697

Pictorial Essay

Primary Craniosynostosis: Imaging Features

1996.166:697-703.

Mark L. Benson 2 Patrick J. , Nancy C. , S. James

In 1 851 , Virchow Introduced the term craniosynostosis to TABLE I : Classification of Primary Cranlosynostosis
describe a variety of abnormalities in calvarial growth [1].
These skull deformities are usually apparent in infancy. When Type Sutures Involved
American Journal of Roentgenology

an abnormal calvarial configuration is detected, a radlologic

Simple synostosis
evaluation is necessary to characterize the deformity and to
Brachycephaly Coronal and/or lambdoidal
guide the corrective surgical procedure. Affected children are
Dolichocephaly Sagittal
believed to have an improved outcome when diagnosis and
(scaphocephaly)
surgical intervention occur at an early age. CT with three-
Tnigonocephaly Metopic
dimensional reconstruction optimally evaluates the presence
Pachycephaly Lambdoidal
and degree of sutural involvement and assesses associated
Plagiocephaly Unilateral coronal or lambdoidal
facial and intracranial abnormalities (2]. This pictorial essay
Compound synostosis
illustrates the Imaging findings, nomenclature, and associated
Acrocephaly All sutures or coronals
abnormalities of the various types of primary craniosynostosis. (oxycephaly/turnicephaly) plus one other
Kleeblattsch#{228}del(cloverleaf Multiple or all
skull deformity) [1]
Classification
Craniosynostosis can occur as a primary abnormality, as
part of a known syndrome, or in association with a host of narrowing, and indistinctness of the suture. These changes may
systemic conditions. Primary craniosynostosis is classified occur over a 1-2-mm segment of suture, or there may be a fibrous
according to the affected calvanial suture(s) (Table 1). union in which no primary nadiologic changes
are noted despite marked secondary signs.
Secondary signs include an altered calvarial shape, changes in
Radiologic Findings
shape and timing of closure of fontanels, and facial
Primary signs of craniosynostosis include bony bridging along anomalies [3, 4]. The lack of growth across a suture often
the suture, heaping up of bone at the suture, sutural results in effacement of the underlying subarachnoid spaces,

Received May 30, 1995; accepted after revision October 12, 1995.

1 Department of Radiology and Radiological Sciences, Neuroradiology Division, The Johns Hopkins Medical Institutions, 600 N. Wolfe St., Baltimore, MD 21287.
2Present address: Department of Radiology, Wheeling Hospital, One Medical Park, Wheeling, WV 26003. Address
correspondence to M. L. Benson.
AJR 1996;166:697-703 036i-803)(/96/1663-697 © American Roentgen Ray Society
 698 BENSON El
implying that there is a restriction on underlying brain growth,
and is considered an indication for surgical repair. Patients
with craniosynostosis may have an enlarged subanachnoid
space beneath regions of compensatory skull growth. If promi-
nent, this condition may require dunal plication to obliterate the
space and to dampen CSF pulsations at the time of repair [5].
The diagnosis of craniosynostosis is based on the calva-nial
shape with relation to a calvarial suture. The presence of
secondary radiographic signs across a suture without pni- many
signs should not delay the diagnosis or surgical conrec-
tion. Children with craniosynostosis who undergo surgical
correction before 3 months of age have a more favorable aesthetic
outcome than do children who undergo surgery at
American Journal of Roentgenology 1996.166:697-703.
AL. AJA:166, March 1996
an older age [6]. The rate of brain growth in older children is
reduced and may not be adequate to promote a normal cal-
varial contour after surgery [7].
Sagittal Synostosis
Sagittal synostosis is the most common type of cranio-
synostosis, accounting for 50% of all cases, and it is usually
an isolated finding [3]. Patients have an elongation of the
skull known as dolichocephaly on scaphocephaly (Fig. 1).
The characteristic frontal and occipital prominence is pre-
dicted by compensatory growth patterns along the metopic,
coronal, and Iambdoidal sutures [7].
Fig. I .-Dollchocephaly (scaphocephaly). Generalized
elongation of caivarlum occurs with overall narrowing of
skull width.
A, Illustration of child with dolichocephaly due to sagittal
suture synostosis. (Reprinted with permission from [1])
B, Axial CT image of 6-month-old child shows narrow
width of calvarium Is more prominent posteriorly (arrows)
due to preferential Involvementof posterior
portion of sag-
ittal suture.
C, Axial CT image of same child shows sclerosis and
bony ridging of posterior portion of sagittal suture (open
arrow) with less prominent involvement of anterior portIon
of suture (arrowhead). Note patentlambdoid sutures (solid
arrow).
D, Three-dimensional CT reconstruction of vertex view
shows dolichocephaly with obliteration of sagittal suture
(arrowheads). Asymmetrical involvement of sagittal suture is
masked by smoothing function of reconstruction algo-
rlthm.
E, Three-dimensional CT reconstruction of lateral view
best shows dolichocephalic configuration. A = anterior, P =
posterior.
 AJR:166, March 1996 IMAGING OF PRIMARY CRANIOSYNOSIOSIS 699

A
American Journal of Roentgenology 1996.166:697-703.

