INVITED REVIEW ARTICLE
Vascular Disease of the Spine
Charles N. Munyon, MD* and David J. Hart, MDw
Abstract: Vascular insults to the spinal cord are substantially less
common than their corresponding events in the brain; it has been
estimated, for example, that spinal cord infarcts make up r1% of
ischemic events in the central nervous system. Although the public
health burden of spinal cord injury remains severe, the majority of this
burden stems from traumatic rather than vascular events. Still, vascular
injuries in the spine are common enough and their consequences
devastating enough that a familiarity with the pathophysiology, diag-
nosis, and treatment of the more common etiologies is essential to any
practitioner of the clinical neurosciences. In this educational review,
we will briefly outline the normal development and anatomy of the
spinal vasculature, then focus on specific mechanisms of vascular
injury to the spine. In particular, we will examine spontaneous and
iatrogenic ischemic insults and their associated clinical syndromes, and
then review vascular neoplasms and malformations of the spine with
attention to the various management strategies that currently exist for
these complex lesions. Finally, we will briefly address the future areas
for exploration, including investigative avenues for neuroprotection, as
well as the possible influence of atherosclerotic disease on spinal
degenerative disease and low back pain.
Key Words: spine, vascular disease, spinal vascular malformation,
spinal embryology and development, spinal radiology, spinal vascular
anatomy
(The Neurologist 2015;19:121–127)
T he spine, like the brain, contains cells that are highly
metabolically active and exquisitely sensitive to ischemia,
requiring a robust and reliable vascular supply. The brain and
spinal cord are also both vulnerable to similar types of vascular
malformations, which can alter neurological function via
hemorrhage, venous congestion, vascular steal, or even direct
compression of neural elements. Additional similarities
between cerebral and spinal vasculature include an anterior
and posterior circulation with different associated clinical
syndromes, as well as a severely limited capacity for regen-
eration in the wake of ischemic or hemorrhagic injury to the
parenchyma. The spine, however, is substantially less prone to
primary vascular injury (as opposed to secondary vascular
injury from trauma) than the brain. The relative rarity of spinal
vascular events is partly explained by the spine’s more direct
and much more collateralized blood supply and its lower ratio
of gray to white matter. This rarity means that the true inci-
dence of spinal vascular disease is unknown and that its
From the *Department of Neurological Surgery, Temple University School
of Medicine, Philadelphia, PA; and wDepartment of Neurological
Surgery, University Hospitals Neurological Institute/Case Western
Reserve University School of Medicine, Cleveland, OH.
The authors declare no conflict of interest.
Reprints: David J. Hart, MD, Department of Neurological Surgery, Uni-
versity Hospitals Neurological Institute/Case Western Reserve Uni-
versity School of Medicine, 11100 Euclid Avenue, Cleveland, OH
44106. E-mail: david.hart@uhhospitals.org.
Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
ISSN: 1074-7931/15/1905-0121
DOI: 10.1097/NRL.0000000000000018
The Neurologist  Volume 19, Number 5, May 2015
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
pathophysiology and natural history remain incompletely
understood. Still, more sophisticated magnetic resonance
imaging (MRI) sequences, coupled with an improved safety
profile and wider availability of spinal angiography, have
begun to substantially improve both our understanding of the
pathophysiology of spinal vascular disease and our ability to
positively influence its course. The greatest obstacle to timely
intervention remains the failure to include spinal vascular
disease in the differential diagnosis, particularly in cases where
symptoms such as severe chest pain draw the attention of the
treating physician away from neurological findings. This
review is therefore offered as a reminder of the many facets of
spinal vascular disease, in the hopes of reinforcing awareness
of these rare but potentially devastating processes.
EMBRYONIC DEVELOPMENT OF THE SPINAL
VASCULATURE
In the second and third weeks of embryonic life, 31 pairs
of segmental vessels arise from the paired dorsal aortas and
extend dorsally to the neural tube, following the path of the
developing nerve roots. Each of these segmental vessels divides
into a ventral and dorsal branch, which will establish a network
of capillaries which become the paired ventral arterial tracts.
