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KMID : 0358819870140020243
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Journal of Korean Society of Plastic and Reconstructive Surgeons
1987 Volume.14 No. 2 p.243 ~ p.250
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AN EXPERIENCE OF CROUZON´S DISEASE
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Kang Yoon-Jae
Kim Jin-Chul
Lim Poong
Kang Joon-Ki
Kim Seung-Nyeun
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Abstract
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Crouzon’s disease, described first by Crouzon in 1912, is one of the craniofacial dysostoses and has been known to be transmitted as an autosomal dominant trait.
The main clinical characteristics of this disease are varing degree of craniosynostosis, exophthalmos and midface recession according to the severity of the involvement. Because of the increased intracranial pressure caused by the premature synostosis of the cranial sutures, neurologic disorders, such as hydrocephalus, epilepsy and mental retardation, are often present. The shallowness of the orbital cavities is the cause of exophthalmos which accompanies many ophthalmologic complications. The midface in general is both retruded and hypoplastic, and pseudoprognathic mandible is seen. The surgical treatment varies depending on it’s severity and patient’s age.
Authors performed Le Fort Ⅲ osteotomy in 16 year-old male patient and obtained acceptable results. Them we report the case with brief discussion and review of the literatures.
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KEYWORD
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