Crouzon syndrome (craniofacial dysostosis) の 1 例

概要

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Crouzon syndrome is characterized by premature craniosynostosis, midfacial hypoplasia, and shallow orbits with exophthalmos. This syndorome is transmitted by autosomal dominant fashion, and its penetrance is complete, with variable expression of symptoms. Our patient is a 35-year-old married woman, with the chief complaint of postextraction pain and swelling in the region of left mandibular third molar area. Radiological examinations revealed hypercementosis of [numerical formula], impacted teeth, calcification of the stylohyoid ligament, multiple digital impressions in the skull, mild brachycephaly, enlarged sella turcica, shallow orbit, maxillary hypoplasia, mandibular prognathism, and scoliosis of the thoracic vertebrae. She showed a characteristic appearance of face, such as parrot-beaked nose, proptosis, hypertelorism, and short upper lip. As intraoral findings, mandibular prognathism, V-shaped maxillary dental arch, and high and narrow palate with pseudocleft were noted. The etiology, differential diagnosis, and treatment of this syndrome were also discussed.
九州歯科学会の論文
1986-04-25