Fig. 2.-Trigonocephaly, due to metopic suture synostosis, results in triangular deformity of frontal
bone wIth central bossing and constriction of temporal areas.
A, Illustration of child with trigonecephaly.(Reprinted with permission from [1])
B, AxIal CT image of 6-month-old child shows ridging along metopic suture gives rise to
characteristic triangular deformity (arrows) of forehead and assocIated flattening of frontal eminences.
C, Axial CT image of same child shows hypotelorism (intercanthal distance of 12 mm, open arrows)
and hypoplastic ethmold sinuses are present.
D, Three-dimensional CT reconstruction of anteroposterlor view In 1-year-old child shows prominent
metoplc suture ridging (open arrows).
Metopic Synostosis Unilateral Coronal Synostosis
Patients with this form of craniosynostosis have a keel- Patients with this type of synostosis have flattening of the
shaped (triangular) frontal deformity known as tnigonoceph- frontal bone, uplifting of the ipsilateral eyebrow, ipsilatenal
aly (Fig. 2). Symmetrical bone growth occurs at the sagittal exophthalmos, and a palpable suture [3]. The radiographic
suture with asymmetric bone growth at the coronal sutures, findings include plagiocephaly (flattening) over the affected
producing the characteristic pear shape with flattened frontal suture with contralateral frontal and panietal expansion and
eminences [7]. Associated radiographic signs include ipsilateral temporal expansion [7] (Fig. 3). In our experience,
hypotelorism (intercanthal distance less than 15 mm in this is often associated with synostosis of the contralateral
infants less than 1 year old), hypopiastic ethmoid sinuses, lambdoidal suture. Additional radiographic findings include a
and anterior bowing of the coronal sutures [3, 4]. shallow anterior cranial fossa, shallow orbits, uplifting of the
700 BENSON El AL. AJA:166, March 1996

A
American Journal of Roentgenology 1996.166:697-703.

Fig. 3.-Unilateral coronal synostosis and plaglocephaly.

A, Illustration of child with unilateral left coronal synostosis. Left frontal area is foreshortened, contralateral frontal area is bossed anteriorly, and
left eyebrow Is uplifted. (Reprinted with permission from [1])
B, Axial CT image of 2-month-old child with right coronal synostosis shows right frontal plaglocephaly (flattening, open arrows) with left frontal
expansion and sclerosis of right coronal suture (solid arrow).
C, Axial CT Image of same child shows effacement of underlying subarachnoid spaces (open arrows) compared with contralateral side (solid
arrows).
0, Three-dImensional CT reconstruction of vertex view shows right frontal plagiocephaly (open arrow), associated facial deformity, and obliteration of right
coronal suture (arrowheads).
E, Three-dimensional CT reconstruction of coronal cut-plane view shows uplifting of right orbital roof (arrows), the harlequin eye deformity.
F; Anteroposterior skull radiograph depicts harlequin eye (arrows) that correlates well with deformity seen in E.

Bilateral Coronal Synostosis

Craniosynostosis of both coronal sutures is often

orbital roof (the harlequin eye deformity), and depression of the associated
petrous bone [3]. with synostosis of other sutures and/or other
malformation
syndromes [3, 4]. Affected patients have a decrease in the
anteroposterior dimension of the calvaria called brachycephaly
(Fig. 4). Asymmetric bone deposition occurs along the squa-
mosal sutures producing a bitemporal bulge. Symmetrical bone
growth along the sagittal and metopic sutures produces
an abnormally tail head [7]. Additional radiographic signs are the
same as in unilateral coronal synostosis, except that they occur
bilaterally.
Unilateral Lambdoidal Synostosis
Patients with this abnormality have flattening of the occipi-
tal bone known as plagiocephaly (Fig. 5). No facial abnor-
 AJA:166, March 1996 IMAGING OF PRIMARY CRANIOSYNOSTOSIS 701

A
Fig. 4.-Bilateral coronal synostosis and bilateral frontal
bone flattening.
A, Illustration of child with bilateral coronal synostosis
and bilateral frontal bone flattening. (Reprinted with per-mission ... .
#{149}: .
from (1])
B, Axial CT image in 9-month-old child shows sclerosis
American Journal of Roentgenology 1996.166:697-703.

