PEDIATRIC/CRANIOFACIAL

Classification of Trigonocephaly in
Metopic Synostosis
Joel S. Beckett, B.A.
Priyanka Chadha, B.Sc.
John A. Persing, M.D.
Derek M. Steinbacher,
M.D., D.M.D.
New Haven, Conn.
Background: The orbitofrontal deformity in metopic synostosis is recognized
clinically but has not been quantitatively defined in a large patient population.
The authors’ purpose was to document the dysmorphology in metopic synostosis
and define subtype gradations.
Methods: Demographic and computed tomographic information was re-
corded. Three-dimensional computed tomographic renderings were created
digitally. Craniometric analysis was conducted for endocranial bifrontal angle,
interzygomaticofrontal suture and interdacryon distance, and angle of orbital
aperture to the midline.
Results: Thirty-five computed tomographic scans were analyzed: 25 affected
infants (median age, 5 months) and 10 controls (median age, 6 months). The
endocranial bifrontal angle ranged from 100 to 148 degrees in metopic patients
and 134 to 160 degrees in controls. The metopic group was split into severe
metopic (100 to 124 degrees) and moderate metopic (124 to 148 degrees)
synostosis. The endocranial bifrontal angle was significantly different among
severe metopic, moderate metopic, and control patients. Interzygomaticofron-
tal suture of the severe group was less than in both moderate (p ⫽ 0.0043) and
control (p ⫽ 0.011) groups. Interdacryon distance was smaller in severe versus
moderate (p ⫽ 0.0083) and control (p ⫽ 0.0002) groups. The orbital rim angle
of the severe group was more acute than that in the moderate (p ⫽ 0.0106) and
control (p ⫽ 0.0062) groups. Except for endocranial bifrontal angle, there was
no difference between moderate metopic and control groups in any analysis.
Conclusions: Metopic synostosis can be divided into two distinct severity indices.
The severe group has significantly narrower orbitofrontal dimensions, whereas
the moderate group does not differ from control. Characterization of trigono-
cephaly may shed light on the etiopathogenesis of disease. (Plast. Reconstr. Surg.
130: 442e, 2012.)
CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

N
onsyndromic, isolated craniosynostosis is
estimated to occur in one in 2000 live
births.1 Fusion of the metopic suture rep-
resents an increasingly prevalent variant of cra-
niosynostosis. Although historically estimated at 3
to 10 percent of all cases, metopic synostosis has
risen in number over the past 25 years and now
represents 23 to 28 percent of craniosynostosis
presentations.2– 4
The synostosis results in a classic dysmorphol-
ogy characterized by trigonocephaly with bitem-
poral narrowing and hypotelorism. The orbito-
From the Section of Plastic and Reconstructive Surgery, Yale
University School of Medicine.
Received for publication January 29, 2012; accepted April
3, 2012.
Copyright ©2012 by the American Society of Plastic Surgeons
DOI: 10.1097/PRS.0b013e31825dc244
frontal deformity is typically symmetric, involving
bilateral supraorbital retrusion with an acute an-
gulation between the forehead halves.5 Clinicians
recognize a phenotype range, but the spectrum
has not been quantitatively defined in a large pa-
tient population.
Surgical correction of metopic synostosis tradi-
tionally occurs in early infancy by means of cranial
reconstruction, orbitofrontal rim advancement, and
metopic suture synostectomy.1 Recently, there has
been a reemergence of endoscopic minimally
invasive techniques for the treatment of isolated
craniosynostosis.6 Compared with the traditional
approach, endoscopic strip craniectomy may re-
Disclosure: The authors have no financial interest
to declare in relation to the content of this article.

