Congenital fusion of the mandible to maxilla can involve soft tissue (synechiae) or bone (syngnathia). Congenital synechiae has been widely reported, whereas congenital syngnathia is rare. Syngnathia severely limits mouth opening. To improve airway control and prevent feeding handicaps, surgical division of the bony fusion or breakdown of the adhesions is recommended within the first few days of life. We described an 8-year-old girl who had unilateral syngnathia with micromandible, reactive coronoid process hyperplasia, cleft palate and congenital corneal opacity. She first underwent surgical treatment to improve jaw function at the age of 8 years. Delayed surgery slightly improved restricted mouth opening.
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