Case report

ARTERIOVENOUS MALFORMATION

Ahmad Brata Rosa, Harsan H

Introduction three types of feeding vessel in AVM: direct

feeders, transit feeders and indirect feeder
Spontaneous Intracerebral hemorrhage (ICH) (artery en passage). Direct feeders are arteries
is one of the disease with high mortality rate that end directly and exlusively in the nidus.
in the world. Many factors that cause the Transit feeders are the arteries that supply
occurence of spontaneous ICH. One of them,
blood to the normal brain but they pass
expecially that attack young age, is through the nidus. Indirect feeders are the
arteriovenous malformation (AVM). arteries that supply blood to the normal brain,
Hemorrhage from cerebral venous pass through the nidus and also contribute to
malformation represents 2% of all the shunt before supply blood to normal
hemorrhagic stroke, which one of the causes
brain. In a similar manner to the arteries, for
of non-hypertensive spontaneous ICH. 1 the draining vein, one should note the
AVM is congenital lession of the complex number, size, and locations of the major
tanggle of the blood vessel (arteries and veins. Like the transit arteries, it is important
veins)connected by one or more fistulae. It to recognize the possible existence of normal
can occured in all over the body including veins draining functional cerebral tissue that
brain and spinal cord. Normally, the blood wil may be adjacent to the lesion.
flow to whole area of the body from the heart
by large artery. Then become samller until a
small cell capillary that supply nutritions and
oxygen to the cell. The place of exchanging
nutrition or oxygenitation is capillary bed. CO2
and other residual of cell metabolisme will be
transfered to vein by capilary bed, and the
back to the heart. But in AVM, arteries direcly
connect to the vein without capilary bed
betwen them. Because theres a different
pressure between arteries (high flow) and the
veins (low flow, then will develop a problem
was called high pressure shunt. The veins will
not be able to withstand a high pressure flow.
So it wil scretch and became larger and thin,
and will be vulnerable to rupture.
Fea ture 1. Arteriovenous malformation. Showed a nidus,
AVM consist of three component. Feeding
s ome feeders a rteri es a nd dra i ni ng vei n
vessels, nidus and darining veins. The vascular
conglomarate is called the nidus. Size of nidus When AVM ruptures, it will has nine fold risk
is resemble the size of the avm. There are for rerupture in the first year.2 A clear
understanding of the diagntic and treatment
of the AVMis imperative.
Epidemiology
Insidence of AVM is vary in each country. In
New York island insidence of AVM is about
1,34 per 100.000 person-years. Theres a
report that insidence rate from australia and
sweden is 0,89-1,24 per 100.000 person-
years, and in scotland about 18 per 100.000
persons-years.3 Al-shahi et al (2001), from his
systematic review, found that insidence of
AVM is 1 per 100.000 persons per year in
unselected population, and in adult is 18 per
100.000 person-years. AVMs account for
between 1 and 2% of all strokes, 3% of strokes
in young adults, 9% of subarachnoid
haemorrhages and, of all primary
intracerebral haemorrhages, they are
responsible for 4% overall, but for as much as
one-third in young adults.
Many factors make the AVMs vurnarable to
rupture. Small nidus and single draining vein is
common factor. Also thre’s connection
between size of the AVM and rupture rate.
Smaller the AVM higher chance to rupture.
May be it’s because the small has the higher
feeding pressure compared to larger AVM, the
high pressure can make the AVM easy to
rupture.4 The other factors that assosiated
with the risk of rupture are previous rupture,
infratentorIal, and deep location. 5,6
AVMs are the most common cause of
spontaneous ICH in young adult. Maybe
because the lessions are congenital lession.
Aproximately it occured on the age of 20-
40.7,8,9 Risk of bleeding can be simply
approximated with the linier formula10 :
% lifetime risk = 105 – patient’s age
For example 25 years old man has % lime time
risk of bleeding by 80%, at least once in a life
time. There’s no distinc between sex for the
AVM. It affects male and female in the same
rate.
Pathological squelae
AVMs make neurological defficites through 3
main mechanisms. 11 First, hemorrhage may
occur in the subarachnoid space, the
intraventricular space or, most commonly, the
brain parenchyma. Second, in the absence of
hemorrhage, seizures may occur as a
consequence of AVM: approximately 15-40%
of patients present with seizure disorder.
