See the Editorial and the Response in this issue, pp 143–144.

J Neurosurg (Spine 2) 96:145–156, 2002

Modified classification of spinal cord vascular lesions

ROBERT F. SPETZLER, M.D., PAUL W. DETWILER, M.S., M.D., HOWARD A. RIINA, M.D.,
AND RANDALL W. PORTER, M.D.

Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph’s Hospital and
Medical Center, Phoenix, Arizona

The literature on spinal vascular malformations contains a great deal of confusing terminology. Some of the nomen-
clature is inconsistent with the lesions described. Based on the experience of the senior author (R.F.S.) in the treatment
of more than 130 spinal cord vascular lesions and based on a thorough review of the relevant literature, the authors
propose a modified classification system for spinal cord vascular lesions.
Lesions are divided into three primary or broad categories: neoplasms, aneurysms, and arteriovenous lesions.
Neoplastic vascular lesions include hemangioblastomas and cavernous malformations, both of which occur sporadi-
cally and familially. The second category consists of spinal aneurysms, which are rare. The third category, spinal cord
arteriovenous lesions, is divided into arteriovenous fistulas and arteriovenous malformations (AVMs). Arteriovenous
fistulas are subdivided into those that are extradural and those that are intradural, with intradural lesions categorized
as either dorsal or ventral. Arteriovenous malformations are subdivided into extradural–intradural and intradural mal-
formations. Intradural lesions are further divided into intramedullary, intramedullary–extramedullary, and conus
medullaris, a new category of AVM.
This modified classification system for vascular lesions of the spinal cord, based on pathophysiology, neuroimag-
ing features, intraoperative observations, and neuroanatomy, offers several advantages. First, it includes all surgical
vascular lesions that affect the spinal cord. Second, it guides treatment by classifying lesions based on location and
pathophysiology. Finally, it eliminates the confusion produced by the multitude of unrelated nomenclatural terms
found in the literature.

KEY WORDS • spine • arteriovenous malformation • arteriovenous fistula • aneurysm •

cavernous malformation • hemangioblastoma • classification

ESPITE the large number of contributions to our pres- Neoplastic Vascular Lesions

D ent understanding of spinal cord vascular lesions,

the literature reflects considerable confusion (Table
1). The existing nomenclature is not always consistent with
Neoplastic vascular lesions include hemangioblasto-
mas49,52,61,81,85 and cavernous malformations (Table 3),1,5,31,33,
38,47,52,78,84
both of which occur sporadically and familial-
the lesions described.9,20,27,45,49–51,75,82 We propose a modified ly.5,23,49,52,70 Hemangioblastomas are composed of the fol-
classification system for spinal cord vascular lesions based lowing three cell types: endothelial cells, endothelial cell–
on pathophysiology, neuroimaging features, intraoperative associated pericytes, and lipid–laden stromal (interstitial)
observations, and neuroanatomy. In the proposed classifi- cells.35,43 Most hemangioblastomas are intramedullary and
cation system, spinal cord vascular lesions are divided into abut the pial surface, although some occur along nerve
three primary or broad categories (Table 2): neoplasms, roots.19,43,56 Histopathologically, cerebellar and spinal le-
aneurysms, and arteriovenous lesions. Arteriovenous le- sions are similar. The arteries and veins associated with a
sions are further subdivided based on their neuroanatomy. hemangioblastoma can enlarge and simulate an AVM.46,52,56
This classification system eliminates the confusion inher- The syrinx associated with some intramedullary heman-
ent in previous classification systems and can be used to gioblastomas can simulate an astrocytoma.29,30,71
determine the appropriate surgical treatment. Endothelial cells form the vascular channels in cav-
ernous malformations.53 Cavernous malformations are al-
Abbreviations used in this paper: ASA = anterior spinal artery; ways associated with a venous anomaly, which facilitates
AVF = arteriovenous fistula; AVFM = arteriovenous fistulous mal- drainage of normal neurological tissue.79 Delayed venous
formation; AVM = arteriovenous malformation; MR = magnetic infarction is a well-known sequela associated with the
resonance. resection of these venous anomalies. The method for re-

