748

congenital abnormalities

748

≤1% of intracranial vascular

malformations

748
748

supplied primarily by the

1. Choroidal.
choroidal arteries

748
748 748 748

feeders connected to a dilated

Lasjaunias three types 2. Mural
vein of Galen

748
748 748

due to an adjacent vascular

3. Secondary. Enlargement of the vein of Galen
malformation

748
748 748

from the pericallosal and

Type I few feeders
posterior cerebral arteries

748

thalamoperforators
748 748

Type II from the

748

posterior cerebral arteries

748

pericallosal arteries

748
748 748 748 748

Type III (aka true, or choroidal

Most common Mixed pattern of feeders from the thalamoperforators
type)

748

posterior cerebral arteries

748

Aneurysmal dilatation

748

748
Type IV (aka secondary type)
748
shunting from adjacent
Angiographic Classi cation parenchymal AVM or dural AV
stula

750

748

Yasargil

#Adicionado

748

connections within the primitive

vascular bed

748

stulous connections normally

involute between the fth and
seventh weeks

748

by 3 months of development, 748

the posterior part of the median
prosencephalic vein joins the form the vein of Galen
748 748
internal cerebral veins and the
Embryology median prosencephalic vein basal veins

748 748

sump effect of the high- ow, leads to the recruitment and

low-resistance venous drainage enlargement of feeding arteries

748

from the interventricular foramen

748 to the choroidal ssure

Vein of Galen malformations

748

laterally to the atria

749
749

(1) neonates (birth to 2 months

cardiac failure
of age)

749 749
749

(2) infants (age 2 months to 2 hydrocephalus and head

three age groups
years) enlargement

749
749

hydrocephalus, headaches, and

(3) older children and adults
developmental delay

749
749 749

dilatation of the right chambers

pulmonary hypertension left heart failure
of the heart
748

KIDS KORNER! Vein of Galen

749 749
Malformations 749

749
May be diagnosed by prenatal have irreversible brain damage
22% of children
ultrasound at birth
749 1. High output cardiac failure.

Clinical Features
749

brief (3-day) period of relative

stability occurs after birth,
followed by acute
decompensation

749

clinical manifestations 749

Intracranial venous hypertension

749
749

cause pre- and postnatal brain

Heart failure
damage
749

2. Neurological manifestations.
749

diffuse brain destruction (i.e.,

melting brain syndrome)

749

Hydrocephalus

749 749

survival rate for neonates is 50–76.9%

749

Natural History and Overall

749
Prognosis 749

Spontaneous thrombosis of a
2.5% of patients
vein of Galen malformation

750

embolization +/− shunt surgery

750 750

Management and Outcomes cornerstones of treatment

750

radiosurgery

750
750 750

poor systemic or neurological

Score <8 withhold therapy
outcome

750 750

normal neurological status

750

Score 8–12
750

emergent embolization

750 750
750

Lasjaunias delay embolization until the child

Score >12
is at least 5 months of age

749

#Adicionado

750

angiography is not indicated

unless embolization is planned.

750

The optimal age for

embolization is ≥5 months

751
751

750
Venous embolization in neonates
used only when an arterial route
is associated with higher
Neonates is not available
morbidity

751 751

Glue (e.g., nBCA) is the it is more resistant to

preferred agent for embolization recanalization
fi
751

Reduction of 30% of shunt

volume is suf cient to improve
cardiac function

751
750

death occurred despite or

Treatment in Neonates because of embolization in 52%;
of the survivors

751

63.6% had moderate or severe

retardation
fi
fi
fl
751

responds to treatment of the

751
lesion

Outcomes in Neonates
751

contribute to venocongestive
751 brain edema
751

CSF shunting
Hydrocephalus
751

risk of subdural hematoma

751

Endoscopic third
fi
fi
ventriculostomy is an acceptable
fi
alternative

751

Outcomes in Infants

751

Children and Adults

751
751

Outcomes in Children
Infants

751

Hemorrhage: 5.6%
751

Complications of Embolization
751
Procedures
Permanent neurological
disability: 2.1%
congenital abnormalities 748

