Yıl 2014, Cilt 31 , Sayı 3, Sayfalar 143 - 148 2014-09-08

A rare clinical presentation of Axenfeld-Rieger syndrome: case report
Nadir görülen Axenfeld-Rieger sendromu: olgu bildirimi

İlkay PEKER [1] , Arzu Zeynep YILDIRIM BİÇER [2] , Erdal BOZKAYA [3] , Meryem Toraman ALKURT [4] , Elif SADIK [5] , Güven KAYAOĞLU [6]


INTRODUCTION: Axenfeld-Rieger syndrome (ARS) is a rare autosomal dominant disorder. It is characterized mainly by anterior segment abnormalities of the eye, and also comprises non-ocular defects including dental, craniofacial and systemic abnormalities. The purpose of this case report was to present clinic and radiographic findings and treatment approach in a patient with ARS.

CASE REPORT: A 21-year-old woman referred to our clinic with complaints of chewing difficulties, esthetic and speech problems. The medical anamnesis revealed several ocular disorders including glaucoma, cataract and strabismus, and the patient had been using eye-glasses since she was six-months-old. The dental history revealed no tooth extraction. In the extraoral examination, hypertelorism, malar hypoplasia, broad nasal bridge, short philtrum and mandibular retrognathia were observed. Intraoral examination disclosed the presence of an anterior open-bite, an Angle Class II malocclusion and four primary and seventeen permanent teeth. Presence of root anomalies (dilaceration, taurodontism, short roots), caries and a partially-erupted mandibular third molar were observed in radiographic examination. Cephalometric measurements suggested the presence of a variety of craniofacial abnormalities and hypoplasia in the maxilla and the mandible. A multidisciplinary operative approach comprising surgical, endodontic, restorative and prosthetic means were implemented for the oral rehabilitation of the patient.

CONCLUSION: The case reported here was a rare presentation of ARS with unique craniofacial features including mandibular hypoplasia and Angle Class II malocclusion. The oral rehabilitation of these patients necessitates a multidisciplinary clinical work.

TANITIM: Axenfeld-Rieger sendromu (ARS), otozomal dominant geçişli, nadir görülen bir hastalıktır. Esas olarak gözün ön bölgesini etkileyen anomalilerle karakterizedir, aynı zamanda dental, sistemik ve kafa-yüz anomalilerini kapsayan göz dışında defektler de izlenmektedir. Bu olgu bildiriminin amacı, ARS’li bir hastadaki klinik, radyografik bulguları ve aynı zamanda tedavi yaklaşımlarını sunmaktır.OLGU BİLDİRİMİ: Yirmibir yaşında kadın hasta estetik problemler, yemek yeme ve konuşma sırasında meydana gelen güçlük şikayetleriyle kliniğimize başvurdu. Medikal anamnezinde hastanın glokom, katarakt ve strabismus gibi bazı göz hastalıkları olduğu ve altı aylıktan itibaren gözlük kullandığı öğrenildi. Dental anamnezinde hiç diş çekimi yaptırmadığı anlaşıldı. Ekstraoral muayenede hipertelorizm, malar hipoplazi, geniş burun köprüsü, kısa filtrum ve mandibular retrognati izlendi. İntraoral muayenede ise ön açık kapanış, Angle Sınıf II maloklüzyon, dört adet süt dişi ve onyedi adet daimi diş mevcuttu. Radyografik incelemede bazı kök anomalileri (dilaserasyon, taurodontizm, kısa kökler) çürük ve yarı sürmüş mandibuler üçüncü molar diş izlendi. Sefalometrik ölçümler hastada çeşitli kafa-yüz anomalileri ile maksilla ve mandibulada hipoplazisi olduğunu gösterdi. Hastaya cerrahi, endodontik, restoratif ve protetik tedaviyi kapsayan multidisipliner tedavi uygulandı.SONUÇ: Burada bildirilen hasta, mandibula hipoplazisi ve iskeletsel Angle Sınıf II maloklüzyonu içeren farklı yüz özellikleri nedeniyle nadir görülen bir ARS olgusudur. Bu hastaların ağız rehabilitasyonu multidisipliner bir klinik yaklaşım gerektirmektedir.
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Birincil Dil en
Konular Sağlık Bilimleri ve Hizmetleri
Bölüm Olgu Bildirimi
Yazarlar

