Original Article
Scalp Arteriovenous Malformation (Cirsoid Aneurysm) in Adolescence: Report of 2
Cases and Review of the Literature
Daphne Li1,3, Daniel M. Heiferman1,3, Brian D. Rothstein1, Hasan R. Syed1,4, Ali Shaibani2, Tadanori Tomita1
- BACKGROUND: Scalp arteriovenous malformations, also
known as cirsoid aneurysms, are rare lesions that are
congenital, traumatic, or postinfectious in nature. These lesions
may be found incidentally or owing to signs and symptoms that
they produce, such as an enlarging pulsatile mass, headache,
tinnitus, or bleeding. These lesions often constitute high-flow
arterial blood from the superficial temporal or occipital arteries
with venous outflow into extracranial venous structures.
- METHODS: We describe diagnosis and management of 2
cases of congenital scalp arteriovenous malformations in
adolescent patients. One case had more typical vascular
supply and outflow, whereas the other case demonstrated
more uncommon arterial blood supply from extracranial
ophthalmic arteries as well as a component of transosseous
venous drainage into the intracranial superior sagittal sinus
via emissary veins.
- RESULTS: Treatment of these lesions usually consists of
endovascular embolization followed by surgical resection.
- CONCLUSIONS: Both scalp AVMs described were
successfully excised after transvenous embolization.
Consideration of risks of intracranial venous drainage must
be taken into account when evaluating AVM anatomy.
INTRODUCTION
S calp arteriovenous malformations (AVMs), also known as
cirsoid aneurysms, are rare lesions that are predominantly
thought to be congenital, but they have also been reported
Key words
- Cirsoid aneurysm
- Scalp arteriovenous malformation
- Transosseous venous outflow
Abbreviations and Acronyms
AVM: Arteriovenous malformation
MRI: Magnetic resonance imaging
STA: Superficial temporal artery
From the 1Division of Pediatric Neurosurgery and 2Department of Radiology, Ann & Robert H.
Lurie Children’s Hospital, Northwestern University Feinberg School of Medicine, Chicago,
Illinois, USA
WORLD NEUROSURGERY -: e1-e5, - 2018
to occur after trauma, infection, and inflammation.1,2 The exact
incidence is unknown, but they have been documented to
account for 8.1% of all AVMs.3 In a more recent series by Visser
et al.,4 4.7% of the vascular anomalies managed by their
institution were extracranial AVMs. Scalp AVMs are anomalous
connections between superficial scalp arteries and veins, often
fed by superficial temporal arteries (STAs) and/or occipital
arteries, but any scalp vessel may be involved.4-6 Patients usually
present with a pulsatile, enlarging mass and may have associated
headaches, tinnitus, or bleeding.
These lesions are often first detected on physical examination,
and the diagnosis is confirmed via computed tomography angi-
ography or diagnostic cerebral angiography. These high-flow
lesions may evolve slowly or have periods of rapid progression
but have not been known to resolve spontaneously. Thus, inter-
vention is commonly recommended. Treatment options include
endovascular embolization, percutaneous injection of sclerosing
agents, and surgical resection.7-12 We report 2 cases of congenital
scalp AVMs in pediatric patients that were successfully treated
with endovascular transvenous embolization with coils and Onyx
(Medtronic, Minneapolis, Minnesota, USA) and subsequent sur-
gical resection. The first of these cases demonstrates the presence
of intracranial venous drainage without concomitant intracranial
vascular abnormalities, which to our knowledge has not been
previously reported in a cirsoid aneurysm. The second case rep-
resents a typical cirsoid aneurysm in presentation, anatomy, and
management.
MATERIALS AND METHODS
The review was approved by the Institutional Review Board at
Ann & Robert H. Lurie Children’s Hospital of Chicago (IRB
2018-1799).
To whom correspondence should be addressed: Daphne Li, M.D.
[E-mail: Daphne.li@lumc.edu]
3
Current affiliation: Department of Neurological Surgery, Loyola University Medical Center,
Maywood, Illinois, USA.
4
Current affiliation: Department of Neurological Surgery, University of Virginia School of
Medicine, Charlottesville, Virginia, USA.
