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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
Assistance Publique – Hôpitaux de Paris, Hôpital Necker Enfants-Malades, Service de Chirurgie Maxillo-faciale et Plastique, Université Paris-Descartes, Paris, France.
The Craniofacial Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
Uppsala University, Disciplinary Domain of Science and Technology, Mathematics and Computer Science, Department of Information Technology, Computerized Image Analysis and Human-Computer Interaction. Uppsala University, Disciplinary Domain of Science and Technology, Mathematics and Computer Science, Department of Information Technology, Division of Visual Information and Interaction.
Assistance Publique – Hôpitaux de Paris, Hôpital Lariboisière, Service de Chirurgie Orthopédique, Université Paris-Diderot, Paris, France.
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2016 (English)In: Journal of Cranio-Maxillofacial Surgery, ISSN 1010-5182, E-ISSN 1878-4119, Vol. 44, no 10, 1567-1575 p.Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
2016. Vol. 44, no 10, 1567-1575 p.
Keyword [en]
Monobloc-distraction, Orbit, 3D-cephalometry, Crouzon–Pfeiffer syndrome, Apert syndrome, FGFR2
National Category
Surgery Medical Image Processing
Identifiers
URN: urn:nbn:se:uu:diva-311642DOI: 10.1016/j.jcms.2016.08.015ISI: 000389106700010PubMedID: 27639780OAI: oai:DiVA.org:uu-311642DiVA: diva2:1060998
Available from: 2016-12-30 Created: 2016-12-30 Last updated: 2017-01-09Bibliographically approved

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Computerized Image Analysis and Human-Computer InteractionDivision of Visual Information and Interaction
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