Crouzon Syndrome

Significant craniofacial and/or oral features:
Lobed tongue
Hamartomas or lipomas of tongue
Cleft of hard or soft palate
Accessory gingival frenulae
Hypodontia
Ocular hypertelorism or telecanthus
Median cleft or pseudocleft upper lip
micrognathia

Etiology:
X-linked dominant genetic disorder and is mostly in females

Prevelene:
Estimates range from 1/50,000 to 1/250,000


Age of Diagnosis:
At birth in infants based on characteristic oral, facial, and digital anomalies, OR after polycystic kidney disease is identified in childhood

Factors that influence dental care or require dental intervention:
Treatment of cleft lip/palate, tongue nodules, accessory frenulae, removal of accessory teeth, and orthodontics for malocclusion
Speech therapy may be necessary


Associated systemic conditions:
Digital abnormalities
Brain abnormalities – intracerebral cysts, corpus callosum agenesis, cerebellar agenesis
Kidney – polycystic kidney disease
Mental retardation – usually mild
Skin and hair disorders such as alopecia