During weeks 3 to 6, the ventral arterial tracts move medially,
eventually fusing to form the anterior spinal artery (ASA).
Concomitantly, a pair of dorsolateral anastomotic pial networks
form which will become the posterior spinal arteries (PSAs).1,2
All 3 arteries supply a ramifying network of smaller vessels
known as the perineural vascular plexus (PNVP); because the
central nervous system (CNS) does not have vascular progen-
itor cells, the developing spinal cord must guide the ingression
of the PNVP into the parenchyma. The pattern of ingression is
highly stereotyped and depends on a network of glioneuronal
progenitor cells called radial glia to guide the vascular endo-
thelial cells to their targets. These endothelial cells, along with
perivascular cells known as pericytes, will interact with neu-
ronal and astrocytic progenitor cells to form the neurovascular
units which compose the blood-CNS barrier.3 Although the
specific molecular mediators of this process are poorly char-
acterized, it is likely that derangements at this stage are
responsible for some types of vascular malformation. As
development progresses, most of the segmental vessels undergo
regression to radicular vessels, supplying the nerve roots, dura,
and vertebral bodies. This disconnects the anterior and PSAs
from aortic supply at all but a few levels.1,2 Levels with per-
sistent aortic supply will therefore be essential for flow aug-
mentation in the postnatal spine, as discussed below.
NORMAL VASCULAR ANATOMY OF THE SPINE
The mature spinal cord and its meninges are supplied by an
anastomosing arterial network arising from 3 longitudinally ori-
ented vessels: the paired PSAs and the solitary ASA. Although the
PSAs have multiple interconnections with each other, they are not
in direct communication with the ASA. This means that the spine,
like the brain, has an essentially independent anterior and posterior
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Munyon and Hart The Neurologist  Volume 19, Number 5, May 2015

circulation.2,4 All 3 longitudinal vessels, however, receive their

rostral supply from the vertebral artery; the ASA is formed from
the union of 1 vessel from each vertebral artery just caudal to the
vertebrobasilar junction, whereas the PSAs arise more caudally
from the ipsilateral vertebral or posterior inferior cerebellar artery.
At their caudal ends, the spinal arteries receive additional blood
supply from the median sacral artery. Between these termini,
blood flow to the spinal arteries is augmented at variable intervals
by those segmental vessels which underwent only partial regres-
sion during development. These vessels are known as anterior or
posterior radiculomedullary arteries, depending on which circu-
lation they augment. The number and configuration of the radi-
culomedullary arteries differs substantially among individuals. In
the thoracic and lumbar spines, they are generally branches of the
dorsospinal artery, but in the cervical spine, they can arise from
branches of the vertebral, subclavian, or supreme intercostal
artery. The largest of the radiculomedullary arteries is the “arteria
radicularis anterior magna,” better known as the artery of
Adamkiewicz, arises at the thoracolumbar junction and supplies
the lumbar enlargement. Generally, the territories supplied by
radiculomedullary arteries can be divided into the cervicothoracic,
midthoracic, and thoracolumbar spines; the junctions between
these territories are watershed areas particularly sensitive to sys-
temic hypotension. Figure 1 shows a schematic of the arterial
supply of the spinal cord.2
The parenchyma of the cord is also divided into vascular
territories, based upon longitudinal supply. The ASA supplies
the anterior horns and central gray matter of the cord via
sulcocomissural or sulcal arteries which run in the anterior
median fissure. Each sulcal artery supplies blood to only 1 side
of the cord, with the subsequent segment alternating laterality.
