ORIGINAL PAPER

Correlations between the abnormal development of the skull

base and facial skeleton growth in anterior synostotic
plagiocephaly: the predictive value of a classification
based on CTscan examination
Sandro Pelo & Giampiero Tamburrini & Tito Matteo Marianetti &
Gianmarco Saponaro & Alessandro Moro & Giulio Gasparini & Concezio Di Rocco
Received: 30 April 2011 / Accepted: 14 June 2011 / Published online: 1 July 2011
# Springer-Verlag 2011
Abstract
Background Anterior cranial plagiocephaly, depending on
the early hemicoronal suture fusion, is the most relevant
form of plagiocephaly in terms of clinical implications. Its
estimated incidence ranges between 0.4 and 1 per 1,000
live births. In the present report, we aim at validating the
classification of Di Rocco and Velardi, proposing a scheme
based on basicranium analysis using CT scans and its
predictive value by evaluating the developmental character-
istics of a population of adult subjects affected by anterior
plagiocephaly who had underwent the surgical correction in
the first months of life.
Materials and methods The group of patients here consid-
ered was retrieved from among all patients operated upon
for craniostenosis in the pediatric neurosurgery unit of
Policlinico Gemelli in Rome between January 1, 1980 and
December 31, 1989. The study group consisted of 13
patients, seven females and six males, affected by anterior
synostotic plagiocephaly ranging in age between 20 and
32 years (mean 25.54 years). We also formed a group of
unaffected patients in order to control for normal variability
in the population. The subjects of the study group were
evaluated using CTscan exams and cephalometric analyses
were performed using three-dimensional reconstruction.
Discussion and conclusion In this study, we were able to
associate a facial phenotype to confirm the predictive value
of the classification proposed. It is highly probable that the
different outcomes depend on the different degrees of
involvement in the synostotic process by the various skull
base sutures which were essentially unaffected by the
surgical procedures.
Keywords Anterior synostotic plagiocephaly
.
Craniostenoses
.
Craniofacial malformations
.
Unicoronal
synostosis
.
Skull base
.
Coronal suture
.
Nasal deformities
.
Facial asymmetries
Introduction
The term plagiocephaly, from the Greek word
(oblique, twisted), introduced by Virchow in 1851, has been
commonly utilized to define those developmental cranial
deformations, either congenital or acquired, resulting in an
asymmetry and scoliotic appearance of the craniofacial
skeleton [1, 2].
Plagiocephaly is characterized by a complex phenotype
which may vary in the different subjects considerably.
Indeed, it depends on the variable combination of anatom-
ical and functional abnormalities. Among the former, the
early fusion of some cranial suture leads to a focally
impaired growth of the bones of the vault and the skull
S. Pelo (*)
:
T. M. Marianetti
:
G. Saponaro
:
G. Gasparini
Maxillo Facial Surgery, Complesso Integrato Columbus,
Catholic University Medical School,
Via Giuseppe Moscati 31-33,
00168 Rome, Italy
e-mail: sandro.pelo@rm.unicatt.it
G. Gasparini
e-mail: giulio.gasparini@rm.unicatt.it
G. Tamburrini
:
C. Di Rocco
Pediatric Neurosurgery, Policlinico Universitario A. Gemelli,
Catholic University Medical School,
Largo Agostino Gemelli 8,
Rome, Italy
A. Moro
Maxillo Facial Unit, Ospedale S. Giovanni Battista,
Foligno, Perugia, Italy
Childs Nerv Syst (2011) 27:14311443
DOI 10.1007/s00381-011-1514-x
base, to malalignment of the cranial and facial bones and to
imbalanced volumetric development of the intracranial
spaces. Ocular, masticatory and cervical muscle functional
abnormalities, varying from mild to severe, also contribute
to the clinical picture. Anterior cranial plagiocephaly,
depending on the early hemicoronal suture fusion, is the
most relevant form of plagiocephaly in terms of clinical
implications. Its estimated incidence ranges between 0.4
and 1 per 1,000 live births [3].
The varying severity of the phenotype depends on the
different involvement at the diagnosis, and subsequently,
during the cranial growth of the fronto-zygomatic, the
fronto-sphenoid, spheno-ethmoidal and sphenozygomatic
sutures [36]. Consequently, the malformation is fully
expressed at the end of the craniofacial growth [79]
(Fig. 1). Until now, the interception of the disease is based
on the correction in early life of the anterior skull base and
orbital anomalies, while the facial skeleton is usually
approached after 67 years of age. The posterior skull base
anomaly and the asymmetry of the cervico-cranial junction
are rarely offered a surgical treatment, although physiother-
apy is nearly always suggested.
In the late seventies and early eighties, the advent of CT
scan examination allowed to differentiate different grades of
severity of the disease based on the skull base abnormalities
characterizing the condition in the single subject but did not
result in specific and different surgical approach. Essential-
ly, a unique surgical modality (which implies the unilateral,
or more rarely bilateral, removal and remodelling and
replacement of the frontal bone flap, opening of the orbit
roof, anterior replacement of the superior orbital ridge) was
offered to all the affected infants. The search for criteria to
predict the late outcomes was stimulated.
