Correction of infraorbital and malar deficiency using costal osteochondral graft along with orthognathic surgery in Crouzon syndrome.

Abstract

In syndromic craniosynostosis, such as Crouzon syndrome, midfacial hypoplasia can cause exophthalmos and concave facial profile. Though midfacial hypoplasia in Crouzon syndrome patients can be treated with midface advancement, known as a Le Fort II or Le Fort III osteotomy, such method can change nasal appearance and frequently fails to achieve class I occlusion after surgery. This report presents a case of an aesthetically and functionally successful midfacial augmentation using rib and cartilage graft along with orthognathic surgery (Le fort I and bilateral sagittal split ramus osteotomy) for patients with Crouzon syndrome. The patient was a 21-year-old male with Crouzon syndrome, who had undergone augmentation rhinoplasty 2 years ago. His main issues were midfacial retrusion and mild anterior open bite and cross bite and, furthermore, did not want any change in his nasal appearance. To augment midfacial volume, rib bone graft was inserted on the inferior orbital rim and costal cartilage graft was done on the zygomatic area. The costal osteocartilage was fixed with titanium screws. Additionally, Le Fort I osteotomy and bilateral sagittal split ramus osteotomy were done to treat the anterior open bite and cross bite. The maxillary segment was advanced 2 mm and posteriorly impacted 2.5 mm. Then, 5 mm of mandibular setback was done and the maxillomandibular segment was rotated clockwise. Finally, genioplasty with 5-mm advancement was done to compensate for the chin retrusion after performing the mandibular setback. The operation took 425 minutes and estimated blood loss was 500 mL. After 6 months since surgery, the patient had convex facial profile and class I occlusion. For the patient with mild midface hypoplasia, good nasal profile, and malocclusion, rib bone graft along with Le Fort I and bilateral sagittal ramus osteotomy can be a good surgical modality.