Crouzon Syndrome

Represents approximately 4.8% of cases of craniosynostosis at birth.

Birth prevalence of 1.6:100,000 births.

Estimated prevalence in general population of Europe is 0.9:100,000.

No race predilection.

Difficult BMV, difficult intubation, massive blood loss, arterial gas embolism

Difficult airway

Intraop blood loss

Inadvertent dural sinus injury

Postextubation subglottic edema

External facial fixation devices

Crouzon syndrome is an autosomal dominant disorder characterized by craniosynostosis causing secondary alterations of the facial bones and facial structure.

Common features include hypertelorism, exophthalmos and external strabismus, parrot beak nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism.

Synonyms: Craniofacial dysostosis type II, FGFR deficiency.

Due to mutation in FGFR2 gene on chromosome 10.

Normal function of FGFRs is to restrain limb growth. FGFR mutations are hypermorphic, causing excessive cranial bone formation.

Inherited in autosomal dominant fashion, but de novo mutations account for 50% of cases.

High penetrance but variable expressivity.

Male to female preponderance of 3:1.