In 1988, Di Rocco and Velardi [10] proposed a classifica-
tion scheme based on basicranium analysis using CT
scans. This classification divides the patients into three
groups. Patients in group I show frontal bone flattening
ipsilaterally to the affected suture, as well as elevation of
the orbital roof without nasal pyramid deviation. In these
children, axial CTscan views show normopositioned vomer
and rocca petrosa.
Patients in group II associate the frontal and orbital
abnormalities with a contralateral deviation of the nasal
pyramid and a variable grade of anterior displacement of the
external ear on the synostotic side. This group is further
divided in two subgroups: IIA and IIB, according to the
severity of rocca petrosa anterior displacement and the
presence of vomer bone deviation. Group IIAincludes patients
with normal positioning of the vomer with a mild or absent
anterior displacement of the rocca petrosa on the affected side.
Group II B includes patients with a moderate grade of vomer
deviation and severe displacement of the rocca petrosa, which
results in reduced size of the middle cranial fossa.
Finally, group III includes patients with a severe nasal
deviation associated with homolateral deviation of the vomer
and anterior displacement of the rocca petrosa besides the
frontal bone flattening and orbital bone anomalies. This
classification is based on the degree of sutural synostosis
extension, which makes it ideal for our research.
It was assumed by the authors that group I only
represented the result of the mere early fusion of the
hemicoronal suture, whereas the other two groups, identified
on the grounds of the skull base anomalies as shown by the
CT scan examination, harboured progressively extended
sutural synostosis of the skull base sutures and corresponded
to different degrees of severity of clinical phenotypes. In the
present report we aim at validating the just mentioned
classification hypothesis and its predictive value by evaluating
the developmental characteristics of a population of adult
subjects affected by anterior plagiocephaly who had under-
went the surgical correction in the first months of life.
Materials and methods
The group of patients here considered was retrieved from
among all patients operated upon for craniostenosis in the
pediatric neurosurgery unit of Policlinico Gemelli in Rome
between January 1, 1980 and December 31, 1989. During
Fig. 1 Typical findings in severe anterior synostotic plagiocephaly: 1
the retrusion of the front on the affected side, 2 retrusion of the roof of
the orbit on the affected side, 3 the contralateral frontal bossing, 4 the
hypertelorism, 5 the deviation of the nasal pyramid controlaterally
from the synostosis, 6 the retrusion of the malar prominence on the
affected side, 7 the mandibular deviation toward the unaffected side
1432 Childs Nerv Syst (2011) 27:14311443
this period of time, 239 patients were treated for syndromic
and non-syndromic craniostenoses. The original group
consisted of 122 patients with scaphocephaly, 40 patients
with trigonocephaly, 40 patients with anterior synostotic
plagiocephaly, nine patients with posterior synostotic
plagiocephaly, two patients with brachicephaly and
patients with complex craniosynostoses, namely: 15 cases of
Crouzons syndrome, 12 cases of Aperts syndrome and a case
of SaethreChotzen syndrome.
Inclusion criteria for this study included the following:
patients affected by anterior synostotic plagiocephaly who
had undergone frontal bone advancement osteotomy as
neonates and had not undergone any other corrective
surgical procedures and who had reached the end of
craniofacial growth. The study group consisted of 13
patients, seven females and six males, affected by anterior
synostotic plagiocephaly ranging in age between 20 and
32 years (mean 25.54 years). We also formed a group of
unaffected patients in order to in order to control for normal
variability in the population.
As control, we considered 15 subjects of similar ages
(2032 years) who were not affected by craniofacial or
dentoskeletal malformation that were clinically detectable and
did not experience any facial correction procedures who had
undergone a CT scan of the cranium, facial skeleton and
mandible, following craniofacial traumatic injuries. The 13
subjects of the study group were evaluated between July and
September 2009 in the Radiology Unit of Complesso
Integrato Columbus using a TC Phillips Brilliance 190P
64-slice with spiral scansion and multiplanar acquisition.
Two-dimensional images were processed with Mimics
v10.01 (Fig. 2), a software package developed by
Materialise Inc. that allows the user to perform three-
dimensional reconstructions from two-dimensional images.
Cephalometric analyses were performed using three-
dimensional reconstruction. As it was not possible to use
standard cephalometric plans in these patients affected by
anterior synostotic plagiocephaly, with the sole exception of
grade I, due to facial asymmetry the most preserved
structure was the complex of sella turcica, which demon-
strated less marked interindividual variability between
affected and unaffected people, were utilized cephalometric
plans passing at this level. Three-dimensional models from
patients CT scans were elaborated using Materialise
Mimics 10.0 software, and the programme was also used
to create a system of three orthogonal planes to be utilized
as references for metrical evaluations of the dysmorphosis.
