European Journal of Radiology 90 (2017) 250–255

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European Journal of Radiology

journal homepage: www.elsevier.com/locate/ejrad

Ultrasonography for the diagnosis of craniosynostosis

Maïa Proisy a,b,∗ , Laurent Riffaud c , Kamal Chouklati a , Catherine Tréguier a ,
Bertrand Bruneau a
a
CHU Rennes, Department of Radiology, Hôpital Sud, F-35203 Rennes, France
b
INSERM U1228 VisAGeS Unit, CNRS UMR 6074, Université de Rennes 1, F-35042 Rennes, France
c
CHU Rennes, Department of Neurosurgery, CHU de Rennes, F-35033 Rennes, France

a r t i c l e i n f o a b s t r a c t

Article history: Objectives: The aim of this study was to report our experience with ultrasonography in our routine practice
Received 17 December 2016 for the diagnosis of cranial deformity in infants.
Received in revised form 9 March 2017 Methods: We conducted a single-institution retrospective study of infants referred to our department
Accepted 15 March 2017
because of skull deformity. We only included in this study infants having undergone both US and 3D-CT to
ensure accurate comparisons. Each cranial suture was described as normal or closed (partial or complete
Keywords:
closure). Sonography examination results were correlated with 3D-CT findings as a gold-standard.
Craniosynostosis
Results: Forty infants were included with a mean age of 5.2 ± 4.9 months. Thirty had a craniosynostosis
Ultrasound
Nonsynostotic plagiocephaly
and 10 children had a postural deformity with normal sutures. Correlation between US and 3D-CT for the
Infant diagnosis of normal or closed suture had a specificity and a sensitivity of 100%. US examination for the
Skull diagnosis of complete or incomplete synostosis had a sensitivity of 100%.
Positional plagiocephaly Conclusions: Cranial US is an effective technique to make a positive or negative diagnosis of prematurely
closed suture. US examination of sutures is a fast and non-radiating technique, which may serve as a
first-choice imaging modality in infants with skull deformity.
© 2017 Elsevier B.V. All rights reserved.

1. Introduction to confirm the diagnosis especially for non-specialized practition-

Cranial deformity is common in children. The 2 main causes
remain positional plagiocephaly and craniosynostosis. Positional
plagiocephaly is becoming more and more frequent since recom-
mendations to place infants on their backs for sleeping in order to
prevent sudden infant death syndrome. On the contrary, craniosyn-
ostosis caused by the premature closure of one or more sutures of
the cranial bones is a much more uncommon condition occurring
in only 3–6 infants per 10,000 live births [1]. Consequences of these
2 deformities may be very different as craniosynostosis may lead
to severe chronic intracranial hypertension and thus need surgical
correction. Differential diagnosis between these 2 malformations
sometimes remains challenging. Although physical examination is
sufficient to differentiate craniosynostosis and deformational pla-
giocephaly in most cases, cranial imaging is still regularly needed
ers [1]. Conventional cranial X-ray has traditionally been the first
imaging modality but its interpretation is particularly demanding
and it is a source of radiation. Three-dimensional computed tomog-
raphy (3D-CT) has high diagnosis accuracy, and is considered as
the gold standard [2] but acquisition may need sedation and it is
also a source of radiation. Very recently, ultrasonography (US) has
been proposed to assist practitioners in the diagnosis of craniosyn-
ostosis with excellent efficiency [3,4]. However, although US has
multiple advantages such as low cost and non-ionising technique
few studies have as yet been conducted [5–10]. To date US is not
yet considered as a screening tool for craniosynostosis.
The aim of this study was to report our experience with US
in our routine practice for the diagnosis of cranial deformity in
infants and to demonstrate its high accuracy in the diagnosis of
craniosynostosis.

