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Definition: An infant's skull is made up of
several
free-floating bones connected by fibrous regions called sutures. These
flexible connections allow the
infant's head to pass through the birth canal and enable the skull to grow
in response to the
brain.
Craniosynostosis refers to the early (premature) fusion of the sutures
of the bones of the skull. Premature fusing of the sutures restricts
and distorts the growth of the skull. These cranial growth
restrictions often
results in increased cranial pressure growth which can cause vision
problems and impede intellectual development. In addition, abnormal
head shape is associated with low self-esteem and behavioral problems.
Correction of these deformities can relieve cranial pressure and in the
long-term, improve the child's self-esteem.
The child's head takes on a distinct form which depend on the fused
suture (or sutures):
- Premature fusion of the
coronal
sutures forces the skull to grow wide relative to its length (see
brachycephaly).
- Premature fusion of the
sagittal suture forces the skull to grow long relative to its width (see
scaphocephaly).
- Premature fusion of the
metopic
suture (metopic craniosynostosis, also know as trigonocephaly) produces a narrow, triangular forehead with lateral pinching of the temples.
(see ).
- Premature fusion of one of the
coronal or
lambdoid sutures
results in asymmetrical head shape with flattening (see
plagiocephaly).
- Premature fusion of both the coronal and sagittal
sutures (oxycephaly) results in a an abnormally high conical
head shape.
- Premature fusion of the coronal, lambdoid and
posterior sagittal sutures [a rare condition] results in a
cloverleaf skull (also known as kleeblattschadel).
Craniosynostosis
occurs in isolation but is also present in many
syndromes, such as Apert,
Crouzon,
Pfeiffer,
Saethre-Chotzen,
Carpenters
and Opitz-C (also known as
Opitz Trigonocephaly)
syndrome.
Incidence:
Diagnosis: A distorted head shape is not always the result of craniosynostosis. The diagnosis of
craniosynostosis begins with an examination by
a
pediatrician, pediatric neurosurgeon or craniofacial surgeon. The initial
examination involves questions about gestation and birth, in utero position, neck
tightness and post-natal positioning (for example, sleeping position). The physical examination
includes inspection of the infant's head and may involve palpation (carefully feeling)
of the child's skull for
suture ridges and soft
spots (the
fontanelles)
as well as checking for neck tightness and other deformities. The
physician may also request x-rays or computerized tomography (a CAT
scan, a series of photographic images of the skull). These images provide the most reliable method
for diagnosing premature suture fusion (craniosynostosis).
In addition, the physician may make (or order) a series of measurements
from the child's face and head [more on
cranial
anthropometry]. These measurements will be used to assess severity
and monitor treatment.
Treatment:
The treatment of
craniosynostosis requires
surgical correction. Please consult a pediatric neurosurgeon or a
craniofacial surgeon to discuss treatment procedures.
Support Groups:
Parent/Child Experiences
Related links:
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