Anterior convex lateral orbital wall: distinctive morphology in Apert syndrome

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dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author LU, X.
FORTE, A. J.
SAWH-MARTINEZ, R.
WU, R.
CABREJO, R.
STEINBACHER, M.
ALPEROVICH, M.
ALONSO, N. FMUSP-HC
PERSING, J. A.
dc.date.issued 2018
dc.identifier.citation BRITISH JOURNAL OF ORAL & MAXILLOFACIAL SURGERY, v.56, n.9, p.864-869, 2018
dc.identifier.issn 0266-4356
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/29773
dc.description.abstract Bony malformations of the orbit and alterations to the soft tissue in Apert syndrome contribute to ophthalmic dysfunction. Recognised structural malformation of the sphenoid and ethmoid sinuses, together with corresponding deformities in the anterior and middle cranial base, are characteristic. Our aim was to explore the underlying structural components of disfigurement and the consequent development of the orbit in patients with Apert syndrome over time by studying 18 preoperative computed tomographic (CT) scans of affected patients and 36 scans from controls. Cephalometric measurements related to the orbit were collected, and analysed with Materialise software. The patients with Apert syndrome had larger than normal external orbital horizontal angles between the ages of 6 months and 2 years. The inside horizontal angle was narrower at 16.36 degrees before 6 months, and continued to decrease into adulthood. The ethmoid and sphenoid side angles in affected patients consistently increased, starting at 7.93% and 14.68% of the external horizontal angle, respectively, during the first 6 months of age, and becoming 20.55% and 11.69%, respectively, in adulthood. In unaffected patients, both angles were less than 3% of the external horizontal angle overall. The orbital vertical angle also changed synchronously, with increasingly wide lateral orbits and shortened anteroposterior orbits. The anterior protrusion of the lateral orbital wall resulted from superior and posterior rotation of a curved, greater wing of the sphenoid, while the widened median orbital wall was caused by the widened ethmoid sinus. These resulted in bony deformities of the orbit, which predisposed to the visual impairments of Apert syndrome.
dc.language.iso eng
dc.publisher CHURCHILL LIVINGSTONE
dc.relation.ispartof British Journal of Oral & Maxillofacial Surgery
dc.rights restrictedAccess
dc.subject Apert syndrome; orbit; malformation; development
dc.subject.other bipartition distraction; crouzon syndromes; surgery; craniosynostosis; advancement; monobloc; pfeiffer; children
dc.title Anterior convex lateral orbital wall: distinctive morphology in Apert syndrome
dc.type article
dc.rights.holder Copyright CHURCHILL LIVINGSTONE
dc.description.group LIM/04
dc.identifier.doi 10.1016/j.bjoms.2018.09.011
dc.identifier.pmid 30337164
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author ALONSO, N.:FM:MCG
hcfmusp.author.external · LU, X.:Chinese Acad Med Sci, Peking Union Med Coll, Plast Surg Hosp, Beijing, Peoples R China
· FORTE, A. J.:Mayo Clin Florida, Div Plast & Reconstruct Surg, Jacksonville, FL USA
· SAWH-MARTINEZ, R.:Yale Sch Med, Sect Plast & Reconstruct Surg, 330 Cedar St,3rd Floor Boardman Bldg, New Haven, CT 06520 USA
· WU, R.:Yale Sch Med, Sect Plast & Reconstruct Surg, 330 Cedar St,3rd Floor Boardman Bldg, New Haven, CT 06520 USA
· CABREJO, R.:Yale Sch Med, Sect Plast & Reconstruct Surg, 330 Cedar St,3rd Floor Boardman Bldg, New Haven, CT 06520 USA
· STEINBACHER, M.:Yale Sch Med, Sect Plast & Reconstruct Surg, 330 Cedar St,3rd Floor Boardman Bldg, New Haven, CT 06520 USA
· ALPEROVICH, M.:Yale Sch Med, Sect Plast & Reconstruct Surg, 330 Cedar St,3rd Floor Boardman Bldg, New Haven, CT 06520 USA
· PERSING, J. A.:Yale Sch Med, Sect Plast & Reconstruct Surg, 330 Cedar St,3rd Floor Boardman Bldg, New Haven, CT 06520 USA
hcfmusp.origem.id WOS:000450589300017
hcfmusp.origem.id 2-s2.0-85054729136
hcfmusp.publisher.city EDINBURGH
hcfmusp.publisher.country SCOTLAND
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dc.description.index MEDLINE
dc.identifier.eissn 1532-1940
hcfmusp.citation.scopus 0
hcfmusp.citation.wos 0
hcfmusp.affiliation.country Brasil
hcfmusp.affiliation.country China
hcfmusp.affiliation.country Estados Unidos


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