One-Stage Treatment for Adult Patients With Crouzonoid Appearance by Orthognathic and Face Contouring Surgery.

Abstract

Crouzon syndrome is a rare genetic disorder with autosomal-dominant inheritance that shows a triad of hallmark characteristics: craniosynostosis, exophthalmos, and midface retrusion. General treatment protocol for patients with Crouzon syndrome has already been established, but there is no standard treatment strategy for adult patients with Crouzonoid appearance. The authors present clinical patients of 1-stage orthognathic and face contouring surgery to achieve functional and aesthetic improvement.One-stage surgery was performed in adult patients with Crouzonoid appearance without a history of facial surgery. Orthognathic 2-jaw surgery was first performed to treat class III malocclusion. Face contouring surgery was conducted next to improve the aesthetic appearance using midface augmentation with onlay rib bone graft, advancement genioplasty, augmentation rhinoplasty, and ancillary procedures such as neck lift, fat graft, or fillers.Five patients (2 men, 3 women) were enrolled in this study. The mean age at the time of surgery was 19.0 years. No specific surgery-related complications such as infection, nerve injury, or recurred malocclusion were observed during the 3.5 years of follow-up. Normal class I occlusion was reached in all patients. The frontonasal angle, nasolabial angle, and labiomental angle improved from 125.1° to 135.1° (P > 0.05), 60.9° to 86.3° (P = 0.018), and 146.3° to 125.0° (P = 0.018), respectively. The Global Aesthetic Improvement Scale score was 4.75 and 5.00 in frontal and profile views, respectively.One-stage orthognathic and face contouring surgery may be an effective surgical option with good postoperative functional and aesthetic improvement in adult patients with Crouzonoid appearance.