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Anatomy

The bones of the skull are the frontal, parietal, temporal,


occipital, and sphenoid bones
The place where two bones come together is called a suture
At birth, the adjacent bones override each other to allow the
infant to pass through the birth canal.
As the child grows the sutures allow for skull expansion to
accommodate the growing brain.
The brain doubles in size by age 6 months and again at age
2 years. These sutures normally begin to fuse around 2 years
of age along with the closure of the fontanel (soft spot)
Anatomy
The seam where two skull bones fuse together is called
a suture. The major sutures are called sagittal, coronal,
metopic and lambdoid
Pathology
Craniosynostosis occurs when one or more of the major
sutures close early
The cause is unknown
It is called 'primary' when not associated with any other
problem. When there is an underlying disorder it is
considered 'secondary
heali
It may be "simple" with only one suture closed or
"compound" when multiple sutures are involved
Pathology
Craniosynostosis occurs when one or more of the major sutures
close early
The cause is unknown
It is called 'primary' when not associated with any other problem.
When there is an underlying disorder it is considered 'secondary
Healing rickets and hypophosphatasia
Idiopathic hyperclcemia
Anemias with diploe involvement
Hyperthyroidism
Brain atrophy
After successful shunting for hydrocephalus
Pathology
Features
Sutural narrowing
Sharpening of the edges
Sclerosis and ridging along the margins of the suture
Bony bridging across the suture line

Three-Dimensional Study: Gold standard


Ultrasound: Preliminary study
Sagittal Craniosynostosis
Most common
Creates an elongated
narrow skull called
'scaphocephaly' or
'dolicocephaly
The compensatory
change causes the front
(frontal) and back
(occipital) areas of the
skull to be pushed
outward causing bossing
(bulging of the skull)
Coronal Craniosynostosis
Second most common
Caused by early closure of the
coronal suture
Produces a calvarium that is short
from front to back and wide from
side to side, called bradycephaly or
brachycephaly
Bregma is somewhat high in
position, and calvarium is wide in
frontal projection
Marked uptilting of the sphenoid
wings
Harlequin appearance of the orbits
Coronal Craniosynostosis
Frontospenoid synchondrosis is also probably involved
Often the primary deformity in Crouzons disease and
Aperts syndrome
Craniofacial dysostosis
Unilateral Coronal Synostosis
Features
Flattening of the ipsilateral frontoparietal region
Elevation of the ipsilateral sphenoid wing
Unilateral harlequin appearance of the involved orbit
Coronal suture on the involved side shows the usual shape
of premature closure
Narrowness of the suture line
Sharpening of the sutural edges
Sclerosis along the bony margins
Skewed Nasal Septal Sign nasal septum is obliquely tilted
Lambdoid Craniosynostosis
Isolated synostosis of the
lambdoid sutures is less common
When present,
Marked flattening and
underdevelopment of the lower
portion of the posterior fossa
Severe overgrowth of the bregma
Oxycpehalic or turricephalic skull
Metopic Craniosynostosis
Forehead becomes pointed
Trigonocephaly
Mixed Craniosynostosis
Cloverleaf Skull most grotesque deformity
Prominent bulging of the temporal region is see
Universal Craniosynostosis
All sutures can close prematurely
Severe microcephaly is seen
Markedly increased inner table convolutions and other
signs of chronically increase intracranial pressure (vs.
brain atrophy)
Summary
Name of Skull Deformity from Craniosynostosis
Suture(s) Fused Early Type of Skull Deformity
Sagittal suture Scaphocephaly (most common)
One coronal suture Anterior plagiocephaly
Both coronal sutures Brachycephaly
One lambdoid suture Posterior plagiocephaly
Metopic suture Trigonocephaly
All skull sutures and basilar
Oxycephaly
skull sutures
Fusion of all sutures except Kleeblattschadel (Cloverleaf
metopic and squamosal skull)