Citation: World Neurosurg. (2018).
https://doi.org/10.1016/j.wneu.2018.05.161
Journal homepage: www.WORLDNEUROSURGERY.org
Available online: www.sciencedirect.com
1878-8750/$ - see front matter ª 2018 Elsevier Inc. All rights reserved.
www.WORLDNEUROSURGERY.org e1

DAPHNE LI ET AL.
Figure 1. (AeH) Digital subtraction angiography. Digital subtraction
angiography of right external carotid artery injection lateral (A and C) and
anteroposterior (E and G) views in late arterial phase and venous phase,
respectively, and left external carotid artery injection lateral (B) and
anteroposterior (F) views in late arterial phase. Right (D) and left (H) internal
carotid artery injection lateral views. There is evidence of transosseous
CASE REPORTS
Case 1
History. A 14-year-old, right-handed boy was referred to the
neurosurgical clinic of Ann & Robert H. Lurie Children’s Hospital
of Chicago for evaluation of magnetic resonance imaging (MRI)
findings obtained for work-up of a prominent, pulsatile forehead
vessel. The patient was otherwise asymptomatic at presentation.
Per his parents, the prominent forehead vessel was first noticed 2
years prior and had not been present at birth. The patient was
otherwise in good health without neurologic abnormalities and
reported no history of trauma or febrile illness. The patient had no
family history of vascular malformations.
Imaging. MRI demonstrated a 4  2 cm focal vascular malfor-
mation with associated dilated vessels including the left STA. On
diagnostic cerebral angiography (Figure 1), the patient was noted
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ORIGINAL ARTICLE
SCALP AVM (CIRSOID ANEURYSM) IN ADOLESCENCE
drainage into the superior sagittal sinus (G). (I and J) Magnetic resonance
imaging. Magnetic resonance imaging of the brain T1 sagittal image
without contrast (I) and T1 axial image with contrast (J). (K and L) Plain
radiography. Native anteroposterior (K) and lateral (L) x-ray images after
lesion embolization.
to have a high-flow scalp AVM with arterial supply from bilateral
STAs and collateral branches of distal ophthalmic arteries,
including the supratrochlear and supraorbital arteries. The lesion
had a focal nidus with drainage into 2 main scalp venous tribu-
taries to the external jugular veins. There was also transosseous
emissary venous drainage into the superior sagittal sinus.
Treatment. The patient underwent transfemoral endovascular
transvenous embolization of the AVM using detachable coils and
Onyx, which reduced the vascularity of the lesion by 90%. The
patient tolerated the procedure well and was taken for surgical
resection of the lesion the following day. At surgery, a zigzag scalp
incision was made posterior to the nidus, which measured 5 cm.
The scalp was elevated to expose the AVM within the galea
aponeurosis. Two distinct transosseous outflow tracts were noted
parasagittally, coagulated, cut, and waxed thoroughly. Residual,
small feeding arteries were coagulated and ligated. Affected galea

DAPHNE LI ET AL.
Figure 2. (AeG) Digital subtraction angiography. Digital subtraction
angiography of right occipital artery injection anteroposterior (A and G) and
lateral (B and H) views in arterial and venous phases, respectively, and right
superficial temporal artery injection anteroposterior (C) and lateral (D)
views in arterial phase. Left external carotid artery injection anteroposterior
was excised. Histologic examination was consistent with AVM
with postembolization changes.
Follow-Up. Pathology showed AVM with postembolization
changes. The postoperative course of the patient was uncompli-
cated. At the 12-month follow-up examination, there was no evi-
dence of residual or recurrent AVM, and the patient was doing
well with a completely healed surgical wound.
Case 2
History. A 14-year-old, right-handed girl was referred to the
neurosurgical clinic of Ann & Robert H. Lurie Children’s Hospital of
Chicago for evaluation of MRI findings performed for work-up of a
mass on the left parieto-occipital scalp that was first noted at age 9
years and had since increased in size. Occasionally, the lesion
became red and warm and was noted to be intermittently pulsatile.
The patient’s past medical history was significant for cyclic vomiting
and migraines. There was no known history of infection or trauma.
Imaging. MRI demonstrated large flow voids in the scalp
concerning for a vascular lesion. Diagnostic angiography revealed
a high-flow left parietal scalp AVM with supply from both occipital
arteries and both STAs and convergence into a single nidus, from
which there was ramification into multiple scalp veins and the left
external jugular vein (Figure 2).
