DURAL AVF –

CLASSIFICATION AND
MANAGEMENT

Presented By : Rakesh K Singh

 BRIEF ANATOMY OF DURA MATER
¾ Dura mater, means "tough mother" (Latin - Toughest tissue
in the body).

¾ Embryologically develops from the mesoderm surrounding

the neural tube.

¾ Forms a tough and fibrous outer covering of CNS

¾ Consists of two layers :

- Outer or endosteal layer (Endocranium)
- Inner or meningeal layer – continuous with
spinal dura mater
 Blood supply of cerebral durameter
A - Vault – Middle meningeal artery
B - Ant. cranial fossa - Ant. Ethmoidal artery
- Post. Ethmoidal artery
- Opthalmic artery
C - Middle cranial fossa - Middle meningeal artery
- Accessory meningeal artery
- ICA
- Ascending pharyngeal artery
D – Post. cranial fossa - Vertebral artery
- Occipital arteries
- Ascending pharyngeal artery
 DURAL AVFs
:INTRODUCTION:
¾ Abnormal direct connection (fistula) between meningeal
artery and a meningeal vein or dural venous sinus within
dural leaflets.

¾ Nidus of AV shunting is contained solely within the dural

leaflets, and is distinguishes DAVFs from pial AVM.

¾ Arterial supply is usually derived from dural arteries and

less frequently from osseous branches.
¾ May drain through an adjacent dural sinus and/or other
dural and leptomeningeal venous channels.

¾ Retrograde leptomeningeal venous drainage is often

tortuous and variceal, and may be frankly aneurysmal.

¾ Broadly divided into –

1 – Intracranial dural AVFs
2 – Spinal dural AVFs
Figure shows a cranial DAVF as seen on a side-on or lateral view of a cerebral
angiogram. The small fistulae are in the dura (red circles). The arterial supply is
marked by the red arrows (branches of the external and internal carotid arteries). In
this case, there is no distinct draining vein, but there is a thrombosed dural venous
sinus in the vicinity of the fistulae. The blocked or thrombosed portion of the sinus is
shown by dark-blue arrow heads, the normal open portion is shown by light-blue arrow
heads.
 Intracranial Dural AV F
:EPIDEMIOLOGY: (Brown RJ et al, 1996).

¾ Incidence= 0.17 per 100,000 persons

¾ Comprise 10-15% of intracranial vascular

malformation.

¾ Found in 1.1% of consecutive angiograms and one

fifth as common as AVMs.
¾ Usually solitary (Multiple in < 7% cases, Malik et al,1988
May occur anywhere in the dura mater covering the
brain (M.C. site - CS and T-S sinuses).

¾ Usually located within or near the wall of a dural venous

sinus, which is often narrowed or obstructed.

¾ Most commonly involve occipital and meningeal arteries.

 ETIOLOGY
¾ May be due to : - Idiopathic – Most common
- Sinus thrombosis
- Head trauma : linear # across T.-sinus
- Cranial surgery
- Hormonal influence
- Sinusitis : in CCF
- Congenital (rare)

¾ May associated with: - Meningioma

- Ehlers-Danlos syndrome
- Fibromuscular dysplasia
- NF-1
 NATURAL HISTORY

¾ Highly variable

¾ No or benign symptoms for many years.

¾ May be incidental finding on angiogram.

¾ May exhibit aggressive behaviours.

¾ Benign DAVFs may spontaneously thrombose, occasionally

after angiography or compression.

¾ Acute or gradual shift of grade may occur.

¾ Overall risk of hemorrhage – 1.6% / yr (Brown et al.)

¾ Annual morbidity and mortality rates of with an aggressive

presentation differ widely (1.8% to 20% per year).

¾ Risk of re bleed – 35% within 2 weeks (Duffau et at.1999).

A largest prospective natural history study:
(Van Dijk et al, 2002)

- Total pt.= 236

- Two groups :

A – Pt. with CVR :

N = 119
- Successful curative treatment =96
- Lost follow up = 3

¾ Rest 20 pt.:
- Refused to treatment = 14
- Partial treatment = 6
Mean follow up = 4.3 yr
¾ RESULT :
- Annual risk of ICH and NHND = 15 % (8.1+6.9)
- Annual mortality rate = 10.4%

B – Pt. without CVR :

N =117
- Lost follow up = 5
- Rest 112 pts. median follow up = 27.9 months
- Only observation = 68
- Palliative treatment =44
(Endovascular = 43,Surgery=1)
¾ RESULT:
- Pts. with benign and well tolerated clinical course
without any ICH or NHND =98%
- Long term angiographic follow up (N=50) shows 2-3%
of risk of developing CVR
 Pathophysiology
¾ Mostly acquired, idiopathic lesion.

¾ Varies in complexity from a single shunt in small region of

the dura to an extensive malformation.

¾ Two theories for origin :

1- Opening of preexisting microscopic vascular channels

within duramater after venous hypertension secondary
to sinus thrombosis.
 2 - Formation of new vascular channels within duramater,
a process stimulated and regulated by angiogenic
factors.
(Pathology specimen contain- Angiogenic growth
factor and basic fibroblast growth factor)

¾ With aggressive symptoms, lesions seem to grow by

recruitment of arterial feeders into nidus, favored by the
AV shunting (Sump effect . Awad et al,1990).