2. Materials and methods

Abbreviations: 3D-CT, three dimensional computed tomography; US, Ultra-
sound.
∗ Corresponding author at: CHU Rennes, Department of Radiology, Paediatric
2.1. Study group
Imaging, Hôpital Sud, 16 Boulevard de Bulgarie, BP 90347, 35203 Rennes Cedex
2, France. We conducted a single-institution retrospective study between
E-mail address: maia.proisy@chu-rennes.fr (M. Proisy). 2004 and 2014 in our paediatric radiology department. During

http://dx.doi.org/10.1016/j.ejrad.2017.03.006
0720-048X/© 2017 Elsevier B.V. All rights reserved.
 M. Proisy et al. / European Journal of Radiology 90 (2017) 250–255
Fig. 1. Diagnostic approach of craniosynostosis in Rennes University Hospital for children under 8 months old.
this period, infants that were referred to our department by a
general practitioner or a paediatrician for skull deformity and sus-
picion of craniosynostosis underwent both a cranial US and a plain
radiography or 3D-CT according to the practitioner’s prescription.
Ultrasonography was performed in all cases by one of the paediatric
radiologist from our department (CT, BB, KC with respectively 30,
15 and 10 years of experience). Given the US experience acquired
during the first 7 years, we changed our practice after 2011 in agree-
ment with the paediatric neurosurgeon (LR). After this date, US was
performed as a first-line radiological examination in cases of suspi-
cion of craniosynostosis and considered as an alternative to classic
plain radiography or 3D-CT for the diagnosis of craniosysnostosis
(Fig. 1).
For this retrospective study, we only included infants having
undergone both US and 3D-CT in order to compare the sutures in
the most reliable manner. We considered that comparison of all
the sutures was not possible with plain radiography. Each child
was examined first by US and then by 3D-CT.
The study was approved by the local ethics committee and did
not require informed consent from the relatives.
2.2. Ultrasonography procedure
All US examinations were performed on a Philips HDI 5000
Sonoct from 2004 to 2008, then a Philips IU 22 machine from
2008 onwards (Philips Medical System, The Netherlands) using
a high frequency linear transducer (12,5 and/or 17,5 MHz trans-
ducer). The probe was positioned perpendicular to the expected
linear course of the suture. Coronal, sagittal, lambdoïd and metopic
sutures were systematically analysed. During US examination, the
cranial sutures were followed along their whole length.
A cranial suture was considered as normal (patent suture) if
a hypoechoic gap was identified between two hyperechoic bony
plates, with end-to-end appearance or bevelled or overlapped
251
appearance [3]. A suture was considered closed (synostosed suture)
if there was a loss of hypoechoic fibrous gap between bony plates
[4]. Paediatric radiologists paid particular attention to partial or
complete closure of sutures. Image and video recordings were
systematically performed during examination and reviewed for
comparison with 3D-CT findings.
2.3. CT acquisition
All CT examinations were performed using the same 16-slice
multidetector CT (Philips Brilliance, Cleveland, Ohio, USA) with
a stereotyped protocol. The imaging parameters used were as
follows: tube voltage 120 kV, tube current 100 mAs, collimation
16 × 0.75, pitch 0.688, rotation time 0.5 s, slice thickness 0.8 mm,
increment 0.4 mm. 3D-CT volume rendering reconstruction were
systematically performed.
2.4. Data analysis
Sonography examination was correlated with 3D-CT findings as
a gold-standard. The sensitivity, specificity, positive and negative
predictive values of ultrasound for the diagnosis of craniosynostosis
was calculated.
3. Results
3.1. Study group description
A total of 40 infants were finally included. There were 7 girls
and 33 boys. Thirty children had a craniosynostosis including
20 scaphocephaly, 4 trigonocephaly, 3 plagiocephaly, 2 brachy-
cephaly, and 1 atypical craniosynostosis. The case of atypical
craniosynostosis was one case with both sagittal and left coro-
nal suture synostosis. Ten children had a postural deformity with
252 M. Proisy et al. / European Journal of Radiology 90 (2017) 250–255

normal sutures including 8 positional plagiocephaly and 2 non-

synostotic scaphocephaly. The mean age of the 40 children was
5.2 ± 4.9 months. There was no significant difference between
ages in the craniosynostosis group (mean age = 4.9 ± 5.0 months)
and in the postural deformity group (mean age = 6.0 ± 4.8 months)
(p = 0.50).

