Case presentation: Vein Of Galen

Malformation

Presenter : Dr. Rasan suresh (DM resident)

Moderator: Dr. Medha Goyal(Asst Professor)
Dr. Ruchi Nanavati (HOD and Professor)

Department of Neonatology, KEM Hospital

 Objectives
• Outline of the case
• Anatomy of Vein of Galen Malformation
(VOGM)
• Literature review – Diagnostics and
Management of VOGM
• Neurointervention techniques
• Bibliography
 History
Maternal details:
• 21 years, housewife
• Married since 3 years, non-consanguineous
• Primigravida
• Regular antenatal visits
• Dating scan and anomaly scan were normal
 Antenatal scans
• Scan at 30 wks gestation: Cystic lesion of size
2.4 x 2.1 cm in supratentorial paramedian
region draining into straight sinus s/o Vein of
Galen Malformation (VOGM)
• Scan at 36 wks showed dilated median
prosencephalic vein (Vein of Galen
Malformation) with associated cardiomegaly.
Vein of Galen Malformation
 Anatomy

• Vein of Galen arteriovenous malformations

(VOGMs) are shunts that form in utero
between the choroidal arteries and the
precursor of the vein of Galen, the Median
Prosencephalic Vein of Markowski.

Berenstein A, Fifi JT, Niimi Y, et al. Vein of Galen malformations in neonates: new
management paradigms for improving outcomes. Neurosurgery 2012;70:1207–13;
discussion 13–14
Early vascularization of choroid plexus
 Anatomy
• It is a congenital vascular malformation
consisting of multiple arteriovenous shunts
draining into the Median Prosencephalic Vein
of Markowski, which is not actually the Vein of
Galen itself, but rather a persistent embryonic
structure that precedes the vein of Galen.
• So the name vein of Galen malformation is a
actually a misnomer
Persistence
of
malformed
Median
Procenceph
alic Vein of
Markowski
Vein of Galen
Malformation
(dilated Median
prosencephalic
vein) draining via
a persistent
falcine sinus
 Case details
• Delivered in at 40wks by LSCS i/v/o CPD (outside
hospital)
• Cried immediately after birth
• Birth weight 3000 gms (between 10th and 50th centile)
• Length 50cm (between 10th and 50th centile)
• Head circumference 34.5cm (between 10th and 50th
centile)
 Case details

• USG skull on day of life 2 was s/o VOGM

• Transferred to KEM on DOL 5 for further
management.
 On Admission to KEM

• On day 5 of life
Vitals at admission
• Temp- 36.6 c
• HR- 168/min
• RR- 72/min
• CRT <3sec, NIBP – 72/44 (53) [between 50th and 95th
centile], Perfusion index- 1.2
• Spo2 70% pre ductal, 68 % postductal
• General examination – normal
• CVS – S1,S2 normal, no murmur
• RS- Bilateral air entry equal,
Bilateral crepts,
Subcostal retractions + (Downe’s score-
5/10)
• Per Abdomen- soft, liver 4.5cm.
• CNS- Conscious, alert, tone normal
AF – 1*1cm
Bruit heard over anterior fontanel
Clinical Impression- Term/AGA/VOGM with
Congestive Heart Failure(CHF)
Chest x ray CTR of 0.70, without pulmonary plethora
Bedside neurosonogram

Vein of Galen
Malformation
USG skull with Colour doppler
USG skull with doppler

Doppler shows
high flow
pattern
suggestive of
arteriovenal
connection
 2D echo On Admission
Day of IVC Fractio TR TAPSE LVO(m PDA
life collapsi nal jet l/kg/m
bility shorte in)
index ning
Day 5 18% 32%
(on
admiss
ion)
• Baby was stabilised.
• CPAP support of 7/55%
• Inotropic and decongestive measures
initiated with Inj. Milrinone and Furosemide.
 Interventional Radiology:
Baby was advised to continue on medical
management and embolization was planned, in
case of further worsening.
 Case continued
On DOL 6:
• Temp 36.6 C
• HR- 180/min
• RR- 84/min
• CRT <3sec, NIBP- 74/48 (57)
• Spo2- 84% on CPAP 7/55%
• CVS- S1,S2 present, no murmur
• Rs- Increase in retractions (DS- 7/10)
• Per Abdomen- Soft, liver 6cm
• CNS- Irritable
 2D echo monitoring
Day of IVC Fraction TR jet TAPSE LVO(ml/ PDA
life collapsibili al kg/min)
ty index shorteni
ng
Day 5 18% 32%
(on
admissio
n)
Day 6 4% 29% 62 7.8 180 3.4mm
L to R
IVC Collapsibility index

