Crouzon Sydrome: Physical Traits
Inside the brain are areas filled with cerebral spinal fluid, called ventricles.
One could think of the ventricles as little lakes, inside the head. Occasionally,
these ventricles become filled with too much fluid. Most all children
with Crouzon syndrome have ventricles that are larger than normal. Usually,
no treatment is required for this enlargement. However, sometimes these
ventricles get so large that they begin to "squish" the brain from the
inside. In this case, a pediatric neurosurgeon might recommend placing
a shunt, or tube, which goes from inside the ventricle and is tunneled
under the skin down to the abdominal cavity in order to drain off the
fluid. Typically, once a shunt is placed, the child requires one for the
rest of his/her life.
Another condition that may occur is called cerebellar tonsillar herniation,
or a Chiari malformation. This may occur in up to 70% of children affected
with Crouzon syndrome, and it is very important to screen for this. The
floor of the skull has a hole called the foramen magnum, through which
the spinal cord exits. Sometimes, part of the brain called the cerebellar
tonsils can get "pushed" down into this hole, like a cork in a wine bottle.
If this happens there can be reduced flow of cerebral spinal fluid, and
a child might develop a particular type of sleep apnea (this means that
the child doesn't get enough oxygen while asleep) called central apnea.
We believe that cerebellar tonsillar herniation should be checked for
at least once a year until the child is grown. Data from our center (see
[ ]) suggests that the development of this tonsillar herniation
might be related to raised intracranial pressure. We believe that this
sign may be an indication for advancing the forehead. Unfortunately, this
condition is difficult to assess on routine CT scans, and is best evaluated
with a specialized CT or an MRI. This should be discussed with either
your craniofacial surgeon or pediatric neurosurgeon.
Raised intracranial pressure is a concern for all Crouzon children until
teenage years. Children with Crouzon syndrome have bigger brains. As these
brains are growing, the skull is not able to keep up with this growth,
at least in part because of the fused coronal sutures. This condition
means that the skull needs to be enlarged with surgery (see treatment
below). It is very important that growing children be closely monitored
by experienced physicians to make sure that the intracranial pressure
is not getting too high. This monitoring is done a number of ways: having
a pediatric neuro-opthalmologist evaluate the optic discs, following head
circumference measurements, checking CT scans, and/or MRIs, looking for
signs such as headaches or vomiting, just to name a few.
Other Crouzon Syndrome Physical
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