TECHNICAL NOTE

Effectiveness of skull models and surgical simulation:

comparison of outcome between different surgical techniques
in patients with isolated brachycephaly
Hakan Emmez & smail Kkdk &
Alp zgn Brcek & Aydemir Kale & Eren Seen &
Gonca Erba & Reha Yavuzer & M. Kemali Baykaner
Received: 13 May 2009 / Published online: 3 July 2009
# Springer-Verlag 2009
Abstract
Purpose The aim of this study was to emphasize the impor-
tance of preoperative surgical planning using 3D skull
models in craniosynostosis surgery.
Methods By using 3D polymethyl methacrylate skull models
manufactured using 3D tomography images, the authors
previously showed that after fronto-parietal osteotomy,
instead of fixing the fronto-parietal bone flap without rotation,
angled advancement with horizontal osteotomy provides
maximum increase in intracranial volume, in a bilateral
coronal craniosynostosis model. After changing the operation
technique using data gathered from previous studies, we
reviewed two bilateral craniosynostosis patients operated with
the new technique and compared it with two patients that
were operated with the old technique.
Results Comparing cranial indexes (CI), significant improve-
ment was detected in both groups. The decrease in CI in the
second group was slightly better than the first group. In the
comparison of intracranial volume (ICV), there was an
increase in ICV values in both groups. The percentage of
increase between two groups was similar. The morphological
outcome was satisfactory in all patients. There were no major
or minor complications and morbidity.
Conclusions Current multislice tomography technology and
stereolithographic procedures provide an excellent surgical
simulation model to find new techniques and predict the
outcome. These models should be used in all complex and
syndromic craniosynostosis for both better results and
reducing the operative time and associated blood loss.
Keywords Brachycephaly
.
Craniosynostosis
.
Skull model
.
Surgical planning
Introduction
Craniosynostosis is the premature ossifications of cranial
sutures that cause specific deformations of the skull. The
incidence is reported to be 0.6/1,000 births [17]. The surgical
correction of craniosynostosis is complex and cosmetic
aspects present a major issue. An interdisciplinary approach
is required for better outcome. Surgical time and blood loss
are important in small children undergoing surgery. Brachy-
cephaly can be isolated or present as a part of a syndrome.
The length of the skull of these children is shortened, leading
to a supraorbitonasal regression, whereas the breadth and the
height are increased. In addition to a morphological
alteration, premature synostosis prevents the adaptation of
the cranial volume to the growing brain and causes
intracranial hypertension which may lead to altered mental
development and blindness [1, 14, 17]. The surgical goal in
brachycephaly is not only to correct the dysmorphology but
H. Emmez
:
A. . Brcek
:
A. Kale
:
E. Seen
:
M. K. Baykaner
Division of Pediatric Neurosurgery, Department of Neurosurgery,
Gazi University Faculty of Medicine,
Ankara, Turkey
. Kkdk
:
R. Yavuzer
Department of Plastic and Reconstructive Surgery,
Gazi University Faculty of Medicine,
Ankara, Turkey
G. Erba
Department of Radiology, Gazi University Faculty of Medicine,
Ankara, Turkey
A. . Brcek (*)
Beyin ve Sinir Cerrahisi AD, Gazi niversitesi Tp Fakltesi,
Poliklinikler 1. Kat,
06500 Ankara, Turkey
e-mail: alpob@yahoo.com
Childs Nerv Syst (2009) 25:16051612
DOI 10.1007/s00381-009-0939-y
also to enlarge the intracranial volume [1, 14]. Current
computed tomography technology allows high-resolution
imaging of craniosynostosis. Multiplane reconstructions can
be performed for both diagnosis and surgical planning [12,
13]. By using 3D tomography images, a polymethyl
methacrylate skull model can be manufactured. Virtual
operative planning is possible by using these models
(Fig. 1). By using these models, the authors previously
showed that after fronto-parietal osteotomy, instead of fixing
the fronto-parietal bone flap without rotation, angled
advancement with horizontal osteotomy provides maximum
increase in intracranial volume, in a bilateral coronal
craniosynostosis model [4]. Depending on this finding, the
authors change the operative technique in the last two cases
and aim to analyze the effectiveness of two different surgical
techniques in brachycephaly. As other malformations are
present in syndromic cases, patients with isolated brachy-
cephaly were selected to achieve better standardization.
