Management of Large Cirsoid Aneurysms of the Scalp Using Tissue Expanders, Intravascular Occlusion, and En Bloc Excision

INTRODUCTION
Cirsoid aneurysms are rare arteriovenous fistulas
of the scalp whose etiology is usually congenital,
although traumatic fistulas have also been reported
[1-3]. The condition is called “cirsoid” (from the
Greek kirsos, meaning "varix," or varicose vein)
because of the characteristic variceal dilatation of
the draining veins. The various names that are used
to describe vascular malformations of the scalp
include aneurysm cirsoide, aneurysma serpentinum,
aneurysm racemosum, plexiform angioma, arteriovenous
fistula and arteriovenous malformation
(AVM) [1,2,4].
Cirsoid aneurysms of the scalp were first described
in 1833 by Brecht [5]. Only 10%-20% of
these arteriovenous fistulas develop following
penetrating trauma to the scalp. Traumatic arteriovenous
malformation of the scalp develops months
or even years after the scalp trauma. While congenital
arteriovenous malformation of the scalp
(the remaining 80%-90%) might be present at birth,
for most patients it is asymptomatic until adulthood
[6-8].
In 90% of patients, the superficial temporal
artery is the main supply to the fistula, with only
one dominant feeding artery in 71% of those patients
[9]. In the remaining 29% of cases usually
involve both the superficial temporal and occipital
arteries [5,9].
The most important distinction regarding AVM
is between single-hole arteriovenous fistula (usually
traumatic) and more complex lesions, which are
thought to be (congenital). These more complex
lesions are characterized by a network of abnormal
channels (nidi) between the arterial feeder (s) and
the draining vein or veins (i.e., an AVM). They
also may have components with no intervening
nidi (i.e., fistulas) [10].
The management of scalp AVM is difficult
because of the scalp's high shunt flow, intracranial
communication, complex vascular anatomy, and
cosmetic problems. The indication of treatment
includes cosmetic relief of the pulsatile or nonpulsatile
mass and the prevention of hemorrhage
and other symptoms such as headache and tinnitus
[5,11-14].
Egypt, J. Plast. Reconstr. Surg., Vol. 42, No. 2, July: 279-284, 2018
Management of Large Cirsoid Aneurysms of the Scalp Using Tissue
Expanders, Intravascular Occlusion, and En Bloc Excision
EMAN NAGY MOHAMED, M.D.; HANY SAAD SETTA, M.D. and AHMED EL-SHAHAT, M.D.
The Department of Plastic Surgery, Faculty of Medicine, Ain Shams University, Egypt
279
It is well known in the literature that the only
effective method of preventing the evolution of
AVM of the scalp is to exclude the lesion completely
from circulation [15]. The management of AVMs
of the head and neck includes observation, ligation
of the feeding arteries, embolization, and surgical
resection [2,7,9,16,17], or a combination of these
elements [18,19]. Ligation of the feeding arteries
was one of the earliest treatment methods for this
condition, although recurrence nearly always ensues
because of the development of collateral vessels
[1,2,17]. The collateral vessels that develop following
ligation of the feeding arteries might parasitize
blood flow from the brain, which can lead to
ischemic complications [20].
Endovascular treatment may be applied in order
to decrease the hemorrhage and to facilitate surgical
treatment or the direct treatment of AVMs [4,9,21,22].
The pre-operative embolization of both feeders
and nidus before surgery is safer than embolization
of the feeders alone for reducing the risk of excessive
hemorrhage [4]. Three different approaches
have been described in the literature for accessing
the fistula; femoral transarterial catheterization,
femoral transvenous catheterization and direct
percutaneous catheterization of the feeding arteries
or draining veins [13,18,23-26].
Surgical excision is the most common and
successful method of dealing with vascular scalp
lesion [6,24,27,28]. Surgical treatment is particularly
indicated for preventing bleeding, the resolution
of cosmetic problems, and for the treatment of the
accompanying issues of tinnitus and headache
[4,21,29]. Various techniques have been used to
control hemorrhage during surgery, including percutaneous
sutures of the feeding vessels [6], Interlocking
sutures along the line of incision, and the
use of a scalp tourniquet and an intestinal clamp
over the base of the flap [30]. A step-wise incision
with careful pressure control is useful for controlling
scalp bleeding [11].
