title: 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.affiliation: The Craniofacial Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom; Assistance Publique - Hôpitaux de Paris, Hôpital Necker Enfants-Malades, Service de Chirurgie Maxillo-faciale et Plastique, Université Paris-Descartes, Paris, France.
authors.forename: Roman H
authors.initials: RH
authors.lastname: Khonsari
authors.affiliation: The Craniofacial Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Way
authors.affiliation: Centre for Image Analysis, Uppsala University, Uppsala, Sweden.
authors.forename: Johan
authors.initials: J
authors.lastname: Nysjö
authors.affiliation: Assistance Publique - Hôpitaux de Paris, Hôpital Lariboisière, Service de Chirurgie Orthopédique, Université Paris-Diderot, Paris, France.
authors.forename: Guillaume A
authors.initials: GA
authors.lastname: Odri
authors.affiliation: Department of Oral and Maxillofacial Surgery, Saint-Luc University Hospital, Catholic University of Leuven, Brussels, Belgium.
authors.forename: Raphaël
authors.initials: R
authors.lastname: Olszewski
authors.affiliation: The Craniofacial Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
authors.forename: Robert D
authors.initials: RD
authors.lastname: Evans
authors.affiliation: The Craniofacial Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
authors.forename: David J
authors.initials: DJ
authors.lastname: Dunaway
authors.affiliation: Centre for Image Analysis, Uppsala University, Uppsala, Sweden.
authors.forename: Ingela
authors.initials: I
authors.lastname: Nyström
authors.affiliation: The Craniofacial Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom. Electronic address: jonathan.britto@gosh.nhs.uk.
authors.forename: Jonathan A
authors.initials: JA
authors.lastname: Britto
mesh_terms: D000168:Acrocephalosyndactylia / Q000000981:diagnostic imaging / Q000601:surgery*; D000293:Adolescent; D016022:Case-Control Studies; D002508:Cephalometry; D002648:Child; D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000000981:diagnostic imaging / Q000601:surgery*; D005147:Facial Bones / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D021621:Imaging, Three-Dimensional; D007223:Infant; D009915:Orbit / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D019857:Osteogenesis, Distraction / Q000379:methods; D010027:Osteotomy / Q000379:methods; D012137:Respiratory System / Q000473:pathology; D014057:Tomography, X-Ray Computed
keywords: 3D-cephalometry; Apert syndrome; Crouzon–Pfeiffer syndrome; FGFR2; Monobloc-distraction; Orbit