Oral duplikasyon-yarık damak sendromu, embriyodaki gelişme kusuru (notokordun çatallanması) sonucu ortaya çıkan, çok ender görülen, genetik altyapısı bilinmeyen bir sendromdur. Bilinen 4 tipi vardır;
- Tek ağız-maksiller ark duplikasyonu
- Süpernümerer ağız (lateralde)-rudimanter mandibula
- Tek ağız-çok segmentli mandibula
- Gerçek yüz duplikasyonu (disprosopus)
Bu tiplerde saptanan bulgulara ek olarak hipertelorizm, yarık dil, yarık damak, Fallot tetralojisi, hipofiz anomalisi, psikomotor gerilik saptanır.
Kaynakça
- ^ a b c 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
- ^ a b 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
- ^ a b 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
- ^ a b 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
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Oral duplikasyon yarik damak sendromu embriyodaki gelisme kusuru notokordun catallanmasi sonucu ortaya cikan cok ender gorulen genetik altyapisi bilinmeyen bir sendromdur Bilinen 4 tipi vardir Tek agiz maksiller ark duplikasyonu Supernumerer agiz lateralde rudimanter mandibula Tek agiz cok segmentli mandibula Gercek yuz duplikasyonu disprosopus Bu tiplerde saptanan bulgulara ek olarak hipertelorizm yarik dil yarik damak Fallot tetralojisi hipofiz anomalisi psikomotor gerilik saptanir Kaynakca a b c 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 a b 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 a b 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 a b 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