J Korean Neurosurg Soc.  1987 Mar;16(1):289-296.

Surgical Treatment of the Craniofacial Dysostoses-Crouzon's Disease

• Kang JK
• Song JU
• Kim JC
• Lim P

Abstract

Craniofacial dysostoses(Crouzon's disease) are characterized by cranial and facial deformities but the relationship between the craniosynostosis and facial deformities has not been clearly established. However, we believe that certain facial hypoplasias are comparable in nature with craniostenosis. Our objective in treatment of the Crouzon's disease is to achieve a postero-anterior movement of the whole facial mass, with the double aim of establishing normal dental occlusion and of increasing orbital capacity. The method we describe uses Tessier's basic procedures for surgical correction of the Crouzon's diseases patient who was 16 year-old boy. It would appear that the best treatment of these requires a radical and total osteostomy of the middle third of the face, to permit complete repositioning of the involved segment. These total osteotomies frequently include the creation of a line of separation between the anterior cranial base above and the facial bone below and also between the pterygoid processes behind and the maxilla anteriorly. Thus one is detaching the face from the cranial base. The authors described the surgical management of the Crouzon's disease and discussed the warning points of intraoperative technique in correction of the craniofacial dysostosis.

Keyword

Craniofacial; dysostoses; Crouzon's disease; Middle facial mass; Total osteotomy

MeSH Terms

Adolescent
Congenital Abnormalities
Craniofacial Dysostosis
Craniosynostoses
Dental Occlusion
Dysostoses
Facial Bones
Humans
Male
Maxilla
Orbit
Osteotomy
Skull Base