Finally, but rarely, a progressive neurological
deficit may occur in 6-12% of patients over a
few months to several years. These slowly
progressive neurological deficits are thought
to relate to siphoning of blood flow away
from adjacent brain tissue that is called the
"steal phenomenon", a concept that has been
recently challenged. Neurological deficits may
be explained alternatively by the mass effect
of an enlarging AVM or venous hypertension
in the draining veins.
Seizure is the second most commont
presenting symptom, assosiated with
supratentorial AVM. Aproximately 15%-30%
AVM patient With focal or generelized
seizure.12,13,14 Angiographic characteristics of
epileptogenic AVMs include cortical location
of the nidus or feeding artery, feeding by the
middle cerebral artery, absence of aneurysms,
presence of varices in the venous drainage,
and association of varix and absence of
intranidal aneurysms. Other factors
significantly associated with the onset of
seizures include AVMs fed by the external
carotid artery and a temporal or parietal
cortical location. 15
Another pathological squelae is headache.
Headaches are the presenting symptom in
approximately 15% of patients without
evidence of rupture. 12 Headache and seizure
also can be assosiated to the hemorrhage.
Classification and Grading System
AVM included into category of dysplastic
vascular malformation, where there have
been four pathological entities:
developmental venous anomalu (venous
angioma), cappilary telangiectasias, cavernous
malformation, and AVM. 16,17,18 One of them can
follow another, altough it’s rare.
Avm may be classified as :
1. Parenchymal AVM
a. Pial
b. Subcortical
c. Paraventricular
d. Combined
2. Pure dural AVM
3. Mix parenchimal and dural AVM
The AVM is classified to know the outcomes after
surgery. So the surgeon can predict the risk of
permanent neurological deficits after surgery. It’s
“Spetzler-Martin Scale” that be used. Grade 1 and
2 has very low risk of morbidity after surgery, but
the grade 4 and 5 has higher risk of neurological
deficits after surgery. It very usefull for surgical
decision making.
Diagnostic Radiology
Many imaging technic can visualize a AVM,
including: MRI, MR angiography (MRA), CT, CT
Angiography (CTA) and angiography. MRI is usefull
in diagnostic and management of AVM. MRI not
only can visualize the AVM but also can show
better visualization of location of the AVMs and
surrounding structure that relate to AVM. On T2-
weighted images, hypointense signal of the lession
are indicated to flow void. Peripheral to the nidus,
hypointense on T2 and gradient echo can also
show hemosiderin depocites that sign a previous
hematoma.
Non contrast CT scan cannot visualize AVM clearly.
But, it’s necessary to recognize the key feature
around hematoma that was suspected AVM. 25%
to 30% AVMs have calcium depocition
(calsification) that may be seen even in the mass of
19
the hematoma. CTA and MRA can deliniate the
nidus and assosiated vessel s without exposed any
complication of angiography.
But in CTA and MRA, is very hard to determine
dinamic aspec of malformation. because in MRA
CTA the vessels enhance contrast simultanly. So,
its dufficult to see where is the nidus, feeding
arteries and draining veins. For that angigraphy is
required. Angiography, expecially digital
substraction angiography (DSA), can substract out
static components of the image, so we can clearly
visualize nidus, feeder artries and darining veins.
Structure visualized in every phase during DSA are
seen on the table 2 below. But the angiography is
lack of geometrical of the nidus.
Treatment
Unlike the anuerisme, risk of rebleeding imediately
for AVM rupture is very rare. Although surgical
evacuation of the hematoma caused by AVM
rupture for life treatening is must be done
imediately, resection of the AVM can be delayed
until hematoma is resol ved and the AVM stabilize
its form. And then wait until swelling decreased for
easier surgical intervention. But if if neurological
deficits happen progressively, surgical intervention
must be done urgently.
The definitif treatments for AVM including:
1. Medical or symptomatic therapy
2. Embolization
3. Microsurgery
4. Radiosurgery
5. Multi modal therapy
 Ta bl e 2. Structure vi s ua l i zed duri ng DSA
That must be considered for treatment of the AVM
are:
1. Size of the AVM
2. Location
3. Vascular anatomy
4. Age
5. Medical condition
The current American Heart Association
multidisciplinary management guidelines for the
treatment of brain AVMs recommend the
20
following approach:
1. Surgical extirpation is strongly suggested
