Frontofacial advancement and bipartition in Crouzon-Pfeiffer and Apert syndromes: impact of fronto-facial surgery upon orbital and airway parameters in FGFR2 syndromes

• Published in: Journal of cranio-maxillo-facial surgery 2016
• Main Authors: Khonsari, Roman H., MD PhD, Benjamin, Way, MD, Johan, Nysjö, PhD, Odri, Guillaume A., MD PhD, Raphaël, Olszewski, MD PhD, Evans, Robert D., DDS, Dunaway, David J., MD, Ingela, Nyström, PhD, Britto, Jonathan A., MD
• Format: Article
• Language: English
• Subjects: Surgery
• ISSN: 1010-5182
• DOI: 10.1016/j.jcms.2016.08.015

Summary A major concern in FGFR2 craniofaciosynostosis is oculo-orbital disproportion, such that orbital malformation provides poor accommodation and support for the orbital contents and peri-orbita, leading to insufficient eyelid closure, corneal exposure and eventually to functional visual impairment. Fronto-facial monobloc osteotomy followed by distraction osteogenesis aims to correct midfacial growth deficiencies in Crouzon-Pfeiffer syndrome patients. Fronto-facial bipartition osteotomy followed by distraction is a procedure of choice in Apert syndrome patients. These procedures modify the shape and volume of the orbit and tend to correct oculo-orbital disproportion. Little is known about the detailed 3D shape of the orbital phenotype in CPS and AS, and about how this is modified by fronto-facial surgery. Twenty-eight patients with CMS, 13 patients with AS and 40 control patients were included. CT scans were performed before and after fronto-facial surgery. Late post-operative scans were available for the Crouzon-Pfeiffer syndrome group. Orbital morphology was investigated using conventional three-dimensional cephalometry and shape analysis after mesh-based segmentation of the orbital contents. We characterized the 3D morphology of CPS and AS orbits and showed how orbital shape is modified by surgery. We showed that monobloc-distraction in CPS and bipartition-distraction in AS specifically address the morphological characteristics of the two syndromes.