¾ Hypertrophy of dural arteries and reappearance of involuted

embryonic arteries occur.
¾ Formed as a consequence of thrombosis and subsequent
recanalization of dural venous sinuses.

¾ Sinus thrombosis seems to be related to either initiating or

healing phases of the disease progression.

¾ Postmenopausal and pregnant females more commonly

experience CS and TS DAVF - thus possibility of a
hormonal influence can not be ruled out.
¾ Progressive arterialization of the pathologic dural leaflet
results in hypertension in adjacent leptomeningeal channels.

¾ Thus retrograde leptomeningeal venous drainage may

occur.

¾ Under arterialized pressure, these venous channels may

become tortuous and eventually, varicose or aneurysmal.

¾ Demyelination may occur around leptomeningeal veins

due to venous hypertension.
 LOCATION

¾ Common locations of dural AVFs:

1 = Cavernous sinus (CS) (20%-40% of cases)

2 = Transverse-sigmoid sinus (TS, SS) (20%-60%)
3 = Tentorium (12%-14%),
4 = Superior sagittal sinus (SSS) (8%), and
5 = Anterior fossa (2%-3%).

¾ CCF and T-S D-AVFs more common in female.

¾ Ant. Cranial fossa and Tentorial D-AVFs more in males.

(IPS = inferior petrosal sinus, ISS = inferior sagittal sinus, JV = jugular vein, MS =
marginal sinus, OS = occipital sinus, SPS =superior petrosal sinus.)
(IPS = inferior petrosal sinus, ISS = inferior sagittal sinus, JV = jugular vein, MS =
marginal sinus, OS = occipital sinus, SPS =superior petrosal sinus.)
 Classification

¾ Most classification systems are based on :

1- Presence of venous stasis or occlusion

2- Direction of flow
3- Presence of leptomeningeal venous drainage,
aneurysm and single or multiple fistulas.
 CLINICAL FEATURES

¾ More common in adults (Age= 40-60 yr).

¾ May be asymptomatic or benign symptoms to fatal

hemorrhage.

¾ Two groups of symptoms –

A- Non aggressive : -Tinnitus (pulsatile)- M.C.
- Headache
B- Aggressive : -Intracranial hemorrhage
-Non hemorrhagic neurological
deficits
Symptoms of cranial dural AVF
¾ Tinnitus :
- Pulsatile
- With high flow fistula in TS or SS location
- Due to recruitment of arterial feeders and close
proximity to middle ear
- Bruit can be auscultated
- Compression of carotid artery, jugular vein or occipital
artery may decrease the bruit intensity.

¾ Headache :
- U/L or generalized
- Increase on physical activity or position change
- Due to .. Engorgement of venous collaterals and dural
sinus distention, edema, compression of 5th nerve or
inflammation d/t venous thrombosis.
¾ Hemorrhage :
- May be - Intra ventricular
- Intraparenchymal
- Subarachnoid
- Subdural

- May be distant from the fistula site.

¾ Most predictive predisposing factors for aggressive
symptom are :
1 - Venous drainage pattern
- Cortical venous drainage
- Venous ecstasies
- Galenic venous drainage

2- Location
- Tentorial incisura (I.=8.4%)
- Ant. Cranial fossa

¾ At the transverse-sigmoid sinuses and cavernous sinuses

- least likely associated with aggressive symptoms.
¾ Any change in tinnitus or bruit warrants re-evaluation.
 INVESTIGATION:

1- CT :
-N
- Hemorrhage
- Hcp

2- MRI and MRA :

-N
- Dilated pial vessels
- White matter edema
- Stenosis or occlusion of dural sinuses
3- Intra arterial catheter angiography
- Gold standard
- Premature visualization of intracranial veins or
venous sinuses during arterial phase- Characteristic
- Best views of nidus are often obtained during the
ultra early arterial phase and by injections of
distant arterial feeders.
- Exact site of CVR must be determined
 MANAGEMENT OPTIONS

1 - Observation
2 – Transarterial Embolisation
3 – Transvenous Embolisation
4 – Surgery:
5 - Stereo tactic Radio surgery
¾ OBSERVATION:

- In lesions without CVR

- Asymptomatic or tolerable symptoms
- Follow up with serial MRI, MRA and DSA after 3 yr
- 2-3% chance of developing CVR
- Any change in symptoms needs evaluation.
¾ TRANSARTERIAL EMBOLIZATION :
- Rarely curative
- Best agent : n-BCA
- Commonly used as an adjuvant before surgery or
TVE
- Palliative treatment for benign lesions with intolerable
symptoms

Complications:
- Stroke
- conversion of benign fistula into aggressive one
¾ TRANSVENOUS EMBOLIZATION :

- Through transfemoral route

- Sacrifice the involve dural venous sinus
- Only done when venous phase of DSA shows that the
involved sinus is not used by brain and alternate
pathway for venous drainage is developed

Complications :
- Venous hypertension and infarction
- SAH
¾ SURGERY:
Indication –
- When endovascular therapy fails
- When endovascular therapy not feasible

- 3 strategies :
1- Obtain venous access for direct packing
2- Complete excision of AVF
3- Disconnection of CVR alone
Complications:
1- Technically difficult
2- Risk of exsanguination
2- Risk of venous infarction
Management Strategy for D-AVF without CVR
Management Strategy for D-AVF with CVR
Management Strategy for D-AVF with CVR and sinus drainage
 STEREOTACTIC RADIOSURGERY
¾ Recent studies have demonstrated promising results in
selected pts.

