Apert syndrome: review and report a case.

Apert’s syndrome is an autosomal dominant inherited disease characterized mainly by achrocephalia due to synostosis of the coronary suture and by usually symmetrical syndactyly involving the four extremities. In most cases the disorder results from a mutation in the father; its prevalence at birth is 1:65,0003 and the incidence in Asians is high.4 Mental retardation is common. The literature shows that this syndrome is caused by one of two mutations in the receptor 2 of the growth factor gene (FGFR2) involving two adjacent aminoacids. Treatment of these patients is done by multidisciplinary teams. Planning of surgery should be done in stages: craniotomy aims to decompress the brain and is done in infancy; advancement of the middle third improves airway-nasal flow, and may be done in puberty; and finally, orthognathic surgery improves occlusion and dental esthetics, and may be done in adolescence.