The axial reference plan was built using the tips of
anterior clinoid processes and the middle point on the line
connecting the tips of the two posterior clinoid processes as
Fig. 2 Interface of materialise mimics software that allows to elaborate three-dimensional reconstruction of soft and hard tissue and permits
cephalometric assessments and surgical simulation
Childs Nerv Syst (2011) 27:14311443 1433
reference points. For the sagittal plane, we used the middle
point on the line connecting the tips of the two posterior
clinoid processes and the middle point on the line linking
the tips of the two anterior clinoid processes. We drew a
line between these two points to define a plane passing
through this line and orthogonal to the axial plane.
The frontal plane is traced using the two middle points
of the two lines linking the tip of the anterior clinoid
processes and the homolateral posterior clinoid processes
orthogonal to the sagittal and axial planes (Fig. 3).
To perform the cephalometric analysis, we chose 22
points (18 paired and 4 unpaired) on which to evaluate the
facial skeleton, the mandible and the basicranium (Fig. 4).
To evaluate the orbit we used:
Lower orbital point (the lower part of the orbital frame)
Upper orbital point (upper orbital incision)
FZ (most medial point of the fronto-zygomatic suture
on the orbital frame)
Lacrimal point (bottom of the lacrimal sac)
NOE (point on the emergence of the optic nerve on the
neuro-orbital plane)
For the nose, we chose a non-paired point:
Nasion (intersection of the internasal suture and the
frontonasal suture)
For malar prominences, we chose three-paired points:
Infraorbital foramen
Temporal intersection of the zygomatic arch
Zygion (most prominent point of the zygomatic arch on
its lower side)
For upper maxilla:
Anterior nasal spine apex
Posterior nasal spine apex
Tip of the 1.3 and 2.3 dental elements
Mesial tips of the 1.6 and 2.6. dental elements
For lower maxilla:
Menton (most anterior point of the mental process)
Condylion (tip of the mandibular condyles)
Coronoid (tips of coronoid processes)
Gnathion (tip of mandibular angulus)
To evaluate the basicranium:
The most anterior point on the curve of the small
sphenoidal wings
The line traced on the main axis of the rocca petrosa
and the angle between this line and the middle plane.
The internal acoustic meatus
To evaluate the posterior section of the basicranium:
The most anterior point of the occipital condyles
The two porions (antero-superior point of the external
meatus)
Mastoid process tips
All selected points were localized in three-dimensional
space by using the reference planes described previously.
We also measured the distance between the gnathion and
menton for improved evaluation of the mandibular body.
We used a minus sign () to describe the position of the
points in relation to frontal and axial planes when the points
were behind the frontal plane or under the axial plane.
Results
These data illustrated the differential development of the
various skeletal segments (Tables 1 and 2).
Fig. 4 View of some of the cranial points used for the cephalometric
assessments
Fig. 3 View of the reference plan system developed specifically for
the purpose of the study
1434 Childs Nerv Syst (2011) 27:14311443
The orbital position was evaluated using the following
points: upper orbital point, lower orbital point, FZ point,
lacrimal point and NOE. The position and shape of the orbit
were different in the various grades.
In grade I, metrical parameters were similar to those
found in unaffected patients. These patients did not show
significant differences between affected and unaffected
sides, with the sole exception of a moderate retrusion of
the upper orbital point on the synostotic side, which
corresponds to a light asymmetry, characterized by a light
retrusion of the upper orbital rim.
In grade IIA, parameters of the orbital points showed a
more evident retrusion compared to unaffected patients and
more evident asymmetry. Upper orbital points (upper orbital
point and FZ) appeared to be more retruded on the
synostotic side. These parameters were compatible with a
moderate retrusion (mean value of 5 mm) that only involves
the upper part of the orbit, with minimal alteration of the
lower orbital frame. The lacrimal point and the lower
orbital point, in fact, did not show evident alterations
between sides or compared to unaffected patients. Upper
orbital points on the non-synostotic side showed a greater
projection compared to those of unaffected patients,
resulting in moderate compensatory growth on the unaf-
fected side. Orbital points of the upper section demonstrat-
ed asymmetry when compared to those on the unaffected
side when evaluated from both the frontal and axial planes.
The orbital points of the lower section exhibited no
alteration. Variability in the position from the axial plane
was not significant from any point, apart from the FZ point,
which was positioned higher on the synostotic side
compared to the contralateral side. Assessments in grade
IIA patients are compatible with moderate asymmetric
patterns that involve both sides of the face, resulting in
only partial expression of the pathology.
In grade IIB, all orbital points on the synostotic side
showed a minor projection fromthe frontal plane as compared
to contralateral points, with a mean difference of approxi-
mately 8 mm for those points situated on the upper section.