¾ Pan and colleagues (2002) demonstrated complete

angiographic resolution of the nidus in 47% of 19 T-S sinus
DAVFs (Median follow up=19 month).

¾ O,leary S et al (2002) in 17 pts of various sites lesion,

demonstrated complete obliteration in 10 pts.
- 2 pts showed considerable reduction in size.
- 1 pts showed detoriation.
- In 4 pts, no angiographic follow up available
¾ Koebbe CJ et al, (2005) in 18 pts (10 pt with embo) with
mean follow up of 43 months and dose 20 GY showed-
- Angiographic obliteration in all 8 cases
- No e/o lesion in 4 pts and decreased size in 3 pts assessed
by MRA/CTA.
- 2 pts developed neurological deficits after embolization
before radiosurgery.
- Temporary hemiparesis in 1 pts.
¾ LIMITATIONS:

1- Long interval b/w treatment and therapeutic effect

- Not acceptable in pts with CVR
2- Without CVR, most pts can be managed with
observation alone or palliative transarterial embolization.
 CAVERNOUS SINUS DURAL AVFs

¾ Cavernous Sinus is the 2nd m. c. involved region.

¾ First recorded treatment by Travers in 1809.

(Ligation of CCA)
¾ Dandy (1935) performed a craniotomy and Clipped the ICA
just proximal to p-com artery.

¾ Nunnelly (1865) and Rivington (1874) described the

pathology and site of lesion.
¾ Parkinson (1965) opened the CS and directly occlude the
fistula.

¾ Serbinenko (1974) developed and used a detachable balloon

for vascular occlusion.

¾ Guglielmi (1991) introduced soft detachable and retrieval

platinum coils.
AA-
– Lateral view

Relation of structures in cavernous sinus

 Classification of CCF
Direct CCF (High-flow)
Most common (70-90%)
75% traumatic (assoc. basal
skull fx)
Defect in intra-cavernous ICA
Spontaneous: mid-age, HTN,
post-menopausal female

Indirect CCF (Low-flow)

Dural Shunt: Meningeal arteries
(ICA, ECA or combo)
Can be spontaneous or
traumatic also
More insidious onset of
symptoms
 Barrow’s Classification (1985)

Type A: Direct between ICA and

CS

Type B: Dural ICA branches to

CS (uncommon)

Type C: Dural ECA branches to

CS

Type D: Dural ICA & ECA

branches to CS
 Angiographic Pattern Classification
(Dae Chul Suh et al. : 2005)

- Categorized by the degrees and patterns of prominent

arteriovenous shunt as well as venous flow.

Type – 1: Proliferative type (40%)

Type – 2: Restrictive type (40%)
Type – 3: Late restrictive type (20%)
¾ Principle ECA feeders are :
1- Int. maxillary artery – distal branches
2- MMA – meningeal branch
3- Accessory meningeal artery – meningeal branch
4- Ascending pharyngeal artery- meningeal branch

¾ ICA feeders – Dural branches from cavernous segment-C5

branches and the inferolateral trunk.
Carotid Cavernous Sinus Fistula
• Symptoms: • Signs:
– Double vision – Proptosis
– eyelid droop – chemosis
– facial pain/numbness – ↑ IOP
– ocular pulse pressure
– orbital/temple bruit
– ptosis
– miosis
– ophthalmoplegia (most commonly
CN VI)
– facial hypoesthesia
– optic disk swelling
– retinal venous dilatation
– intraretinal hemorrhage
Classic Sign:
Limbal injection with arterialized conjunctival & episcleral vessels
¾ Symptoms and signs are usually benign because CS has
sufficient venous drainage routes.

¾ Because the symptoms are very diverse and they fluctuate,

analysis of the symptomatology related to the angiographic
findings does not always correspond to the disease status.

¾ Symptoms depend on the direction of venous drainage.

¾ Severity depends on the rate of blood flow through shunt.

¾ C/L eye symptoms in 11% of patients.

¾ More common in -Postmenopausal elderly women

- Pregnancy
- Sphenoid sinusitis
- Trauma

¾ Over all mortality = 1-2%

 Pathophysiology
¾ Retrograde venous drainage into orbit :
–Venous HTN
Æ Enlarged EOM’s
Æ Restriction Æ Diplopia
Æ Proptosis Æ Exposure keratopathy

Æ Chemosis & Injection Æ Red Eye

Æ Increased episcleral & vortex venous pressure
Æ Increased IOP Æ Secondary Glaucoma

–Venous & Arterial Stasis

ÆDecreased ocular/retinal perfusion
Æ Decreased visual acuity
Æ Anterior Segment Ischemia

Æ Decreased perfusion to intra CS cranial nerves

Æ Ophthalmoplegias Æ Diplopia
 Pathophysiology

¾ Venous HTN in other directions may also occur -

– Retrograde cortical venous drainage

• 10-55% of CCF cases
Æ Severe HA
Æ Contralateral neurological deficits
Æ 30-40% risk of intracerebral hemorrhage
Æ May be fatal
 Diagnostic Studies
Orbital color Doppler U/S:
- Reversed, arterialized flow
in S. Ophthalmic v.

CT/MRI:
- Enlarged S. Ophthalmic v.
- Enlarged EOM
- Proptosis

Cerebral Angiography
-“Gold Standard” diagnosis
- View ICA, ECA, &
vertebral circulations
Schematic presentation of the types and progression of CSDAVF.

