Mehmet Sinan DOĞAN, Osman ATAŞ, İzzet YAVUZ, Samet TEKİN

Ektodermal Displazili Olgularda Klinik ve Radyolojik Bulguların İncelenmesi

Amaç: Ektodermal displazi; deri, tırnak, saç, ter bezleri ve diş gibi ektoderm kaynaklı dokuları etkileyen ve nadir olarak rastlanan kalıtsal bir hastalıktır. Bu çalışmada fakülte hastanemize başvuran ektodermal displazili olgulardaki; sistemik ve dental bulguların klinik ve radyolojik olarak değerlendirilmesi amaçlanmıştır. Gereç ve Yöntem: Çalışmamızda 2006-2017 yılları arasında Dicle Üniversitesi Diş Hekimliği Fakültesi hastanesine diş eksikliği şikayetiyle başvuran, 49 ektodermal displazi olgusu retrospektif olarak değerlendirildi. Klinik değerlendirmede saç, tırnak, deri, diş, burun, ter bezleri ve benzeri malformasyonlar incelendi. Radyolojik muayenede; geleneksel radyografi ve konik ışınlı bilgisayarlı tomografi kullanılarak diş germleri, çene kemiği ve diş köklerine bakıldı. Bulgular: Çalışmamızda yaş ortalaması 11,9±4,6 olan, 25’i kadın (%51,1), 24’ü erkek (%48,9) toplam 49 hasta değerlendirildi. Çalışmadaki ED’li hastaların oral bulguları ile ilgili olarak; %100 (n =49) diş eksikliği, %100 (n =49) mandibular protrüzyon ,%75,51 (n =37) konik diş, %6,12 (n =3) kök şekil anomalileri görülmüştür. Klinik muayenesinde ise terleme problemi %77.55 (n =38), saç-kıl anomalisi %95.91 (n =47), anormal parmak ve tırnaklar %83.67 (n =41) belirlendi. Sonuç: ED’nin temel bulguları oral ve maksillofasiyal bölgede oluştuğundan, estetik ve çiğneme problemleri ortaya çıkmaktadır. Bu nedenle diş hekimliğinde multidisipliner tedavi gerektiren özel bir yere sahiptir. Bu hastalarda yaşam kalitesini artırmaya yönelik olarak tıp ve diş hekimlerinin koordineli çalışması oldukça önem kazanmaktadır.

The Investigation of Clinical and Radiological Findings in Ectodermal Dysplasia

Objective: Ectodermal dysplasia (ED) is a rare hereditary disease that affects ectoderm-derived tissues such as skin, nails, hair, sweat-glands and teeth. In this study it is aimed to evaluate systemic and dental findings of patients with ED who applied to our faculty hospital; clinically and radiologically. Material and Method: We retrospectively evaluated 49 cases of ED who were admitted to Dicle University Faculty of Dentistry with complaint of lack of teeth between 2006-2017. During clinical evaluation, hair, nails, skin, teeth, nose, sweat glands and similar malformations were examined. On radiological examination, dental germs, jawbone and tooth roots were examined using conventional radiography and conical beam computed tomography. Results: A total of 49 patients consisting of 25 female, (51.1%) and 24 male patients (48.9%) with the mean age of 11,9±4,6 years were evaluated in our study. Oral findings of patients with ED in the study revealed; 100% (n =49) absence of the tooth, 100% (n =49) mandibular protrusion, 75.51% (n =37) conical tooth and 6.12% (n =3) root shape anomalies. In the clinical examination, the sweating problem was determined as 77.55% (n =38), hair-bristle anomaly 95.91% (n =47) and abnormal fingers and nails 83.67% (n =41). Conclusion: As the main findings of ED are in the oral and maxillofacial region, aesthetic and chewing problems arise. For this reason, ED has a special place in dentistry requiring multidisciplinary treatment. Coordinated study of medical doctors and dentists is very important for increasing the quality of life in these patients.

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