Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion Associated With Gum Fusion

J Craniofac Surg. 2016 Jan;27(1):e20-3. doi: 10.1097/SCS.0000000000002293.

Abstract

A congenial syngnathia is very rare condition. It can be simple mucosal fusion (synechiae), or complete bony fusion (synostosis) between the maxilla or zygoma and the mandible. Fusion of the ascending ramus of mandible to maxilla and zygoma is less common than fusions of the alveolar ridges of the mandible to the maxilla. Bony syngnathia is either isolated or complex in form. There are 59 cases of congenital bony syngnathia reported in the literature: the first report was by Burket in 1936. There are 16 reported cases of zygomatico-maxillo-mandibular fusion. In the reported cases, women expressed the isolated form more commonly whereas men demonstrated a more complex pattern of disease. The authors present another patient of bony syngnathia involving bilateral fusion of the ascending ramus and body of the mandible with the maxillary complex in a young man. Early surgery was performed to release the bony and soft tissue fusion on the eighth day from the baby's birth. A second operation was performed for recurrence when the baby was 2.5 months old. A customized splint, an intense postoperative program of mouth exercises, and close follow-up aims to prevent further refusion.

Publication types

  • Case Reports

MeSH terms

  • Alveolar Process / abnormalities
  • Alveolar Process / surgery
  • Exercise Therapy / methods
  • Follow-Up Studies
  • Gingiva / abnormalities*
  • Gingiva / surgery
  • Humans
  • Infant, Newborn
  • Male
  • Mandible / abnormalities*
  • Mandible / surgery
  • Maxilla / abnormalities*
  • Maxilla / surgery
  • Osteotomy / methods
  • Recurrence
  • Reoperation
  • Splints
  • Synostosis / surgery*
  • Zygoma / abnormalities*
  • Zygoma / surgery