.
Schematic presentation of the types and progression of CSDAVF
 Treatment Options for Cavernous Sinus Dural AVFs
(Listed in increasing order of potential risk and technical difficulty)
 Management
¾ Indications for Tx:
- Lack of spontaneous closure
- Risk to eye/vision,
- Intolerable symptoms
-“High-risk” for stroke,
-Venous thrombosis,
-Mental status changes
Surgical closure
- Rare in last 30 years
- Can be salvage option in : In failed embolization,
contraindication of embo, in occluded ICA with
patent fistula d/t previous intervention.

Interventional Radiology :
(balloon occlusion/embolization)
- Primary treatment modality.
- Trans-arterial route directly through tear or
embolization of feeding vessels.
- Trans-venous through sup. ophthal. v. or inferior
petrosal sinus.
 Endovascular Management
•Meyers, et al. Am J Ophthalmology, 2002
–Retrospective interventional case series
•121 (90%) patients were cured clinically (mean f/u 56 mos)
•4% patients with moderate/severe disability
•6% with symptomatic complications
•133/135 consecutive cases had tx
–Cerebral infarction, Decreased VA (2), Diabetes Insipidus, orbital eccymosis,
retroperitoneal hematoma, DVT’s (2)
•No operative mortality

Conclusions:
•High success rate
•Low complication/morbidity rate
•Patient’s ocular symptoms may be transiently worsened post
procedure
 Superior Ophthalmic Vein Approach
¾ First proposed by Hanneken, et al. in 1989.
–Direct access to cavernous sinus
Potential complications: puncture of S. ophthal v.,
orbital hemorrhage, infection, trochlea or other structure
damage

Conclusions
•Especially effective with significant ICA
contribution to CCF
•“technically straightforward, safe, and
effective treatment”
¾ RADIORURGERY :
- May be effective in treating some indirect CCF.
(Type –B,C or D)
Dose : 20 Gy
¾ LIMITATIONS are –
- Takes a mean of 7.5 month to show effect on lesion.
- Inappropriate for pts. with progressive visual loss,
neurological deficit, or cortical venous drainage.
- 15% recurrence rate (Pollock et al.)
- Risk of radiation induced malignancies.

¾ Thus precise role remains to be determined.

 Treatment of Choice in CCF

Type – A : TA detachable balloon (Silicon balloons

filled with HEMA)

Type – B : TVE

Type – C : TAE

Type – D : TAE, if fail then – TVE or Surgery

 Prognosis
¾ Direct CCF:
-Poor visual prognosis (90% with severe vision loss)
. -Ocular & optic nerve damage,
-Exposure keratopathy, secondary glaucoma,
-Ant segment ischemia,
-CRVO
-Ischemic ON
-Concern for intra-cerebral hemorrhage
¾ Indirect CCF:
-Prognosis less severe
-Also concern for intra-cerebral hemorrhage
-Exacerbation & remission is the hallmark
-May close spontaneously (10-60%)
 Transverse-Sigmoid Sinus Dural AVFs
¾ Most common location of IC dural AVFs.

¾ Most common symptoms are benign (pulsatile tinnitus and

headache).

¾ More frequently associated with hemorrhagic and non

hemorrhagic aggressive neurologic symptoms than CS dural
AVFs.

¾ Spontaneous regression is relatively rare (approx.5%) and

usually occurs following hemorrhagic events.
¾ Epicenter of fistula most often found at junction between
the transverse and sigmoid sinus.

¾ Arterial feeders :
1- Occipital artery branches
2- MMA – posterior and petrosal branches
3- ICA- marginal tentorial branch
4- Post. auricular artery
5- Vertebral artery – post. meningeal branch
6- A. pharyngeal artery – meningeal branch
¾ Require treatment because of :
- Low rate of spontaneous regression without
symptomatic events.
- Relatively high rate of aggressive symptoms.

¾ Recent studies of stereo tactic radiation therapy for

transverse-sigmoid sinus dural AVFs showed a relatively
high occlusion rate of the AVF (approximately 60% of
cases) several months after treatment without significant
complications.
¾ Although TVE showed higher occlusion rates (80%–100%
of cases), this procedure requires sacrifice of sinus flow and
may cause venous infarction if the sinus contributes to the
drainage of normal cerebral tissue.

¾ The rate of permanent complications in TVE is

approximately 4%.
¾ In the treatment of Grade 2 lesions, occlusion of the normal
cortical venous drainage system should be avoided.

¾ When there is a high risk of normal cortical venous drainage

sacrifice at TVE, other treatments such as radiation therapy
should be applied.

¾ Surgical isolation of the sinus with preservation of normal

cortical venous drainage may also be performed but is more
invasive.
¾ Grade 3 lesions can be treated with TVE, during which
time the affected sinus and retrograde cortical drainage
outlet should be tightly packed with coils.

¾ Loose packing might cause recanalization, resulting in

delayed hemorrhagic infarction after embolization.
¾ Grade 4 lesions are the most difficult type of dural AVF to
treat.

¾ The standard techniques combine endovascular and

neurosurgical elements (eg, TVE combined with a surgical
approach).