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Volume 130, Number 3 • Trigonocephaly in Metopic Synostosis

sult in less blood loss and shorter hospital stay and
can be performed at an earlier age.7 Depending
on the severity of dysmorphology, the procedure
relies on helmet therapy for up to 1 year postop-
eratively to assist the correction of skull shape. The
decision between traditional and endoscopic re-
pair is typically surgeon dependent, and the age of
presentation may play a role. The purpose of this
study was to objectively document the orbitofron-
tal dysmorphology in metopic synostosis, define
subtype gradations compared with normal con-
trols based on craniometric data, and consider
possible etiopathogenetic and treatment implica-
tions.
MATERIALS AND METHODS
This is a retrospective analysis preformed in
concordance with the Yale University Institutional
Review Board (protocol no. 1101007932). Demo-
graphic data and computed tomographic scan in-
formation were obtained for metopic synostosis
and control subjects. All metopic synostosis sub-
jects included in this study presented before 8
months of age with a metopic ridge and some
amount of bitemporal constriction or trigonoceph-
aly. Fusion of the metopic suture was confirmed on
computed tomographic scan. Age and sex were
recorded at the time of computed tomographic
scanning for each subject. Children with additional
synostosis or other craniofacial or intracranial ab-
normality were excluded. Three-dimensional com-
puted tomographic scans were analyzed using a sur-
gical planning program (SurgiCase; Materialise,
Leuven, Belgium). Craniometric analysis was per-
formed on a number of parameters in the orbito-
frontal region (Table 1 and Fig. 1). The null hy-
pothesis was used and statistical analysis involved
Kruskal-Wallis analysis of variance (JMP 9; SAS In-
stitute, Inc., Cary, N.C.), group pairs were compared
directly with the Mann-Whitney U test, the Bonfer-
roni correction was used, and a value of p ⬍ 0.017 was
considered significant.
RESULTS
Three-dimensional computed tomographic
scans were identified in 25 infants with metopic
synostosis and 10 corresponding control infants.
There were 18 male and seven female metopic
patients, with a mean median age of 5 months.
The control group contained four male and six
female infants, with a median age of 6 months
(Table 2).
The endocranial bifrontal angle ranged from
100 to 148 degrees in metopic patients and 134 to
160 degrees in controls. The metopic group was
split into severe metopic (100 to 124 degrees, n ⫽
12) and moderate metopic (124 to 148 degrees,
n ⫽ 13) synostosis. The endocranial bifrontal an-
gle differed significantly among the three groups
(H ⫽ 25.972, df ⫽ 2, p ⬍ 0.0001) (Table 3).
Interzygomaticofrontal suture distance was
significantly different among the three groups
(H ⫽ 25.02, df ⫽ 2, p ⬍ 0.0001). The distance in
severe metopic group was significantly less (70.1
mm) than in both control (76.5 mm, p ⫽ 0.0011)
and moderate metopic (75.5 mm, p ⫽ 0.0043)
groups. There was no significant difference between
moderate metopic and control groups (Table 4).
Interdacryon distance varied significantly
among the three groups (H ⫽ 15.74, df ⫽ 2, p ⫽
0.0004). The severe metopic group was again sig-
nificantly smaller (14.1 mm) than both control
(19.0 mm, p ⫽ 0.0002) and moderate metopic
(18.0 mm, p ⫽ 0.0083) groups. There was no sig-
nificant difference between moderate metopic
and control groups (Table 5).
There was no statistical difference between
right and left orbital rim angles in intragroup anal-
ysis of any group. In intergroup analysis, again the
three groups were significantly different (H ⫽
9.923, df ⫽ 2, p ⫽ 0.0070). The orbital rim angles

Table 1. Craniometric Parameters

Abbreviation Parameter Description
ECA Endocranial bifrontal Angle of the frontal bone in a single axial plane at the level of the superiormost
angle aspect of the crista galli with the vertex (A) located on the endocranial side of
the frontal bone at the metopic suture and terminal points at the lateral
borders of the respective orbital apertures (B right, B left)
ZF Interzygomaticofrontal Distance between zygomaticofrontal sutures (ZF)
suture distance
ID Interdacryon distance Distance between dacryons
ORA Orbital rim angle Angle of the plane of the orbital aperture, as defined by points on the orbital
rim: supraorbital notch (SON), zygomaticofrontal suture (ZF),
zygomaticomaxillary suture (ZM), with the midsagittal plane defined by the
following points: sella turcica (ST), nasal spine (NS), and posteromedial
aspect of foramen magnum (PMFM)