≤1% of intracranial vascular malformations 748

Angiographic Classification 748

Lasjaunias 748

three types 748

1. Choroidal. 748

supplied primarily by the choroidal arteries 748

2. Mural 748

feeders connected to a dilated vein of Galen 748

3. Secondary. 748

Enlargement of the vein of Galen 748

due to an adjacent vascular malformation 748

Yasargil 748

Type I 748

few feeders 748

from the pericallosal and posterior cerebral arteries 748

Type II 748

from the 748

 thalamoperforators 748

posterior cerebral arteries 748

Type III (aka true, or choroidal type) 748

Most common 748

Mixed pattern of feeders 748

from the 748

pericallosal arteries 748

thalamoperforators 748

posterior cerebral arteries 748

Type IV (aka secondary type) 748

Aneurysmal dilatation 748

shunting from adjacent parenchymal AVM or dural AV fistula 748

750

#Adicionado
Embryology 748

median prosencephalic vein 748

connections within the primitive vascular bed 748

fistulous connections normally involute between the fifth and seventh weeks 748

by 3 months of development, the posterior part of the median prosencephalic vein joins the internal cerebral veins and the basal veins 748

form the vein of Galen 748

sump effect of the high-flow, low-resistance venous drainage 748

leads to the recruitment and enlargement of feeding arteries 748

Vein of Galen malformations 748

from the interventricular foramen to the choroidal fissure 748

laterally to the atria 748

Clinical Features 749

three age groups 749

(1) neonates (birth to 2 months of age) 749

cardiac failure 749

(2) infants (age 2 months to 2 years) 749

hydrocephalus and head enlargement 749

(3) older children and adults 749

 hydrocephalus, headaches, and developmental delay 749

clinical manifestations 749

1. High output cardiac failure. 749

dilatation of the right chambers of the heart 749

pulmonary hypertension 749

left heart failure 749

May be diagnosed by prenatal ultrasound 749

22% of children 749

have irreversible brain damage at birth 749

brief (3-day) period of relative stability occurs after birth, followed by acute decompensation 749

2. Neurological manifestations. 749

Intracranial venous hypertension 749

Heart failure 749

cause pre- and postnatal brain damage 749

diffuse brain destruction (i.e., melting brain syndrome) 749

Hydrocephalus 749

Natural History and Overall Prognosis 749

survival rate for neonates 749

 is 50–76.9% 749

Spontaneous thrombosis of a vein of Galen malformation 749

2.5% of patients 749

Management and Outcomes 750

cornerstones of treatment 750

embolization +/− shunt surgery 750

radiosurgery 750

Neonates 750

Lasjaunias 750

750

Score <8 750

poor systemic or neurological outcome 750

withhold therapy 750

 Score 8–12 750

normal neurological status 750

emergent embolization 750

Score >12 750

delay embolization until the child is at least 5 months of age 750

749

#Adicionado

Treatment in Neonates 750

angiography is not indicated unless embolization is planned. 750

The optimal age for embolization is ≥5 months 750

Venous embolization in neonates is associated with higher morbidity 751

 used only when an arterial route is not available 751

Glue (e.g., nBCA) is the preferred agent for embolization 751

it is more resistant to recanalization 751

Reduction of 30% of shunt volume is sufficient to improve cardiac function 751

Outcomes in Neonates 751

death occurred despite or because of embolization in 52%; of the survivors 751

63.6% had moderate or severe retardation 751

Hydrocephalus 751

responds to treatment of the lesion 751

CSF shunting 751

contribute to venocongestive brain edema 751

risk of subdural hematoma 751

Endoscopic third ventriculostomy is an acceptable alternative 751

Infants 751

Outcomes in Infants 751

Children and Adults 751

Outcomes in Children 751

Complications of Embolization Procedures 751

Hemorrhage: 5.6% 751

Permanent neurological disability: 2.1% 751

 Intracranial Aneurysm Treatment
5

5.1 Intracranial Aneurysm Embolization

Indications and Contraindications

Indications for the endovascular treatment of intracranial aneurysms are discussed in depth in
Chap. 12.

