include that all patients were exam-
ined by a neurologist at the time of presentation, and there-
fore, reliable clinical history and physical examination find-
ings were consistently available. Weaknesses of this study
include that delayed time of diagnosis was calculated on the
basis of the time of symptom onset reported by the patient
upon evaluation in our clinic, often many months after
symptoms had started. Thus, there is a possibility of recall
bias. Not all patients had MRA and consequently we could
not reliably assess the sensitivity of this imaging modality
in this study.
CONCLUSION
Delayed diagnosis of SDAVF is common due to nonspecific
presenting features, diagnostic confusion with other more
prevalent conditions, or failure to consider SDAVF in the
differential diagnosis. Leg weakness exacerbated by exer-
tion, likely due to worsening of hypertension in the arte-
rialized draining vein, is a frequent manifestation particu-
larly in patients with thoracic fistula and extensive T2 signal
abnormality in the cord. Although a pinprick level is com-
monly found, it cannot reliably guide the level of imaging,
and therefore, whole spine MRI should be performed when
suspecting SDAVF.
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 CLINICAL CASE SERIES
Key Points
Ascending weakness and sensory changes are the
predominant presenting symptoms of SDAVFs.
Pain in the back or legs is present in only one of five
patients initially, and sphincter disturbances are very
rare upon presentation.
T2 signal abnormality involving the conus is almost
invariably present on MRI.
Leg weakness exacerbated by exercise, likely due to
worsening hypertension in the arterialized draining
vein, is a common manifestation of thoracic SDAVF.
Although a sensory level is often found, it correlates
poorly with the level of the fistula and cannot reliably
guide the level of imaging. Thus, the entire spine should
be examined with MRI when an SDAVF is suspected.
Acknowledgment
The authors thank Donna Larkin for her invaluable help in
the preparation of this manuscript.
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