BECKWITH - WIEDEMANN SENDROMU (OLGU BİLDİRİSİ)*-THE BECKWITH - WIEDEMANN SYNDROME (CASE REPORT)
Beckwith-Wiedemann Syndrome is a growth disorder, with cardinal symptoms of exomphalos, macroglossia and gigantism, and the review of the cases reported shows that the syndrome is inherited by way of an austosomal dominant gene with various expressivity.
Abstract
OZET Beckwith-Wiedemann sendromu, ana belirtileri 'exomp-halos', makroglossi ve gigantizm olan ve etiyolojisi hen uz tamamen aydinlanmamis bir buyume hastaligidir. Ancak bugune kadar sendromun degisik expresivite gosteren oto-somal dominant bir genle gectigini bildiren vakalar vardir. Bazi yazarlar sendromun gorulme sikligim 1/13.700 olarak bildirmislerdir. Kizlarda yaklasik %60 oraninda daha fazla gorulmektedir. Dis Hekimligi acisindan bu sendrom, makroglossi, mandi-buler prognatiye egilim ile birlikte malokfuzyonlarin gorulmesi nedeniyle onemlidir. Bu yazida dogumda tanimlanmis 'omphalocele' ve mak-roglosi ameliyat i gecirmis olan ve klinigimize dis curukleri sebebiyle basvuran bir Beckwith-Wiedemann olgusu sunulmustur. Anahtar sozcukler: Beckwith-Wiedemann sendromu, malokluzyonlar, makroglossi. ABSTRACT Beckwith-Wiedemann Syndrome is a growth disorder, with cardinal symptoms of exomphalos, macroglossia and gigantism. The etiology of the disorder is unknown. However, the review of the cases reported, shows that the syndrome is inherited by way of, an austosomal dominant gene with various expressivity. Some authors report its prevalance as 1/13.700. Females are more affected by 60%. The Syndrome is as important condition in dental practice as it has features like macroglossia, malocclusion with tendency toward mandibular prognathism. This article, is the report of a case of Beckwith-Wiedemann Syndrome which was diagnosed at birth. The case was previously operated for omphalocele and macroglossia; and later applied to our clinic for the treatment of dental caries. Key words: Beckwith-Wiedemann Syndrome, malocclusion, macroglossia.