The ASA also sends out the arteriae vasocoronae, which
course around the sides of the cord and penetrate the pia to
supply the anterior and lateral funiculi. The PSAs also send out
pial perforators, which supply the posterior funiculi as well as
a variable portion of the posterior horns. Figure 2 shows a
cross-sectional representation of the arterial supply of the
spinal cord, with the approximate territories supplied by the
ASA and PSAs marked out on the right.2
Venous drainage of the spinal cord occurs centrifugally
along pathways similar to those taken by the arteries. The
anterior median vein, however, drains both sides of the gray
matter in contrast to the unilaterally alternating sulcoco-
missural arteries. There is also a posterior median vein, which
drains out to the dorsal median sulcus. Larger veins on the
surface of the spinal cord form a network of up to 6 channels, 6
ventral and 3 dorsal, which receive drainage from the anterior
and posterior median veins as well as the smaller perforating
veins draining the white matter. These intradural anastomotic
networks send out ventral and dorsal radicular veins which
then drain along the nerve roots into the internal vertebral
venous plexi. These plexi are composed of the basivertebral,
anterior and posterior longitudinal, and retrocorporeal veins, as
well as the aforementioned radicular veins.5 They are valve-
less, tortuous channels which anastomose extensively with the
intracranial venous sinuses rostrally and the deep pelvic and
thoracic veins more caudally. The caudal anastomoses appear
to be a frequent route for metastasis of pelvic malignancies to FIGURE 1. Schematic diagram of the arterial supply to the
the spine, as first hypothesized by Batson in 1940.2,5,6 spine (posterior view), showing typical locations for the anterior
and posterior radiculomedullary arteries. The dashed lines
represent divisions between the cervicothoracic, midthoracic,
and thoracolumbar vascular territories, with the hatch marks
RADIOGRAPHIC EVALUATION OF SPINAL showing regions of potential watershed ischemia. Reprinted
VASCULATURE with permission from Wells-Roth and Zonenshayn.2
Historically, diagnostic modalities for spinal vascular disease Copyright [Elsevier], [Cleveland, OH]. All permission requests for
have lagged significantly behind those for cerebrovascular disease, this image should be made to the copyright holder.

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The Neurologist  Volume 19, Number 5, May 2015
FIGURE 2. Cross-sectional representation of the arterial supply of
the spinal cord, including depiction of both an anterior and a
posterior medullary artery. The right hand side shows the terri-
tories typically supplied by the sulcocommisural arteries (vertical
stripes), the arteriae vasocoronae (horizontal stripes), and the
posterior spinal arteries (diagonal stripes). Reprinted with per-
mission from Wells-Roth and Zonenshayn.2 Copyright [Elsevier],
[Cleveland, OH]. All permission requests for this image should be
made to the copyright holder.
not only because of the relative rarity of spinal vascular insults, but
also because of the technical challenges in imaging the vasculature
of the spine. Although Moniz first described cerebral angiography
in 1927, early neuroangiography relied on direct, or later catheter-
based, injection of high concentrations of contrast into proximal,
large-caliber vessels.7,8 Radiographic localization and character-
ization of vascular lesions in the spine was therefore essentially
limited to looking for alterations in the bony spine through an x-
ray or in the subarachnoid or epidural space via myelography.
Distinction between vascular versus infectious or neoplastic eti-
ologies had to be undertaken on clinical grounds, with many
misdiagnoses not discovered until surgery or autopsy.4,8,9 Gradual
refinements in technique and technology allowed Hook and
Lidvall10 to demonstrate 2 cervical arteriovenous malformations
(AVMs) by vertebral arteriography, and Djindjian et al (1962) to
use aortography for gross diagnosis of spinal vascular lesions, but
real progress would not begin until 1967, forty years after Moniz’s
initial monograph. In that year, DiChiro, Doppman, and Ommaya
first described selective catheterization of the radiculomedullary
arteries, and all subsequent techniques in spinal angiography have
been built upon their work.8,11 While catheter angiography
remains the gold standard, in the last two decades MR angiog-
raphy has become progressively better at delineating the anatomy
of vascular malformations and detecting ischemic lesions with
substantially better temporal and spatial sensitivity.12–15 For vis-
ualization of vascular lesions in relationship to the surrounding
bony anatomy, CT angiography (CTA) has recently become a
viable diagnostic modality. Utility had historically been limited by
an inability to scan more than a few vertebral segments in the
narrow window of peak arterial contrast concentration; this limi-
tation appears to be mitigated, however, by the use of multi-
detector row helical CT (MDCT). MDCT allows for increased
longitudinal resolution with shorter acquisition times, allowing for
CTA of the neuraxis during peak contrast concentration.16 For
precise localization, moreover, most modern fluoroscopy suites
will allow for selective catheterization followed by CTA acquis-
ition with intra-arterial contrast injection.16,17
None of these diagnostic modalities will be of any use,
however, if they cannot be brought to bear in a focused and
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Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Vascular Disease of the Spine
timely manner. Technologic advancement cannot replace a
thorough history and physical examination and the generation
of an appropriate differential diagnosis to guide further
investigations.
