Black Sea Journal of Health Science

Yazarlar: Ayşe Feyda NURSAL, Kemal TÜMER, Serbülent YİĞİT, Akın TEKCAN

Konular:Tıp

Anahtar Kelimeler:Apert's syndrome,Dental anomalies,Orthodontic treatment

Özet: Viral Apert's syndrome (AS) is a rare congenital disorder with autosomal dominant inheritance and is characterized by irregular craniosynostosis, syndactylia of hands and feet, mid-face hypoplasia, hypertelorism and anomalies of central nervous system, heart and kidneys. AS has been associated with mutations in Fibroblast growth factor receptor 2 (FGFR2) located on chromosome 10q (10q26). Dental anomalies are common in AS. We report on a 6-year-old AS patient with complex dental anomalies. A 6 year-old female patient with AS was presented to the dental clinic with complaints of teeth decay and embedded teeth. She had dysmorphic facial symptoms including mid-face hypoplasia, low-set ears, hypertelorism, prognathic mandible, steep wide forehead, down-slanting lateral canthi and palpebral fissures. She had syndactyly of third and fourth digits of both hands. Arachnoidal cyst was diagnosed previously. She had intellectual disability. Radiography showed that there were more than one embedded teeth. Upper first premolar and canine teeth were displaced. She had teeth agenesis of the maxillary lateral incisor. Her maxilla and mandible were narrow. The maxillary dental arch was v-shaped. Orthodontic treatment was planned for the future because the patient was too young. The aim of the present report is to show the dental manifestations in case with AS. The treatment and management of AS require a multidisciplinary approach.

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