Points on the contralateral side displayed greater projections
in unaffected people than in grade IIA patients, suggesting
compensatory growth. The lower orbital point was also
involved in this grade, although with minimal severity,
whereas the lacrimal point showed no alterations. Grade IIB
Table 1 The differential development of the various skeletal segments of unaffected individuals (assessments are expressed in mm)
Points Front Sn Front Dx Front Sagitt Sn Sagitt Dx Sagitt Axial Sn Axial Dx Axial
Unaffected individuals
Lower Orbital Point 45.5 44.95 35.10 35.10 22.45 22.40
Upper Orbital Point 55.08 57.98 24.97 25.12 14.70 15.0
FZ 47.56 48.01 48.77 49.01 1.25 1.60
NOE 16.10 15.97 15.69 15.49 8.35 7.98
Infraorbital Foramen 46.00 45.00 23.80 24.0 36.40 35.98
Ins temp zyg 8.26 8.13 56.66 55.96 16.36 16.28
Zygion 35.34 34.89 52.46 52.66 28.59 29.04
Canine 41.4 40.78 18.50 18.70 78.31 78.29
Condylion 21.03 20.83 44.38 44.42 22.22 22.40
Coronoid 15.97 16.20 44.39 44.39 34.96 35.13
Gnation 18.31 17.91 44.50 44.49 72.44 72.35
Small Sphenoid Wing 18.37 17.98 35.68 34.98 10.00 9.87
Ang RP p med 57.35 SN
57.68 DX
Internal acoustic meatus 29.64 30.04 23.80 24.10 11.98 12.22
Occipital Condyle 28.50 28.68 9.36 9.46 38.88 38.75
Porion 28.11 27.91 52.49 52.50 17.89 18.01
Mastoid Proc 43.47 43.50 50.34 49.34 38.98 39.20
Lacrimal 50.04 51.24 10.61 10.58 18.30 18.45
Molar 31.06 31.16 21.46 21.55 70.23 69.87
Points unpaired
Nasion 70.00 0.6 7.02
Ant Nasal Spine 62.03 0.23 50.30
Post Nasal Spine 11.03 0.1 43.98
Menton 44.57 0.3 111.02
Childs Nerv Syst (2011) 27:14311443 1435
Table 2 The differential development of the various skeletal segments of grades I, IIA and B and III (assessments are expressed in mm)
Points Front I Front C Front Sagitt I Sagitt C Sagitt Axial I Axial C Axial
Grade I
Lower Orbital Point 47.99 50.31 32.51 33.53 22.81 22.00
Upper Orbital Point 57.21 55.68 28.14 23.75 13.83 14.1
FZ 43.5 46.25 47.35 49.04 1.29 0.81
NOE 15.51 15.8 14.52 16.69 6.9 5.84
Infraorbital Foramen 47.41 46.47 23.8 25.26 32.06 30.3
Ins temp zyg 11.25 11.45 56.67 56.75 13.02 12.86
Zygion 33.88 35.25 54.87 53.56 29.42 29.24
Canine 50.17 50.02 15.24 18.29 74.01 74.81
Condylion 16.09 13.34 45.27 43.98 18.92 24.05
Coronoid 17.27 16.96 43.84 45.43 30.77 28.76
Gnation 17.11 17.4 45.14 43.69 69.44 68.44
Small Sphenoid Wing 21.17 22.76 35.67 36.87 6.69 9.87
Ang RP p med 59.25 I
59.06C
Internal acoustic meatus 27.69 26.53 23.80 25.40 13.23 12.5
Occipital Condyle 27.84 29.99 5.95 10.74 38.86 38.91
Porion 24.65 24.2 51.09 52.69 16.00 15.8
Mastoid Proc 41.90 39.58 47.75 50.22 36.95 36.02
Lacrimal 48.82 40.3 19.17 10.19 17.94 17.09
Molar 30.74 30.02 19.74 20.22 66.51 65.70
Points unpaired
Nasion 63.80 0.51 I 5.72
Ant Nasal Spine 62.64 1.01 C 49.13
Post Nasal Spine 12.03 0.4 I 41.97
Menton 45.67 1.33 C 108.52
Grade IIA
Lower Orbital Point 46.13 45.82 42.4 39.34 28.02 30.96
Upper Orbital Point 51.26 64.82 28.8 21.34 3.59 2.69
FZ 43.57 45.09 52.4 49.64 2.58 9.47
NOE 16.1 14.83 19.16 18.87 7.52 8.04
Infraorbital Foramen 47.12 48.15 24.53 29.6 39.03 39.99
Ins temp zyg 14.79 12.8 56.61 58.61 16.18 17.01
Zygion 31.75 34.29 53.54 57.04 40.47 37.38
Canine 51.76 49.88 15.00 18.87 82.64 81.68
Condylion 16.00 18.87 51.13 47.32 20.72 19.72
Coronoid 14.30 12.90 45.58 44.85 36.18 32.92
Gnation 21.37 22.04 50.4 46.59 83.45 82.25
Small Sphenoid Wing 18.02 16.96 38.26 41.1 11.78 7.72
Ang RP p med 60.03 I
52.61C
Internal acoustic meatus 24.21 31.32 31.15 23.22 11.09 10.41
Occipital Condyle 31.01 32.62 11.71 9.87 36.88 35.93
Porion 20.86 22.48 56.69 52.83 14.87 16.87
Mastoid Proc 40.7 51.62 54.65 51.09 44.7 41.16
Lacrimal 53.77 53.79 10.11 8.87 18.7 19.69
Molar 23.20 21.46 24.39 23.81 71.32 71.32
1436 Childs Nerv Syst (2011) 27:14311443
Table 2 (continued)
Points Front I Front C Front Sagitt I Sagitt C Sagitt Axial I Axial C Axial
