Arteriovenous Malformations

Definition

AVMs are fistulous connections of cerebral arteries and veins without a normal
intervening capillary bed, which creates high- flow shunting of arterial blood into
the venous system. Due to the direct transmission of arterial pressure to the venous
structures, dilation, tortuosity, and arterialization of the draining vein(s) occur and
venous hypertension may result
Epidemiology
 Among patients ages 15–45 years presenting with intracerebral hemorrhage, 38% of cases are due to AVM
 Population-based studies estimate the sex- and age-adjusted incidence at 0.51–1.34 per 100,000 persons
 Autopsy and clinical magnetic resonance imaging (MRI) studies suggest a prevalence of approximately
0.2%–1.0%
 In autopsy studies, only 15% of patients had symptoms related to the AVM
 Patients are typically diagnosed in the third to fourth decade
 AVMs are common in men and women but there may be a slight male predominance
 Many AVMs are situated at the border zone areas of the anterior, middle, and posterior cerebral arteries.
They are often pyramidal shaped, with the base parallel to the cortex and the apex pointing inward toward
the ventricle
Pathology, Pathogenesis, and Pathophysiology
 AVMs are high-flow cerebrovascular lesions consisting of a tangle of abnormal blood vessels

 Three morphologic features are typical of these lesions:

 Feeding arteries
 Draining veins
 Dysplastic vascular nidus composed of a tangle of abnormal vessels that acts as a shunt from the arterial to venous
system

 Gross pathologic features include the absence of a capillary bed and the presence of small feeding arteries composed
of variable amounts of smooth muscle and elastic laminae, with single or multiple direct arterio- venous (AV)
connections

 AVM-associated aneurysms are associated with 2.3% to 16.7% of AVMs

 AVMs are increasingly recognized as dynamic lesions that may enlarge, regress, obliterate, and recur
Axial and coronal T2-weighted (A and B) and
sagittal T1-weighted (C) magnetic resonance
images show multiple vascular flow voids
within a left frontal arteriovenous
malformation extending from the cortex
down to the frontal horn of the left lateral
ventricle. Left internal carotid injection
(lateral projection) during cerebral
angiography shows that the arterial supply is
via anterior left middle cerebral artery
branches (D) with early venous drainage (E).
The nidus is more clearly defined with
superselective angiography (F)
Etiology
 AVMs may be classified as being sporadic or syndromic in origin. Sporadic AVMs are by far the most
common with a global preva- lence of 0.04% to 0.52%

 There is increasing evidence that they occur as the result of upregulation or downregulation of multiple
homeobox genes, which are involved in angiogenesis, such as HOXD3 and HOXB3

 Syndromic AVMs account for approximately 2% of cases. Familial mul- tiple AVMs are seen in hereditary
hemorrhagic telangiectasia (HHT)

 AVMs are the most common cause of spontaneous brain hemorrhage in children (excluding the neonatal
period)

 Large AVMs generate an arterial steal phenomenon, and older children may present with progressive
neurological deterioration and chronic epilepsy
MIXED LESIONS: TRUE ARTERIOVENOUS MALFORMATIONS WITH OTHER
VASCULAR MALFORMATIONS

Mixed lesions that include a component of a true AVM are relatively uncommon

 Arteriovenous Malformations and Capillary Telangiectasia :

 The growth of the capillary telangiectasia may have been caused by the decompressive effect or
changes in hemodynamics associated with the AVM resection or by the release of angiogenic factors
after surgery

 Arteriovenous Malformations and Developmental Venous Anomalies

 The coexistence of DVAs and AVMs is well recognized despite being rare

 Arteriovenous Malformations and Cavernous Malformations

 smooth muscle may develop in the wall of the CM as a reaction to angiogenic factors
 microhemorrhage from an occult AVM precipitates development of the CM
Clinical Presentation

 Up to 40% of patients with AVM present with symptoms unrelated to the AVM

 The remainder of patients present with symptoms related to the AVM, including focal neurological deficit,
headache, and seizure. These clinical symptoms may be associated with hemorrhage or simply be due to
mechanical compression or irritation of the surrounding brain

 Up to 50% (range: 30%–70%) of patients with AVM present to medical attention due to a ruptured AVM
with hemorrhage

 Intraparenchymal hemorrhage is the most common, but intraventricular hemorrhage, subarachnoid

hemorrhage, and rarely subdural hemorrhage may occur

 Patients with hemorrhage at initial presentation often have significant morbidity and mortality (10%–40%)

 Approximately 15%–35% of patients with AVM will first present to medical attention with a seizure
AVM Diagnostic and Grading
 CT Scan

 Diagnosis can be difficult on non-contrast CT.

