"Bochdalek" skull (syngnathia): CT examination.
Bony fusion of the jaws (syngnathia) without any other anatomic oral anomalies is an unusual condition. It is believed that important factors can be congenital. Some cases with combination of cleft palate, aglossia, and soft or bony adhesion between the maxilla and mandible have been reported. Congenital syngnathia could also occur with Treacher-Collins syndrome, pterygium syndrome and van der Woude syndrome. In this study, girl skull with jaw anomaly depicted by prof. Bochdalek in XIX. century was re-examined using CT method to explain possible mechanism of this anomaly development. Our report presents a case of syngnathia with bilateral vision where mandible, maxilla, zygomatic and palatal bones are mutually connected. CT findings strongly support the idea about of mechanical trauma triggering a chain of bone disturbances in facial skeleton. With high probability most of the teeth were extracted later to keep the oral cavity open (Fig. 9, Ref. 32).
Klepacek
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Department of Anatomy, First Faculty of Medicine, Charles University, Prague, Czech Republic. klepacek@seznam.cz
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D000293:Adolescent; D005260:Female; D049671:History, 18th Century; D006801:Humans; D007569:Jaw Abnormalities / Q000000981:diagnostic imaging / Q000266:history*; D011859:Radiography; D012886:Skull / Q000002:abnormalities*; D013580:Synostosis / Q000000981:diagnostic imaging / Q000266:history*