Fig. 1 A1A5 Left anterior plagiocephaly model in five projections; B1B5 scaphocephaly model in five projections; C1C5 Aperts syndrome
model in five projections; D1D5 hypertelorism model in five projections; E1E5 trigonocephaly model in five projections
1606 Childs Nerv Syst (2009) 25:16051612
Patients and methods
We found that instead of fixing the fronto-parietal bone flap
without rotation, angled advancement with horizontal
osteotomy provides maximum increase in intracranial
volume (ICV) and maximum decrease in cephalic index
(CI) in a bilateral coronal craniosynostosis model. After this
finding, we changed our surgical technique. We reviewed
the charts of two children with isolated brachycephaly who
were operated with the old fronto-orbital advancement
technique (180 rotation with horizontal osteotomy and
angling) and the last two cases who were operated with new
fronto-orbital advancement technique (only horizontal
osteotomy and angling) in Gazi University Medical School
between 2004 and 2008. Patients characteristics and
outcome measures are shown in Table 1. Preoperative and
postoperative computed tomography scans taken under
similar conditions were compared in terms of ICV, CI,
and morphological outcome. CI is the ratio of the maximum
width of the head to its maximum length (i.e., in the
horizontal plane, or front to back), sometimes multiplied by
100 for convenience. CI value is in close relation with
morphology and has less effect on ICV.
ICV measurement
All patients underwent cranial computed tomography (CT)
in both preoperative and postoperative period for assess-
ment of intracranial volumes. CT examinations were
performed with a 64-multidetector computed tomography
(Lightspeed VCT, GE Healthcare, WI, USA). Scanning
parameters were selected as follows: 100 kV, 180 mA,
0.625 mm slice thickness, and interval, field of view
22 cm. Patients were scanned from vertex to foramen
magnum. After acquisition, images obtained were trans-
ferred to a work station (GE Advantage Windows v.4.3).
Axial images were used for volumetric reconstruction. The
outline of each fifth section was traced manually. The
computer stacked all data from each section together to
build a virtual model. Volumetric values were obtained by
the inherent software volume rendering algorithm. The
calculated preoperative and postoperative intracranial
volumes were compared. Morphological outcome is also
evaluated.
Surgical technique
All of the operations were performed by the same surgeons
in the craniofacial surgery team which includes neuro-
surgeons and plastic and reconstructive surgeons. After
injection of a local anesthetic, the operative procedure
began with bicoronal skin incision behind the ears. The
scalp was reflected in the subperiosteal layer up to the
superior orbital rim. The temporalis muscle was also
elevated and moved laterally in the subperiosteal plane to
expose the pterion. Burr holes were placed according to the
proposed craniotomy lines. The fronto-parietal bone flap
involving the coronary sutures was elevated in one piece
after the craniotomy which was performed 1 cm superior to
the supraorbital bar. In the older technique, fronto-orbital
bone flap was rotated 180 clockwise and divided into two
pieces with horizontal osteotomy. Then, the pieces were
fixed with angling using absorbable miniplates and screws.
The new bone flap was fixed to supraorbital bar with
absorbable miniplates and screws. The new bone flap was
also fixed to temporal and parietal bones bilaterally with
1.2 cm advancement (Fig. 2). In the new technique after
horizontal osteotomy to fronto-parietal bone flap, the bone
flaps were fixed to each other with angling and then fixed
to a supraorbital bar with absorbable miniplates and screws.
The new bone flap was fixed to temporal and parietal bones
bilaterally with 1.2 cm advancement (Fig. 3). We always
use absorbable plating system since it allows the infants
skull to grow once the system is absorbed, thus not
inhibiting the necessary developmental growth seen with
Table 1 Patients characteristics and operative techniques
Patients Sex Age Operative technique
1 M 10 years Fronto-orbital advancement (180 rotation of fronto-parietal flap with horizontal osteotomy and angling)
2 F 36 months Fronto-orbital advancement (180 rotation of fronto-parietal flap with horizontal osteotomy and angling)
3 M 40 months Fronto-orbital advancement (only horizontal osteotomy and angling)
4 F 48 months Fronto-orbital advancement (only horizontal osteotomy and angling)
Fig. 2 a, b Fronto-orbital advancement model with 180 rotation of
fronto-parietal flap with horizontal osteotomy and angling
Childs Nerv Syst (2009) 25:16051612 1607
the titanium system. After fixing with absorbable miniplates
and screws, a silicone drain is placed under periosteal layer.