SPINAL CORD ISCHEMIA
As a result of its rich anastomotic supply and higher ratio of
white to gray matter, the spinal cord is substantially better pro-
tected than the brain from symptomatic ischemia. While reliable
data on incidence are lacking, one large autopsy series showed
only 9 spinal infarctions in 3784 patients (0.23%). Sandson and
Friedman (1989) reported that 1.2% of the admissions for stroke
at their institution were spinal ischemic events.18 It is widely
accepted that spontaneous spinal cord infarctions make up less
than 1% of ischemic insults to the CNS.4
Anterior Spinal Artery Syndrome (ASAS)(Aka
Beck’s Syndrome)
Because the ASA is primarily responsible for blood sup-
ply to the anterior and lateral funiculi as well as the spinal gray
matter, insults due to occlusion or hypoperfusion of the ASA
lead to destruction of the corticospinal and spinothalamic tracts
with relative preservation of the dorsal columns.19 ASAS is
most frequently described in association with surgery for aortic
aneurysm (discussed below), but can also present sponta-
neously.19–21 The spontaneous presentation generally involves
sudden onset of severe pain in the affected dermatome, para-
lysis and loss of pain and temperature sensation below the
lesion, and sparing of vibration sense and proprioception. Loss
of bladder and bowel function is a frequent but not universal
occurrence.19,21 In instances of lower cervical lesions, ASAS
can be mistaken for myocardial infarction due to involvement
of afferent visceral pathways from the cardiac plexus.22,23
Prognosis for recovery depends on age, longitudinal extent of
signal abnormality on MRI, and evolution of proprioceptive
deficits (thought to be due to cord edema causing dorsal column
compression and ischemia).
Sulcal Artery (SA) Syndrome
Because the sulcal (sulcocommissural) arteries alternate
between supplying the left and right sides of the spine,
infarction in the territory of a sulcal artery can lead to a cluster
of findings relatively similar to a Brown-Sequard injury.24,25
Hemiparesis starts at the level of the lesion, with a loss of pain
and temperature sensation starting roughly two dermatomes
below the level of the infarct. As with ASA syndrome, there is
dissociated sensory loss with preservation of dorsal column
signals. Bowel and bladder effects are variable, but continence
is usually preserved. SA syndrome is generally embolic in
etiology, and has been described in association with dissecting
thoracoabdominal aneurysms with mural thrombus,25 with
iatrogenic injury from manipulation of the aorta (see below),
and recently by Li et al (2010)24 in a case of vertebral artery
dissection.
PSA Syndrome
Posterior spinal artery syndrome (PSAS) is substantially
less common than ASAS, likely due to both the dual, inter-
connected PSAs and the greater number of posterior radi-
culomedullary arteries.26 As would be expected, PSAS
involves loss of proprioception and vibration sense below the
level of the lesion; segmental sensory loss to all modalities can
be seen with involvement of the dorsal horns, and involvement
of the lateral corticospinal tracts can lead to paresis. Symptoms
can be unilateral or bilateral.27 Reported causes of PSAS
www.theneurologist.org | 123
Munyon and Hart
include syphilitic arteritis, cholesterol emboli, vertebral dis-
section, and iatrogenic injection or embolization.26–28 As with
ASAS, prognosis appears to correlate with the extent of
longitudinal involvement.
