DUDAK SİNEŞİSİ VE POLİDAKTİLİ ÇOCUK OLGU; ELLIS VAN CREVELD SENDROMU

Gaye Filinte, Mehmet Ersin Gönüllü, Nujen Gülçin Ayçiçek çardak, Güniz Eker uluçay, Tayfun Aköz

Özet


Ellis Van Creveld sendromu iskelet sistemi anomalileri ile karakterize herediter bir tablodur. Kondrodisplazi, ektodermal displazi, konjenital kardiak defektler ve polidaktili karakteristik özelliklerindendir. Alt alveoler arkta çentiklenme, üst dudak ve gingivomukozal marjinde füzyon, sıklıkla neonatal diş varlığı, oligodonti ve anterior kısımdaki dişlerde konik şekil bulunması gibi oral mukozal ve dental değişikliklerin varlığı Ellis van Creveld sendromu tanısını kuvvetlendirmektedir. 13 aylık kız çocuğu bilateral postaksiyal polidaktili ve üst dudak ve gingivomukozal bileşkede füzyon deformitelerinin rekonstrüksiyonu nedeniyle tarafımıza refere edildi. Detaylı muayenesinde kondroektodermal displazi ve kardiak anomali saptandı. Aile genetik inceleme yapılmasını kabul etmedi fakat hastanın klinik bulguları Ellis van Creveld Sendromu tanısını güçlü bir şekilde destekliyordu.
Anahtar Kelimeler: Ellis Van Creveld sendromu postaksiyal polidaktili, maksiller jinjiva labiyal füzyon

Referanslar


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Online ISSN:2528-8644
Basılı ISSN:1300-6878 (önceki)

Türk Plastik Rekonstrüktif ve Estetik Cerrahi Dergisi (Turk J Plast Surg),
Türk Plastik Rekonstrüktif ve Estetik Cerrahi Derneği'nin, Estetik Plastik Cerrahi Derneği'nin, Türkiye Interplast Derneği'nin ve Çene Yüz Cerrahları Derneği'nin yayın organıdır.