Öz
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.
Anahtar Kelimeler
Kaynakça
- Bhatia PV, Patel PS, Jani YV, Soni NC. 2013. Apert's syndrome: Report of a rare case. J Oral Maxillofac Pathol. 17: 294-297.
- Cohen MM Jr, Kreiborg S, Lammer EJ, Cordero JF, Mastroiacovo P, Erickson JD, Roeper P, Martínez-Frías ML. 1992. Birth prevalence study of Apert syndrome. Am J Med Genet. 42: 655–659.
- Cohen MM Jr, Kreiborg S. 1990. The central nervous system in the Apert syndrome. Am J Med Genet, 35: 36-45.
- Fadda MT, Ierardo G, Ladniak B, Di Giorgio G, Caporlingua A, Raponi I, Silvestri A. 2015. Treatment timing and multidisciplinary approach in Apert syndrome. Ann Stomatol (Roma), 6: 58-63.
- Gupta M, Pai AA, Bhattacharya A, Ramachandra R, Sawarappa R, Mohapatra S, Kanoi A. 2013. Anterior plagiocephaly in an atypical case of apert syndrome. World J Plast Surg, 2: 115-118.
- Ibarra-Arce A, Ortiz de Zárate-Alarcón G, Flores-Peña LG, Martínez-Hernández F, Romero-Valdovinos M, Olivo-Díaz A. 2015. Mutations in the FGFR2 gene in Mexican patients with Apert syndrome. Genet Mol Res, 14: 2341-2346.
- Kreiborg S, Cohen MM Jr. 1992. The oral manifestations of Apert syndrome. J Craniofac Genet Dev Biol, 12: 41-48.
- Kumar GR, Jyothsna M, Ahmed SB, Sree Lakshmi KR. 2014. Apert's Syndrome. Int J Clin Pediatr Dent, 7: 69-72.
Öz
Kaynakça
- Bhatia PV, Patel PS, Jani YV, Soni NC. 2013. Apert's syndrome: Report of a rare case. J Oral Maxillofac Pathol. 17: 294-297.
- Cohen MM Jr, Kreiborg S, Lammer EJ, Cordero JF, Mastroiacovo P, Erickson JD, Roeper P, Martínez-Frías ML. 1992. Birth prevalence study of Apert syndrome. Am J Med Genet. 42: 655–659.
- Cohen MM Jr, Kreiborg S. 1990. The central nervous system in the Apert syndrome. Am J Med Genet, 35: 36-45.
- Fadda MT, Ierardo G, Ladniak B, Di Giorgio G, Caporlingua A, Raponi I, Silvestri A. 2015. Treatment timing and multidisciplinary approach in Apert syndrome. Ann Stomatol (Roma), 6: 58-63.
- Gupta M, Pai AA, Bhattacharya A, Ramachandra R, Sawarappa R, Mohapatra S, Kanoi A. 2013. Anterior plagiocephaly in an atypical case of apert syndrome. World J Plast Surg, 2: 115-118.
- Ibarra-Arce A, Ortiz de Zárate-Alarcón G, Flores-Peña LG, Martínez-Hernández F, Romero-Valdovinos M, Olivo-Díaz A. 2015. Mutations in the FGFR2 gene in Mexican patients with Apert syndrome. Genet Mol Res, 14: 2341-2346.
- Kreiborg S, Cohen MM Jr. 1992. The oral manifestations of Apert syndrome. J Craniofac Genet Dev Biol, 12: 41-48.
- Kumar GR, Jyothsna M, Ahmed SB, Sree Lakshmi KR. 2014. Apert's Syndrome. Int J Clin Pediatr Dent, 7: 69-72.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Klinik Tıp Bilimleri |
| Bölüm | Olgu Sunumu |
| Yazarlar | |
| Yayımlanma Tarihi | 1 Ocak 2018 |
| Gönderilme Tarihi | 1 Ocak 2018 |
| Yayımlandığı Sayı | Yıl 2018 Cilt: 1 Sayı: 1 |