J. Neurosurg: Spine / Volume 96 / March, 2002 145

 R. F. Spetzler, et al.

TABLE 1 section of both cavernous malformations and heman-

Old nomenclature for spinal cord vascular lesion gioblastomas is similar to that used in cases of intradural
intramedullary AVMs—that is, sharp dissection of the
angioma arteriovenosum discrete lesion to separate it from surrounding neurologi-
angioma cavernosum
angioma racemosum
cal tissue. Violation of the surface of either lesion can lead
angioma racemosum arteriovenosum to significant blood loss.
angioma racemosum venosum
angioreticuloma
classic AVM
dorsal extramedullary
epidural
extradural
Foix–Alajouanine
glomus
intradural extramedullary
intramedullary
intraspinal extramedullary
intraspinal extramedullary radiculomeningeal
juvenile
metameric
others
perimedullary
radiculomeningeal fistula
retromedullary
Types I, II, III, & IV
varicosis spinalis
ventral

TABLE 2
Proposed classification of spinal cord vascular malformations
neoplastic vascular lesions
hemangioblastoma
cavernous malformation
spinal aneurysms
arteriovenous fistulas
extradural
intradural
ventral*
dorsal†
AVMs
extradural–intradural
intradural
intramedullary
compact
diffuse
conus medullaris
* Includes the following subtypes: A, small shunt; B, medium shunt;
and C, large shunt.
† Includes the following subtypes: A, single feeder; and B, multiple feeders.

TABLE 3
Summary of clinical characteristics in neoplastic vascular
lesions and spinal cord aneurysms*
Hemangio- Cavernous Spinal Cord
Characteristic blastoma Malformation Aneurysm
FIG. 1. Upper: Anteroposterior angiogram demonstrating sub-
pathophysiology tumor, cyst diapedesis, blood flow, arachnoid hemorrhage associated with a dissecting aneurysm on the
hemorrhage dissection artery of Adamkiewicz. Lower: Intraoperative photograph. The
presentation mass effect mass effect SAH, stroke lesion was wrapped with muslin gauze and has been asymptomatic
diagnostic modality MR imaging, MR imaging, MR imaging more than 5 years. From Vishteh AG, Brown AP, Spetzler RF:
angiography angiography
Aneurysm of the intradural artery Adamkiewicz treated with muslin
previous nomenclature same same same
wrapping: technical case report. Neurosurgery 40:207–209, 1997.
* SAH = subarachnoid hemorrhage. With permission from Lippincott-Williams and Wilkins.

146 J. Neurosurg: Spine / Volume 96 / March, 2002

Classification of spinal vascular lesions

TABLE 4
Summary of clinical characteristics in AVFs
Characteristic Extradural Dorsal Intradural Ventral Intradural

pathophysiology spinal cord compression, venous venous congestion, rare compression (venous aneurysm),
congestion, vascular steal hemorrhage hemorrhage, vascular steal
presentation progressive myelopathy progressive myelopathy progressive myelopathy
diagnostic modality MR imaging, angiography MR imaging, angiography MR imaging, angiography
previous nomenclature epidural dural AVF, long dorsal, Types IVA (small), B (medium),
Type IA, others & C (large), perimedullary

