The vein of Galen malformation is a rare cerebrovascular disorder which is characterized by an abnormal direct communication between one or several cerebral arteries and the vein of Galen. In neonates, it usually causes congestive heart failure. Infants, older children, and adults usually present with mass effect, seizures, or intracranial hemorrhage. Here, we report a 6-month-old infant diagnosed with the vein of Galen malformation antenatally and presented to us with acute hydrocephalus. She underwent emergency embolization, developed in right femoral artery thrombosis as a complication requiring emergency thrombectomy and end-to-end repair. After embolization, she stabilized but hydrocephalus and raised intracranial tension persisted for which she required endoscopic third ventriculostomy.

1. Intracranial arteriovenous malformation in an infant-vein of
Galen malformation

2. Apollo Medicine 2011 December
Case Report
Volume 8, Number 4; pp. 307–309
© 2011, Indraprastha Medical Corporation Ltd
Intracranial arteriovenous malformation in an infant—vein of
Galen malformation
Vineet B Gupta*, Hemant Choudhary**, Harsh Rastogi†
, VP Singh‡
*Senior Consultant, Paediatric Neurology, **DNB Fellow, †
Senior Consultant, Neuroradiology, ‡
Senior Consultant, Neurosurgery,
Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi – 110076, India.
ABSTRACT
The vein of Galen malformation is a rare cerebrovascular disorder which is characterized by an abnormal direct
communication between one or several cerebral arteries and the vein of Galen. In neonates, it usually causes con-
gestive heart failure. Infants, older children, and adults usually present with mass effect, seizures, or intracranial
hemorrhage. Here, we report a 6-month-old infant diagnosed with the vein of Galen malformation antenatally and
presented to us with acute hydrocephalus. She underwent emergency embolization, developed in right femoral
artery thrombosis as a complication requiring emergency thrombectomy and end-to-end repair. After embolization,
she stabilized but hydrocephalus and raised intracranial tension persisted for which she required endoscopic third
ventriculostomy.
Keywords: Hydrocephalus, noninvasive treatment, vein of Galen malformation
Correspondence: Dr. Vineet B Gupta, E-mail: vineetbgupta@hotmail.com
doi: 10.1016/S0976-0016(11)60014-8
INTRODUCTION
The vein of Galen malformation is a rare cerebrovascular
disorder, which is characterized by an abnormal direct com-
munication between one or several cerebral arteries and the
vein of Galen. Posterior choroidal artery, anterior cerebral
artery, and transmesencephalic artery are the most common
arteries that join the malformation.1
It may appear in the
neonatal period or afterwards.2
CASE REPORT
A 6-month-old female child was brought to our hospital by
her parents with complaints of lethargies, vomiting, drowsi-
ness, decreased activity, not responding to the mother, and
no eye contact with the mother for the past 3 days.
There was no history of fever, cough, loose stools,
breathlessness, or seizures. The child was diagnosed as ven-
triculomegaly on antenatal ultrasonogram. Birth history and
postnatal histories were uneventful. The child had attained
developmental milestones appropriately for her age.
Physical examination showed her weight 6.5Kg (15th
percentile), length 65cm (15–50th percentile) and head
circumference 46cm (>97th percentile). Her anterior fonta-
nel was full and sutural diastasis was noted. She had a down-
ward gaze. Pupils were bilaterally equally reacting. Her limb
movements were normal. Vital signs were within normal
range. Rest of the systemic examination was unremarkable.
A diagnosis of the acute hydrocephalus was made. She
was started on intracranial hypertension management pro-
tocol. An urgent MRI brain performed showed the vein of
Galen arteriovenous fistula compressing on the posterior third
ventricle and cerebral aqueduct with obstructive hydrocepha-
lus and periventricular ooze. Echocardiogram was normal.
The child was immediately taken for digital subtraction
angiography (DSA) and glue embolization. The DSA showed
a large vein of Galen aneurism with a single feeding arterial
channel from a common trunk from the left posterior choroi-
dal and anterior cerebral artery.There was negligible drainage.
The single arterial feeder was super selectively embolized suc-
cessfully using 80% glue. No residual filling of aneurysmal
sac was seen on the postembolization check angiography.
However, at the end of the procedure, there were weak
distal pulses in the right lower limb. Intra-arterial nitroglyc-
erin and papaverine and heparin were given to overcome
the arterial spasm but not much of improvement was seen.
As further thrombolysis was contraindicated, the vascular
surgeon was requested to see the case.