The excision of large arteriovenous malformation
of the scalp leaves a large defect that requires
reconstruction; the Ideal reconstruction is the use
of hair-bearing skin flaps. Marotta et al., [31] expanded
the scalp adjacent to the arteriovenous
malformation to prepare a hair-bearing skin flap
that they used for reconstruction.
The lack of descriptions of this technique in
the literature, together with its absence in plastic
surgery journals, encouraged us to revisit this
technique. The aim of this work is thus to present
a complete excision using a combined approach
of preoperative endovascular occlusion followed
by radical excision and coverage of the defect via
expanded adjacent scalp.
PATIENTS AND METHODS
This is a retrospective case series study on five
patients with cirsoid aneurysms who were treated
from March 2012 to December 2015. Diagnosis
was based on both a clinical picture of pulsatile
swelling in the scalp with bruit and thrill and on
the intravascular dye injection (angiography). None
of our patients presented with hemorrhage, and
none had neurological deficits. The patients' lesions
were located in the temperoparietal region in two
patients, in the frontal region in two patients and
in the occipital region in one patient. No intracranial
lesion was demonstrated in any patient. Table (1)
shows the data of the five patients included in this
study.
280 Vol. 42, No. 2 / Management of Large Cirsoid Aneurysms of the Scalp Using Tissue Expanders
Table (1): Data of the 5 patients included in this study.
Patient 1
22 years
Male
None
Temporoparietal
12cm diameter
One rectangular
3 months
3 years
None
Age
Sex
History of trauma
Site
Size
Expanders
Duration of expansion
Follow up period
Recurrence
26 years
Male
None
Temporoarietal
15cm diameter
Two rectangular
3.5 months
2 years
None
Patient 2
29 years
Male
None
Frontal
9cm diameter
Two rectangular
3.5 months
One years
None
Patient 3
30 years
Male
None
Frontal
8cm diameter
Two rectangular
2.5 months
9 months
None
Patient 4
19 years
Male
None
Occipital
10 cm diameter
One rectangular
4 months
6 months
None
Patient 5
In an attempt to completely eradicate the AVM,
one or two large, rectangular tissue expanders were
then placed in the less-affected scalp area in the
subgaleal plane at least 3cm from the edge of the
AVM. All incisions for the expander placement
were perpendicular to the expansion axis and were
distant from the AVM. Ports were buried in all
patients; the use of suction drains for a few days
was the routine for all cases. In the second stage
and before excision, aggressive preoperative coiling
(occlusion) of the malformation was performed by
the intervention neuroradiology department in our
hospital. One day after endovascular occlusion,
complete excision of the malformation was then
performed. Interlocking sutures along the line of
incision were used in all patients prior to surgical
excision to decrease blood loss. The expanded
scalp was then transposed for coverage of the entire
defect.
RESULTS
All patients were treated according to the proposed
protocol. Expansion was successful in all
patients despite the seropurulent collection that
was detected in one patient around the expanders;
this was managed successfully by exteriorization
of the ports. The operating time during the application
of the tissue expanders ranged from 30 to
45 minutes in the five cases, while en bloc excision
and reconstruction ranged from 90 to 120 minutes.
Blood loss ranged from 350 to 1000ml in the en
bloc excision. Blood transfusion of 500ml whole
blood was the routine in all five cases.
All the preoperative symptoms and signs were
eliminated. The expanded flaps survived completely,
and all the patients had excellent postoperative
cosmetic appearance; No postoperative complications
related to the surgery had occurred. The
patients were followed-up with for a period ranged
from 6 to 36 months; no recurrences occurred
during the follow-up period. Figs. (1-6) show three
patients after application of the tissue expanders
and before excision of the lesions (1,3,5) and after
excision of the cirsoid aneurysms and advancement
of the expanded flaps (2,4,6).
Egypt, J. Plast. Reconstr. Surg., July 2018 281
Fig. (1): A photo shows large cirsoid aneurysm in scalp (left
temporoparietal region) of 26 year old male patient
with two inflated tissue expanders underneath the
adjacent sound scalp.