Spinal Cord Aneurysms intradural; the latter are further subdivided into intramed-
Spinal aneurysms unrelated to AVMs are rare. Their ullary, intramedullary–extramedullary, and conus medul-
pathophysiology is related to blood flow and dissection laris AVMs. Conus lesions are a newly proposed category
(Table 3).28,74,79 We have treated two cases of spinal cord (Detwiler, et al., unpublished data).
aneurysms, one located in the thoracic region and the Extradural Arteriovenous Fistulas
other in the cauda equina. Both patients presented with
subarachnoid hemorrhage and sudden-onset low-back Extradural (epidural) AVFs (Fig. 2) rarely require open
pain. The thoracic lesion involved the artery of Adam- surgery because they can be treated very effectively by
kiewicz, which precluded resection because primary re- endovascular procedures. Their pathophysiology and clin-
anastomosis or bypass of the ASA would create an unac- ical presentation have been well described (Table 4).6,33,
ceptable risk to the blood flow to the distal ASA (Fig. 1).
40,57,72
A direct connection between an extradural artery and
Consequently, the thoracic lesion was dissected free from vein leads to the development of a high-flow fistula, en-
the spinal cord and wrapped with muslin gauze.79 Five gorgement of the epidural venous system, compression of
years postoperatively, the patient is functioning normally the spinal cord, and resultant progressive myelopathy. The
and has no symptoms. In the other patient, MR imaging high venous pressure in the epidural venous system can
and angiography of the spine demonstrated an isolated lead to intradural venous hypertension by increasing the
aneurysm located on a radicular artery coursing along the resistance to outflow. The shunting of large quantities of
cauda equina. This lesion was primarily resected, and arterial blood into the venous system can also steal blood
there were no neurological complications. The results ob- flow from the spinal cord.
tained in the latter case demonstrate that an aneurysm of a Intradural Dorsal Arteriovenous Fistulas
radicular artery coursing along the cauda equina can be
resected safely by sacrificing the involved vessel. Intradural AVFs are the most controversial lesions in
terms of origin, pathophysiology, and treatment (Table 4).
Intradural dorsal (Fig. 3) and intradural ventral lesions
Arteriovenous Fistulas and Malformations (Fig. 4) are distinct entities. Terms for intradural dorsal
Spinal cord arteriovenous lesions represent the most AVFs have included long dorsal,49,50 angioma racemo-
confusing category. We have divided these lesions into sum,45 dorsal extramedullary, angioma racemosum veno-
AVFs and AVMs (Tables 4 and 5). Arteriovenous fistulas sum, and Type I.45,49,50,59,82 They are the most common type
are subdivided into extradural and intradural lesions, with of spinal AVF and usually occur in the thoracic region.
the latter being either dorsal or ventral. Arteriovenous The primary pathophysiology of intradural dorsal AVFs
malformations are divided into extradural–intradural and is venous hypertension. In very rare cases do patients

TABLE 5
Summary of clinical characteristics in AVMs
Characteristic Extradural–Intradural Intramedullary Conus Medullaris

pathophysiology compression, vascular steal, hemorrhage, compression, venous hypertension, compression,

hemorrhage vascular steal hemorrhage
presentation pain, progressive myelopathy acute myelopathy, pain, progressive myelopathy, radiculopathy
progressive myelopathy
diagnostic modality MR imaging, angiography, MR imaging, angiography MR imaging, angiography
high-flow, multiple feeders
previous nomenclature juvenile AVM, metameric AVM classic AVM, glomus type none

J. Neurosurg: Spine / Volume 96 / March, 2002 147

 R. F. Spetzler, et al.

FIG. 2. Left: Artist’s rendering of an extradural AVF. Right: Right anteroposterior vertebral artery angiogram reveal-
ing a large serpiginous fistula in the epidural space compressing the spinal cord in a 47-year-old woman who developed
progressive quadriparesis.

FIG. 3. Upper Left and Center: Artist’s rendering of an intradural dorsal AVF. Upper Right: Artist’s rendering of
an intradural dorsal fistula that has its recruited blood supply from several levels. Lower Inset: Selective angiogram
obtained in a 41-year-old woman who developed progressive paraparesis, demonstrating a fistula between the radicu-
lomedullary artery and the coronal venous plexus. The fistula was coagulated and transected.

