2. Overview
Named after Octave Crouzon,
a French Neurologist who first described this disorder in 1912.
Octave Crouzon (1874-1938)
3. Epidemiology
Estimated birth prevalence ranges from 1 per 25,000 births to 1 per 67,000 births
Accounts for 5% of all cases of craniosynostosis
Almost 60% of cases are new mutations
Associated with paternal age > 35 years
5. Physical Findings
Tall, flattened forehead (secondary to bicoronal synostosis)
Brachycephaly (disproportionately wide head)
Exophthalmos (due to shallow eye sockets after early fusion of surrounding bones)
Hypertelorism (greater than normal distance between the eyes )
Maxillary hypoplasia (insufficient growth of the midface)
Beaked nose
Low-set ears
Ear canal malformations
7. Diagnosis
Physical exam, usually at birth
CT scan
Measuring intracranial pressure
FGFR mutational screening
8. Special considerations
Cervical spine abnormalities present in up to one-third of patients.
**must be thoroughly evaluated before surgical correction of Craniosynostosis.
9. Treatment
Surgery
- to prevent the closure of sutures of the skull from damaging the brain's development.
- should be done as early as possible for optimal results