IVC full
TR jet regurgitation on Apical view

TR jet regurgitation
TR jet regurgitation
• Baby was ventilated (TCPL-AC mode) with
settings of PIP- 20, PEEP- 7, fio2- 80% , MAP- 10
• 2D echo: Worsening hemodynamic parameters
and severe PPHN

• In view of clinical worsening on medical

management and the Bicêtre Score of 12, baby
was planned for embolization.
The Bicêtre Score
• The Bicêtre Score– uses indices of systemic organ failure
• Useful predictor of survival and outcome
1. Score >12 – continue medical management and
embolization at 5-6month
2. Score 8 -12 – Emergent embolization
3. Score <8 – often signifies poor prognosis and does not
warrant treatment

Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, GarciaMonaco R. The management
of vein of Galen aneurysmal malformations. Neurosurgery 2006;59(05, Suppl 3):S184–S194,
discussion S3–S13
 The Bicêtre Score (pre procedure)

1. Cardiac function - 1 (intubated)

2. Cerebral function – 5 (normal)
3. Respiratory function – 1 ( fio >25%)
4. Hepatic function – 2 ( liver span 6cm below
costal margin with normal LFT)
5. Renal function – 3 ( normal)
Total - 12
 EMBOLIZATION
• CHF refractory to medical management in a
newborn necessitates emergency embolization
• It will relieve the hemodynamic load on the
heart.
• Partial, rather than complete, obliteration of
the shunt is the goal
Gupta AK, Rao VR, Varma DR, Kapilamoorthy TR, Kesavadas C, Krishnamoorthy T, et al:
Evaluation, management, and long-term follow up of vein of Galen malformations. J
Neurosurg 105:26–33, 2006
 EMBOLIZATION
• This allows for redistribution of blood flow to
the heart and brain to allow normal cardiac
and neurological development.
• The residual shunt can then be occluded at a
later time to reduce complications.

• Gupta AK, Rao VR, Varma DR, Kapilamoorthy TR, Kesavadas C, Krishnamoorthy
T, et al: Evaluation, management, and long-term follow up of vein of Galen
malformations. J Neurosurg 105:26–33, 2006
 Angiogram Pre Procedure
• VOGM embolization
was done on DOL6.
• Diagnostic Angiogram
showed very high flow
Vein of Galen
malformation with
multiple dilated
anterior and posterior
choroidal arteries
draining into dilated
VOGM
Feeding Arteries of VOGM
 Angiogram Post Procedure

• Two of the feeding

arteries were
embolized with
80% glue(N-butyl-
cyanoacrylate)
• Angiogram after
that showed
marked reduction
in flow to the fistula
MRI Angiogram Before MRI Angiogram After
embolization Embolization
Glues cast
Bicêtre Score( post procedure)
1. Cardiac function - 1 (intubated)
2. Cerebral function – 5 (normal)
3. Respiratory function – 1 ( fio >25%)
4. Hepatic function – 2 ( liver span 6cm with
normal LFT)
5. renal function – 3 ( normal)
Total - 12
 Post procedure
• CHF improved: Decrease in tachycardia, liver
size regressed from 6.5cm to 3cm over 6-8
hours.
• Ventilator settings reduced from 20/7/80% to
16/7/40%
 Serial 2D echo monitoring
Day of life IVC Fractional TR jet TAPSE LVO(ml/kg/ PDA
collapsibility shortening min)
index
Day 5 18% 32%
(on
admission)

Day 6 4% 29% 62 7.8 180 3.4mm

(Pre L to R
procedure)

Day 6 20% 45% 50 8 210 3.4mm

(Post L to R
procedure)

Day 7 26% 37% 60 8 184 3.4mm

L to R
Day 8 6% 28% 40 9 210 3.5mm
L to R
 Second Session of Glue
Embolization
• In view of persistent CHF with severe PPHN
baby was planned for repeat embolization on
day 11
• MRI angiography was done to detect feeding
vessels
• Baby underwent 2nd session of glue
embolization of VOGM.
Indications of repeat embolization

• Worsening after the procedure.

• No improvement after embolization.

• Initial improvement followed by worsening.

2D Echo Findings Post 2nd Time Embolization

IVC TR jet TAPSE

collapsib Fraction
ility al
index shorteni
ng
Day 12 20% 43% 50 9
Day 13 30% 42% 52 8.9
Currently baby is on
TCPL- AC mode,
antibiotics, heart failure
and PPHN line of
management.
 Vein Of Galen Malformation

• Current prevalence estimates of VOGM are

quite low, often cited at 1 of 25,000 deliveries

Lasjaunias P, Hui F, Zerah M, et al. Cerebral arteriovenous malformations in

children: management of 179 consecutive cases and review of the literature. Childs
Nerv Syst 1995;11:66 –79;
 Classification of VOGM
MURAL TYPE
• High-flow shunts
• Supplied by the collicular
and posterior choroidal
arteries
• Better tolerated clinically
CHOROID type
• Involves extensive arterial
network between the
arterial feeder
• More severe symptoms
 Pathophysiology of VOGM
• After birth and removal of the placenta, flow
through the fistula suddenly increases.
• Large arteriovenous shunts reduce the
diastolic pressure within the aorta.
• Subsequently, reduced coronary blood flow,
coupled with the high ventricular pressure,
can lead to myocardial ischemia.