148 J. Neurosurg: Spine / Volume 96 / March, 2002

Classification of spinal vascular lesions

FIG. 4. Upper Left: Artist’s rendering of an intradural ventral AVF. Upper Right: Anteroposterior spinal angiogram
obtained in a 16-year-old woman with progressive headache and back pain, revealing a small AVF (Type A) located on
the anterior surface of the spinal cord. From Carter LP, Spetzler RF: Spinal arteriovenous malformations. Surgical treat-
ment, in Carter LP, Spetzler RF, Hamilton MG (eds): Neurovascular Surgery. New York: McGraw-Hill, 1995, pp
1197–1212. With permission from McGraw-Hill. Lower Left: Sagittal T2-weighted MR image of the cervical spine
obtained in a 34-year-old woman with progressive left upper-extremity pain, revealing serpiginous flow voids ventral and
lateral to the spinal cord. The lesion was exposed by a cervical corpectomy and durotomy. The AVF was coagulated and
sharply transected, completely relieving the patient’s preoperative pain. From Spetzler RF, Koos WT (eds): Color Atlas
of Microneurosurgery, ed 2. Volume III: Cerebral Revascularization, Extracranial Vascular Disease, and
Intraspinal Pathology. Stuttgart: Georg Thieme Verlag, 1999, p 413. With permission from Georg Thieme Verlag
Medical, Stuttgart. Lower Right: Axial T1-weighted MR images obtained in a 10-year-old boy with severe progressive
thoracolumbar pain, demonstrating a large, circular flow void in the anterior spinal canal that is indenting the cord.
Selective angiography demonstrated a large ventral AVF (Type C). The lesion was occluded using an endovascular
approach. With permission from Barrow Neurological Institute.

J. Neurosurg: Spine / Volume 96 / March, 2002 149

 R. F. Spetzler, et al.

experience clinical deterioration secondary to hemor-

rhage.2,12 Selective angiography demonstrates a character-
istic slow-flow pattern produced by the feeding dorsal
radiculomedullary artery. The artery enters the dura mater
at the dural root sleeve and forms a fistula, arterializing
the coronal venous plexus.39,54,55 These lesions have been
divided into two subtypes: Type A, with a single feeding
artery, and Type B, with multiple feeding arteries (Fig. 3
right).4
Clinically, patients usually present with venous hyper-
tension–induced progressive myelopathy, which can be
misdiagnosed as lumbar stenosis. Aminoff and Logue3
have detailed the clinical prognosis of these lesions in 60
cases. In seven patients who presented with acute-onset
symptoms, there was no further progression. In the remain-
ing 53 patients, symptoms progressed gradually, and five of
FIG. 5. Intraoperative photograph demonstrating the intradural
these patients suffered acute neurological events. Within 3 location of the fistula (F), feeding artery (A), and associated arteri-
years of the onset of functional lower-extremity impair- alized veins (V). The nerve root (N) is seen coursing below the
ment, half of the patients had become severely disabled. fistula.
These outcomes led to the present dogma that progressive
neurological deterioration can only be halted by eliminat-
ing the fistula.
Intraoperative inspection of the dorsal nerve roots
reveals a vascularized pedicle or pedicles, which feed the
AVF in the subarachnoid space (Fig. 5). The presence of an (Fig. 4). They are clearly located in the subarachnoid
AVF in the dural root sleeve has been repeatedly verified space. They originate directly from the ASA and have a
histologically, but no extradural arteriovenous abnormali- direct fistula to an enlarged venous network.10,14 There is
ty exists; otherwise, engorgement of the epidural venous no intervening capillary system. Blood flow through the
plexus would be expected. Although there are numerous lesions is rapid and can produce flow-related aneurysms
extradural and dural vessels, they compromise the dural and venous hypertension.
recruitment process to feed the intradural fistula. This same Intradural ventral Type A fistulas are small shunts in
vascularity can be observed when a meningeal feeding ves- which blood flow is slow and venous hypertension is
sel supplies a cortical AVM. If the fistula were extradural moderate. Types B and C have progressively larger
or within the dura, the drainage would empty into the rich shunts; the latter are characterized by a giant fistula and a
vascular epidural or dural venous system instead of into the markedly distended venous network. As the size and flow
high-resistance coronal venous plexus. When more than of the fistula increases, the signs and symptoms attribut-
one feeding vessel is present (Type B), they form a single able to progressive vascular steal and spinal cord com-
intradural fistula, providing further support for the intradu- pression become more pronounced.
ral location of the AVF.
Repeated measurements of epidural venous pressure, Extradural–Intradural Arteriovenous Malformations
which is the same as central venous pressure, support this Fortunately, extradural–intradural AVMs (Fig. 6) are un-
contention. The mean invasive pressure recorded in 12 pa- common. In the past they have been referred to as juve-
tients with intradural dorsal fistulas was 40 mm Hg within nile, metameric, or Type III AVMs (Table 5).44,69,73,75 They
the coronal venous plexus.12 When the fistula is closed, dis- respect no tissue boundary. These lesions require a multi-
tal pressure drops to a mean of 23 mm Hg, twice the level disciplinary approach in which the team emphasizes em-
of the average epidural venous pressure (same as central bolization of the multiple feeding arteries, sometimes fol-
venous pressure) into which it drains. Furthermore, angiog- lowed by staged resection. Complete surgical resection is
raphy routinely demonstrates slow flow;13 this finding sup- often difficult without significant risk of neurologic mor-
ports the contention that resistance to venous outflow must bidity.
be high where the coronal venous plexus exits the dura.39
Intramedullary Arteriovenous Malformations

