Crouzon's syndrome
 

The patient has dysmorphic features with prominent forehead, maxillary hypoplasia, a hooked nose and prognathism  (the lower jaw shows forward protrusion). The eyes are proptosed with shallow orbits. There may be divergent strabismus. Mention that you like to examine: the optic nerve function by examining the fundi for optic atrophy

Questions:

1. What is the cause of Crouzon's syndrome?

Answer
It is a form of craniosynthosis caused by premature closure of the cranial fissures.

2. What ocular features may occur with craniosyntosis syndrome?

Answer
visual failure:    this is commonly caused by optic atrophy. This may result from intracranial hypertension, abnormal skull and brain development or narrow optic canal.
proptosis:       this is caused by shallow orbits and maxillary hypoplasia. The lower sclera is often visible due to a lowered and relatively posterior origin of the lower lid.
strabismus:    this can be caused by the divergent line of the orbital axes, short anterioposterior orbital dimensions and hypertelorism
hypertelorism:    this is caused by wide separation of the orbits.

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