and heaping up of bone at both coronal sutures (open

arrows). Bilateral frontal flattening results In triangular con-
figuration of anterior portion of calvarlum. This occurs
above level of metopic suture and should not be confused
with trigonocephaly.
C, Axial CT image in same child shows shallow orbits
and exophthalmos (curved arrow and dotted line) are present
I’.
bilaterally.
“ “I
D, Three-dimensional CT reconstruction of lateral view
shows shortening of calvarium in anteroposterior dimen-
sion resulting in brachycephaly. Because of smoothing
function of reconstruction algorithm, neither coronal nor
lambdold suture is visualized. A = anterior, P = posterior
E, Three-dimensional CT reconstruction of frontal view
shows uplifting of both orbital roofs (arrows), the bilateral
harlequin eye deformity.

malities are seen [4]. Compensatory bone growth occurs at both squamosal sutures, resulting in a bilateral temporal
the ipsilateral squamosal, contralateral Iambdoidal, and sag- prominence with anterior and inferior displacement of the
ittal sutures, resulting in an ipsilateral temporal bulge with pinnae. Bony expansion on both sides of the sagittal suture
depression of the pinna and contralateral panieto-occipital elevates the vertex [7].
expansion [7]. Synostosis of the Iambdoidal suture may pro-
duce only a mild deformity owing to the small amount of Not all cases of craniosynostosis will have a calvanial
bone growth that occurs along this suture [7]. deformity. The typical calvanial deformity seen in primary
craniosynostosis is absent in cases of secondary synostosis
that are due to inadequate brain growth (Fig. 7).
Bilateral Lambdoidal Synostosis Not all pediatric calvarial deformities are due to cranio-
This is the least common type of craniosynostosis [4]. synostosis. Calvarial asymmetry may result from the position
Patients present with brachycephaly (Fig. 6). Radiographic of the fetus in utero or of the neonate in the crib (Fig. 8). The
findings include flattening across the occiput and a shallow flattening of the calvania must cross a suture for the diagno-
posterior fossa [4]. Enhanced bone growth occurs along sis of craniosynostosis to be made.
 702 BENSON El AL. AJA:166, March 1996

Fig. 5.-4-month-old child with right iamb-

doidal plaglocephaly.
A, Axial CT image shows bone heaping up
at margins of right lambdoid suture (open
arrow) with plagiocephaly (flattening) across
suture. Note normal left lambdold suture (solid
arrow).
B, Axial CT Image shows right parleto-
occipital plagiocephaly (open arrows) with
right lambdold suture
narrowing. There Is com-
pensatory right temporal bulging
(arrowheads)
and centralateral parleto-occipital expansion
(solid arrow).
C, Three-dimensional CT reconstruction of
vertex view shows right lambdold plagioceph-
aly (open arrow) and contralateral parleto-
occipital expansion (solid arrow).
D, Three-dimensional CT reconstruction of
angled frontal-basal view accentuates right
parieto-occipital piaglocephaly (open arrows),
ipsilateral temporal bulge (arrowheads), and
contralateral parleto-occipital expansion
(solid
arrow).
American Journal of Roentgenology 1996.166:697-703.

Fig. 6.-Brachycephaly.
A, Axial CT Imageof 5-month-old child shows marked shortening of calvarium In anteroposterior dimension (brachycephaly) with bilateral parieto-
occipital plagiocephaly (arrows).
B, Three-dimensional CT reconstruction of lateral view In same child shows brachycephalic configuration of calvarium with
elevation of vertex. A=
anterior, P = posterior
 C, Three-dimensional CT reconstruction of posterior view in different patient with multiple suture synostosis. Ridging (arrows) of both lambdoid sutures is
seen.
AJA:166, March 1996 IMAGING OF PRIMARY CRANIOSYNOSTOSIS 703

Fig. 7.-i-year-old child with microcephaly and secondary synostosis of all Fig. 8.-9-month-old child
with clinically
major cranial sutures. apparent calvarial deformity, presumably
A, Axial CT Image shows diffuse cerebral atrophy. due to persistent positioning of child on his
B, Image shows bony ridging and sclerosis of both lambdoid and coronal left side in crib. Three-dimensional CT
sutures (arrows). Due to lack of underlying brain growth, characteristic calva- reconstruction of vertex view confirms that
rial deformity Is not present. flattened segment (white arrow) lies
1996.166:697-703.

between left coronal suture (straight black

arrows) and metopic suture (curved black
arrow) and does not traverse either suture.
Not all calvarial deformities are the result of
craniosynostosis.

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American Journal of Roentgenology

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2. Vannier M, Hildebolt C, Marsh J, et al. Craniosynostosis: cess of surgical correction of craniosynostosis. J Neurosurg i990;72:
diagnostic value 22-26
ofthree-dimensional CT reconstruction. Radiology 1989;173:669-673 7. Delashaw J, Persing J, Jane J. Cranial deformation in craniosynostosis: a
3. Fernbach 5, Feinstein K. Radiologicevaluationof the child with cranio- new explanation. Neurosurg Clin NAm 1991 2:611-620
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