Fibrocartilaginous Emboli
In addition to atheroemboli, thromboemboli from atrial
fibrillation, paradoxical venous emboli, and mycotic emboli,
the spine is susceptible to fibrocartilaginous emboli (FCE)
from herniation of intervertebral disc material into the spinal
vasculature.29 This rare syndrome is classically associated with
axial loading to the spine with accompanying valsalva, but has
also been described after minor trauma, particularly to the
cervical spine. Onset of symptoms may be almost instanta-
neous, but can be delayed by hours or even days.4,30 Autopsy
findings in patients with infarction of the cervical spine and
respiratory compromise have confirmed the presence of carti-
lage within both the arteries and veins of the spinal cord, with a
predilection for medium sized vessels.29,30 While clinical
suspicion can often provide a putative diagnosis, confirmatory
testing is only truly available at autopsy; FCE should therefore
remain a diagnosis of exclusion, with an appropriate workup
for transverse myelitis, multiple sclerosis, and other clinically
appropriate syndromes remaining mandatory even in cases
with classical mechanism and clinical picture.
Ischemia and Aortic Aneurysms
As discussed above, the spinal arteries rely on blood from
the aortic trunk to perfuse the length of the spine. The blood
supply to the lumbar enlargement is particularly vulnerable to
interruption if flow from the aorta is compromised, because the
ASA typically becomes atretic in the lower thoracic spine,
reconstituting distally with the input of the artery of Adam-
kiewicz.3,31 Atherosclerotic disease of the aorta can cause
direct compromise of segmental vessels by atheroma for-
mation, aneurismal dilation, or dissection causing occlusion.
Thromboembolic events from mural thrombi have also been
described in the literature. An imaging clue that may suggest
an aortic etiology is the presence of vertebral body infarction.
Vertebral body ischemia often becomes apparent on MRI
before parenchymal infarction, and can help confirm infarction
as the cause of new neurologic deficit.32 Cheng et al33, show
that vertebral body infarction in the same vascular territory
as the affected level correlates with aortic pathology in their
series.
Many cases of spinal infarction associated with aortic
aneurysms are related to surgical or endovascular repair of the
aneurysms. Paraplegia has been a known complication of aortic
surgery since its inception, and multiple neuroprotective, neu-
romonitoring, and neuroimaging strategies have been devised
to minimize the risks associated with thoracoabdominal aortic
reconstruction and repair.34 The use of somatosensory evoked
potentials (SSEPs), preoperative localization of the artery of
Adamkiewicz, systemic hypothermia, and distal perfusion with
shunting or cardiopulmonary bypass have all improved the
safety profile of aortic aneurysm repair.34,35 Additional suc-
cessful strategies include the preoperative drainage of CSF to
reduce the intradural pressure and thus increase the perfusion
pressure of the spine, as well as direct epidural cooling to
obviate the need for systemic hypothermia. In addition to
reducing the rate of iatrogenic injury, these strategies have
helped to provide insight into potential neuroprotective meas-
ures against spontaneous vascular insult.35–37
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The Neurologist  Volume 19, Number 5, May 2015
Other Iatrogenic Injuries
There are several reports in the literature of spinal
infarction complicating coronary artery bypass grafting, par-
ticularly with use of the intra-aortic balloon pump.38 While
some of these injuries are likely due to systemic hypo-
perfusion, other causes include creation of atheroemboli due to
plaque rupture by the pump, or even direct occlusion by the
balloon of a segmental artery.39,40 Other thoracic procedures
that require mobilization or manipulation of the aorta have also
been associated with infarction, particularly transthoracic
esophagectomy.41 Additionally, there are several reported
cases of spinal infarction causing severe neurologic deficit or
even death following epidural corticosteroid injection for the
relief of radicular pain. While the mechanism of infarction is
incompletely understood, the prominent hypothesis is that
intra-arterial injection of steroids associated with a particulate
vector may cause agglomeration and downstream occlusion of
the vessel. This is borne out by the observation that some
steroid formulations appear to carry significantly higher risk
than others.42,43 Spinal infarction is also a known complication
of spinal angiography, even in the absence of endovascular
intervention.44 Finally, injury to the spinal vasculature is
unsurprisingly a possible complication of spine surgery. While
direct injury accounts for some of these cases, there are also
many reports of ischemia related to deformity correction,
particularly in larger scoliosis repair. While some of these
events may be attributable in part to systemic hypotension,
mechanical impingement on arterial supply or venous drainage
appears to be the principal cause in many such events.45 SSEPs
have therefore become indispensible to the safe correction of
spinal deformity, optimally allowing for the rapid detection
and reversal of mechanical disruption to the vascular supply.
