Aneurysmal dilation of the vein of Galen and straight
sinus with resultant obstructive hydrocephalus
Faiza Jamil,1 Hajira Farooq Khan,2 Iftikhar Ahmad,3 Faiz Anwer1
1

Hematology Oncology,
University of Arizona Medical
Center, Tucson, Arizona, USA
2
Radiology, Khyber Teaching
Hospital, Peshawar, Pakistan
3
Neuroradiology, IUPUI,
Indianapolis, Indiana, USA
Correspondence to
Dr Faiz Anwer,
​anwerf@​email.​arizona.​edu
Accepted 7 September 2018

Description

A 20-year-old woman was admitted to the emergency department on a previous occasion with tonicclonic seizures. Her medical history was significant
for headaches and hypertension. The headaches
were associated with nausea and vomiting. She had
no history of developmental delay or epilepsy. CT
scan of the brain showed signs of obstructive hydrocephalus due to venous hypertension as a result of
vein of Galen malformation, and hence a ventriculoperitoneal shunt was placed. Now on follow-up,
her MRI brain demonstrates a round flow void
in the suprapineal cisterns with engorged straight
sinus, compatible with vein of Galen arteriovenous
fistulous communication and shunting (figure 1).
The resultant venous hypertension leads to mild to
moderate compensated hydrocephalus.
Vein of Galen aneurysm (VGA) is an unusual
pathology, typically found in children, accounting
for less than 1% of all the intracranial vascular
malformations.1 It originates from persistent arteriovenous shunting from primitive choroidal vessels
into the median prosencephalic vein of Markowski,
the embryonic precursor of the vein of Galen.2 It
is commonly diagnosed in neonates or children,
with signs and symptoms of heart failure, macrocephaly and cranial murmurs.3
Common presentation in adults includes headaches, hydrocephalus, seizures, rounded calcified
masses in the pineal region and fistulas which
change blood flow in the dural sinuses.3 Blood flow
reorganisation to pial veins can lead to subarachnoid and intracerebral haemorrhages. Despite many
studies, there is still little known about this disease
in adults.
Vascular association between the cerebral venous
system and fistula is demonstrated by cerebral
angiography, which is the gold standard for the

Patient’s perspective
The operation has changed things for me. I can
now manage to do my daily activities, which
I had stopped doing due to the severity of my
problems. I no longer spend the major portion of
my day in bed. The sleepiness has gone, there is an
improvement in my mood. The feeling of fullness
in my head and blurring eyesight has shown
improvement. I keep visiting my doctor. According
to the latest scans, they have seen an improvement
in my condition. I was amazed to find out that, I
have a vessel malformation which has led to this
and has been there my entire life. At least the
excessive fluid in my brain led to finding it. Now
we know what is causing all the problems.

Learning points
►► Vein of Galen aneurysm is a rare finding to be

diagnosed in adults.

►► Common presentation in adults includes

headaches, hydrocephalus, seizures, rounded
calcified masses in the pineal region and
fistulas which change blood flow in the dural
sinuses.
►► Blood flow reorganisation to pial veins can
lead to subarachnoid and intracerebral
haemorrhages.
►► Cerebral angiography is the gold standard for
the diagnosis of vein of Galen malformation.
►► Intravascular embolisation is the treatment of
choice in adults, but carries a risk of permanent
neurological disability.

diagnosis of VGA. Intravascular embolisation is the
treatment of choice in adults, but carries a risk of
permanent neurological disability.2

© BMJ Publishing Group
Limited 2018. No commercial
re-use. See rights and
permissions. Published by BMJ.
To cite: Jamil F, Khan HF,
Ahmad I, et al. BMJ Case
Rep Published Online First:
[please include Day Month
Year]. doi:10.1136/bcr-2018226742

Contributors FJ contributed as the first author. FJ, HFK, IA and FA
planned and designed the study. FJ, HFK and IA searched for the
relevant studies. FJ and HFK developed the manuscript. IA helped
with the interpretation of the image. FA helped with the final
edits. FA is the senior author of this manuscript and serves as the
corresponding author.

Figure 1 (A) Axial T2, (B) axial T1 and (C) sagittal T2
MRI brain. MRI brain demonstrates a round flow void
in the suprapineal cisterns with engorged straight sinus
(green arrow), compatible with vein of Galen (yellow
arrow) arteriovenous fistulous communication and
shunting. The resultant venous hypertension leads to mild
to moderate compensated hydrocephalus.

Funding The authors have not declared a specific grant for this
research from any funding agency in the public, commercial or
not-for-profit sectors.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally
peer reviewed.

Jamil F, et al. BMJ Case Rep 2018. doi:10.1136/bcr-2018-226742

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References

1 Hosmann A, El-Garci A, Gatterbauer B, et al. Multimodality management of
vein of galen malformations-an institutional experience. World Neurosurg
2018;112:e837–47.

2 Moizuddin M, Graham B, Owens W. 1126: vein of galen malformation in adultmanagement dilemma in intensive care unit. Critical Care Medicine 2013;41:A285.
3 Recinos PF, Rahmathulla G, Pearl M, et al. Vein of Galen malformations: epidemiology,
clinical presentations, management. Neurosurg Clin N Am 2012;23:165–77.

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Jamil F, et al. BMJ Case Rep 2018. doi:10.1136/bcr-2018-226742

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