as the primary treatment for Spetzler-
Martin grade I and II if surgically
accessible with low risk.
2. Radiation therapy alone is recommended
for Spetzler-Martin grade I or II if the
AVM is less than 3 cm in size and surgery
has an increased surgical risk based on
location and vascul ar anatomy.
3. Brain AVM of Spetzler-Martin grades III
can often be treated by a multimodal
approach with embolization followed by
surgical extirpation. If the lesion has a
high surgical risk based on location and
vascular anatomy, radiation therapy may
be performed after embolization.
4. AVMs of Spetzler-Martin grade IV and V
are often not amenable to surgical
treatment alone because of the high
procedural risk. These AVMs can be
approached by a combined multimodal
approach of a combination of
embolization, radi osurgery, and/or
surgery.
5. In general, embolization should only be
performed if the goal is complete AVM
eradication with other treatment
modalities. The only exception is palliative
embolization in patients with an AVM of
Spetzler-Martin grade IV or V with venous
outflow obstruction or true steal
phenomenon in order to reduce arterial
inflow to control edema or to reduce the
amount of shunt, respectively.
Medical treatment
Indications for medical treatment including:
1. Malformation is very extensive
2. Deep location in the brain
3. Primary blood supply origin from deep
perforating vessels
4. Advance age
5. Poor medical condition. (such as: advance
heart disease, insuficiency respiratory, or
cancer with metastatic.
Embolization
Embolization is used for pre operative, pre
radiosurgery or paliatif embolization. Embolization
involves occluding blood flow to an arteriovenous
malformation by using endovascular catheters to
deposit occlusive materials into the feeding
arteries and nidus. It can be used to reduce size of
the AVMs, and symptoms. Embolization can be
curative in a minority of cases, particularly for
lesions less than 1 cm in diameter that are fed by a
21,22
single artery. It is usually inadequate by itself
to treat the AVM, because it may recanalize later.
AVMs that are embolized have a rate of
23
permanent morbidity between 4% and 14%.
If the embolization is followed by surgical
intervention, the surgery can be done 3-30 days
after embolization. If its followed by radiosurgery,
the surgery can be done 30 days after the
24
embolization.
The agents for embolization are classified to three Ta bl e 3. Percentage of defici ts a fter s urgery ba s ed on
Spetxel -ma rti n gra de.
cathegories, they are: occlusive devices,
microparticles, and liquids. Occlusive devices
include balloons for large vessel occlusions,braided
silk threads which are highly thrombogenic, and
coils. Many earlier reports of embolizations used
particles, specifically, polyvinyl alcohol (PVA)
(Counter PVA particles, Boston Scientific, Fremont,
CA). Two liquid agents are currently in use in the
Case report
United States for embolization: NBCA and EVOH
(Onyx). Improved obliteration rates (of A 42 years-old man was pressented with
approximately 20%) have been reported for the spontaneous inrtracerebral hemorrhage 3 moths
use of a recently developed embolic agent ago. He was underwent surgery for evacuation clot
25
(Onyx). emergency. No history of hypertension before.
Patient has hystory of the seazure 2 years ago,
Radiosurgery
twice, generelize seizure, but controled by the
drug. He complaint mild headache sometimes, but
Radiosurgery is an option that is generally used to
it didn’t really disturbe his daily activities. Phsisical
treat AVMs that are approximately 3 cm in
diagnostic was normal and neurological state in
diameter or less. Proton beam, linear accelerator,
the normal range.
or gamma knife methods are used to deliver a high
From the CT finding shows calsification feeture on
dose of radiation to the AVM, while minimizing the
the left temporal near the remains of hematoma.
effects to surrounding brain tissue; a single dose
Susupicious for the AVM. The MRI was done, and
generally is given. AVMs with nidal volumes less
showed some heterogen lession with a flow void
than 10 mL (diameter <3 cm) are frequently
feature. And from CTA showed a tanggle of some
curable by radiosurgery, with rates of obliteration
26,27 vessels that refers to AVM with size aproximately
at 2 years estimated at between 80% and 88%.
5.6 cc. From angiography found involvement of
Radiosurgery may take 1-3 years to achieve
two feeder arteries branch of MCA and one
thrombosis of an AVM, thus the patient remains at
darining vein.
risk for hemorrhage from AVM during the
treatment period.