Treatment. The patient underwent transfemoral endovascular
transvenous embolization of the parietal scalp AVM. Detachable
coils and Onyx were used to achieve 90% flow reduction of lesion.
The patient then underwent resection 2 days later. At surgery, a
curvilinear incision was made in the scalp over the nidus. Doppler
was used intraoperatively to detect any residual arterial feeders.
The AVM was superficial to the galea, and the scalp was elevated
to expose the AVM for excision. Histologic examination was
consistent with AVM with postembolization changes.
WORLD NEUROSURGERY -: e1-e5, - 2018
ORIGINAL ARTICLE
SCALP AVM (CIRSOID ANEURYSM) IN ADOLESCENCE
(E) and lateral (F) views. (IeL) Plain radiography and digital subtraction
angiography. Native anteroposterior (I) and lateral (J) x-ray images and
digital subtraction angiography of left occipital artery injection
anteroposterior (K) and lateral (L) views after lesion embolization.
Follow-Up. Pathology showed AVM with postembolization
changes. The postoperative course for this patient was also un-
complicated. The patient was most recently seen for a 6-month
postoperative follow-up visit and had no symptoms of pain,
redness, or swelling. Physical examination showed no evidence of
recurrent AVM and a well-healed incision.
DISCUSSION
AVMs are abnormal fistulous connections between feeding
arteries and draining veins without an intervening capillary bed.
Located in the scalp, these rare entities are also known as cirsoid
aneurysms, plexiform angiomas, scalp arteriovenous fistulas, and
arteriovenous aneurysms. These were first described by Brecht in
1833 and derive their colloquial name, cirsoid aneurysm, from
their resemblance to venous varix.
Pathophysiology
There is no clear consensus in the literature on the etiology of
these lesions, either congenital or traumatic. Congenital AVMs are
thought to be the more predominant etiology, especially in pedi-
atric patients. Generally, congenital AVMs are thought to arise
from failure of regression or apoptosis of primitive embryonic
plexus. These remain through embryologic development as
nonfunctional shunts and are thought to later canalize to form
small arteriovenous connections that eventually start a cycle of
dilation, causing local ischemia and recruitment of vascular col-
laterals.12 Trauma results in a fistulous connection secondary to
mechanical damage to apposing arterial and venous walls,
which eventually enlarges into a shunt owing to the high-flow
arterial blood flowing into a low-pressure venous system.13 This
trauma can involve penetrating trauma, history of prior surgical
interventions, or mild, blunt trauma.14 In addition, a few scalp
AVMs are thought to be due to infection or, rarely, familial.13
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DAPHNE LI ET AL.
Table 1. Schobinger Classification of Arteriovenous
Malformations
Stage Features
I (quiescence) Cutaneous blush/warmth
II (expansion) Bruit, audible pulsations, and expanding lesion
III (destruction) Pain, ulceration, bleeding, and infection
IV (decompensation) Cardiac failure
Subsequent growth of these AVMs may be attributed to
increased concentrations of circulating hormones during puberty
resulting in an increased production of vascular endothelial
growth factor leading to neovascularization.11,15-17 One retrospec-
tive study demonstrated a 2-fold increased risk in rapid progres-
sion of scalp AVMs, regardless of gender, during puberty.17 In
both of our patients, there was a history of an enlarging lesion
over the last couple of years, with the patient finally presenting
for evaluation at age 14. Without any notable history of trauma,
we believe our cases to be examples of congenital AVMs.
Diagnosis
Diagnosis of these vascular malformations begins with computed
tomography angiography or diagnostic cerebral angiography, usu-
ally after a patient has noted a persistent or progressively enlarging
scalp mass that may be associated with pain, a loud bruit, head-
aches, or bleeding. Vascular imaging may also aid in differentiating
scalp AVMs from other extracranial vascular pathology, such as
scalp hemangiomas or sinus pericranii. In contrast to a scalp AVM,
sinus pericranii is strictly a venous anomaly in which there is direct
communication between intracranial and extracranial venous
systems. Scalp AVMs often receive their arterial supply from the STA
or occipital arteries before draining into extracranial venous struc-
tures. Similar to AVMs in other locations, the nidus may have
associated aneurysms. Vascular studies help to aid in guiding
intervention and management approaches for delineating AVM
feeders and venous outflow.