Intradural Ventral Arteriovenous Fistulas Intramedullary AVMs have a nidus similar to intracranial
AVMs. In the past these lesions have been referred to as
In 1977, Djindjian, et al.,27 found six intradural ventral classic AVMs, glomus-type lesions, Type II AVMs,
AVFs in a series in which there were 150 lesions. Heros, et angioma arteriovenosum, and angioma racemosum arteri-
al.,41 introduced the term Type IV lesion in 1986, and ovenous lesions.5,21,22,63–65,82,83 They can be supplied by mul-
Gueguen, et al.,34 divided ventral fistulas into three sub- tiple branches of the anterior and posterior spinal arteries
types: 1, 2, and 3. To avoid confusion, Anson and Spetzler4 and are characterized by high pressure, relatively low resis-
reclassified the subtypes as IV-A, IV-B, and IV-C. The tance, and high blood flow (Table 5). Associated aneu-
reclassified subtypes have been maintained in the present rysms are common.17 The nidus can be compact or diffuse.
modification. We have therefore subdivided intramedullary AVMs into
These lesions are located ventrally and in the midline compact (Fig. 7) and diffuse (Fig. 8).

150 J. Neurosurg: Spine / Volume 96 / March, 2002

Classification of spinal vascular lesions

FIG. 6. Left: Artist’s rendering of an extradural–intradural AVM. Right: Coronal T1-weighted MR image obtained
in an 8-year-old girl who developed severe paraparesis, revealing involvement of the spinal cord, vertebral column, and
extraspinal soft tissue.

Based on their experience in the treatment of 26 cases, improve dramatically over time when these lesions are suc-
Djindjian, et al.,27 recommended subclassifying these le- cessfully treated. Their numerous feeding arteries and mul-
sions into three types based on the volume of the spinal tiple niduses create a potential for recurrence.
cord affected: normal volume (Type 1), enlarged volume
(Type 2), and extramedullary and intramedullary AVM
(Type 3) with further subclassifications of each. This sys- Discussion
tem was not adopted clinically because of its complexity. It
also provides no useful prognostic information and can be In early classification schemes the authors stratified le-
confused with other grading systems.4 sions based on pathological type—not location or patho-
The most frequent clinical presentation of intradural in- physiology.51 In 1969 Yaşargil used the histology-based
tramedullary AVMs is hemorrhage- or compression-in- classification proposed by Bergstrand, et al.,16 which di-
duced acute myelopathy. Progressive myelopathy can oc- vided spinal vascular malformations into angioma caver-
cur secondary to vascular steal.26 The diagnosis can be nosum, angioma racemosum, and angioreticuloma. The
made using MR imaging, but angiography is necessary to 17 lesions that he reported were all categorized as an-
define the exact angioarchitecture. Obtaining the correct gioma racemosum.
exposure is the cornerstone of treating these lesions suc- Recent classification systems have stressed the concept
cessfully. of the nidus and accounted for the lesion’s location. Bao
and Ling8 have reported a series of spinal vascular mal-
formations in 80 patients. Similar to Rosenblum, et al.,67
Conus Medullaris Arteriovenous Malformations they classified lesions as intramedullary AVMs, intradural
The conus malformation (Fig. 9, Table 5) is a newly pro- AVFs, dural AVFs, paravertebral AVMs, and Cobbs syn-
posed category of lesions characterized by multiple feeding drome. Intramedullary lesions were subclassified into glo-
arteries, multiple niduses, and complex venous drainage mus AVMs and juvenile AVMs. Intradural AVFs were
(Detwiler, et al., unpublished data). These lesions fit none subclassified into Type I, which were small low-flow sin-
of the aforementioned categories. They have multiple gle-hole fistulas; Type II, which were high-flow single-
direct arteriovenous shunts that derive from the anterior hole fistulas; and Type III, which were giant high-flow fis-
and posterior spinal arteries and have glomus-type niduses tulas with multiple feeding arteries. Between Type I (no
that are usually extramedullary and pial based, but they obvious dilation) and Type III lesions (markedly dilated
may also have an intramedullary component. They are vein), the intradural feeding artery and draining vein dilate
location specific (that is, they are always located in the progressively. Ultimately, Bao and Ling concluded that
conus medullaris and cauda equina) and can extend along their classification system offered limited guidance for the
the entire terminal filum. Symptomatically, they can mani- treatment of spinal vascular malformations. Furthermore,
fest with venous hypertension, compression, or hemor- they added that successful treatment requires a precise
rhage. Unlike other spinal arteriovenous lesions, they fre- understanding of the lesion’s anatomical location and an-
quently produce radiculopathy and myelopathy at the same gioarchitecture.
time. The radicular deficits are often prominent but may Rosenblum, et al.,67 distinguished AVFs from AVMs

