TY - JOUR T1 - Ellis-van Creveld Sendromunda Oral Bulgular TT - Oral Findings of Ellis-van Creveld Syndrome AU - Zengin, Ayşe AU - Çelenk, Peruze AU - Mutlu, Birgül PY - 2023 DA - June DO - 10.58711/turkishjdentres.vi.1253583 JF - Türk Diş Hekimliği Araştırma Dergisi JO - J Turkish Dent Res PB - Ondokuz Mayıs Üniversitesi WT - DergiPark SN - 2822-4310 SP - 163 EP - 167 VL - 2 IS - 1 LA - tr AB - Ellis-van Creveld Sendromu, nadir görülen herediter bir hastalıktır. Sendromun belirgin özellikleri; ellerde bilateral postaksiyal polidaktili, akromesomelik dwarfizme yol açan ve uzun kemiklerde görülen kondrodisplazi, diş ve tırnakları etkileyen ektodermal displazi ile konjenital kalp anomalileridir. Dörtlü major bulguları arasında kraniofasial ve dental anomalilerin bulunması nedeniyle diş hekimleri tarafından kolayca teşhis edilebilir. Literatürde Ellis-van Creveld Sendromu ile ilgili çok sayıda yayın olmasına rağmen oral bulgularını tanımlayanlar sadece birkaç tanedir. Bu yazıda diş eksikliği şikayeti ile başvuran 6 yaşındaki kız çocuğunda görülen ve oral bulgularıyla tanı konulan Ellis-van Creveld Sendromu, genel klinik karakteristikleri ve oral bulgularıyla sunulmuştur. KW - dental KW - oral KW - Ellis van creveld N2 - Ellis-van Creveld Syndrome is a rare hereditary disease. Distinctive features of the syndrome; Bilateral postaxial polydactyly in the hands, chondrodysplasia in long bones causing acromesomelic dwarfism, ectodermal dysplasia affecting teeth and nails and congenital heart anomalies. It can be easily diagnosed by dentists due to the presence of craniofacial and dental anomalies among the four majorfindings. Although there are many studies about Ellis-van Creveld Syndrome in the literature, only a few describe its oral findings. In this article, Ellis-van Creveld Syndrome, which was diagnosed in a 6-year-old girl who presented with the complaint of missing teeth and was diagnosed with oral findings, is presented with its general clinical features and oral findings. CR - 1. Atasu M, Biren S. Ellis-van Creveld syndrome: dental, clinical, genetic and dermatoglyphic findings of a case. J Clin Pediatr Dent 2000; 24:141-5. CR - 2. Eswar N. Chondroectodermal dysplasia: a case report. J Indian Soc Pedod Prev Dent 2001; 19:103-6. CR - 3. Hattab FN, Yassin OM, Sasa IS. Oral manifestations of Ellis-van Creveld syndrome: report of two siblings with unusual dental anomalies. J Clin Pediatr Dent 1998; 22:159-165. CR - 4. Gorlin RJ, Cohen MM, Hennekam RCM. Syndromes of the Head and Neck. 4th ed, New York: Oxford University Press; 2004. 239-41. CR - 5. Hunter ML, Roberts GJ. Oral and dental anomalies in Ellisvan Creveld syndrome (chondroectodermal dysplasia): report of a case. Int J Peidatr Dent 1998; 8: 153-7. CR - 6. Cahuana A, Palma C, Gonzales W, Gean E. Oral manifestations in Ellis-van Creveld syndrome: report of five cases. Pediatr Dent 2004; 26:277-82. CR - 7. Arya L, Mendiratta V, Sharma RC, Solanki RS. Ellis-van Creveld Syndrome: A report of two cases. Pediatr Dermatol 2001; 18:485-9. CR - 8. Varela M, Ramos C. Chondroectodermal dysplasia (Ellisvan Creveld syndrome): a case report. Eur J Orthod 1996; 18:313-8. CR - 9. Lyon Jones K. Smith’s recognizable patterns of human malformations. Elsevier Saunders, Philadelphia, PA. 2006 UR - https://doi.org/10.58711/turkishjdentres.vi.1253583 L1 - https://dergipark.org.tr/tr/download/article-file/2962657 ER -