Crouzon Sydrome: Physical Traits
The Eyes and Midface:
Children with Crouzon syndrome seem to have big eyes. In fact, the eyeballs
are a normal size. The problem is that the bones surrounding the eyes
(the orbits) cannot grow forward in a normal way. In addition, the eyelid
openings usually slant downwards. Some children's eyes can "bulge" so
far forward (this condition is called proptosis, or exophthalmos) that
the cornea of the eye can become exposed. Occasionally, when children
with severe proptosis cry, their eyelids can get temporarily caught behind
the eyeball. This condition obviously requires immediate treatment either
with a tarsorrhaphy (sewing the outside part of the upper and lower eyelids
together) or by surgically moving the bones of the orbits forward (this
is usually done at the same time that the forehead is brought forward).
The eye problem most frequently found is an imbalance of the muscles
that move the eye (called "strabismus"). Some children with Crouzon syndrome
may require eye muscle balancing surgery. If this is not done, children
learn to stop seeing out of one eye ("amblyoplia") in order to prevent
double vision. Some children with Crouzon syndrome may also develop optic
nerve atrophy (weakening, or wasting), which can diminish a child's ability
to see. It is not clear what causes this optic nerve atrophy, but it may
be related to chronically elevated intracranial pressure.
The mid face is the part of the face that extends from below the eyes
down to the upper teeth. The midface is always small (also called "hypoplastic",
meaning lack of growth) in Crouzon syndrome. The deepest part of the face
is usually centered at the top of a short nose. Because the midface cannot
grow forward very well, it is usually surgically moved forward a couple
of times over the child's lifetime (see the
section). From measurements taken from our center in Dallas, it appears
that the face in children with Crouzon syndrome grows only about 1/3 normal
speed (depending on the severity of Crouzon syndrome), and there is additional
evidence to suggest that all forward growth stops about age 9 (see
[ ]).
Other Crouzon Syndrome Physical
Traits:
Skull |
Brain |
Ears |
Mouth
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