J. Neurosurg: Spine / Volume 96 / March, 2002 151

 R. F. Spetzler, et al.

FIG. 7. Left: Artist’s rendering of a compact intramedullary AVM. Right: Anteroposterior right vertebral artery
angiogram obtained in a 32-year-old woman who developed acute onset of upper- and lower-extremity weakness,
demonstrating multiple fistulas feeding an intramedullary malformation and associated aneurysm. The malformation and
associated aneurysm were exposed and resected by a laminectomy and dorsal myelotomy. Postoperative right lateral ver-
tebral angiography (not shown) demonstrated the patency of the ASA and no residual malformation.

based on pathogenesis, pathophysiology, radiographic nile and glomus) and direct AVFs, which were extra-
findings, and response to treatment in 81 patients. Intra- medullary or intramedullary in location. Dural AVFs were
dural AVMs were subclassified as intramedullary (juve- supplied by an artery originating from the dura and drain-

FIG. 8. Artist’s rendering of a diffuse intramedullary AVM.

152 J. Neurosurg: Spine / Volume 96 / March, 2002

Classification of spinal vascular lesions

FIG. 9. Upper: Artist’s rendering of a conus AVM. Selective angiography (not shown) demonstrated a T-10 fistula in a
36-year-old Hispanic man with progressive paraparesis and lower-extremity pain. The fistula fed into the coronal venous sys-
tem. Lower: Intraoperative photograph showing exposure of the conus and cauda equina achieved through a multilevel lum-
bar laminoplasty. The lesion consisted of multiple fistulas feeding the malformation located on the posterior surface of the
conus. Preoperative spinal angiography (not shown) showed only one fistula fed from above. Intraoperatively, three additional
fistulas, originating from vessels coursing along the cauda equina nerve roots and being fed from below, were identified. The
lesion was resected. The patient’s neurological condition remained stable, and her lower-extremity pain resolved.

