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Crouzon Syndrome

Terms in this set (10)

Crouzon Definition
Genetic disorder characterized by the premature fusion of certain skull bones (craniosynostosis).
Crouzon Alternate Names
-Craniofacial dysarthrosis
-Craniofacial dysostosis
-CFD1
Crouzon Features
-Autosomal dominant genetic disorder that affects the first brachial arch, which is the precursor of the maxilla and mandible.
Crouzon Etiology
-due to the mutation of the fibroblast growth factor receptor 2 (FGFR2)
-associated with increased paternal age.
Crouzon Clinical Features
-Brachycephaly
-Scaphocephaly
-Trignocephaly
-Ocular proptosis
-Blindenss/Deafness
-Higher incidence of headache
-Beaten Copper appearance (radiographic)
Crouzon Oral Manifestation
-Midface hypoplasia
-Crowding of maxillary teeth and malocclusion
-Lateral Palatal Swellings (pseudo cleft)
Crouzon Demographics
Most common craniosyntosis syndrome. 16 per million newborn.

1 in 65,000
Crouzon Treatment
Surgery - early craniotomy
Crouzon DDX
Apert
Achrondroplasia
Jackson-Weiss Syndrome
Crouzon
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