Points unpaired
Nasion 65.84 4.35 I 1
Ant Nasal Spine 65.25 0.87 C 54.99
Post Nasal Spine 11.31 0.58 I 45.94
Menton 49.15 1.59 C 113.91
Grade IIB
Lower Orbital Point 46.83 48.14 47.9 39.17 28.89 28.24
Upper Orbital Point 58.07 67.14 33.38 19.16 2.29 3.27
FZ 41.03 49.15 55.75 47.32 2.48 5.09
NOE 20.45 17.54 18.58 18.43 10.06 8.86
Infraorbital Foramen 49.15 51.18 24.82 30.92 38.41 26.93
Ins temp zyg 14.35 4.78 57.19 61.83 18.04 12.57
Zygion 37.7 37.41 51.95 57.04 37.38 34.74
Canine 52.63 49.76 17.99 14.08 82.33 81.37
Condylion 8.7 16.82 47.46 47.03 21.52 18.9
Coronoid 21.46 20.3 46.74 45.14 37.21 32.55
Gnation 10.87 17.68 50.22 46.79 86.50 83.99
Small Sphenoid Wing 24.79 21.89 38.90 38.17 5.07 5.16
Ang RP p med 64.93 I
54.76C
Internal acoustic meatus 29.69 49.90 26.7 22.2 11.65 11.54
Occipital Condyle 27.55 30.59 13.06 7.11 38.85 38.19
Porion 16.67 28.85 58.78 52.83 18.37 15.17
Mastoid Proc 35.09 45.96 57.04 49.21 43.25 39.56
Lacrimal 54.34 54.08 18.14 7.84 22.0 19.93
Molar 26.68 23.68 25.84 25.84 74.39 73.95
Points unpaired
Nasion 68.59 6.24 I 1.46
Ant Nasal Spine 68.14 2.47 C 53.22
Post Nasal Spine 14.64 0.43 I 49.18
Menton 51.62 1.71 C 115.83
Grade III
Lower Orbital Point 47.42 53.58 40.68 47.9 17.99 28.89
Upper Orbital Point 51.78 57.76 26.92 33.38 12.78 8.23
FZ 41.91 48.8 46.94 55.75 6.76 2.48
NOE 19.31 20.77 15.27 18.58 5.00 4.39
Infraorbital Foramen 51.67 55.42 26.04 30.92 38.41 26.8
Ins temp zyg 8.03 2.66 61.83 53.38 15.78 18.04
Zygion 25.37 33.00 54.47 57.04 25.68 37.38
Canine 62.10 57.74 12.42 14.42 68.87 65.50
Condylion 4.79 11.19 41.53 48.46 22.11 16.67
Coronoid 28.42 19.15 41.28 45.33 28.79 26.43
Gnation 5.51 10.92 42.81 44.45 76.00 72.1
Small Sphenoid Wing 20.28 53.58 30.50 47.9 11.9 28.89
Ang RP p med 73.42 I
52.76C
Childs Nerv Syst (2011) 27:14311443 1437
patients are characterized by an augmented distance of the
points from the middle plane on the affected side of both the
upper section and the lower section, with a mean value of
7 mm, which is bigger than in grade IIA. Similar to grade IIA,
there were no significant signs of asymmetry from the axial
plane, with the sole exception of the FZ point, which is
situated in a higher position on the synostotic side.
In grade III, all orbitary points were retruded compared
to the non-synostotic side, with a mean value of 7 mm. This
retrusion involved all orbital points, including the lacrimal
point, which showed no alterations in the other grades.
Points on the non-synostotic side displayed greater projec-
tion than in unaffected patients. We found a larger distance
from the middle plane for points on the synostotic side,
with a mean value of 7 mm, similar to grade IIB. All points
were positioned higher from the axial plan on the synostotic
side, with the exception of the lower orbital point, whose
position was lower than that on the non-synostotic side.
These assessments are significant for an orbit of
increased dimension whose upper section is positioned
upwards from the axial plan, configuring the so-called
Harlequins phenomenon. Hypertelorism was present in
three patients and did not appear to be linked with grades.
Nose position was analysed through the position of the
nasion and the anterior nasal spine.
In grade I, the nasion showed a light displacement toward
the affected side. This displacement was minimal and was
present in the group of unaffected patients, potentially
representing anatomical variability. The nasal anterior spine
was also slightly deviated contralaterally from the synostosis,
and its entity was bigger than in unaffected individuals. These
parameters describe a slight deviation of the nasal pyramid,
with a minimally oblique axis top-down from the synostotic
side toward the non-synostotic side that is superimposable to
the normal anatomic variability.