Periosteum and scalp were closed.
Results
Forty-six patients with craniosynostosis were operated by
the craniofacial surgery team in Gazi University Medical
School between 2002 and 2008. This series includes
hypertelorism (two patients), Aperts syndrome (three
patients), Crouzon syndrome (two patients), plagiocephaly
(13 patients), scaphocephaly (14 patients), and trigono-
cephaly (eight patients). There were four isolated brachy-
cephaly in this series. As the long-term follow-up period
was not identical between patients, the authors have to
analyze the short-term outcome. Fronto-orbital advance-
ment with 180 rotation and angling was performed in two
of the cases (patients 1 and 2), and fronto-orbital advance-
ment with only horizontal osteotomy and angling of the
bone flap was performed in the last two patients (patients 3
and 4). The CI, ICV, and morphological outcome in short
term were compared. As the groups consist of two patients,
no statistically significant data can be obtained.
In the comparison of CI, significant improvement was
detected in both groups. The decrease in CI in the second
group was slightly better than the first group (Table 2). In
the comparison of ICV, increase in ICV was found in both
groups. The percentage of increase between the two groups
was similar (Table 3). The morphological outcome was
satisfactory in all patients (Fig. 4). There were no major or
minor complications and morbidity.
Discussion
In 1851, Virchow [16] first suggested that premature fusion
of cranial sutures is responsible for cranial dysmorphism.
Since then, according to Virchows concept, to cure
excision of the prematurely fused cranial suture had been
tried by surgeons. Because of the refusion of the suture
after the operation, this technique was abandoned. Today,
we know that the local dura plays an important role.
Secretion of transforming growth factor (TGF) and fibro-
blast growth factor (FGF) from the local dura is responsible
for inducing new bone formation and cranial suture fusion
[10]. According to this theory, mere suture resection is
followed by rapid osteogenesis and bone edge re-growth
induced by TGF and FGF excreted from the dura. Single
fused bone line excision cannot prevent the bone suture
from refusion.
In 1967, Tessier developed the fronto-orbital advance-
ment method [15]. The main purpose of this method was to
release any osseous constriction, correct the deformity, and
allow for further normal growth. Marchac and Renier have
utilized fronto-orbital advancement in treating craniosynos-
tosis with encouraging results [8].
Brachycephaly is characterized by short anteroposterior
cranial diameter, retropositioned supraorbital bar with
abnormally wide cranium, and absence of frontal angle or
even reversed. Usually, approximately 1-cm advancement is
sufficient [2]. Brachycephaly most frequently occurs in
syndromic craniosynostosis: Crouzon, Pfeiffer, and Apert
syndromes. Fourteen percent of the cases are familial [1].
Bilateral Harlequin eye sign can be seen in plain skull
radiographs (superior displacement of the lesser wings of
both sphenoid bones). Brachycephaly causes functional and
morphological problems that require fronto-orbital advance-
ment in infancy to correct the brachycephalic deformity and
Fig. 3 a, b Fronto-orbital advancement model with only horizontal
osteotomy and angling
Table 2 Cranial index values and changes
Patients Preoperative
CI
Postoperative
CI
Decrease
in CI (%)
Interval between
two CT (months)
1 95.2 86.7 9.2 3
2 93.6 84.2 10 2
3 93.7 84.9 11.3 1
4 99.2 86.3 13 3
CI cranial index, CT computed tomography
Table 3 Intracranial volume measurements and changes
Patients Preoperative
ICV (cm
3
)
Postoperative
ICV(cm
3
)
Increase in
ICV (%)
Interval between
two CT (months)
1 1,407.9 1,462.5 3.6 3
2 993.3 1,052.7 5.9 2
3 1,207 1,259 4.1 1
4 1,267.1 1,373.9 7.7 3
ICV intracranial volume, CT computed tomography
1608 Childs Nerv Syst (2009) 25:16051612
to prevent mental impairment caused by the intracranial
hypertension. The prevalence, natural history, and determi-
nants of the condition are unclear. The mean CI by 2 years
was 81.6% [1]. Postoperative mental status is better when
surgery is performed before the patient reached 1 year of
age. Although both morphological and functional outcomes
are better in the subgroup that does not carry the FGFR3
P250R mutation, the differences were not statistically
significant. Prominent bulging of the temporal fossa was
frequently responsible for poor morphological outcome in
carriers of this mutation [1].