3.2. US accuracy for the diagnosis of craniosynostosis

Correlation between US and 3D-CT for the diagnosis of normal or

closed suture was excellent. Cranial US for the diagnosis of a closed
suture had a specificity of 100%, a sensitivity of 100%, positive and
negative predictive values of 100% and 100%.

3.3. US accuracy for the diagnosis of complete/incomplete

synostosis

The correlation between US and 3D-CT for the diagnosis of

complete or incomplete synostosis was excellent. There was a sen-
sitivity of 100%.

3.4. Case illustrations

Figs. 2–5 .

4. Discussion

During recent years, the incidence of positional plagiocephaly in

young children has increased by up to 20% [11] due to recommenda-
tions to position infants on their back [12]. Thus, it is now a frequent
finding [13,14]. In such cases, it is important to exclude craniosyn-
ostosis. Although diagnosis of positional plagiocephaly could be
assessed by physical examination with some typical features such
as trapezoidal head shape, mastoid bulge, tilt of the cranial base and
pushed-forwards ear position [15], clinical differentiation with iso-
lated unilateral lambdoid or coronal synostosis could be difficult,
especially for general practitioners. A CT scan is then considered
as the gold standard to distinguish these cranial deformities but
it requires sedation in young children and exposure to ionizing
radiations in this more radiosensitive population [16,17]. For this
last reason, several studies have investigated the impact of dose
reduction on image quality for specific 3D-CT protocols [18,19] but
alternative procedures without any ionizing radiations should be
considered.
Sonographic appearance and measurement of normal major cra-
nial sutures in neonates and infants were first described in 1997 [3]
and high-resolution sonography of the abnormal cranial suture was
described one year later by the same author [4]. Only a few studies
have shown the effectiveness of US in craniosynostosis. So far, US is
not yet used as a routine screening tool. Its use in diagnosing cran-
iosynotosis would become far more common if more specifically
adapted examinations were requested and carried out. Sze et al.
[5] demonstrated that sonography of the lambdoid sutures had
excellent results as a screening test of lambdoid sutural patency
among 41 children. In another study concerning 100 paediatric
patients who were admitted with an abnormal head shape suggest-
ing deformational plagiocephaly, Regelsberger et al. [9] described
that patency of the lambdoid sutures was confirmed by US in 99
cases. In one patient, partial synostosis was diagnosed. Further
reports demonstrated that US was an effective method for the other
sutures [6,7,10]. In our study, cranial US for the diagnosis of a closed
suture had a specificity of 100% and a sensitivity of 100%. These
results are similar to those of another recent study [8], but we also
evaluated complete or incomplete closure of the sutures with a sen-
sitivity of 100%. For these reasons, we think that US is an effective
and reliable technique which may serve as a first imaging tool when
Fig. 2. Posterior positional plagiocephaly in a 4-month-old boy. Vertex view of 3D-
CT (A) shows the head shape and demonstrates the parallelogram appearance of the
skull. The US image of the left coronal suture performed at the level of the blue line
positioned on the 3D-CT shows the hypoechoic gap between hyperechoic calvarial
bones (B). Lambdoid sutures were also patent on US examination and 3D-CT (not
shown on this Figure).
a clinical diagnosis of craniosynostosis is suspected. Moreover, US
has many advantages: it is a fast, non-ionising technique that does
not require any sedation.
However, there are some limitations with US examination.
Ultrasound imaging is often described as an “operator-dependent”
technique. Although education of radiologists is recommended,
imaging of the cranial sutures is relatively easy to learn. Fur-
thermore, this method did not show any interobserver variability
between two independent investigators in Regelsberger et al’s
study [9]. Another limitation of US is the inability to assess sutu-
ral patency after a certain age. In our experience, the earlier the
US is performed the more reliable the examination is. Reliability
of US decreased with age because of hair growth, skull growth and
thinner sutures, especially coronal sutures. According to our expe-
rience, US is limited by age with the upper limit at 8 months old.
Regelsberger et al. considered the age limit of 13 months [9] for
the technique and Sze et al. [5] were able to distinguish patent
from fused sutures in one patient scanned at 18 months. As most
children with cranial deformity are usually seen before the age of
10 months, we recommend carrying out US examination as soon
as possible when diagnosis is suspected, ideally before the age of
8 months. Special attention must be paid to the metopic suture.
 M. Proisy et al. / European Journal of Radiology 90 (2017) 250–255 253