¾ In patients in poor general condition, other techniques (eg,

TVE combined with other approaches, TAE with n-butyl-
2-cyanoacrylate) may be used; however, they require more
skill.
 Recanalization
of a grade 3
transverse-
sigmoid sinus
dural AVF after
TVE. (a) Early
arterial phase
left common
carotid
angiogram
shows a Grade 3
transverse-
sigmoid sinus
dural AVF.
outlet (arrows).
Recanalization of
a grade 3
transverse-
sigmoid sinus
dural AVF after
TVE.
(b) Late arterial
phase left
common carotid
angiogram shows
that the left
sigmoid sinus is
occluded (arrow)
and the dural
AVF drains
mainly into
cortical veins and
the posterior
condylar vein
(arrowheads).
Recanalization
of a grade 3
transverse-
sigmoid sinus
dural AVF
after TVE.

(c) Superselective
venogram shows
a microcatheter
that has been
advanced via the
posterior condylar
vein (arrowheads)
into the affected
sinus.
 Recanalizatio
n of a grade 3
transverse-
sigmoid sinus
dural AVF
after TVE.

d) Left
common
carotid
angiogram
obtained after
TVE shows
disappearance
of the AVF.
Recanalization
of a grade 3
transverse-
sigmoid sinus
dural AVF after
TVE.
(e) CT scan
obtained 2
months after
TVE shows a
massive
hemorrhage in
the left temporal
lobe.
 Recanalization of
a grade 3
transverse-
sigmoid sinus
dural AVF after
TVE.
f) Left common
carotid angiogram
shows
recanalization of
the dural AVF at
the retrograde
cortical drainage
outlet (arrows).
Treatment Options for Transverse-Sigmoid
Sinus Dural AVFs

Treatment options are listed in increasing order of potential risk and

technical difficulty.
(Numbers in parentheses indicate percentages of cases)
Recommended Treatment Strategies for
Transverse-Sigmoid Sinus Dural AVFs
 Tentorial Dural AVFs
¾ Incidence of intracranial hemorrhage _ 60% to 74%.

¾ May cause fatal bleeding in the posterior fossa.

¾ Tentorial dural AVFs drain through the retrograde

leptomeningeal-cortical venous drainage system only
(Cognard types III and IV, Borden type III), resulting in a
high risk of hemorrhagic or nonhemorrhagic aggressive
symptoms (19% and 10% of cases per year, respectively).
¾ Complete cure requires aggressive treatment.

¾ Interventional and surgical procedures are both used to

disconnect the venous drainage system.

¾ Because of the deep-seated location of such lesions, the

difficult access route, and the need for n-butyl- 2-
cyanoacrylate, these techniques require a high level of skill.
¾ Treatment selection depends on :
- Skill of the neurosurgeon and interventional
radiologist
- On lesion accessibility

¾ Stereotactic radiosurgery should be considered an option,

especially in older patients or in those in poor general
condition.
Treatment Options for Tentorial Dural AVFs
Type IV
tentorial dural
AVF with
intracranial
hemorrhage.
(a)
Unenhanced
CT scan
shows
intracranial
hemorrhage in
the left
occipital lobe
and the lateral
ventricle.
 Type IV
tentorial dural
AVF with
intracranial
hemorrhage.
(b) Left external
carotid
angiogram shows
a tentorial dural
AVF
(arrowheads)
with
leptomeningeal-
cortical venous
drainage and
venous ectasia
(arrow).
Type IV tentorial
dural AVF with
intracranial
hemorrhage

(c) Digital
subtraction
angiogram
obtained during
the injection of
diluted n-butyl-2
cyanoacrylate
demonstrates the
tip of a
microcatheter
(arrow).
Type IV
tentorial
dural AVF
with
intracranial
hemorrhage
(d) Left
common
carotid
angiogram
obtained after
TAE shows
complete
obliteration of
the AVF.
 SDAVFs
Arterial Supply of the Spinal Cord

¾ By the vertebral arteries.

¾ Also from the branches ultimately from the thoracic and

abdominal aorta ( Radicular arteries).

¾ Each vertebral artery (or PICA) gives rise to a posterior

spinal artery, which proceeds along the line of attachment
of the dorsal roots.

¾ Each vertebral artery also gives rise to an anterior spinal

artery.
¾ The two ant. spinal arteries fuse to form a single midline
vessel along the anterior median fissure.

¾ The posterior spinal arteries and the anterior spinal artery

supply upper cervical levels with from the vertebral
arteries.

¾ Below this, all 3 spinal arteries form a more or less

continuous series of anastomoses with radicular arteries.
¾ Great radicular artery (of Adamkiewicz), present at the
spinal cord level T12-L2 may provide the entire arterial
supply for the caudal 2/3 of the spinal cord.

¾ Very long anterior spinal artery is usually a continuous

vessel for the length of the spinal cord.

¾ It gives rise to hundreds of central and circumferential

branches.

¾ These supply the anterior 2/3 of the spinal cord.

( posterior horn and a variable portion of the lateral
corticospinal tract )
¾ The posterior spinal arteries are really more of a plexiform
network of small arteries.

¾ They supply the posterior columns, substantia gelatinosa,

dorsal root entry zone, and a variable portion of the lateral
corticospinal tract.
Venous Drainage of the Spinal Cord

¾ By 6 irregular, plexiform channels.

¾ There is one along:

- The anterior and posterior midlines;
- Along the line of attachment of the dorsal roots of
each side;
- Along the line of attachment of the ventral roots of
each side.
¾ These are drained by the radicular veins.

