Definition
Craniostenosis is when premature synostosis of cranial
bone sutures occurs, normal head growth is inhibited. Various
characteristic anomalies of the head develop as a result, and in
extreme cases the development of the brain and sensory organs is
hindered
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Radiographic Appearance
The cranium radiograph, (6), shows rivela microcephaly
with acro-brachicephaly; accentuation of the digited impressions;
absence or low development of frontal and mastoid sinus; maxillary
hypoplasia with relative mandibular prognathism and and
temporo-mandibular joint ankylosis.
In the rachid occasionally we can find vertebral anomalies, while it
is frequent to find short clavicula with ipoplasic extremity.
The basin presents small iliac wings and big ischium, with valgus
coxa.
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Pathology
The skull consists of five thin, curved, bony plates that meet along
lines called sutures. At birth, the bony plates of the skull are not
completely joined along the sutures. This allows the baby's head and
brain to grow and develop after birth. Usually a baby's brain and
skull double in size in the first 6 months of life and again by age
2.1 After age 2, the sutures begin to close so that the bones can
join together (fuse). The skull and brain continue to grow
throughout early life but at a much slower rate.
Craniosynostosis (craniostenosis) is a rare condition in which the
skull sutures close and the bony plates of the skull become fused
too early in life. This causes a baby to have an abnormally shaped
head. Craniosynostosis usually develops while the fetus is still in
the mother's womb and often is evident at birth or soon after. It
develops in about 1 in every 2,000 births.2
Having less room for the baby's brain to grow and develop can also
cause increased pressure on the brain. If untreated, the increased
pressure can lead to brain injury. This may cause developmental
delays, seizures, blindness, and other problems. Fortunately, the
majority of children with craniosynostosis do not have these
problems. The effects of craniosynostosis depend on how many sutures
are affected and whether the problem develops as part of a genetic
disorder or other condition.The cause of craniosynostosis cannot
always be determined. However, 10% to 20% of babies with
craniosynostosis develop it as part of a genetic syndrome. The genes
responsible for this syndrome may come from one or both parents.
Other factors have been associated with craniosynostosis, including
having a mother who smokes or lives in a high altitude during
pregnancy.The condition has also been associated with a fetus lying
in a breech position in the womb or a baby being a twin.
Craniosynostosis tends to run in families. If one parent
and one child have craniosynostosis, the chance that another child
will develop it is about 50%. If two children in the same family
have craniosynostosis, the chance that another child will develop
the condition is about 25%.
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Treatment:
Should surgery prove to be necessary, it is usually performed
between the 4th and the 7th month after birth. The cranial bone is
reshaped to enable uninhibited further growth of the head and a
normal development of the brain and sensory organs. |
Images
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Radiographic Report for the above three images
Skull x-rays show complete fusion of the coronal sutures
bilaterally
with a very abnormal appearance of the frontal region as a result.
The orbits are distorted in appearance also as a result of the
coronal sutural fusion. The lateral view shows at least partial
fusion of the lambdoid sutures also. The AP view shows some
patency of the sagittal suture over the vertex but I suspect
there may be some posterior fusion of this suture also. Referral
to the specialist surgical centre is recommended.
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Useful Link: http://www.danhosp.org
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