Le Fort III bipartition osteotomy to treat a rare craniofacial anomaly: frontofacionasal dysostosis.
Frontofacionasal dysplasia or dysostosis (Mendelian Inheritance in Man 229400) is composed of cranial, ophthalmic, nasal, and lip and palate deformities. It is a rare and separate entity because it contains none of the extracranial defects seen in frontonasal dysplasia and related syndromes. Four patients with frontofacionasal dysplasia were treated in Hacettepe University between 2000 and 2007. A new surgical approach, less invasive and risky than an intracranial procedure and more effective and quicker than an orthodontic molding/distraction therapy, was developed during the treatment course of this group of patients. Le Fort III bipartition osteotomy was performed to reduce the distance between maxillary segments in very wide cleft lip and palate repair. No surgical complication was encountered, and all the patients healed well and were discharged from the hospital without a problem. Craniofacial deformities of the patients with frontofacionasal dysplasia should be repaired to have a socially acceptable facial appearance. We suggest Le Fort III bipartition osteotomy in selected cases to reduce the distance between maxillary segments in patients with very wide clefts.
Tunçbilek
Gökhan
G
Department of Plastic and Reconstructive Surgery, Hacettepe University Faculty of Medicine, Sihhiye, Ankara, Turkey. gokhantuncbilek@gmail.com
Alanay
Yasemin
Y
Kayikçioğlu
Aycan
A
D002971:Cleft Lip / Q000601:surgery; D002972:Cleft Palate / Q000601:surgery; D019465:Craniofacial Abnormalities / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D019340:Osteotomy, Le Fort; D035583:Rare Diseases; D016896:Treatment Outcome