Fronto-facial advancement and bipartition in Crouzon-Pfeiffer and Apert syndromes: Impact of fronto-facial surgery upon orbital and airway parameters in FGFR2 syndromes.

Abstract

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.

• Authors: Roman H Khonsari, Benjamin Way, Johan Nysjö, Guillaume A Odri, Raphaël Olszewski, Robert D Evans, David J Dunaway, Ingela Nyström, Jonathan A Britto
• Journal: Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery (J Craniomaxillofac Surg) Vol. 44 Issue 10 Pg. 1567-1575 (Oct 2016) ISSN: 1878-4119 [Electronic] Scotland
• PMID: 27639780 (Publication Type: Journal Article)
• Copyright: Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Topics

• Acrocephalosyndactylia (diagnostic imaging, surgery)
• Adolescent
• Case-Control Studies
• Cephalometry
• Child
• Child, Preschool
• Craniofacial Dysostosis (diagnostic imaging, surgery)
• Facial Bones (diagnostic imaging, surgery)
• Humans
• Imaging, Three-Dimensional
• Infant
• Orbit (diagnostic imaging, pathology, surgery)
• Osteogenesis, Distraction (methods)
• Osteotomy (methods)
• Respiratory System (pathology)
• Tomography, X-Ray Computed