¾ Each, in turn empty into the epidural venous plexus.

 Spinal Dural AVFs
INTRODUCTION :
¾ Rare and enigmatic disease entity
¾ Also known as
- Long dorsal AVM
- Angioma racemosum venosum (Wyburn-Mason)
- Malformation retromedullaire (Djindjian)
- Dorsal extra medullary AVMs

¾ Clinical features and structural changes have been

recognized since 1926 (Foix and Alajouanine), and the
pathophysiology and the essentials of treatment since 1974.
¾ Etiology unknown
¾ Probably the first successful operation on an SDAVF was
performed by Elsberg in 1916.

¾ He ligated and excised an enlarged and thickened vein at

the level of T8 in a patient with a sensory level at T9.

¾ The patient made a full recovery

¾ Kendall and Logue (1977) showed that the site of the fistula
was not located in the spinal cord but on or in the dural root
sleeve.
¾ Caudal end of the spinal -first affected by congestive edema
and ultimately infarction, regardless of the level of the
fistula.

¾ Thus initial clinical features often consist of sensory and

motor symptoms ascending from the feet, suggesting a
polyneuropathy or polyradiculopathy
 Epidemiology
¾ SDAVFs are rare, but still make up the most common
vascular anomaly of the spine, with a proportion of 60–
80%.

¾ 5–10/million/year in the general population

¾ Underdiagnosed disease.

¾ Mostly in middle aged men.

¾ Mean age at the time of diagnosis is 55–60 years.

¾ Patients under the age of 30 are rarely reported

¾ M:F = 5:1

¾ M. C. location - Thoracolumbar region

¾ Cervical and sacral SDAVF constitute just under 6% of

patients with SDAVF.

¾ Multiple SDAVFs are uncommon (0.5-4%).

Classification of spinal vascular lesions (Spetzler et al,2002 )
¾ Neoplastic vascular lesions
- Haemangioblastoma
- Cavernous malformations
¾ Aneurysms
¾ Arteriovenous lesions
- Arteriovenous malformations
- Arteriovenous fistulas
: Extradural
: Intradural
Ventral - A: Small, B: Medium, C: Large
Dorsal - A: Single arterial feeder
B: Multiple arterial feeder
Type 1a: Single Feeders Type 1b: Multiple feeders
Spinal Dural AVF, Extradural type
 Causal factors and pathophysiology
¾ An acquired condition – 0nset in middle age.

¾ Venous hypertension - the main pathophysiological factor.

¾ The shunt is most often formed within the dorsal surface of

the dural root sleeve in the intervertebral foramen, where
the radicular vein pierces the dura, together with one or
more dural branches of the radicular artery.
¾ Shunt is sometimes situated along the dura between two
adjacent nerve roots.

¾ Increased pressure causes the venous system to

‘arterialize’.
¾ The radicular feeding artery is often a dural branch and in a
minority, the medullary artery.

¾ The shunt (low flow) results in venous hypertension in the

spinal cord, because the intramedullary veins and the
radicular vein share a common venous outflow.
¾ Reduced arteriovenous pressure gradient:

- Decrease in tissue perfusion and venous infarction.

¾ Apart from the increased pressure caused by the shunt, the

venous outflow may be less efficient to start with than is the
case in healthy individuals.

¾ Lower thoracic region has relatively fewer venous outflow

channels at a segmental level than the cervical or
lumbosacral region.
¾ These differences in segmental outflow probably contribute
to the phenomenon that venous congestion is transmitted in
a caudo-cranial direction throughout the spinal cord.

¾ Thus, first symptoms of myelopathy tend to reflect

dysfunction of the lowest part of the cord, (conus
medullaris), even though the shunt is at the higher level, or
in some cases even near the skull base.
¾ Venous outflow through the medullary vein and venous
plexus is dorsal from the cord in 80–90%, and combined
ventral and dorsal in 10–20%.
Table 3 Proportion of patients with symptoms present at the onset of the
disease and symptoms present at the time of diagnosis
Initial Symptoms at
symptoms (%) diagnosis (%)

Sensory disturbances 17–72 63–100

Gait difficulties and motor 50–81 78–100
disturbances

Pain (either pain in the back or 13–64 17–86

radicular pain)

Micturition difficulties 4–75 62–91

Defaecation problems 0–38 30–100
Sexual dysfunction 0–17 11–80
 Clinical diagnosis

¾ SDAVF is notoriously hard to diagnose, because of the

misleading nature of the initial symptoms and the rarity of
the disease.

¾ The median time between onset and diagnosis ranges

between 12 and 44 months.
¾ Erroneous diagnoses often made initially are -
- Sensory polyneuropathy,
- Acute or chronic inflammatory
demyelinating polyneuropathy
- Spinal muscular atrophy
- Medullary tumour

¾ Not infrequently patients are unsuccessfully operated for a

lumbar disc prolapse.
¾ At the time of diagnosis, two-thirds of patients show a
combination of gait difficulties, sensory disturbances and
involvement of sacral segments (micturition, defaecation or
sexual dysfunction).

¾ Bowel and micturition problems frequently occur, mostly

later in the course of the disease .

¾ Micturition disturbances consist of urinary retention.