In grade IIA, the nasion showed a larger deviation
toward the non-synostotic side than in grade I; the anterior
nasal spine was also minimally deviated toward the non-
synostotic side. The reciprocal position of the two points is
suggestive of a nasal pyramid deviation with the same
direction of grade I, but the deviation is of a bigger entity
than grade I, and it is not superimposable on unaffected
individuals. In grade IIB, the displacement of the nasion
from the sagittal middle plane was greater than in grade
IIA, whereas anterior nasal spine deviation was superim-
posable with regard to entity and direction.
In grade III, nasion displacement was superimposable for
entity and direction to grade IIB, whereas anterior nasal
spine deviation was bigger than grade IIB. This pattern was
suggestive of a more severe nasal deviation but with the
same direction of the other grades. Malar prominences were
evaluated through three-paired points: infraorbital foramen,
zygion and temporal insertion of the zygomatic arch.
In grade I, the coordinates of the three points did not differ
significantly on the two sides; coordinates were symmetrical
and superimposable in unaffected individuals expressing a
pattern of normoprotrusion and malar symmetry. In grade IIA,
there was an evident retrusion of the malarzygomatic
complex on the affected side, with a reduction of all projection
assessments. On non-synostotic side projections, assessments
were superimposable to those of unaffected patients. We
therefore conclude that no compensatory growth was present
in this grade. All malar points on the synostotic side were
closer to the sagittal middle plane than on the contralateral
side, whereas no difference was assessed in the distance from
the axial plane with the exception of the zygion, which was
higher on the affected side.
In grade IIB, there was an evident retrusion of the
middle third of the face on the synostotic side; all points
were, in fact, closer to the frontal plane compared to those
on the contralateral side. In this grade, all points on the non-
synostotic side had a moderately increased projection from
the frontal plane, supposedly for compensatory growth. All
points were closer to the sagittal plane on the synostotic
Table 2 (continued)
Points Front I Front C Front Sagitt I Sagitt C Sagitt Axial I Axial C Axial
Internal acoustic meatus 17.50 22.36 26.70 21.7 11.65 15.98
Occipital Condyle 20.53 20.23 8.49 12.44 38.85 40.75
Porion 15.45 26.67 57.72 57.75 18.37 17.72
Mastoid Proc 28.46 35.09 51.64 50.84 43.25 42.12
Lacrimal 56.52 54.34 10.76 6.3 22.0 4.1
Molar 34.65 31.50 22.30 25.26 74.39 55.66
Points unpaired
Nasion 67.52 6.67 I 1.46
Ant Nasal Spine 74.55 3.14 C 53.22
Post Nasal Spine 20.94 0.5 I 49.18
Menton 61.24 4.3 C 115.83
1438 Childs Nerv Syst (2011) 27:14311443
side than on the contralateral side; this distance was smaller
than that observed in grade IIA patients. All points,
including the zygion, were positioned closer to the axial
plane on the affected side, as in grade IIA.
Data fromgrade III confirmed the retrusion pattern that was
present in grade IIA, and the difference between the synostotic
side and the contralateral side was associated with a more
evident hemifacial hypoplasia, compatible with full expres-
sion of the disease. The unaffected side was characterized by
increased values, as observed for compensatory growth. A
pattern of retrusion was also found in the distances from the
middle plane, with the exception of the temporal insertion of
the zygomatic arch, which was situated further from the
middle plane on the synostotic side because of temporal
bulging that is always present in severe grades of plagioce-
phaly. Distances from the axial plane were bigger on the
synostotic side, as in grade IIB. The stato-acoustic apparatus
was evaluated through two-paired points (the internal acoustic
meatus and porion) and the angle between the main axis of the
rocca petrosa and the sagittal middle plane.
In grade I, there were no significant asymmetries
between the two sides, and the two angles had the same
amplitudes on both sides. In grade IIA a light anterior
displacement and an increased distance from the middle
plane of both the porion and the internal acoustic meatus
were observed. The amplitude of the angle between the
rocca petrosa main axis and the middle plane was more
ample on the synostotic side than on the non-synostotic
side. In grade IIB, there was a severe anterior displacement
of the internal acoustic meatus and porion on the affected
side, and the angle between the rocca petrosa main axis and
the middle plane was wider than on the contralateral side
and wider than in grade IIA. Grade III alterations were
superimposable to those of grade IIb, and there was an
internal meatus displacement in a lower position, whereas
in grade IIB, the two points were on the same plane. The angle
between the main axis of the rocca petrosa and the midplane
was wider on the synostotic side than on the non-synostotic
side, and its amplitude was larger than in grade IIB. The
maxilla was evaluated using two-paired points (tips of upper
canines, mesial cusped of second molar) and two unpaired
points (anterior nasal spine and posterior nasal spine).