ing into the coronal venous plexus via an arteriovenous lesions that affect the spinal cord. Second, it guides treat-
shunt located in the intervertebral foramen.68 The arterio- ment by classifying arteriovenous lesions based on their
venous shunt in the intramedullary lesion was located par- location and pathophysiology. Finally, it eliminates the
tially within the spinal cord or pia mater and supplied by confusion produced by the multitude of unrelated nomen-
the medullary arteries. clature used in the literature.
Borden and colleagues18 have proposed a classification
for both cranial and spinal dural AVFMs. They introduced Treatment of Spinal Vascular Lesions
the term dural AVFM to account for single dural AVMs Hemangioblastomas and cavernous malformations
with multiple fistulas. Type I lesions drain directly into the should be treated with resection. Embolization plays a
dural venous sinus or meningeal vein intracranially or into
the Batson epidural venous plexus in the spine. Type II
lesions drain into the venous sinus and have retrograde
flow into subarachnoid veins or into both the epidural
venous plexus and perimedullary veins. Type III AVFMs
drain into subarachnoid veins. They are supplied by a TABLE 6
meningeal branch of a radicular artery and drain into the Spinal cord arteriovenous lesions treated surgically
coronal venous plexus. This is the same lesion termed by the authors
“angioma racemosum venosum” by Wyburn-Mason,82
Postoperative Status (%)
Type I by Di Chiro with colleagues,24 and Wener25 and in-
tradural dorsal in our modified classification. No. of Lesions Improved Same Worse
Our proposed classification represents an evolution
extradural AVF 2 100 0 0
from the earlier, most commonly used classification sys- dorsal intradural AVF 32 80 10 10*
tem (Types I–IV).4,8,16,18,24,25,67,82 The new classification in- ventral intradural AVF 7 75 25 0
cludes categories for the previously undescribed conus extradural–intradural AVM 5 100 0 0
medullaris AVM as well as aneurysms and vascular neo- intramedullary AVM† 27 68 29 8
plasms, all of which are being diagnosed with increasing conus AVM 16 35 65 0
frequency.79,80 We have adopted this modified classifica- * Vein stripping occurred.
tion for several reasons. First, it includes all vascular † Characterized by 8% residual tumor.

J. Neurosurg: Spine / Volume 96 / March, 2002 153

 R. F. Spetzler, et al.

small role in the treatment of hemangioblastomas but not 3. Aminoff MJ, Logue V: The prognosis of patients with spinal
in the treatment of cavernous malformations. In both of vascular malformations. Brain 97:211–218, 1974
our patients with spinal cord aneurysms cure was effected 4. Anson JA, Spetzler RF: Classification of spinal arteriovenous
by surgical means.77,79 malformations and implications for treatment. BNI Quarterly
8:2–8, 1992
Extradural AVFs are primarily treated endovascularly, 5. Anson JA, Spetzler RF: Surgical resection of intramedullary spi-
as are extradural–intradural AVMs. In the latter case, sur- nal cord cavernous malformations. J Neurosurg 78:446–451,
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tion,11,15 or both. In some institutions embolization is used ology 36:490–491, 1994
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mum for two reasons. First, the rates of recurrence and embolization for obliteration of an intramedullary AVM of the
progressive myelopathy associated with embolization are spinal cord. Technical note. J Neurosurg 47:119–125, 1977
high.37,58,60 Second, in surgical series, the reported morbid- 8. Bao Y-H, Ling F: Classification and therapeutic modalities of
spinal vascular malformations in 80 patients. Neurosurgery
ity rate is extremely low and the success rate high.42,45,48,62,76 40:75–81, 1997
Small- and medium-sized intradural ventral fistulas can 9. Barontini F, Tonini R: A case of necrotic myelopathy due to
be treated surgically whereas large-sized fistulas are best a spinal vascular malformation (Foix-Alajouanine’s disease).
suited to occlusion by embolization.32,36,66 During surgery, Schweiz Arch Neurol Neurochir Psychiatr 128:5–12, 1981
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be demonstrated on postoperative angiography and the 11. Barth MO, Chiras J, Rose M, et al: [Results of embolization of
patient’s neurological status worsened. Although intradu- spinal dural arteriovenous fistulas with perimedullary venous
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12. Bederson JB, Spetzler RF: Pathophysiology of type I spinal dural
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entire lesion was not possible. Finally, conus AVMs can hypertension and the effects of venous outflow obstruction in a
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definitive treatment is best achieved using aggressive em- 14. Benhaiem-Sigaux N, Zerah M, Gherardi R, et al: A retromedul-
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technical case report. Neurosurgery 40:207–209, 1997 Address reprint requests to: Robert F. Spetzler, M.D., Neuroscience
80. Vishteh AG, Sankhla S, Anson JA, et al: Surgical resection of Publications, Barrow Neurological Institute, 350 West Thomas Road,
intramedullary spinal cord cavernous malformations: delayed Phoenix, Arizona 85013–4496. email: [email protected].

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