Gupta AK, Varma DR: Vein of Galen malformations: review. Neurol India 52:43–53,
2004
 Systemic Manifestations

CARDIAC NEUROLOGICAL
Manifestations Manifestations
• Cerebral low-resistance Are due to
arteriovenous shunts in VGM lead 1. Tremendous flow of blood to the
to fistula, leads to ischemic damage to
cerebral parenchyma secondary to
1. Increased venous return to the blood diversion.
right atrium, 2. Reduction in venous drainage
2. Subsequent pulmonary secondary to poorly developed
hypertension due to increased venous system or venous stenosis
pulmonary blood flow, and 3. Altered hemodynamics due to cardiac
3. Ultimately congestive heart failure manifestations
as a result of the increased 4. High cerebral venous pressure and
preload cerebral edema.
 Antenatal Detection Of VOGM
Complications

• Congestive heart failure developed prenatally can be

detected on ultrasound
• In severe cases it can lead to multiorgan failure and
irreversible brain damage.
• While antenatal diagnosis is not an indication for abortion
or early or cesarean delivery, in utero cardiac failure and
brain damage represent indications for abortion.

Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R: The management of
vein of Galen aneurysmal malformations. Neurosurg 59:S184–S94, 2006
 Clinical Presentation
• Gold et al. described typical clinical presentations that
remain valid today for 3 age groups of patients with VOGMs.
1. Neonates
2. Infants
3. Older children and Adults

• The clinical pictures are correlated with the age of

presentation and the underlying pathophysiology.

Gold A, Ransohoff J, Carter S: Vein of Galen malformation. Acta Neurol Scand Suppl 40 (11
Suppl):1–31, 1964
 Clinical Presentation

Neonates Older Children

Infancy
and Adults
• Headache and
•Severe •Mild cardiac seizures
cardiorespiratory manifestations caused by
failure including intraparenchy
hydrops (94%) mal or
• These patients
most frequently subarachnoid
• It presents at or present with hemorrhage.
shortly after birth. hydrocephalus or
seizures
 Additional considerations

• Severe pulmonary hypertension may

complicate management.
• Cyanosis may be present and may thus be
mistaken for congenital heart disease.
• An electrocardiogram can detect some
features of myocardial infarction.

Kothari SS, Naik N, Juneja R, Saxena A: Aneurysm of the vein of Galen in neonates:
report of four cases. Indian Heart J 53:499–502, 2001
 Current scenario

• In any infant born with high-output cardiac

failure, a VGM should be ruled out.
• The mortality rate in the past was close to
100%,
• Recent advances in endovascular techniques
have greatly improved the survival rate.
 Imaging Modalities

(1) Ultrasound
• Noninvasive technique for bedside evaluation
of the cerebral vascular system
• Quick and convenient, although strongly
operator dependent.

Taylor GA: Intracranial venous system in the newborn: evaluation of normal

anatomy and flow characteristics with color Doppler US. Radiology 183:449–452,
1992
 (2) Computed Tomography

• Contrast-enhanced axial CT scanning of the

brain can demonstrate
 multilobulated, intensely enhancing lesion,
 ventricular dilation,
 periventricular white matter hypodensities,
 diffuse cerebral atrophy, and
 thrombosis within the aneurysm sac.
(3) Magnetic Resonance Imaging

MR imaging is increasingly used, not only for

the characterization of the lesion but also the
documentation of brain atrophy and cardiac
abnormalities.

Jones BV, Ball WS, Tomsick TA, Millard J, Crone KR: Vein of Galen aneurysmal
malformation: diagnosis and treatment of 13 children with extended clinical
follow-up. AJNR Am J Neuroradiol 23:1717–1724, 2002
 (4) Angiography

• Angiography is the gold standard for precise

evaluation of VOGM angioarchitecture.
• It includes
o Detailing the anatomy of arterial feeders
o The hemodynamics of venous drainage, and
o It provides access for endovascular
management.
 Management

• A comprehensive multidisciplinary approach

in the intensive care units, has significantly
improved the poor prognosis of patients with
VGM.
Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R: The
management of vein of Galen aneurysmal malformations. Neurosurg 59:S184–S94,
2006
 1. Microneurosurgery

• Despite advances in Microneurosurgery,

complete removal of the lesion in newborns is
rarely achieved or advised, because of
1. The hemodynamic instability
2. Location of the lesion
3. Poor myelination of the brain parenchyma,
and
4. Cerebral venous hypertension.
 2. Endovascular Treatment

• Endovascular access can be gained via the

transarterial or transvenous route.
• In the newborn arterial access can be
achieved through the umbilical artery if patent
or through the femoral artery.
• The embolic glue of choice to occlude the
arteriovenous fistula on the arterial side is N-
butyl-cyanoacrylate, and more recently
Ethylene vinyl alcohol (Onyx) has been used
as well.