Fig. (2): A photo of the same patient in Fig. (1) after en block
excision of the cirsoid aneurysm and advancement
of expanded scalp flaps
Fig. (3): A photo shows cirsoid aneurysm in the right frontal
region of a 30 year old male patient with two inflated
tissue expanders in the adjacent scalp and cheek
Fig. (4): A photo shows the same patient in Fig. (3) after en
block excision of the cirsoid aneurysm and advancement
of expanded flaps to reconstruct the right frontal
region and anterior hair line.
DISCUSSION
Cirsoid aneurysms are rare despite the intense
vascularity of the scalp and the relatively high
frequency of trauma to this region [3]. Most of the
reports in the literature consist of individual case
reports; very few studies consist of sufficient
number of patients [7,13,18]. One study from the
literature mentioned that these lesions usually begin
as a small, subcutaneous lump on the head that
over a period of time evolves into a grotesque,
deforming mass [13 ]. A bruit or a throbbing headache
might be the chief complaint in some patients;
other presenting symptoms include pulsatile tinnitus
or (rarely) hemorrhage from the mass [25]. Large
lesions may occasionally be associated with scalp
necrosis. Congestive heart failure has occurred
with large fistulas [18]. The same sequelae were
found in our patients, except for the absence of
hemorrhage or congestive heart failure.
Definitive treatment for the condition requires
controlled en bloc excision, in which the entire
malformation is excised [15,32]. In the five patients
included in this study coiling (occlusion) of the
arteriovenous malformations was done before the
en bloc excision to limit intraoperative bleeding;
interlocking sutures were also used for the same
purpose. Blood loss still took place despite these
precautions, so blood transfusion was the routine.
Many techniques have been used to cover scalp
defects; some of these include direct closure in
small AVM defects, skin and galeal-pericranial
flaps, compound musculocutaneous flaps, as well
as free-tissue transfer [34,35]. These techniques
were not applicable in the five patients included
in this study, however, because the lesions were
large and the defects that were left after en bloc
excision were even larger. Although Free-tissue
transfer can cover large defects, it requires normal
vessels for anastomosis and the skin provided is
non hair bearing. Donor-site morbidity is another
disadvantage of the use of free flaps.
Similarly to the technique Marotta et al., [31]
used, planned tissue expansion of the adjacent
scalp allowed for large hair-bearing flaps to cover
the resultant defects after en bloc excision.
Periexpander seropurulent collection in one
patient was managed by exteriorization of the
buried ports for drainage in order to salvage the
infected expanders (as mentioned by Elshahat [36]);
this management was successful, and the expansion
was completed to the planned-for volume. A double
back-cut of the expanded flaps [37] was performed
to maximize the gain from the expanded flaps; this
allowed for full coverage of the resultant defects.
One point of criticism is that bearing a tissue
expander on the head for 3-4 months will likely
cause patients social- and work-related difficulties,
so economic and social factors should be taken
into account when indicating such a procedure.
On the other hand, the non-treated patients suffer
repeated attacks of headache and subsequently
have repeated need for sick leave in addition to
the psychological effects of the cosmetic disfigurement
from the lesion. Therefore, bearing a tissue
expander on the head for 3-4 months is made
tolerable by the elimination of these symptoms.
Conclusion:
Careful planning for the management of large
cirsoid aneurysm is crucial to assure uneventful
282 Vol. 42, No. 2 / Management of Large Cirsoid Aneurysms of the Scalp Using Tissue Expanders
Fig. (5): A photo shows cirsoid aneurysm in the right occipital
and postauricular regions in a 19 year old male
patient with inflated tissue expander underneath the
adjacent sound scalp.
Fig. (6): A photo the same patient in Fig. (5) after en block
excision of the cirsoid aneurysm and advancement
of the expanded scalp flap.
Egypt, J. Plast. Reconstr. Surg., July 2018 283
outcomes. Three-stage management with application
of tissue expanders and endovascular occlusion
shortly before en bloc excision and reconstruction
resulted in excellent results with no recurrence, no
morbidity and an appealing cosmetic outcome.