J Korean Soc Plast Reconstr Surg.  1999 Jul;26(4):590-596.

Le Fort III Advancement for the Correction of Crouzon's Disease

• Park CG
• Park SJ

Abstract

Crouzon's disease, first described by Crouzon in 1912, is characterized by craniosynostosis, exorbitism and midface hypoplasia. As well, it has been known to be transmitted as an autosomal dominant trait. Clinically, Crouzon's disease not only has aesthetic problems, but also many functional disabilities, such as increased intracranial pressure, hydrocephalus, visual disturbance. difficulty in nasal breathing and malocclusion. The surgical correction of Crouzon's disease includes: 1) frontal bone advancement and release of the craniosynostosis; 2) correction of the midface retrusion; and 3) other ancillary procedures for better aesthetic results. The authors performed Le Fort III advancement for the correction of midface retrusion in 5 cases of Crouzon's disease. These advancements were performed at about the age of 15 in consideration of facial bone growth disturbance, psychosocial effects, and the recurrence of class III malocclusion after operation. The average advancement of the midface was 9. 2 mm. We experienced favorable aesthetic and functional results without severe complication in all cases or relapse of the advanced segment.

MeSH Terms

Craniofacial Dysostosis*
Craniosynostoses
Facial Bones
Frontal Bone
Hydrocephalus
Intracranial Pressure
Malocclusion
Recurrence
Respiration