VASCULAR MALFORMATIONS OF THE SPINE
Spinal vascular malformations are diverse and challeng-
ing lesions requiring a multidisciplinary approach to diagnosis
and treatment. Their presentation can range from incidental
radiologic finding to sudden, catastrophic neurologic injury,
and the spectrum of intervening clinical syndromes can mimic
such diverse pathologies as spinal stenosis, multiple sclerosis,
and even intracranial aneurismal rupture.46 Classification of
these complex lesions has evolved substantially based on
advances in histopathologic and radiographic evaluation as
well as surgical and endovascular therapies. In 2002, Spetzler
and colleagues proposed a revised classification system with
three broad categories: vascular neoplasms, spinal aneurysms,
and arteriovenous lesions. The latter category is further sub-
divided into arteriovenous malformations (AVMs) and arte-
riovenous fistulae (AVFs). Finally, AVMs are subdivided into
extradural-intradural, intramedullary, and conus medullaris
subtypes, whereas AVFs are subdivided into extradural,
intradural dorsal, and intradural ventral groups.46,47 An alter-
nate classification system was also proposed in 2002 by
Lasjaunias and colleagues, reflecting the suspected etiology of
a spinal AVM: under this system, type I AVMs are hereditary
genetic lesions (typified by the macrofistula found in patients
with Hereditary Hemorrhagic Telangectasia), type II AVMs
are those found in genetic but nonhereditary syndromes such as
the Cobb and Klippel-Trenaunay-Weber syndromes, and type
III AVMs are sporadic/isolated findings.48 Please see the
above-referenced publications for a comprehensive explan-
ation of these 2 classification schemes.
The multidisciplinary management of spinal vascular
malformations is beyond the scope of this review and
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The Neurologist  Volume 19, Number 5, May 2015 Vascular Disease of the Spine

continues to evolve rapidly. Strategies for endovascular, sur-

gical, and radiosurgical approaches to these lesions abound in
the literature, and selection of treatment modality will depend
on multiple factors including institutional practice patterns and
the preferences of the treating physician.49–53 What is uni-
versally and unsurprisingly true is that the prognosis for these
lesions improves when they are detected before the onset of
major deficit. Prompt diagnosis is therefore of the utmost
importance. Patients with even minor neurological deficit and
stigmata of vascular syndromes such as Cobb or Klippel-
Trenaunay-Weber syndromes should be of particular concern;
examination of the skin for cutaneous angiomata as well as
examination of the extremities for limb hypertrophy is essen-
tial, particularly in younger patients presenting with signs of
spinal cord dysfunction.50,54,55 While these syndromes are rare
enough that no guidelines currently exist, early and thorough
workup (including MRI of the brain and spine as well as spinal
angiography), although expensive and invasive, is likely to FIGURE 3. Cavernous malformation, with large, thin walled
prove cost effective, given the available therapeutic options vascular spaces uninterrupted by normal parenchyma. Reprinted
and the potentially devastating consequences should a mal- with permission from Edgar.59 Copyright [Elsevier], [Cleveland,
formation go undetected. OH]. All permission requests for this image should be made to
the copyright holder.
Another clinical picture that should be of concern is the
gradual evolution of an apparent polyradiculopathy or myel-
opathy in a middle-aged male. Dural arteriovenous fistulae challenge of markedly decreased sensitivity of MRI within the
represent the most common vascular malformation of the spine spinal canal. Nardone and colleagues recently reported the use
and generally present with symptoms related to venous con- of motor evoked potentials to confirm spinal pathology in
gestion or vascular steal rather than frank hemorrhage or patients with acute ischemic insults to the spine and normal
infarction.51,52,56,57 Fluctuating intensity of symptoms should MRIs.33,65 There are also multiple recent reports of improved
raise particular concern for a vascular etiology, as variations in MR diffusion-weighted imaging techniques which may
regional hemodynamics can cause claudication of the spine. As improve the sensitivity for acute spinal infarct.66 The devel-
with many other spinal vascular lesions, prognosis is princi- opment of improved imaging and ancillary testing will be
pally dependent on the extent of neurological deficit at time of critical to any organized effort to study the timely treatment of
diagnosis; it has been estimated that the mean time between spinal ischemia.