General Indications

1. Aneurysmal subarachnoid hemorrhage

2. Unruptured intracranial aneurysms
3. Poor surgical candidates:
(a) Elderly patients
(b) Patients with significant medical problems
(c) Patients requiring chronic systemic anticoagulation (i.e., patients with atrial fibrillation)
4. Posterior circulation aneurysms
5. Cavernous segment ICA aneurysms

Relative Contraindications

1. Vascular anatomy that is prohibitive (e.g., some giant, wide-necked aneurysms, exaggerated vessel
tortuosity)
2. Significant atherosclerotic disease or other abnormalities affecting the parent vessel (e.g., signifi-
cant atherosclerotic stenosis of the carotid bifurcation)
3. Coagulation disorders or heparin hypersensitivity
4. Active bacterial infection (i.e., bacteremia at the time of endovascular treatment)

© Springer International Publishing AG 2018 249

M.R. Harrigan, J.P. Deveikis, Handbook of Cerebrovascular Disease and Neurointerventional
Technique, Contemporary Medical Imaging, https://doi.org/10.1007/978-3-319-66779-9_5
13.10 Specific Considerations 737

KIDS KORNER! Vein of Galen Malformations

Vein of Galen malformations are congenital abnormalities that are distinct from other intracra-
nial vascular malformations. They have a spectrum of severity and may present at birth, during
infancy, or during later childhood. They are rare, and are believed to comprise ≤1% of intracra-
nial vascular malformations [240]. Management of these lesions is presently handled primarily
by pediatric neurosurgeons and neurointerventionalists.
Angiographic Classification
Lasjaunias and colleagues divided congenital vein of Galen lesions into three types [241]:
1. Choroidal. Network of feeders, resembling a nidus, supplied primarily by the choroidal
arteries.
2. Mural. One or more arterial feeders connected to a dilated vein of Galen.
3. Secondary. Enlargement of the vein of Galen due to an adjacent vascular malformation, fis-
tula, or venous outlet obstruction.
Yasargil introduced the following classification Scheme [242]:
1. Type I. Relatively few feeders, arising principally from the pericallosal and posterior cere-
bral arteries.
2. Type II. Feeders arise mainly from the thalamoperforators and posterior cerebral arteries.
3. Type III (aka true, or choroidal type). Most common type. Mixed pattern of feeders, arising
from the pericallosal arteries, thalamoperforators, and posterior cerebral arteries.
4. Type IV (aka secondary type). Aneurysmal dilatation (i.e., varix) of the vein of Galen result-
ing from shunting from adjacent parenchymal AVM or dural AV fistula, or outlet obstruction
in the straight sinus.
Development, Anatomy, and Pathophysiology
Embryology
The embryonic precursor to the normal vein of Galen is the median prosencephalic vein, which
has arteriovenous fistulous connections within the primitive vascular bed. The fistulous connec-
tions normally involute between the fifth and seventh weeks of development [243], and by
3 months of development, the posterior part of the median prosencephalic vein joins the internal
cerebral veins and the basal veins to form the vein of Galen. Persistence of the median prosen-
cephalic vein and its primitive arteriovenous connections leads to a true vein of Galen malfor-
mation. The sump effect of the high-flow, low-resistance venous drainage leads to the recruitment
and enlargement of feeding arteries.
Anatomy
Vein of Galen malformations are midline structures, extending from the interventricular fora-
men to the choroidal fissure, and laterally to the atria [241].
1. Arterial supply is usually bilateral and symmetrical [244].
(a) Choroidal arteries. In most cases all of the choroidal arteries contribute feeders.
(b) Subependymal arterial network arising from the posterior circle of Willis.
(c) Thalamoperforators—rare.
2. Venous drainage is into a dilated median vein of the prosencephalon, which drains into a
falcine sinus and then into the superior sagittal sinus or the posterior venous sinuses.
3. The straight sinus is absent in most patients [245].
(a) Venous outlet obstruction may be present.
(b) There may be no effective connection to the deep cerebral venous system.
738 13 Arteriovenous Malformations