The nidus is blood density and therefore
usually somewhat hyperdense compared to
adjacent brain. Enlarged draining veins may
be seen. Although they might be very large in
size, they do not cause any mass effect
unless they bleed.
 Following contrast administration, and
especially with CTA, the diagnosis is usually
self-evident, with feeding arteries, draining
veins, and intervening nidus visible in the so-
called "bag of worms" appearance. The exact
anatomy of feeding vessels and draining veins
can be difficult to delineate, so angiography
remains necessary.
 MRI

Fast flow generates flow voids, easily seen on T2 weighted

images. Complications, including previous hemorrhage and
adjacent edema, may be evident.

MRA: phase-contrast MR angiography is often useful for

subtracting the hematoma components when an
arteriovenous malformation complicated by an acute
hemorrhage needs to be imaged
Radiology reports should include certain key points that help
the clinician in deciding the management. Radiological
evidence of previous hemorrhage, intranidal aneurysm,
ectasia or stenosis of draining veins, single draining vein or
deep draining vein, or deep or posterior fossa location of the
arteriovenous malformation is associated with a high risk of
future hemorrhage .
Risk of non-hemorrhagic complications like focal neurological
deficit increases with a long pial course of a draining vein,
arterial steal, mass effect, and perinidal gliosis .
 Cerebral Angiography (DSA)

Cerebral angiography remains the gold standard,

able to exquisitely delineate the location and
number of feeding vessels and the pattern of
drainage. Ideally, angiography is performed in a bi-
plane system with a high rate of acquisition, as
shunting can be very rapid.
On angiography, an arteriovenous malformation
appears as a tightly packed mass of enlarged
feeding arteries that supply a central nidus. One or
more dilated veins drain the nidus and abnormal
opacification of veins occurs in the arterial phase
(early venous drainage), represents shunting.
CLASSIFICATION AND GRADING

 Brain arteriovenous malformations can be divided into two types:

• Compact (or glomerular) nidus: abnormal vessels without any interposed
normal brain tissue. More common than diffuse nidus type.
• Diffuse (or proliferative) nidus: no well-formed nidus is present, with
functional neuronal tissue interspersed amongst the anomalous vessels.
• when early venous drainage is absent, this is considered cerebral proliferative angiopathy
• The Spetzler Martin - AVM System relates morphology and location to the risk of surgery.
SPETZLER-MARTIN GRADING
SYSTEM
•Stratify morbidity risk in patients following complete
microsurgical resection of brain AVM to help inform
treatment decision
•AVM size (largest diameter of nidus)
• < 3 cm = 1 point
• 3-6 cm = 2 points
• > 6 cm = 3 points
•Deep venous drainage = 1 point
• Deep veins include internal cerebral veins, basal
veins, precentral cerebellar vein
• All veins in posterior fossa are deep except
for cerebellar hemispheric veins that drain into
straight sinus transverse sinus
•AVM adjacent to eloquent brain areas = 1 point
• Eloquent indicates identifiable neurological
function and disabling neurological deficit if
injured
• Includes sensorimotor, language, and visual
cortices; hypothalamus and thalamus; internal
capsule, brainstem, cerebellar peduncles, and deep
cerebellar nuclei
•Grade = sum of scores (total score range 1-5 points)
TREATMENT
• Embolization
• Microsugery
• Radiosurgery
Choise of therapy
 Small AVM: microsurgery is a choise: Role of embolization
 Supp point 2 and 3: radiosurgery
 Supp point 2 and 3 with size 3-6cm or 14cc Volume: radiosurgery
 Volume > 14cc: embolization
Embolisation
 The advancement in neuroradiologY interventional
 Early microsurgery following embolization must be institute before
recanalization

Goal of Embolization
Pre surgical: to control the feeders
Pre SRS: to reduce the size of nidus amenable for radiation
Embolic Agents

Embolic agents generally ckassified 3 categories

 Occusive devices
 Mivroparticles and
 Liquid
Occlusive Devices
 Balloons for large vessel occlusions
 Braided silk threads which are highly thrimbogenic and coils
 Coils can be used to permanently occlude larger arterial
Particles
 Polyvinyl alcohol (PVA): radiolucent
 Irregularly shaped: 50um – 100um
 selected sizes 45 to 1180 um

 Particles must not reflux into en passage or transit vessels

and cclude beds of normal brain tissue
Stereotactic radiosurgery (SRS)
 Radiation induces a biological effect that is depend on cellular mitosis
 The is to ionizing radiation to caompelete nidus volume
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