443e

Fig. 1. Three-dimensional computed tomographic scan show-
ing the skull of a 6-month-old infant with the craniometric points
used in this study.
Table 2. Demographic Information
Metopic
No. of subjects 25
Sex
Male 18
Female 7 6
Age, mo
Median 5 6
First to third quartile 4–7
Range 2–8
of severe metopic was significantly more acute
than control (p ⫽ 0.0062) and moderate metopic
(p ⫽ 0.0106) groups. There was no significant
difference between moderate metopic and con-
trol groups (Table 6 and Figs. 2 and 3).
DISCUSSION
Metopic synostosis is a surgical problem of
growing incidence. Traditionally thought to occur
in one in 15,000 births, the incidence is now es-
timated as high as one in 5000 births.8 The cau-
sation of increase is not known, but some studies
postulate that the increase of positional plagio-
cephaly from the Back to Sleep campaign may be
providing biomechanical forces that stimulate
early metopic fusion.9
In this study, we show that those cases with
severe metopic synostosis, as characterized by an
endocranial bifrontal angle of less than 124 de-
grees, have significant orbitofrontal narrowing
and medially slanted orbital rims compared with
controls. In contrast, moderate synostosis (en-
docranial bifrontal angle ⬎124 degrees) does not
result in significant morphologic orbitofrontal dif-
ferences compared with controls.
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Plastic and Reconstructive Surgery • September 2012
This study indicates that two patterns of sever-
ity exist in metopic synostosis from which one
could hypothesize that there may be different gen-
eral contributing pathophysiologic mechanisms.
The most severely affected metopic group pres-
ents with marked trigonocephaly, likely from a
longer duration of growth restriction caused by in
utero fusion.10 By distinction, less severe presen-
tations of metopic synostosis with trigonocephaly
(moderate metopic group) may result from later
in utero fusion or early postnatal fusion. The su-
ture still fuses prematurely and imparts growth
constriction, vis-à-vis bitemporal narrowing and
lateral supraorbital retrusion, but the magnitude
is less corresponding to decreased time of fusion
against a growing brain. Stressors such as posi-
tional plagiocephaly may be one etiologic factor.9
Teasing out the relationship between timing of
fusion and severity of deformity will be the focus
Control of future research.
10 It is important to note the difference between
the moderate metopic group and those infants with
4 metopic ridging at or after 8 months of age. The
moderate metopic group represents subphysiologic
metopic suture closure in younger infants. The
3–8 quantitative findings in our study, including en-
2–9
docranial bifrontal angle and interorbital distance,
confirmed the representation of trigonocephaly in
these infants compared with normal controls. In-
fants that present at or after 8 months of age with a
metopic ridge and absence of trigonocephaly were
not included in this study. These children should be
objectively similar to normal controls, and the com-
parison could be entertained; however, computed
tomographic scans are not routinely obtained for
infants older than 8 months with normal physiologic
metopic suture closure. The ridge present from
physiologic metopic closure ordinarily will decrease
over time.
Treatment objectives in the surgical correction
of metopic synostosis include obviating growth re-
striction and intracranial pressure, normalizing su-
praorbital retrusion, and rounding the forehead
shape, in an “overcorrected” fashion if possible.11,12
Early synostectomy has been advocated by some,
hoping that a combination of brain growth and pro-
longed helmet use will normalize the forehead.6
However, the ability to fully correct the deformity
with these lesser techniques is still being debated.13,14
In addition, studies conducted within the past
decade indicate that a high degree of brain dys-
morphology is present in patients with simple cra-
niosynostosis both before and after surgical
correction.15,16 An intrinsically dysmorphic brain,
which is unable to maintain suture patency, may
Volume 130, Number 3 • Trigonocephaly in Metopic Synostosis

Table 3. Endocranial Angle of Metopic Synostosis and Control Groups

C* MM* SM*
No. 10 13 12
Median, mm 147.2 (137.3–153.5) 136.4 (132.4–140.9) 113.0 (106.7–118.4)
Statistical comparison
MM p ⫽ 0.0101 —
SM p ⬍ 0.0001 p ⬍ 0.0001
C, control; MM, moderate metopic; SM, severe metopic.
*First to third quartile.

Table 4. Interzygomaticofrontal Suture Distance of Metopic Synostosis and Control Groups

C* MM* SM*
No. 10 13 12
Median, mm 76.5 (74.9–78.9) 75.5 (73.5–80.1) 70.1 (66.0–72.5)
Statistical comparison
MM p ⫽ 0.7330 —
SM p ⫽ 0.0011 p ⫽ 0.0043
C, control; MM, moderate metopic; SM, severe metopic.
*First to third quartile.