¾ Erectile dysfunction exists in 11–80% of men patients, and

unwanted and involuntary ejaculations may occur after
exercise.
¾ In the majority of patients (40–63%) progression lasts for
1–3 years before the diagnosis is made, but a protracted
course with a duration of >3 years occurs in 10–34%.

¾ A gradually progressive course with stepwise deterioration

is recorded in 11–32% of patients.

¾ A spontaneous and complete disappearance of the fistula

has occasionally been described, though the clinical deficits
remained unchanged in these patients.
¾ If symptoms develop within minutes to hours they can
mimic an anterior spinal artery syndrome.

¾ The sudden episodes mostly occur after exercise, prolonged

standing and even singing and may disappear after rest.

¾ Acute worsening of symptoms may also be related to

changes in posture such as bending over; even eating has
been related with worsening of symptoms
 INVESTIGATION
¾ The essential investigations to establish the diagnosis are
MRI and catheter angiography.

¾ MRI findings - Hypointens -T1WI

- Hyperintens-T2-WI
- Increased signal intensity in the centre of the spinal
cord and peripheral sparing on T2-WI is found in 67–
100% of patients.

¾ Hyper intensities extend over an average level of 5–7

vertebrae, with conus involvement over 80%, and are
typically homogeneous.
MRI and angiography of SDAVF. (A) T2-weighted MRI image of a
66-year-old man with SDAVF. Multiple flow voids resembling an
enlarged medullary draining vein can be seen. (B) Angiogram of a
57-year-old man showing an SDAVF at T7 on the right.
Increased
signal intensity
in the centre of
the spinal cord
with peripheral
sparing on a
T2-weighted
MRI image of
a 60-year-old
male with a
left-sided
SDAVF at T8
¾ Abnormal blood vessels may be seen on either the ventral
or the dorsal side of the spinal cord (flow void phenomena
in 35-91% patients).

¾ Marked enhancement occurs some 40–45 min after

contrast injection.

¾ M R Angiography:
- reveals flow in serpentine perimedullary structures in up
to 100% of patients.
Catheter angiography:
- Gold standard in the diagnosis of SDAVF
- Not only the intercostal and lumbar arteries should be
visualized as potential feeding arteries of an abnormal
shunt but also the median and lateral sacral artery, the deep
cervical and ascending cervical arteries.
Figure 3 (above) shows a spinal DAVF during selective spinal angiography. The catheter through
which the contrast is injected is marked by the light-blue arrow heads. The fistula itself (F; red
circle) is in the spinal nerve root dural sleeve. The arterial supply is marked by the red arrow and
the draining vein is marked by the dark-blue arrows.
 Methods of treatment
¾ The choice of treatment is between endovascular
embolization and surgical ligation of the fistula.

Embolization :
- Liquid polymers [such as isobutyl 2-cyanoacrylate
(IBCA), n-butyl 2-cyanoacrylate (NBCA)] is preferred
over particles such as polyvinyl alcohol (PVA), because
the use of particles leads to a recurrence rate as high as 30–
93% (Nichols et al.,1992). In contrast, occlusion is
successful with liquid polymers in 44–100%.
¾ Embolization of SDAVF is not possible in -.

1- If the arterial feeder of the fistula is a segmental

medullary artery.
( high risk of spinal cord ischemia)

2- In technical difficulties such as arterial wall dissection

of the feeding vessels during the embolization
procedure may prohibit introduction of the micro
catheter close enough to the fistula.
¾ If embolization is possible, then the success of treatment
depends on endovascular occlusion of the draining vein,
which means that recanalization will rarely occur when the
draining vein is filled with glue (Jellema et al., 2005).

¾ The reason is probably that the fistula is often made up of

several small feeding arteries and a single draining vein, so
that occlusion of only (one of) the arterial feeder(s) to the
fistula will generally lead to development of new arterial
feeders.
Intradural
localization
of the glue
cast,
indicating
that the
draining
vein is
filled with
glue.
Surgical treatment
¾ Multi-level laminectomy with stripping of the draining
vein and decompression of the spinal cord
(when pathophysiology of SDAVF was not clear )

¾ Presently an intradural interruption of the vein draining the

fistula is advocated (Huffmann et al., 1995).

¾ As effective as total removal of the draining vein.

Figure shows the draining vein complex of a spinal DAVF immediately
following open surgical disconnection of the fistula. The draining vein
complex, a tangle of red-purple veins, can be seen. Normally, there
should be no such tangle of veins on the spinal cord surface.
¾ A meta-analysis of patients with SDAVF who were
treated with either embolization or operation showed that
almost 98% of surgical procedures were technically
successful; this stands in contrast to the 46% of patients
who were successfully treated with embolization
(Steinmetz et al., 2004).
 Outcome after treatment
¾ Depends on several factors:
- Duration of symptoms
- Pre-treatment disability
- Success of the procedure to close the fistula

¾ Treatment is directed at halting the progression of

symptoms or even reversing them.

