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

Khonsari, Roman H.;Way, Benjamin;Nysjö, Johan;Odri, Guillaume A.;Britto, Jonathan A.;et.al.
(2016) Journal of Cranio-Maxillofacial Surgery — Vol. 44, n° 10, p. 1567-1575 (2016)

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Authors
  • Khonsari, Roman H.
    Author
  • Way, Benjamin
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  • Nysjö, Johan
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  • Odri, Guillaume A.
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  • Olszewski, Raphaëlorcid-logoUCLouvain
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  • Britto, Jonathan A.
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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. © 2016 European Association for Cranio-Maxillo-Facial Surgery
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Khonsari, R. H., Way, B., Nysjö, J., Odri, G. A., Olszewski, R., Evans, R. D., Dunaway, D. J., Nyström, I., & Britto, J. A. (2016). Fronto-facial advancement and bipartition in Crouzon–Pfeiffer and Apert syndromes: Impact of fronto-facial surgery upon orbital and airway parameters in FGFR2 syndromes. Journal of Cranio-Maxillofacial Surgery, 44(10), 1567-1575. https://doi.org/10.1016/j.jcms.2016.08.015 (Original work published 2016)