• Journal of the korean academy of Pediatric Dentistry
• /
• v.29 no.1
• /
• pp.44-50
• /
• 2002

Apert syndrome is a kind of congenital-acrocephalosyndactyly syndrome which was first reported by Apert in 1906 and characterized by its acrocephaly and syndactyly. Clinical characteristic features are cone-shaped skull morphology due to early fusion of coronal suture, fusion of fingers of hands and toes of feet. It is an autosomal dominant-heritable syndrome. Due to hypo-development of midface region, Apert syndrome patients have a tendency to have ocular proptosis, hypertelorism, maxillary deficiency. High palate and soft palate cleft are common findings in these patients. In general, mandibular growth pattern is normal, but relative maxillary deficiency exaggerates mandibular forward position, so relative mandibular prognathism is inevitable. Narrow maxillary and mandibular dental arch worsen teeth alignment and crowding. Skeletal malocclusion and open bite are also common. This is a case report of a Korean 3 year 1 month male Apert syndrome child referred by department of plastic surgeon for the possibility of orthodontic treatment. General features of Apert syndrome, patient's medical history, radiographic evaluation, clinical examination, orthodontic and surgical treatment planning are discussed in this report.