CRANIOSYNOSTOSIS PRIMER: GAMBARAN RADIOLOGI

In 1851 , Virchow Introduced the term craniosynostosis to describe a variety of

abnormalities in calvarial growth.1 These skull deformities are usually apparent in
infancy. When an abnormal calvarial configuration is detected, a radlologic
evaluation is necessary to characterize the deformity and to guide the corrective
surgical procedure. Affected children are believed to have an improved outcome
when diagnosis and surgical intervention occur at an early age. CT with
threedimensional reconstruction optimally evaluates the presence and degree of
sutural involvement and assesses associated facial and intracranial abnormalities.2
This pictorial essay illustrates the Imaging findings, nomenclature, and
associatedabnormalities of the various types of primary craniosynostosis.

Classification
Craniosynostosis can occur as a primary abnormality, as part of a known syndrome,
or in association with a host of systemic conditions. Primary craniosynostosis is
classified according to the affected calvanial suture(s) (Table 1).

Radiologic Findings
Primary signs of craniosynostosis include bony bridging along the suture, heaping up
of bone at the suture, sutural narrowing, and indistinctness of the suture. These
changes may occur over a 1-2-mm segment of suture, or there may be a fibrous union
in which no primary nadiologic changes are noted despite marked secondary signs.
Secondary signs include an altered calvarial shape, changes in shape and timing of
closure of fontanels, and facial anomalies.3,4 The lack of growth across a suture often
results in effacement of the underlying subarachnoid spaces, 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 prominent, 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 calvanial shape with
relation to a calvarial suture. The presence of secondary radiographic signs across a
suture without pnimany signs should not delay the diagnosis or surgical conrection.
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
an older age.6 The rate of brain growth in older children is reduced and may not be
adequate to promote a normal calvarial contour after surgery.7

Sagittal Synostosis
Sagittal synostosis is the most common type of craniosynostosis, 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 predicted by compensatory growth patterns along the
metopic, coronal, and Iambdoidal sutures.7

Figure 1. Dolichocephaly (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 Involvement of posterior portion of sagittal 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
patent lambdoid 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 algorlthm.
E. Three-dimensional CT reconstruction of lateral view best shows dolichocephalic configuration. A =
anterior, P = posterior.

Metopic Synostosis
Patients with this form of craniosynostosis have a keelshaped (triangular) frontal
deformity known as tnigonocephaly (Fig. 2). Symmetrical bone growth occurs at the
sagittal suture with asymmetric bone growth at the coronal sutures, producing the
characteristic pear shape with flattened frontal eminences.7 Associated radiographic
signs include hypotelorism (intercanthal distance less than 15 mm in infants less than
1 year old), hypopiastic ethmoid sinuses, and anterior bowing of the coronal
sutures.3,4

Figure 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
metopic suture ridging (open arrows).

Unilateral Coronal Synostosis

Patients with this type of synostosis have flattening of the frontal bone, uplifting of
the ipsilateral eyebrow, ipsilatenal exophthalmos, and a palpable suture.3 The
radiographic findings include plagiocephaly (flattening) over the affected suture with
contralateral frontal and panietal expansion and ipsilateral temporal expansion (Fig.
3).7 In our experience, this is often associated with synostosis of the contralateral
lambdoidal suture. Additional radiographic findings include a shallow anterior cranial
fossa, shallow orbits, uplifting of the orbital roof (the harlequin eye deformity), and
depression of the petrous bone.3

Figure 3. Unilateral coronal synostosis and plagiocephaly.

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
plagiocephaly (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).
D. 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 associated 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 squamosal 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.

Figure 4. Bilateral coronal synostosis and bilateral frontal bone flattening.

A. Illustration of child with bilateral coronal synostosis and bilateral frontal bone flattening. (Reprinted
with permission from (1])
B. Axial CT image in 9-month-old child shows sclerosis and heaping up of bone at both coronal
sutures (open arrows). Bilateral frontal flattening results In triangular configuration of anterior portion
of calvarlum. This occurs above level of metopic suture and should not be confusedwith
trigonocephaly.
C. Axial CT image in same child shows shallow orbits and exophthalmos (curved arrow and dotted
line) are present bilaterally.
D. Three-dimensional CT reconstruction of lateral view shows shortening of calvarium in
anteroposterior dimension resulting in brachycephaly. Because of smoothing function of reconstruction
algorithm, neither coronal nor lambdoid 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.

Unilateral Lambdoidal Synostosis

Patients with this abnormality have flattening of the occipital bone known as
plagiocephaly (Fig. 5). No facial abnor malities are seen.4 Compensatory bone growth
occurs at the ipsilateral squamosal, contralateral Iambdoidal, and sagittal sutures,
resulting in an ipsilateral temporal bulge with depression of the pinna and
contralateral panieto-occipital expansion.7 Synostosis of the Iambdoidal suture may
produce only a mild deformity owing to the small amount of bone growth that occurs
along this suture.7

Figure 5. month-old child with right lambdoidal plagiocephaly.

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 parletooccipital plagiocephaly (open arrows) with right lambdoid
suture narrowing. There is compensatory right temporal bulging (arrowheads) and centralateral
parleto-occipital expansion (solid arrow).
C. Three-dimensional CT reconstruction of vertex view shows right lambdold plagiocephaly (open
arrow) and contralateral parletooccipital expansion (solid arrow).
D. Three-dimensional CT reconstruction of angled frontal-basal view accentuates right parieto-
occipital piagiocephaly (open arrows), ipsilateral temporal bulge (arrowheads), and contralateral
parleto-occipital expansion (solid arrow).

Bilateral Lambdoidal Synostosis

This is the least common type of craniosynostosis.4 Patients present with
brachycephaly (Fig. 6). Radiographic findings include flattening across the occiput
and a shallow posterior fossa.4 Enhanced bone growth occurs along both squamosal
sutures, resulting in a bilateral temporal prominence with anterior and inferior
displacement of the pinnae. Bony expansion on both sides of the sagittal suture
elevates the vertex.7

Figure 6. Brachycephaly.
A. Axial CT Image of 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.

Not all cases of craniosynostosis will have a calvanial 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). Not all pediatric calvarial
deformities are due to craniosynostosis. Calvarial asymmetry may result from the
position of the fetus in utero or of the neonate in the crib (Fig. 8). The flattening of
the calvania must cross a suture for the diagnosis of craniosynostosis to be made.
Figure 7. 1-year-old child with microcephaly and secondary synostosis of all major cranial sutures.
A. Axial CT image shows diffuse cerebral atrophy.
B. Image shows bony ridging and sclerosis of both lambdoid and coronal sutures (arrows). Due to lack
of underlying brain growth, characteristic calvarial deformity is not present.

Figure 8. 9-month-old child with clinically apparent calvarial deformity, presumably due to persistent
positioning of child on his left side in crib. Three-dimensional CT reconstruction of vertex view
confirms that flattened segment (white arrow) lies 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.