Surgical treatment
Decisions as to which lesions are most amenable
to surgery are commonly based on the Spetzler–
Martin scale. Grade I, II, or III AVMs were found to
have low treatment-associated morbidity. In case
series for grade I – III, reported rates of permanent
weakness or paralysis, aphasia, and hemianopsia
28,29
are 0 to 15%, and most report no deaths.
However, grade IV lesions conferred 31.2%
treatment-associated morbidity, and grade V
lesions had 50% new treatment-associated
30
morbidity. Surgery is recomended for all grade I
and II lesions. Grade III lesions should be treated
on a case-by-case basis. Grade IV and V lesions
require a multidisciplinary approach with Fea ture 2. Non contra s t CT s ca n of the pa ti ent a fter
individual analysis. Surgical outcome predicted by previ ous s urgery, s hows s ome ca l ci fi ca ti on on l eft
Spetzler–Martin scale are showed on the table tempora l .
31
below.
The patient was diagnosed with arteriovenous resection. No significant neuroligical deficits was
malformation. And performed surgery for AVM found after surgery.
resection. And the AVM was resected by en-block

Fea ture 3. MRI a xial section of the patient. T1 (l eft) shows some heterogen lession like a tanggle of vessels. T2 (ri ght) shows
s ome fl ow voi d ni ea r the l es s i on.

Fea ture 4. CTA recons tructi on. Shows AVM fea ture, wi th ni dus a nd s ome a s s os i a ted ves s el s .

Fea ture 4. Non contra s t CT s ca n a fter res ecti on of the AVM.

Discussion
Spontaneous ICH from rupture of the AVM are still
a rare case. Neverthless, to know the things
relating to the AVM as etiology of cerebral
hemorrhage is imperative. It’s including age,
simptom, history of hypertension and feature of
radio imaging,
This patient is 42 years -old, no history of the
hypertension and has hystory of generalized
seizure. With this datas, it’s supposedly the priority
of it’s etiology is AVM. And phsysician before
prepare for the resection of the AVM. Because the
ruptered AVM has the high risk of reccurent
bleeding as 18% in the first year. This may be
resulting poorer prognosis.
In caso, presenting lobar AVM with 2,5 cm
diameter, located in non-eloquen area, with
superficial venous drainage. With this so the
spetzel-martin grade is 1, and its recomended for
surgical resection of the AVM, suitable to the
surgical intervention that already done. Because
from the some trials, surgical is generally
considered appropriate for AVM that are grade I
and II.
Summary
Comperhension of natural history, signs and
symptoms, and imaging for the AVM case is
necessary for receiving an optimal treatment.
Decision making for treatment of the AVM is
according to spetzel-martin grade, age of the
patient, presenting of assosiated aneurisme, and
pattern of the vessels.
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