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Fronto-facial advancement and bipartition in Crouzon-Pfeiffer and Apert syndromes: Impact of fronto-facial surgery upon orbital and airway parameters in FGFR2 syndromes.
- Source :
-
Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery [J Craniomaxillofac Surg] 2016 Oct; Vol. 44 (10), pp. 1567-1575. Date of Electronic Publication: 2016 Aug 21. - Publication Year :
- 2016
-
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.<br /> (Copyright © 2016 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)
- Subjects :
- Acrocephalosyndactylia diagnostic imaging
Adolescent
Case-Control Studies
Cephalometry
Child
Child, Preschool
Craniofacial Dysostosis diagnostic imaging
Facial Bones diagnostic imaging
Humans
Imaging, Three-Dimensional
Infant
Orbit diagnostic imaging
Orbit pathology
Osteogenesis, Distraction methods
Osteotomy methods
Respiratory System pathology
Tomography, X-Ray Computed
Acrocephalosyndactylia surgery
Craniofacial Dysostosis surgery
Facial Bones surgery
Orbit surgery
Subjects
Details
- Language :
- English
- ISSN :
- 1878-4119
- Volume :
- 44
- Issue :
- 10
- Database :
- MEDLINE
- Journal :
- Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery
- Publication Type :
- Academic Journal
- Accession number :
- 27639780
- Full Text :
- https://doi.org/10.1016/j.jcms.2016.08.015