Gailloud P, O’Riordan DP, Burger I, Levrier O, Jallo G, Tamargo RJ, et al: Diagnosis
and management of vein of Galen aneurysmal malformations. J Perinatol 25:542–
551, 2005
Studies On Clinical Outcomes Following
Embolization
 Complication and Prognosis

• Treatment during the neonatal period has

guarded neurologic outcome due to

 Poorer cardiologic status at presentation and

 Increased severity of disease.
 Complication and Prognosis

• Perioperative complications are not negligible

• It is more frequent in neonates than in infants.
• Treated according to the The Bicêtre score
guidelines are more likely to experience good
outcomes than those who were not.

Endovascular Treatment of Vein of Galen Malformations: A Systematic Review and

Meta-Analysis X W. Brinjikji, X T. Krings, X M.H. Murad, X A. Rouchaud, and X D.
Meil 2017
 Endovascular Treatment of Vein of Galen
Malformations: A Systematic Review and
Meta-Analysis
• A total of 27 series with 578 patients were included
with 41.9% of patients were neonates
• Endovascular treatment of VOGMs is generally safe
and effective
• Can result in good long-term outcomes for patients in
experienced centers.
• An emphasis on patient selection and timing is the key.
Endovascular Treatment of Vein of Galen Malformations: A Systematic Review and Meta-
Analysis X W. Brinjikji, X T. Krings, X M.H. Murad, X A. Rouchaud, and X D. Meil 2017
 Take home messages

• Vein of Galen is an arteriovenous malformations

though uncommon, should be considered in cases
of high output cardiac failure.
• Emergency embolization is the treatment of choice
in newborn with CHF refractory to medical
management .
• The Bicêtre Score is an useful predictor of survival
and outcome.
• Patients requiring treatment in neonatal period
have a guarded neurologic outcome.
 Bibliography
• Berenstein A, Fifi JT, Niimi Y, et al. Vein of Galen malformations in neonates: new
management paradigms for improving outcomes. Neurosurgery 2012;70:1207–
13; discussion 13–14
• Gupta AK, Varma DR: Vein of Galen malformations: review. Neurol India 52:43–
53, 2004
• Gupta AK, Rao VR, Varma DR, Kapilamoorthy TR, Kesavadas C, Krishnamoorthy T,
et al: Evaluation, management, and long-term follow up of vein of Galen
malformations. J Neurosurg 105:26–33, 2006
• Lasjaunias P, Hui F, Zerah M, et al. Cerebral arteriovenous malformations in
children: management of 179 consecutive cases and review of the literature.
Childs Nerv Syst 1995;11:66 –79;
• Garcia-Monaco R, Lasjaunias P, Berenstein A: Therapeutic management of vein of
Galen aneurysmal malformations, in Vinuela F, Halbach VV, Dion JE (eds):
Interventional Neuroradiology: Endovascular Therapy of the Central Nervous
System. New York: Raven Press, 1992, p 1
• Gailloud P, O’Riordan DP, Burger I, Levrier O, Jallo G, Tamargo RJ, et al: Diagnosis
and management of vein of Galen aneurysmal malformations. J Perinatol
25:542–551, 2005
• Casasco A, Lylyk P, Hodes JE, Kohan G, Aymard A, Merland JJ: Percutaneous
transvenous catheterization and embolization of vein of Galen aneurysms.
Neurosurgery 28:260–266, 1991 y also occur
• Chevret L, Durand P, Alvarez H, Lambert V, Caeymax L, Rodesch G, et al: Severe
cardiac failure in newborns with VGAM. Prognosis significance of hemodynamic
parameters in neonates presenting with severe heart failure owing to vein of
Galen arteriovenous malformation. Intensive Care Med 28:1126–1130, 2002
• Vein of Galen malformation Stanley Hoang, B.S., Omar Choudhri, M.D., Michael
Edwards, M.D., and Raphael Guzman, M.D. Department of Neurosurgery,
Division of Pediatric Neurosurgery, Lucile Packard Children’s Hospital, Stanford
University School of Medicine, Stanford, California
Thank you.