A CLINICAL CASE OF CRANIOCLAVICULAR DYSOSTOSIS COMPLICATED BY MANDIBULAR PHLEGMON IN THE PRACTICE OF A MAXILLOFACIAL SURGEON

Cranioclavicular dysostosis is an inherited orphan disease associated with a mutation in the Runx2 gene located on the short arm of chromosome 6. Clavicular cranial dysostosis is transmitted by an autosomal dominant type and is manifested by delayed ossification of the skull, large fontanelles and closure of cranial sutures. Retention and dystopia of permanent teeth is associated with the presence of one or more super-complete teeth on the upper and lower jaws. One of the characteristic symptoms of the disease is hypoplasia or aplasia of the collarbones.
The article describes the case of cranioclavicular dysostosis (CСD) in a 42-year-old man with severe purulent complication due to lack of timely treatment in childhood. The patient went to the maxillofacial surgery clinic complaining of pain and swelling in the submandibular region on the left side. A series of CT scans of the skull bones revealed a defect in the cranial vault, a lot of retarded teeth of the lower and upper jaws. After the examination, the diagnosis was made: odontogenic phlegmon of the left submandibular region, masticatory, pterygoid-maxillary space on the left side, cranioclavicular dysostosis. The causal tooth of the development of the purulent-inflammatory process turned out to be a retinated 3.8 tooth located next to a group of dystopian and retinated chewing teeth of the lower jaw on the left side. An operation was performed to open a purulent-inflammatory focus, remove a retarded 3.8 tooth. The article presents photos of the patient, CT scans of the skull and chest.

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