¾ Symptoms that generally respond well to treatment

are :
- Gait difficulties
- Muscle strength
(resulting in less disability and dependence)
¾ Symptoms that often respond less well to
treatment are :

- Micturition,
- Pain
- Muscle spasms
 Aminoff–Logue disability scales for gait and micturition
(Aminoff and Logue, 1974b)
Gait
0 Normal
1 Leg weakness, abnormal gait or stance, but no restriction of activity
2 Restricted activity but not requiring support
3 Requiring one stick for walking
4 Requiring two sticks, crutches or walker
5 Confined to wheelchair
Micturition
0 Normal
1 Hesitancy, urgency, frequency, altered sensation, but continent
2 Occasional urinary incontinence or retention
3 Total incontinence or persistent retention
 REFRENCES
1 – YOUMANS Neurological Surgery : Vol.-2
2 – Textbook of Neurological Surgery : Vol.-3
- H. Hunt and Christopher M.
3 - Textbook of Neurosurgery : 2nd edition
- Prof. B. Ramamurtthi and Prof. P.N. Tandon
4 – Neurosurgery : Vol.-2
- Setti S. Rengachary
5 – Operative Neurosurgical Techniques : Vol.-2
- Schmidek and Sweet
6 – Operative Neurosurgery : Vol.-2
- Kaye and Black
7 - Internet
. Images of a brain dural arteriovenous fistula (DAVF).

Figure 2 (above collage) shows a cranial (brain) DAVF. Top left: Axial CT scan shows a ruptured
DAVF (circled in red) located in the right paramedial cerebellum and located in the dura
surrounding a very high-risk venous structure known as the torcula herophili. The patient
presented with abrupt-onset headache and impaired consciousness. Bottom left: Preoperative
cerebral angiogram (right external carotid artery injection) showing the actual fistula (circled in
red) with its blood supply arising mainly from the occipital artery (OA) via many arterial
channels (red arrow heads). The fistula is drained by an abnormal sac-like draining vein (DV).
Top right: Intraoperative photograph showing the fistula being surgically disconnected by the
placement of titanium microclips via a neurovascular clip applier (CA) across the draining vein.
Bottom right: Postoperative cerebral angiogram (right external carotid artery injection) following
surgical disconnection of the fistula. The angiogram shows complete obliteration of the fistula
(nothing left to see in the red circled area!). The patient was discharged from hospital
neurologically intact
External layer- derived from the internal layer
of
periosteum and adherent to the inner
surface of the skull.

Continuous with the ectocranial periosteum

through the
foramina of the skull.

Internal layer - True meningeal duramater

(cranial dura
mater) and adheres in most places to the
external layer.

Separations of the two layers create the dural

sinuses, the
dural reflections, and the trigeminal cave.

Internal layer continues through the foramen

magnum to
encloses the spinal cord ending at S2 level.
Internal layer forms the five dural reflections :
1 - Falx cerebri
2 - Falx cerebelli
3 - Tentorium cerebelli
4 - Diaphragma sellae
5 - Tentorium bulbus
olfactorium

Cranial duramater contains :

- 8 paired venous sinuses
- 7 unpaired venous sinuses
A - Valt – Ophthalmic branch of trigeminal
nerve

B - Ant. cranial fossa :

- Ant. Ethmoidal nerve
- Maxillary nerve
C – Middle cranial fossa :
Ant. half - Maxillary
nerve
- Mandibular
nerve
Post. half- Trigeminal
ganglion
D – Posterior cranial fossa :
- C1, C2, C3
- 9th and 10th cranial
nerves
ANATOMY OF CS
Plexus of veins encased in a double layer of
dura.

Located on either side of the body of sphenoid

sinus.

Length = 2 cm, Width = 1 cm.

Extends from SOF to apex of petrous

temporal bone

ICA given of 3 branches in the CS :

1 – Meningohypophyseal trunk
2 – A. of inf. CS
3 – McConnell,s capsular arteries (30-
50%)
Venous drainage of CS include:
- Superior ophthalmic vein (SOV)
- Superior petrosal sinus (IPS)
- Inferior petrosal sinus
- Superficial middle cerebral vein (SMCV)
- Coronary sinus to the opposite side of
CS.

CS and IPS drain veins of the brain after birth.

All the cerebral veins converge toward the

posterior
sinuses, depending on the rapidity with
which CS matures
and venous communication between CS and
cerebral veins
takes place.
Djindjian Classification (1977)

Type -1: Immediate drainage into a sinus

with antegrade
flow.

Type -2 : Initial drainage into a sinus with

reflux into other
sinuses or cortical veins.

Type -3 : Initial drainage into cortical vein.

Type -4 : Initial drainage into cortical vein

with a venous
pouch.
Borden Classification

- Three types: based upon their venous

drainage:

Type I: Dural arterial supply drains

anterograde into
venous sinus.
Type II: Dural arterial supply drains
into venous sinus.
High pressure in sinus
results in both anterograde
drainage and retrograde
drainage via
subarachnoid veins.

Type III: Dural arterial supply drains

retrograde into
subarachnoid veins.
Type I :
- Suppied by meningeal arteries
and drain into a
meningeal vein or dural venous
sinus. The flow
within the draining vein or
venous sinus is
anterograde.

Type Ia -Simple dural arteriovenous

fistula have a single
meningeal arterial supply.

Type Ib -More complex arteriovenous

fistulas
are supplied my multiple
meningeal
arteries.
Type II
The high pressure causes blood to flow in
a retrograde
fashion into subarachnoid veins which
normally drain into
the sinus.

Typically this is because the sinus has

outflow obstruction.

Such draining veins form venous varices

or aneuryms
which can bleed.

Type II fistulas need to be treated to

prevent hemorrhage.
Type III

Drain directly into subarachnoid veins.

These veins can form aneurysms and

bleed.

Type III dural fistulas need to be treated to

prevent
hemorrhage.