In grade I, the values of the coordinates of the points
considered were similar on the two sides, and there were no
differences when compared to unaffected individuals.
Grade IIA points were displaced more anteriorly on the
synostotic side than on the non-synostotic side, with a
slight deviation of the anterior nasal spine toward the non-
synostotic side and a posterior nasal spine deviation to the
synostotic side. This framework is compatible with a slight
rotation of the upper maxilla in the axial plane. There were
no differences in the axial plane coordinates of the points
matched; this situation is typical of the absence of tilting.
Grade IIB yielded similar findings to grade IIA, again
suggesting rotation of the maxilla in the absence of tilting.
Grade III displayed advancement of points on the
synostotic side, and the anterior nasal spine was tilted to
the synostotic side, while the posterior nasal spine was
Fig. 5 Sample of evolution of anterior synostotic plagiocephaly during the growth in a patient with grade III
Childs Nerv Syst (2011) 27:14311443 1439
tilted toward the synostotic side. These changes were similar
to those found in previous grades, but they were larger. There
were also imbalances in the positions of the points matched
against the axial plane. The tips of the canines and the mesial
cuspeds of the molars were, in fact, lower on the synostotic
side. This framework has significant tilting of the maxilla,
which is tilted in the frontal plane, higher on the synostotic
side and lower on the non-synostotic side.
The mandible has been studied by means of three-paired
items (condylion, apex of coronoid and gnathion) and a
non-matched point (the menton). To evaluate the mandib-
ular body, we compared the distance between the gonion
and menton on the two sides.
In grade I, we did not note significant asymmetries on
the two sides; only the condylion was slightly forward with
a slight drop in the affected side. All other values were
symmetrical and similar in the two hemifaces with values
similar to healthy subjects. The menton was moved to the
side contralateral to the lesion; this shift was probably due
to the, albeit limited, condylion/glenoid fossa anterioriza-
tion. The distances between the gnathion and menton were
the same in the two hemimandibles and superimposable to
healthy subject findings. The mandibular deviation was
likely due to displacement of the glenoid fossa when we
consider the condylion as an indirect index of its position.
In the grade IIA samples, paired points showed a greater
distance from the frontal plane on the synostotic side than on
the contralateral side. They were also more distant from the
sagittal plane, and they were further down fromthe axial plane
on the synostotic side. The distance between the menton and
gonion was less on the hemimandibula of synostotic side, and
it was equal to that of healthy subjects on the non-synostotic
side. The jaw was moved to the front and down on the
synostotic side, with a slight deviation from the middle plane
of the menton to the side contralateral to the lesion. The
deviation was less than expected because this anteriorization
of the glenoid fossa is compensated for by hemimandibular
hypoplasia on the affected side.
Patients in grade IIB had similar values to the grade IIA,
but greater anteriorization and lowering of the points of the
synostotic side were detectable. The deviation of the
menton and the distance between the menton and gonion
on the affected side were similar in magnitude to those
observed in grade IIA, and the distance between the menton
and gonion in the unaffected side showed compensatory
growth and was increased compared to normal individuals.
This condition was significant for the presence of compen-
sation implemented by hemimandibular hypoplasia on the
synostotic side and elongation on the non-synostotic side
compensating for glenoid fossa anteriorization.
Fig. 6 Picture, soft tissue three-
dimensional reconstruction
and facial skeleton three-
dimensional reconstruction
of a patient with grade I
Fig. 7 Picture, soft tissue three-
dimensional reconstruction
and facial skeleton three-
dimensional reconstruction
of a patient with grade IIA
1440 Childs Nerv Syst (2011) 27:14311443
In grade III, a further increase in the entity of mandible
condyle anteriorization on synostotic side, due to probable
altered position of the glenoid fossa, was accompanied by
increased shortening of the gonionmenton distance on the
synostotic side and an increase of the same distance on the
contralateral side. In this grade, the menton was deviated
contralaterally to the lesion to a greater extent than the
grades IIA and IIB measurements. In this case, the real
deviation was masked by the compensation implemented
by the two mandibular bodies.
Discussion
Anterior synostotic plagiocephaly is a complex develop-
mental disorder (Fig. 5) that manifests itself in an
incremental manner with the progression of somatic growth
and is expressed in a non-homogeneous pattern in various
subjects. The severity of the deformity is closely related to
the extension of the synostosis [11], which differs among
the three groups. Neurosurgical operation solves the
problem in the frontal region [12, 13] but does not appear
to prevent the development of craniofacial dysmorphoses,
with the partial exception of patients in grade I.
We have noticed that the severity of facial abnormalities
was directly related to the degree of the classification
method created by Di Rocco, which takes into account the
changes in skull base analysed using two-dimensional CT
axial cuts. With our study, we typed these changes,
analysing bone alterations with respect to the classification
method, establishing a pattern of skeletal and facial pheno-
types associated with the different levels of classification. The
data revealed that there was evidence of a clear facial
phenotype for each different grade; this allows us, as early
as during childhood, to anticipate the development character-
istics of the splanchnocranium in a single patient.
