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Oral duplikasyon-yarık damak

Çok ender

Embriyondaki gelişme kusuru (notokordun çatallanması) sonucu

Bilinen 4 tipi vardır;

1. Tek ağız-maksiller ark duplikasyonu

2. Süpernümerer ağız (lateralde)-rudimanter mandibula

3. Tek ağız-çok segmentli mandibula

4. Gerçek yüz duplikasyonu (disprosopus)

Bunlara ek olarak: hipertelorizm, yarık dil, yarık damak, Fallot tetralojisi, hipofiz anomalisi, psikomotor gerilik

 

• Avery JK, Hayward JR. Case report: duplication of oral structures with cleft palate. The Cleft Palate Journal, 1969 Oct; 6:506-515, 1969

• Yaqoob M, Mahmood F, Hanif G, et al. Etiology and genetic factors in clefts of lip and/or palate reported at children's hospital, Lahore, Pakistan. Indian Journal of Human Genetics, 19(2):136-43, 2013

• Maeda Y, Suenaga H, Sugiyama M, et al. Clinical presentation of epignathus teratoma with cleft palate; and duplication of cranial base, tongue, mandible, and pituitary gland. Journal of Craniofacial Surgery, 24(4):1486-1491, 2013

• Louw JJ, Corveleyn A, Jia Y, et al. MEIS2 involvement in cardiac development, cleft palate, and intellectual disability. American Journal of Medical Genetics A, 167A: 1142-1146, 2015

• Noguchi T, Sugiyama T, Sasaguri KI, et al. Surgical management of duplication of the pituitary gland-plus syndrome with epignathus, cleft palate, duplication of mandible, and lobulated tongue. Journal of Craniofacial Surgery, 28(2):e141-e144, 2017