| Craniosynostosis
is a congenital or birth defect, characterized by the premature
closure of one or more sutures, which causes an abnormally shaped
skull. The skull is normally composed of bones which are separated
by sutures. As an infant’s brain grows, open sutures allow
them to expand and develop a relatively normal head shape. When
one or more sutures close early, it can cause the skull to expand
in the direction of the open sutures. This often results in an abnormal
head shape and in severe cases, places excessive and damaging amounts
of pressure on a growing brain.
There are different types of craniosynostosis. The most common is
scaphocephaly in which the sagittal suture is closed.
This particular form of craniosynostosis causes the head to grow
long and narrow to accommodate the growing brain. It occurs in
approximately 1 in 2000 births. In addition, males are affected
about three times as often as females.
Another type of craniosynostosis is metopic
hypostasis. It typically manifests itself as a
visible ridge down the child's forehead and the eyebrows seem to
be pinched on either side. The eyes may also appear to be close
together.
A third form of craniosynostosis, known as
unilateral coronal synostosis, is the one which
affected our son, Jorge Luis. This type affects the coronal suture
which extends from ear to ear on the top of the head. Early closure
of one side results in the forehead and orbital rim having a flat
appearance on that side. The other side is pushed forward (sort
of like a bump in the forehead),and also one eyebrow is higher than
the other one.
Finally, when both sides are fused it is called bicorporal hypostasis.
In these cases, the child may have a very flat and recessed forehead.
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The cause of craniosynostosis is unknown.
It can be hereditary and caused by a genetic defect, or it can occur
sporadically in a family with no other affected relatives, like
our case with Jorge Luis.
In order to be able to diagnose this condition it is necessary to
measure the circumference of the infant’s head. If deformities
are noted, the doctor should order x-rays and confirm the diagnosis
by a physical evaluation. In addition, a CT scan should also be
performed to ensure that there are no underlying abnormalities in
the brain.
The primary treatment for craniosynostosis during
infancy is surgery. There are three goals for the surgery. First,
relieve intracranial pressure. Second, assure that the skull has
the capacity to accommodate the brain’s growth. Third, attempt
to cosmetically improve the appearance of the child’s head.
While the deformity may vary from mild to severe, the psychological
effect of a noticeably misshapen forehead, face and head can cause
great problems and is a very important reason for seeking treatment.
Disclaimer:
The preceding information should not be viewed as
advise on the treatment and/or diagnosis of craniosynostosis or
any other medical condition. Advice on the treatment or care of
a child suffering from craniosynostosis should be obtained only
through consultation with a physician who has examined that child
or is familiar with that child's medical history.
National Institute of Neurological Disorders
Children's Hospital at Montefiore
Children's Craniofacial Association (PDF Document)
Foundation for Faces of Children
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