Table 5. Interdacryon Distance of Metopic Synostosis and Control Groups

C* MM* SM*
No. 10 13 12
Median, mm 19.0 (17.6–20.3) 18.0 (15.9–18.8) 14.1 (12.5–14.8)
Statistical comparison
MM p ⫽ 0.1287 —
SM p ⫽ 0.0002 p ⫽ 0.0083
C, control; MM, moderate metopic; SM, severe metopic.
*First to third quartile.

Table 6. Orbital Rim Angle of Metopic Synostosis and Control Groups

C* MM* SM*
No. 10 13 12
Median
Right, degrees 59.5 (55.3–61.4) 56.7 (55.1–62.4) 53.5 (51.7–56.6)
Left, degrees 57.6 (55.8–61.7) 56.6 (54.9–63.4) 53.6 (50.2–56.3)
Statistical comparison
MM p ⫽ 0.5980 —
SM p ⫽ 0.0062 p ⫽ 0.0106
C, control; MM, moderate metopic; SM, severe metopic.
*First to third quartile.

not be capable of the growth required to correct
skull deformity by means of the functional matrix
concept.
However, if it follows that a moderate defor-
mity corresponds to a more normally behaving
brain, it may warrant consideration that a minor
synostectomy alone could effectively treat this
group. This is because the magnitude of change
required for correction is less and the brain may
be more capable of expansion. Such treatment
corollaries deserve attention in future studies.
Perhaps the critical piece of information is that
a significant proportion of children with surgically
corrected nonsyndromic craniosynostosis have learn-
ing disabilities.17,18 The cognitive profile is thought to
be secondary to an underrecognized increase in in-
tracranial pressure and/or intrinsic brain malfor-
mation. Currently, there is little evidence that sever-
ity of skull deformity and timing/type of surgical
correction have an impact on cognitive develop-
ment, but additional study is needed.19 –21 As learning
disability in craniosynostosis may be related to in-
creased intracranial pressure, one could postulate
that the severe metopic group in this study would
have more impairments. Moreover, if learning dis-
ability arises from primary brain malformation, it

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 Plastic and Reconstructive Surgery • September 2012

Fig. 2. Craniometric analyses of the orbitofrontal region of 6-month-old infants with moderate (left) and severe (right) metopic
synostosis. The moderate metopic infant has an endocranial bifrontal angle of 138 degrees, an interzygomaticofrontal suture
distance of 79.1 mm, and an interdacryon distance of 18.6 mm. The severe metopic infant has an endocranial bifrontal angle
of 121 degrees, an interzygomaticofrontal suture distance of 68.2 mm, and an interdacryon distance of 12.6 mm.

tal narrowing is not significantly different. Further

Fig. 3. Orbital rim angle analysis of left orbital aperture of
6-month-old infant with moderate metopic synostosis.
also is reasonable to hypothesize that the severe
metopic group is indicative of a more profound
brain dysmorphology. Future studies that use a com-
bination of cognitive testing with functional and mi-
crostructural neuroimaging may provide more robust
evidence and allow for teasing apart the relationship
between skull deformity and neurofunction.22,23
CONCLUSIONS
Metopic synostosis patients can be placed into
two severity indices—moderate and severe—
based on the endocranial angle. Severe metopic
synostosis is accompanied by significantly more
acute endocranial bifrontal angle and greater or-
bitofrontal narrowing versus controls. Moderate
disease, in contrast, has a more acute endocranial
bifrontal angle than controls, but the orbitofron-
study is needed to investigate etiopathology, as the
two groups may arise from different mechanisms;
also, the groups may have differential impact on
neurodevelopment and may be amenable to tai-
lored treatment approaches.
Derek M. Steinbacher, M.D., D.M.D.
Craniofacial Program
Yale Plastic and Reconstructive Surgery
3rd Floor, Boardman Building
330 Cedar Street
New Haven, Conn. 06520
derek.steinbacher@yale.edu
ACKNOWLEDGEMENTS
The authors sincerely thank the Charles W. Ohse
Grant for Surgical Research (Department of Surgery,
Yale University) and the Doris Duke Charitable Foun-
dation for supporting this study.
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