Of interest and novelty to the literature, our first case had
atypical arterial supply and unique venous drainage. In addition to
bilateral STA feeders, there were distal extracranial branches of the
bilateral ophthalmic arteries, the supratrochlear and supraorbital
arteries, that contribute to the anterior portion of the nidus.
Branches of the ophthalmic artery from the internal carotid artery
(supraorbital and supratrochlear arteries) normally contribute
blood supply to the scalp; thus, although rare, it is not unexpected
to note ophthalmic supply to a cirsoid aneurysm.18 Additionally,
there is evidence of transosseous channels that drain from the
nidus into the superior sagittal sinus likely through emissary
veins. If the lesion had not been effectively treated, we speculate
that with arterialized veins draining into the intracranial venous
circulation, a potential existed for the development of venous
hypertension and subsequent elevated intracranial pressure or
cortical venous reflux. To our knowledge, intracranial venous
drainage from a cirsoid aneurysm without concomitant
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ORIGINAL ARTICLE
SCALP AVM (CIRSOID ANEURYSM) IN ADOLESCENCE
intracranial vascular malformations has not been previously
reported in the literature.19,20
Management
Scalp AVMs can be classified per the Schobinger classification for
peripheral AVMs (Table 1). Generally, treatment is indicated for
lesions that are symptomatic or causing tinnitus or headache or
that are Schobinger grade II or higher. Often, owing to the
superficial nature of these AVMs, even lower grade lesions are
treated for cosmetic reasons. Treatment of these lesions, other
than for cosmesis, is to prevent sequelae of continued shunting of
a high-pressure arterial system into the low-pressure venous
system. Over time, lesions can progress to high Schobinger grades,
resulting in recurrent hemorrhage, scalp necrosis, cardiac failure,
seizures, mental retardation, and intracranial ischemia.1,21-23 In
addition to clinical features, morphology of the AVM should be
considered in making decisions for treatment. For example,
evaluation for intracranial venous drainage should occur, given the
theoretical risk of venous hypertension and potential for cortical
venous reflux, similar to in dural arteriovenous fistulas.
Subsequent management of scalp AVMs includes transarterial
or transvenous embolization, percutaneous intranidal injection of
sclerosing agents, and surgical resection. Management of an AVM
with embolization may be associated with risks of embolization of
nontarget arteries, local inflammation, and soft tissue tattooing. It
is also associated with a higher rate of recurrence compared with
surgical excision.11,24 However, embolization alone, whether
endovascular or percutaneous, can still effectively manage small
AVMs with few feeding arteries.9 It may also be the intervention of
choice when considering extremely large or diffuse aneurysms in
patients whose comorbidities would preclude them as surgical
candidates.
Surgical excision, with the goal of gross total resection of the
nidus, is the most commonly employed method for definitive
management of scalp AVMs.7,8,11,12,25,26 The nidus is often local-
ized in the galeal aponeurosis. Resection is aimed at removing the
affected galea from the subcutaneous tissue and the pericranium,
which are not involved directly. Dilated arteries and veins of the
AVM may extend into the subcutaneous tissue, which can be
surgically separated without causing scalp ischemic necrosis.
Preoperative embolization, performed within 24e48 hours of
surgical resection, may be employed to reduce intraoperative
blood loss and decrease the risk of recurrence.25
CONCLUSIONS
Scalp AVMs are rare lesions that are typically due to anomalous
connections between superficial STA or occipital arteries and
venous outflow into extracranial veins. We report a scalp AVM
with venous outflow that had a transosseous component into the
superior sagittal sinus without associated intracranial vascular
abnormalities. As with entirely extracranial lesions, scalp AVMs
with limited transosseous venous outflow into intracranial sinuses
may be successfully managed with preoperative embolization and
surgical excision. Preoperative vascular imaging is crucial in
demonstrating AVM morphology and guiding management
decisions.

DAPHNE LI ET AL.
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Conflict of interest statement: The authors declare that the
article content was composed in the absence of any
commercial or financial relationships that could be construed
as a potential conflict of interest.
Received 24 April 2018; accepted 23 May 2018
Citation: World Neurosurg. (2018).
https://doi.org/10.1016/j.wneu.2018.05.161
Journal homepage: www.WORLDNEUROSURGERY.org
Available online: www.sciencedirect.com
1878-8750/$ - see front matter ª 2018 Elsevier Inc. All
rights reserved.
www.WORLDNEUROSURGERY.org e5