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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 CT scan 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 and32 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 CT scan 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 πλαγιος

(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. GaspariniMaxillo Facial Surgery, Complesso Integrato Columbus,Catholic University Medical School,Via Giuseppe Moscati 31-33,00168 Rome, Italye-mail: [email protected]

G. Gasparinie-mail: [email protected]

G. Tamburrini : C. Di RoccoPediatric Neurosurgery, Policlinico Universitario “A. Gemelli”,Catholic University Medical School,Largo Agostino Gemelli 8,Rome, Italy

A. MoroMaxillo Facial Unit, Ospedale S. Giovanni Battista,Foligno, Perugia, Italy

Childs Nerv Syst (2011) 27:1431 – 1443

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 0.4and 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 [3 – 6]. Consequently, the malformation is fully

expressed at the end of the craniofacial growth [7 – 9]

(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 6 – 7 years of age. The posterior skull base

anomaly and the asymmetry of the cervico-cranial junctionare 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 CT scan 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 theseverity of rocca petrosa anterior displacement and the

presenceof vomer bone deviation. Group IIA includes 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 thehypertelorism, 5 the deviation of the nasal pyramid controlaterallyfrom the synostosis, 6 the retrusion of the malar prominence on theaffected side, 7 the mandibular deviation toward the unaffected side

1432 Childs Nerv Syst (2011) 27:1431 – 1443



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

plagio cephaly, two patien ts wit h brachicep haly and

patients with complex craniosynostoses, namely: 15 cases of

Crouzon’s syndrome, 12 cases of Apert ’s syndrome and a caseof Saethre – Chotzen 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

(20 – 32 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 usedto 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 permitscephalometric assessments and surgical simulation

Childs Nerv Syst (2011) 27:1431 – 1443 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 cephalometricassessments

Fig. 3 View of the reference plan system developed specifically for the purpose of the study

1434 Childs Nerv Syst (2011) 27:1431 – 1443



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 from the 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) 27:1431 – 1443 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



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


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


Ang RP p med 59.25° I

59.06°C



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



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


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 7.72Ang RP p med 60.03° I

52.61°C



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) 27:1431 – 1443



Table 2 (continued)


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



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


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



54.76°C



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



Menton 51.62 1.71 C −115.83

Grade III



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


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


52.76°C

Childs Nerv Syst (2011) 27:1431 – 1443 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

“Harlequin’s 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 entitythan 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 malar – zygomatic

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)




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



Menton 61.24 4.3 C −115.83

1438 Childs Nerv Syst (2011) 27:1431 – 1443



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 theside 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 from the axial plane

on the synostotic side. The distance between the menton and

gonion was less on the hemimandibula of synostotic side, andit 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 thoseobserved 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 reconstructionand facial skeleton three-dimensional reconstructionof a patient with grade I

Fig. 7 Picture, soft tissue three-dimensional reconstructionand facial skeleton three-

dimensional reconstructionof a patient with grade IIA

1440 Childs Nerv Syst (2011) 27:1431 – 1443



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