Various surgical procedures have been devised to
manage brachycephaly, but there is no standard surgical
method in the treatment of brachycephaly. Suboptimal
results may be related to the tendency of misdirected bone
growth of the remaining cranium in brachycephaly. There-
fore, the abnormal growth vectors should be corrected
toward a more normal configuration [3]. In 1979, Marchac
and Renier proposed the floating forehead concept.
Elevation of supraorbital bar releases the fronto-ethmoidal
and fronto-sphenoidal stenosis. Because the forehead is
only attached to the face after advancement with no
posterior fixation, this procedure enables the expanding
brain to maintain and continue its advancement [3, 9].
Early surgery is recommended for all types of cranio-
synostosis from both functional and technical points of
view. Several studies showed that early intervention in
other cases of craniosynostosis such as Apert syndrome and
trigonocephaly prevents deterioration in mental function,
which reinforces our conviction to operate early [11].
Regarding brachycephaly, surgical decompression may also
be required in very young infants. A recent study conducted
Fig. 4 Photograph showing the four patients and their 3D CT preoperatively and postoperatively (1a1d, 2a2d, 3a3d, 4a4d)
Childs Nerv Syst (2009) 25:16051612 1609
among 99 children showed the necessity of treating them
before the age of 1 year to prevent both morphological and
functional impairment. The surgery may not improve a
childs mental status but may prevent deterioration in mental
function [1]. It had been suggested that early surgery might
be deleterious to the fronto-glabellar morphology, with
adverse effects on frontal sinus growth. However, it has
been previously shown that the fronto-glabellar morphology
was less dependent on frontal sinus growth after advance-
ment and more on the morphology of the supraorbital bar
itself. Brachiocephalic children who do not undergo surgery
rarely develop a frontal sinus [1]. In this series, all the
patients were older than 1 year, and increased intracranial
pressure signs were observed in the cranial X-rays and bone
flaps intraoperatively in all patients (Fig. 5).
Cranioorbital normalization has become an objective of
synostosis surgery with attention paid to the correction of
forehead contour, browprojection, and the naso-frontal angle.
Surgical reshaping, advancement, and stabilization of the
caudal aspect of the frontal bones, the superior orbital rims,
and the naso-frontal junction are performed in an attempt to
achieve these goals. The standard operation includes bifrontal
craniotomy with preservation of the caudal 1 cm of the frontal
bones in continuity with the superior orbital rims, extended
bilateral coronal suturectomies, re-contouring and anterior
and caudal displacement of the supraorbital bar, and fixation
of the advanced bar at the nasion (two different fixation
methods) and at both pterions [6, 7].
The majority of published reports describe good result
using postoperative photographs and images. These images
provide objective outcome information, but they are not
quantifiable and not available for comparison to any group.
Recently, anthropometric measures are used to quantify the
outcomes of different treatments for a given condition [17].
Although normative values often do not exist and there are
no natural history studies that use such measurements as
parameters, we evaluate the outcome in terms of cephalic
index and intracranial volume. ICV is an important aspect
of brachycephaly as mostly it is decreased and causes
intracranial hypertension. CI can be used as an objective
Fig. 6 1a, b Multiple osteotomy technique in brachycephaly in
anteroposterior (a) and sagittal view (b); 2a, b fronto-orbital
advancement technique with 180 rotation of fronto-parietal flap with
horizontal osteotomy and angling in anteroposterior (a) and sagittal
view (b); 3 fronto-orbital advancement technique with only horizontal
osteotomy and angling in anteroposterior (a) and sagittal view (b); 4
only rotation technique in anteroposterior (a) and sagittal view (b); 5a
only advancement technique
Fig. 5 a Cranial X-ray showing
the increased intracranial
pressure sign; b fronto-parietal
bone flap showing increased
intracranial pressure
1610 Childs Nerv Syst (2009) 25:16051612
measurement of morphological outcome as it indicates the
changes in the ratio of maximum width to maximum length
of the cranium.