3. 308 Apollo Medicine 2011 December; Vol. 8, No. 4 Gupta et al
© 2011, Indraprastha Medical Corporation Ltd
On exploration, it was found that she had a thrombus in
the right femoral artery and that had resulted in acute ischemia
of the limb. Vascular surgeon did end-to-end repair of a seg-
ment of the right femoral artery. Postvascular repair, there
were good pulses in the right lower limb (Figures 1 and 2).
Following surgery, the child was regularly monitored
for the perfusion in the right lower limb as well as for
signs of raised intracranial pressure. She was put on low-
molecular-weight heparin.
The child remained asymptomatic for a week, but then
developed vomiting and increased irritability. In view of the
increased intracranial tension, a computerized tomography
scan of the brain was done which showed increasing hydro-
cephalus.
The child was then taken for endoscopic third ventricu-
lostomy, which she tolerated well.
The child was discharged after 3 weeks of hospitaliza-
tion in a stable condition.
DISCUSSION
Intracranial arteriovenous malformations (AVM) are com-
plex medical emergencies in infants. It is reported that 30%
of AVM in childhood is the vein of Galen malformation.3
Systemic manifestations and congestive heart failure
are the most common presentations encountered in the neo-
natal period and infancy whereas neurological signs and
symptoms and hemorrhage belong mostly to the adult
symptomatology.4
The malformation develops between the 6th and 11th
weeks of gestation after the development of the circle of
Willis. It is thought to result from the development of an
arteriovenous connection between the primitive choroidal
vessels and the median prosencephalic vein of Markowski.
The abnormal flow through the connection retards the nor-
mal involution of this embryonic vein and thus prevents the
development of the vein of Galen.5
Figure 1 (A) Pre-embolization angiogram, and (B) postembolization angiogram.
A B
A B
Figure 2 (A) Postembolization computerized tomography, and (B) postembolization magnetic imaging source.

4. Intracranial arteriovenous malformation in an infant Case Report 309
© 2011, Indraprastha Medical Corporation Ltd
Neonatal Rating Score6
(Table 1)
Lasjaunias and Ter brugge score is derived from the measures
of cardiac, cerebral, hepatic, renal and respiratory function.
• <8 results in a decision not to treat,
• 8–12 prompts emergency endovascular intervention,
• 12 recommends medical treatment alone and delayed
embolization at 5 months.
CONCLUSION
With more and more technological advances in the develop-
ments in the field of interventional neuroradiology and
availability of better postprocedure intensive care, these
once nontreatable conditions with a very high mortality rate
are now potentially curable using interventional neuroradi-
ological techniques with excellent clinical results, low com-
plication rate and very low morbidity and mortality. In the
future with more centers having advanced neuroradiologi-
cal facilities, the treatment of this condition will be well
within the reach of many patients.
Table 1 Neonatal rating score.
Score Cardiac Cerebral Respiratory Renal Hepatic
5 Normal Normal Normal – –
4 Nontreated Infraclinical EEG Tachypnea bottle – –
overload anomalies finished
3 CCF stable with Nonconvulsive Tachypnea bottle Normal Normal
treatment CNS signs not finished
2 CCF unstable with Isolated convulsion Assisted ventilation Transient anuria Hepatomegaly
treatment normal function
1 Need ventilation Seizure permanent Assisted ventilation Unstable diuresis Moderate hepatic
CNS signs FiO2 >25% with treatment impairment
0 Resistant to Rx Coma Assisted ventilation Anuria Coagulation disorder
CCF: congestive cardiac failure; EEG: electroencephalogram; CNS: central nervous system.
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