A grade I classification level corresponds to minor
changes in facial morphology whereas subjects with grade
II and III express more accentuated facial deformities. The
facial phenotype of grade I (Fig. 6) is characterized by very
slight alterations; therefore the morphological aspect of
affected subjects is very similar to normal subjects.
In these patients, we found a slightly decreased projection
of the roof of the orbit on the affected side. The nasal
pyramid is slightly deviated toward the non-synostotic
side, and the malar prominences are symmetrical on both
sides and are not altered compared with healthy subjects.
The bony components of the acoustic system on the
Fig. 8 Picture, soft tissue three-
dimensional reconstruction
and facial skeleton three-
dimensional reconstruction
of a patient with grade IIB
Fig. 9 Picture, soft tissue three-
dimensional reconstruction
and facial skeleton three-
dimensional reconstruction
of a patient with grade III
Childs Nerv Syst (2011) 27:14311443 1441
synostotic side are aligned with those on the unaffected
side. The maxilla and the mandible are normopositioned
and normally developed.
The facial phenotype of grade IIA (Fig. 7) is character-
ized by retraction of the roof of the orbit on the synostotic
side and the side wall, with slight enlargement of the orbit
itself. The nasal pyramid is deviated toward the side
contralateral to the lesion more evidently than in grade I.
At the level of the zygomaticmalar complex, there is a
slight retrusion on the synostotic hemiface. The external
acoustic meatus is slightly anteriorly displaced on the
synostotic side. The maxilla is slightly rotated in the axial
plane toward the non-synostotic side, in the absence of frontal
tilting. The jaw is slightly deviated (1 mm approximately) to
the non-synostotic side and shows a slight tilt such that it is
lower on the synostotic side. These alterations can be seen on
CT measurements but are not evident clinically, owing to
compensational shortening of ipsilateral hemimandible that
masks the glenoid fossa anterior displacement.
The facial phenotype of grade IIB (Fig. 8) is character-
ized by evident retraction of the roof of the orbit and the
side wall on the synostotic side, and it is accompanied by
enlargement of the orbit on the affected side. Patients also
show a slight advancement of the roof of the orbit on the
contralateral side, a sign of compensatory growth. The
nasal pyramid always shows a deviation toward the
unaffected side (more severe than in other grades), and a
serious retrusion of the middle third of the malar promi-
nence on synostotic hemiface was also evident.
In this grade, there was a serious anteriorization of the
external acoustic meatus on the side of the lesion
accompanied by a slight sag. The jaw shows a slight
rotation toward the side contralateral to the lesion in the
absence of tilting. The mandible is tilted, such that it is
lower on the synostotic side, with a slight deviation to
the non-synostotic side. The deviation from the axial
plane is identical to that of grade IIA; this is due to the
fact that the shortening of the mandibular body on the
synostotic side compensates for the glenoid fossa
anteriorization.
The facial phenotype of grade III (Fig. 9) is character-
ized by marked retrusion of the whole orbital frame of
the synostotic side. The orbit of the affected side is also
increased in size, resulting in an obvious so-called
Harlequins phenomenon. The orbital frame on the non-
synostotic side shows a larger projection compared to
non-affected patients, indicating compensatory growth.
The nasal pyramid shows a detour to the synostotic side
that is more severe than other grades.
There is a complete severe retrusion of the middle third
of synostotic hemiface, with an evident involvement of the
malar prominence, which is more prominent in the
unaffected side because of compensatory growth. The
external auditory canal shows severe anteriorization and
sag on the synostotic side. The upper jaw is rotated in the
axial plane toward the non-synostotic side. The amount of
rotation is identical to that observed in grade IIB. It also
shows tilting, being positioned lower on the synostotic side
than the contralateral side.
The mandible showed anteroposition and sag on the
synostotic side and a clear deviation of the chin toward the
non-synostotic side. The deviation is, again, less than
expected considering the extent of structural alterations of
the skull base because the anteroposition of the glenoid
fossa of the affected side would provoke a serious deviation
contralateral to the lesion; however, this does not happen
because there is a shortening of the mandibular hemibody
on the synostotic side, which leads to a more moderate
deviation.
Conclusions
In this study, we were able to associate a facial phenotype
to confirm the predictive value of the classification
proposed by Di Rocco and Velardi. It is highly probable
that the different outcomes depend on the different degrees
of involvement in the synostotic process by the various
skull base sutures which were essentially unaffected by the
surgical procedures.
This kind of surgical failure obviously indicates the
necessity to individuate more effective and extended
surgical modalities which should address the anterior skull
base. The model we have devised allows us to inform the
parents of the affected children about the type and the
extent of the facial abnormalities, which should be faced
during the facio-cranial growth. It also could be utilized for
a more appropriate time for uniforming the correction of the
maxillary, mandibulary and orbital abnormalities which
take place after the primary correction.
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