Yazar: İlkay PEKER

Yazar: Arzu Zeynep YILDIRIM BİÇER

Yazar: Erdal BOZKAYA

Yazar: Meryem Toraman ALKURT

Yazar: Elif SADIK

Yazar: Güven KAYAOĞLU

Tarihler

Yayımlanma Tarihi : 8 Eylül 2014

Bibtex @olgu sunumu { gaziaot86638, journal = {Acta Odontologica Turcica}, issn = {}, eissn = {2147-690X}, address = {}, publisher = {Gazi Üniversitesi}, year = {2014}, volume = {31}, pages = {143 - 148}, doi = {10.17214/aot.12475}, title = {A rare clinical presentation of Axenfeld-Rieger syndrome: case report}, key = {cite}, author = {Peker, İlkay and Yıldırım Biçer, Arzu Zeynep and Bozkaya, Erdal and Alkurt, Meryem Toraman and Sadık, Elif and Kayaoğlu, Güven} }
APA Peker, İ , Yıldırım Biçer, A , Bozkaya, E , Alkurt, M , Sadık, E , Kayaoğlu, G . (2014). A rare clinical presentation of Axenfeld-Rieger syndrome: case report . Acta Odontologica Turcica , 31 (3) , 143-148 . DOI: 10.17214/aot.12475
MLA Peker, İ , Yıldırım Biçer, A , Bozkaya, E , Alkurt, M , Sadık, E , Kayaoğlu, G . "A rare clinical presentation of Axenfeld-Rieger syndrome: case report" . Acta Odontologica Turcica 31 (2014 ): 143-148 <https://dergipark.org.tr/tr/pub/gaziaot/issue/6534/86638>
Chicago Peker, İ , Yıldırım Biçer, A , Bozkaya, E , Alkurt, M , Sadık, E , Kayaoğlu, G . "A rare clinical presentation of Axenfeld-Rieger syndrome: case report". Acta Odontologica Turcica 31 (2014 ): 143-148
RIS TY - JOUR T1 - A rare clinical presentation of Axenfeld-Rieger syndrome: case report AU - İlkay Peker , Arzu Zeynep Yıldırım Biçer , Erdal Bozkaya , Meryem Toraman Alkurt , Elif Sadık , Güven Kayaoğlu Y1 - 2014 PY - 2014 N1 - doi: 10.17214/aot.12475 DO - 10.17214/aot.12475 T2 - Acta Odontologica Turcica JF - Journal JO - JOR SP - 143 EP - 148 VL - 31 IS - 3 SN - -2147-690X M3 - doi: 10.17214/aot.12475 UR - https://doi.org/10.17214/aot.12475 Y2 - 2013 ER -
EndNote %0 Acta Odontologica Turcica A rare clinical presentation of Axenfeld-Rieger syndrome: case report %A İlkay Peker , Arzu Zeynep Yıldırım Biçer , Erdal Bozkaya , Meryem Toraman Alkurt , Elif Sadık , Güven Kayaoğlu %T A rare clinical presentation of Axenfeld-Rieger syndrome: case report %D 2014 %J Acta Odontologica Turcica %P -2147-690X %V 31 %N 3 %R doi: 10.17214/aot.12475 %U 10.17214/aot.12475
ISNAD Peker, İlkay , Yıldırım Biçer, Arzu Zeynep , Bozkaya, Erdal , Alkurt, Meryem Toraman , Sadık, Elif , Kayaoğlu, Güven . "A rare clinical presentation of Axenfeld-Rieger syndrome: case report". Acta Odontologica Turcica 31 / 3 (Eylül 2014): 143-148 . https://doi.org/10.17214/aot.12475
AMA Peker İ , Yıldırım Biçer A , Bozkaya E , Alkurt M , Sadık E , Kayaoğlu G . A rare clinical presentation of Axenfeld-Rieger syndrome: case report. Acta Odontol Turc. 2014; 31(3): 143-148.
Vancouver Peker İ , Yıldırım Biçer A , Bozkaya E , Alkurt M , Sadık E , Kayaoğlu G . A rare clinical presentation of Axenfeld-Rieger syndrome: case report. Acta Odontologica Turcica. 2014; 31(3): 143-148.
IEEE İ. Peker , A. Yıldırım Biçer , E. Bozkaya , M. Alkurt , E. Sadık ve G. Kayaoğlu , "A rare clinical presentation of Axenfeld-Rieger syndrome: case report", Acta Odontologica Turcica, c. 31, sayı. 3, ss. 143-148, Eyl. 2014, doi:10.17214/aot.12475