Correction of facial deformity using a Red III device in a patient with Antley-Bixler syndrome.
Antley-Bixler syndrome was first described in 1975 and to date at least 44 cases have been reported. In addition to brachycephaly, this syndrome is associated with midface hypoplasia, bilateral radiohumeral synostosis, multiple joint contractures, long bone fractures, dysplastic ears, "pear shaped" nose, and occasionally urogenital or cardiac defects. The authors report a case in a 23-year-old man with severe midface deficiency treated using distraction osteogenesis. A modified Le Fort III osteotomy was performed, and nasal subunit and remaining Le Fort III segment was distracted separately. The classic RED II design was found to be insufficient, so the authors added an extra horizontal bar to the system and converted it to a RED III design. Midface advancement was 11 mm from the porion to the orbitale and 31 mm at point A. Nasal advancement at the nasal root level was 5 mm. There were no complications, and at 4 months after surgery, advancement was stable without any relapse.
Tunçbilek
Gökhan
G
Faculty of Medicine, Department of Plastic and Reconstructive Surgery, Hacettepe University, Ankara, Turkey. gtunc@ada.net.tr
Vargel
Ibrahim
I
Mavili
M Emin
ME
D000015:Abnormalities, Multiple; D000328:Adult; D002508:Cephalometry; D002754:Choanal Atresia; D003398:Craniosynostoses; D005147:Facial Bones / Q000002:abnormalities / Q000601:surgery*; D006801:Humans; D017880:Limb Deformities, Congenital; D008297:Male; D008313:Malocclusion, Angle Class III; D019767:Maxillofacial Abnormalities / Q000601:surgery*; D019857:Osteogenesis, Distraction / Q000295:instrumentation*; D019340:Osteotomy, Le Fort; D019291:Skull Base / Q000002:abnormalities; D013577:Syndrome