(c) Because of this, deep brain structures use alternative drainage pathways. These typically
include thalamic and subtemporal or lateral mesencephalic veins, which have an epsilon
shape on a lateral angiogram.
Clinical Features
The clinical manifestations of patients with vein of Galen malformations can be divided into
cardiac and neurological problems. Patients may be divided into three age groups [246]: (1)
neonates (birth to 2 months of age), (2) infants (age 2 months to 2 years), and (3) older children
and adults. Neonates typically appear with cardiac failure; infants usually present with hydro-
cephalus and head enlargement [247]; older patients frequently present with hydrocephalus,
headaches, and developmental delay.
1. High output cardiac failure.
(a) The intracranial AV shunt can be hemodynamically significant, resulting in dilatation of
the right chambers of the heart [248], pulmonary hypertension, and left heart failure.
(b) A loud, machine-like bruit may be present over the head and chest [246].
(c) May be diagnosed by prenatal ultrasound; 22% of children with a prenatal diagnosis
have irreversible brain damage at birth and die [244].
(d) The severity of cardiac symptoms varies widely, from asymptomatic cardiomegaly to
cardiogenic shock.
(i) In some cases, a brief (3-day) period of relative stability occurs after birth, followed
by acute decompensation [244].
(ii) Some patients require emergent embolization of the intracranial lesion, whereas oth-
ers may be medically stabilized for a while, and undergo embolization later in life.
2. Neurological manifestations.
(a) Neurological symptoms are attributable to:
(i) Intracranial venous hypertension, resulting from AV shunting and venous outflow
obstruction.
(ii) Heart failure, which may cause pre- and postnatal brain damage.
(iii) MRI may show white matter lesions or diffuse brain destruction (i.e., melting brain
syndrome) [249].
(iv) Hydrocephalus.
• The intracranial venous system normally possesses a pressure gradient that facilitates
absorption of CSF. Intracranial venous hypertension interferes with this process.
Natural History and Overall Prognosis
Although many publications about vein of Galen malformations include a discussion of the
“natural history” of these lesions, a firm understanding of the prognosis of untreated patients is
nearly impossible to obtain. The rarity of these lesions, the wide spectrum of severity at presen-
tation, and the variety of treatment approaches currently in use (treatment vs. no treatment,
arterial vs. venous embolization, ventricular shunting vs. no shunting) make it difficult to gen-
eralize about outcome. Clinical results reported below are from large series published by the
Hopital de Bicêtre in France and the Hospital for Sick Children in Toronto.
1. Overall, the survival rate for neonates with vein of Galen malformations is 50–76.9% [246, 250].
2. Spontaneous thrombosis of a vein of Galen malformation is rare, and has been reported to
occur in some 2.5% of patients (with half of them neurologically normal) [244].
13.10 Specific Considerations 739

Management and Outcomes

Most authors agree that patients at the two ends of the spectrum of severity—those with pro-
found symptoms and multisystem failure and those with completely asymptomatic lesions—
may be followed expectantly [246]. The cornerstones of treatment are embolization +/− shunt
surgery; radiosurgery has been reported [251].
Neonates
Lasjaunias and colleagues have developed a scoring system to help with decision-making
(Table 13.4) [252].
1. Score <8 is associated with a poor systemic or neurological outcome and may be an indica-
tion to withhold therapy.
2. Score 8–12 is associated with normal neurological status by medically refractory heart fail-
ure, and emergent embolization should be considered.
3. Score >12 indicates that the patient may be stable enough to delay embolization until the
child is at least 5 months of age.