symptom onset and diagnosis in dural arteriovenous fistulae is In the interim, iatrogenic ischemic insults to the spine,
around 10.5 months.58,59 Awareness of these lesions and although unfortunate, provide another possible means to
maintenance of an appropriate index of suspicion is therefore explore treatment options for spinal ischemia. Lee and col-
paramount in the avoidance of permanent neurological injury. leagues report a recent case of spinal cord ischemia after
embolization of a high cervical lesion; the patient was treated
Vascular Neoplasms of the Spine with thrombolytics, hypothermia, and hyperbaric oxygen, with
The 2 common vascular neoplasms intrinsic to the spinal improvement from hemiplegia (grade 0/5) at onset to grade 4/5
cord are hemangioblastomas and cavernous angiomas (cav- by the end of the treatment.64 Although it is impossible to
ernomas). Both lesions can also be found in the brain (although generalize from this anecdotal report, it seems likely that
hemangioblastomas are generally limited to the posterior fossa),
and both can present with hemorrhage or local mass effect.60
Finally, both lesions can be found in sporadic and familial forms,
with hemangioblastomas associated with Von Hippel-Lindau
disorder and multiple cavernomas associated with mutations in
CCM1, CCM2, and CCM3.60–62 Although these lesions are pri-
marily located along the cord, they can also be found in the
epidural space or along the nerve roots.60,61,63 Treatment for
symptomatic lesions is generally surgical, although hemangio-
blastomas can be embolized preoperatively to minimize blood
loss. Figures 3 and 4 show characteristic histologic presentations
of cavernomas and hemangioblastomas, respectively.

FUTURE AREAS OF INVESTIGATION

Intervention in Acute Ischemia of the Spine
As with diagnostic modalities, in the realm of therapeutic
intervention, spinal vascular disease lags significantly behind
its cerebrovascular counterpart. Although thrombolytic thera-
pies would appear to have a logical role in the management of
acute spinal stroke, there are substantial barriers to initiation of
thrombolysis within the therapeutic window.4,32,64 In addition
to the decreased clinical awareness of spinal stroke, there is the
FIGURE 4. Hemangioblastoma, with pale, eosinophilic stromal
cells surrounding sinusoidal vascular channels. Reprinted with
permission from Edgar.59 Copyright [Elsevier], [Cleveland, OH].
All permission requests for this image should be made to the
copyright holder.

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Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Munyon and Hart
aggressive intervention by neurocritical care teams may have a
substantive impact on the outcome in cases of spinal ischemia.
Atherosclerotic Disease and the Spinal Column
Low back pain is one of the leading causes of disability in
the industrialized world; although not nearly as potentially
devastating in individual terms as the entities discussed above,
its overall financial impact is tremendous. Observational
studies have shown good correlation between atherosclerotic
burden and degenerative disease of the spine, as well as
between cardiovascular risk factors and evidence of low back
pain. In particular, atherosclerotic stenosis of the lumbar
arteries was strongly correlated with degenerative disease of
the spine in 2 cadaveric studies.67–69 Given the already
extraordinary burden that atherosclerotic disease inflicts upon
the developed world, further prospective studies into a possible
link to back pain as well as efficacy of risk factor management
in alleviating back pain seem warranted.
CONCLUSIONS
Vascular disease of the spine remains poorly understood
and often devastating. Although substantial progress has been
made since the latter half of the 20th century, it remains a fertile
and potentially very rewarding area of research. Even with
recent advances in diagnostic imaging and ancillary testing, the
most important tool for prompt identification of a vascular
insult to the spine remains a high index of suspicion. We hope
that this review, although not comprehensive, will help to
reinforce awareness of the myriad presentations and patho-
physiological mechanisms underlying spinal vascular disease.
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