Fig. 3. Unilateral coronal synostosis in a 2-month-old boy. Antero-superior 3D-CT

view (A) shows the elevated left orbital roof and the complete fusion of the left
coronal suture. The US image performed at the level of the blue line positioned on
the 3D-CT shows the synostosed left coronal suture with a continuous hyperechoic
calvarian bone and no hypoechoic gap (B).

Indeed, physiological closure of this suture occurs much earlier

than the others. As fusion normally arises between the age of 3
and 9 months [20], complete fusion of the metopic suture does not
necessary mean trigonocephaly. Typical triangular frontal defor-
mation and hypotelorism should also be associated with complete
fusion of this suture to diagnose trigonocephaly with certainty.
3D-CT is an important imaging technique in diagnosis of cran-
iosynostosis. However, 3D-CT should not be used as a first tool
imaging procedure as is still the case in many centres. We con-
sider that it should concern only children with uncertain clinical
situations and those with positive findings at US as a preoperative
planning. Regarding preoperative surgical planning in our institu-
tion, 3D-CT examination was only performed in cases of metopic,
coronal or complex craniosynostosis. US demonstrated diagno-
Fig. 4. Scaphocephaly in a 1-month-old boy. Superior view of the 3D-CT (A) shows
sis of craniosynostosis in an initial step and then allowed us to the particular head shape with elongated cranium and the sub-total sagittal syn-
wait for the right moment to perform 3D-CT (one month before ostosis, with the corresponding US images (B) showing a continuous hyperechoic
surgery). Furthermore, we considered that a child with a charac- bone at the anterior part of the suture, with bridging at the half-posterior part and
a very small part of the posterior patent suture with persistence of an hypoechoic
gap.
254 M. Proisy et al. / European Journal of Radiology 90 (2017) 250–255
Fig. 5. Nonsynostotic scaphocephaly in a 10 month-old boy. Vertex view of the
3D-CT shows the head deformity with an elongated cranium suggestive of scapho-
cephaly but patent coronal, sagittal and lambdoid sutures. Corresponding US images
at the level of blue lines show patent sutures with persistence of a hypoechoic gap
between calvarial bones (B).
teristic nonsyndromic scaphocephaly and a positive US for sagittal
synostosis should be operated on without additional imaging stud-
ies. In the literature some authors suggest that operative correction
of isolated sagittal synostosis can proceed without any radiolog-
ical investigations, unless clinical examination exhibits atypical
features [20]. Considering potential side effects from ionizing
radiation, risks of sedation, and costs, Fearon et al. [21] also sug-
gested that surgeons should reserve computed tomographic scans
for infants in whom physical examination was not clear. Finally
and regarding literature [22] and our 2 cases of nonsynostotic
scaphocephaly (illustration: case 5), we advise that craniosynosto-
sis should always be confirmed by radiological imaging except for
typical positional plagiocephaly, with US as a first step diagnosis.
5. Conclusion
US examination of sutures is a fast, effective and non-irradiating
technique that may serve as a first choice imaging modality in
infants (particularly under 8 months old) with skull deformity
when craniosynostosis is suspected. In our study, we demonstrated
that US was an effective technique for positive diagnosis of a pre-
maturely closed suture. Diagnosis of craniosynostosis can be ruled
out if US examination of the entire coronal, sagittal and lambdoid
sutures demonstrates normal patent sutures. 3D-CT should only
be recommended either in atypical cases or in a pre-operative set-
ting when the diagnosis of craniosynostosis has been acquired on
US examination. Thus 3D-CT could be organised at the appropriate
time before surgery according to the surgeon’s recommendations
in order to optimize surgical planning.
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