Table 13.4 Bicêtre neonatal evaluation score [252]

Respiratory Renal
Points Cardiac function Cerebral function function Hepatic function function
5 Normal Normal Normal – –
4 Overload, no Subclinical, isolated Tachypnea, –
medical treatment EEG abnormalities finishes bottle
3 Failure; stable Nonconvulsive Tachypnea, does No hepatomegaly, Normal
with medical intermittent not finish bottle normal hepatic
treatment neurological signs function
2 Failure; not stable Isolated convulsion Assisted Hepatomegaly, Transient
with medical ventilation, normal normal hepatic anuria
treatment saturation function
FIO2 < 25%
1 Ventilation Seizures Assisted Moderate or transient Unstable
necessary ventilation, normal hepatic insufficiency diuresis with
saturation treatment
FIO2 > 25%
0 Resistant to Permanent Assisted Abnormal Anuria
medical therapy neurological signs ventilation, coagulation, elevated
desaturation enzymes
EEG electroencephalogram, FIO2 fraction of inspired oxygen. Maximum score = 21. Reproduced from Vascular
Diseases in Neonates, Infants and Children: Interventional Neuroradiology Management, 49, Lasjaunias P:
“Neonatal evaluation score (Bicêtre).” © 1997 Springer Science and Business Media with permission

Treatment in Neonates
1. Evaluation of neonates with vein of Galen malformations should include the following [245]:
(a) Weight and head circumference
(b) Renal and liver function tests
(c) Cranial and cardiac ultrasound exams
(d) Brain MRI, to provide information about lesion anatomy and the status of myelination.
(e) Catheter angiography is not indicated unless embolization is planned.
2. The optimal age for embolization is ≥5 months [244]. Treatment should be delayed until
then when possible.
740 13 Arteriovenous Malformations

3. The interventional strategy consists of arterial embolization to reduce the shunt.

(a) Venous embolization in neonates is associated with higher morbidity [253–256] and
should be used only when an arterial route is not available [244].
(b) Glue (e.g., nBCA) is the preferred agent for embolization because it is more resistant to
recanalization than particles or coils.
4. Reduction of 30% of shunt volume is sufficient to improve cardiac function and permit
weaning of the ventilator [244].
(a) Angiographic cure of the lesion is not necessary to control symptoms and allow the brain
to develop normally.
Outcomes in Neonates
Of 23 neonates treated at the Hopital de Bicêtre, death occurred despite or because of emboliza-
tion in 52%; of the survivors, 36.4% were neurologically normal and 63.6% had moderate or
severe retardation [245].
Infants
1. Hydrocephalus in vein of Galen malformation patients is due to intracranial venous hyper-
tension, and frequently responds to treatment of the lesion.
2. CSF shunting should be reserved until after endovascular treatment has been undertaken
[244, 247].
(a) Less than 50% of patients with hydrocephalus go on to require a shunt [246].
(b) CSF shunting can be problematic in these patients. Shunting may contribute to venocon-
gestive brain edema [257], and carries a risk of subdural hematoma development.
(c) Endoscopic third ventriculostomy is an acceptable alternative to ventricular shunting in
selected patients [244].
Outcomes in Infants
Of 153 infants treated at the Hopital de Bicêtre, death occurred despite or because of emboliza-
tion in 7.2%; of the survivors, 78.9% were neurologically normal and 21.1% had moderate or
severe retardation [245].
Children and Adults
Developmental delay is part of the natural history of untreated symptomatic vein of Galen mal-
formations [245], because of sustained, long-term intracranial venous hypertension and
hydrocephalus.
1. Elevated intracranial venous pressures are well documented in patients with vein of Galen
malformations.
2. Some authors argue strenuously that embolization should be done to normalize venous pres-
sure, and treat hydrocephalus, prior to CSF shunt placement [245].
3. Adults: Very rare [258–260].
Outcomes in Children
Of 40 children treated, no deaths occurred, 67.5% were neurologically normal and 32.5% had
moderate or severe retardation [245].
Complications of Embolization Procedures
At Hopital de Bicêtre, total of 196 patients, 1981–2002 [245]:
1. Transient neurological disability: 1.6%
2. Permanent neurological disability: 2.1%
3. Nondisabling nonneurological complications: 6.7%
4. Hemorrhage: 5.6%