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Syngnathia

Syngnathia, altçenenin üstçene ya da yüz kemikleriyle kaynaşması olgusudur.[1][2] Çoğu gen mutasyonu sonucu oluşan izole olgulardır; sendroma-özgü bir bulgu olduklarında, otosomal dominant ya da otosomal resesif yolla aktarılırlar.[3][4][5]

4 fenotip saptanmıştır:[2][6]

  • Tip 1a: alt ve üst çene ön bölge alveol kretleri kaynaşması,
  • Tip 1b: alt ve üst çene ön bölge alveol kretleri kaynaşması ile birlikte başkaca baş-boyun anomalileri,
  • Tip 2a: altçene ve zigomatik kemik kaynaşması,
  • Tip 2b: altçene ve zigomatik kemik kaynaşması ile birlikte yarık damak ve temporomandibular eklem ankilozu.


  1. ^ Cohen MM Jr. Malformations of the Craniofacial Region: Evolutionary, Embryonic, Genetic, and Clinical Perspectives. American Journal of Medical Genetics (Seminars in Medical Genetics), 115:245-268, 2002
  2. ^ a b Laster Z, Temkin D, Zarfin Y, Kushnir A. Complete bony fusion of the mandible to the zygomatic complex and maxillary tuberosity: case report and review. International Journal of Oral & Maxillofacial Surgery, 30: 75-79, 2001
  3. ^ Parkins GE, Boamah MO. Congenital maxillomandibular syngnathia: Case report. Journal of Cranio-Maxillofacial Surgery, 37 (5): 276–278, 2009
  4. ^ Ugurlu K, Turan T, Urganci N, et al. Fusion of maxillary and mandibular alveolar process together with a median mandibular cleft: a rare congenital anomaly. Journal of Craniomaxillofacial Surgery, 27: 105-108, 1999
  5. ^ Villanueva-Garcia D, Contreras-Masse G, Villa-Guillen M, et al. Syngnathism in an infant born to consanguineous parents. American Journal of Medical Genetics, 149A: 2303-2305, 2009
  6. ^ Arshad AR, Goh CS. Hypoglossia congenita with anterior maxillo-mandibular fusion. British Journal of Plastic Surgery, 47: 139-141, 1994
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Syngnathia
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