We choose isolated brachycephaly to assess the effective-
ness of using skull models since in syndromic cases
associated malformations impair the standardization. Addi-
tionally, in other non-syndromic craniosynostosis, procedures
are well-defined with satisfactory outcomes. In brachy-
cephaly, different advancement protocols are used, and
increased intracranial pressure is an important aspect. We
compared the changes in CI and ICV between five different
surgical techniques including only fronto-orbital advance-
ment, only rotation, advancement with 180 rotation and
angling, only advancement with angling and multiple
osteotomy technique, and only advancement technique
(Fig. 6). We found that instead of fixing the fronto-parietal
bone flap without rotation, angled advancement with
horizontal osteotomy provided maximum increase in ICV
and maximum decrease in CI in a bilateral coronal
craniosynostosis model [13]. These findings encourage us
to abandon our usual technique (advancement with 180
rotation, horizontal osteotomy, and angling) and perform
only advancement with angling. After performing this
technique in two children, we aimed to analyze the outcome
in real patients. Since the number of patients is not enough,
unfortunately, we could not have the chance to compare the
patients with identical characteristics like age and follow-up
period. The statistical analysis could not be performed also.
In the comparison of two techniques, the decrease in CI was
slightly better in the last technique; however, ICV increase
was similar in both techniques. Morphological outcome was
satisfactory in all patients. Since the number of patients is
limited, statistical data could not be obtained. Also, patients
characteristics like age and follow-up interval were not very
similar. Larger series with similar patient characteristics can
give us more objective information about the effectiveness
of the surgical techniques.
The surgical correction of craniosynostosis is a complex
procedure and presents as an interdisciplinary challenge, as
the procedure is comparatively rare even at specialized
centers. As early surgery is beneficial, there is a demand for
less drastic but effective techniques. Among other issues,
surgical time and blood loss are of importance in small
children undergoing surgery.
Various surgical techniques have been described for
brachycephaly. We think that to provide the best outcome
with lower risks, virtual surgical planning is necessary for
all patients with complex craniosynostosis.
Current multislice computed tomography technology can
be used for diagnosis and surgical planning with computer-
assisted 3D visualization and surgical simulation. Three-
dimensional models were created for the purpose of
surgical simulation. These allow planning the course of
the osteotomy and individual placing of the different bony
fragments by an assigned matrix to simulate the surgical
result and to assess the positioning of the individual bony
fragments [57, 12]. Postprocessing time is the major
disadvantage of 3D CT and limits the routine use of it.
Stereolithography defines a 3D printing process that
makes a solid object from a computer image by using a
computer-controlled laser to draw the shape of the object
onto the surface of a liquid plastic. We use skull models
made of polymethyl methacrylate for operative simulation
especially in patients with complex craniosynostosis and
syndromic cases (Fig. 1). These models have important
advantages like better evaluation than the 3D images,
providing easy and virtual operative planning; shortening
the operation time; and reducing the risks and complication,
providing an excellent tool for parents cooperation and
understanding, useful for training. Rotation and translation
of the 3D model of the osteotomy segments was easy to
handle. It provides better understanding of the pathology;
operative simulation is easier and more real than the 3D CT
models. Thus, the preoperatively planned surgery could be
transferred directly to the operating table. Working on the
model helps us to define the craniotomy borders, the bone
flaps, and the new position of the flaps better. To know
what to do and seeing the result of the procedure before the
operation provides shorter operation time and more satis-
factory outcome.
Conclusion
Although the expectation of genetic therapy for craniosy-
nostosis will be possible in the future, today, as a surgeon,
we have to find and try new techniques and procedures for
better outcomes. Current multislice tomography technology
and stereolithographic procedures provide excellent surgi-
cal simulation models to find new techniques and predict
the outcome. These models should be used in all complex
and syndromic craniosynostosis.
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