title: Comparing patients with Apert and Crouzon syndromes--clinical features and cranio-maxillofacial surgical reconstruction.
abstract: Cranio-maxillofacial malformations, as seen in Crouzon and Apert syndromes, may impose an immense distress on both function and aesthetics of the person affected. The aims of this study were to describe and compare the main facial and intraoral features of patients with Apert and Crouzon syndromes, the clinical manifestations that may be present, additionally to the main syndromic traits, as well as the cranio-maxillofacial surgical treatment protocols followed.Twenty-three patients with Apert syndrome (6 males, 17 females), and 28 patients with Crouzon syndrome (20 males, 8 females) were evaluated for general medical aspects, craniofacial characteristics, dentoalveolar traits before and after the final orthognathic surgery, and types and timing of cranio-maxillofacial operations. Mental retardation, associated additional malformations, cleft palate, and extensive lateral palatal soft tissue swellings were more common in children with Apert syndrome. In both syndromes, clinical findings included concave profile, negative overjet, posterior crossbites, anterior openbite, and dental midline deviation, which were corrected in almost all cases with the final orthognathic surgery, with the exception of the lateral crossbites, including more than one tooth pair, which were persisting in about half of the cases. Cranial vault decompression and/or reshaping, midfacial and orbital advancement procedures, often in conjunction with a mandibular setback, were the most frequent cranio-maxillofacial operations performed. In conclusion, Apert syndrome is more asymmetric in nature and a more severe clinical entity than Crouzon syndrome. The syndromic dentofacial features of both conditions could be significantly improved after a series of surgical procedures in almost all cases with the exception of the posterior crossbites, with haIf of them persisting post-surgically.
authors.affiliation: Department of Orthodontics, Institute of Odontology, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden. Dimitrios.Stavropoulos@odontologi.gu.se
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Stavropoulos
authors.forename: Peter
authors.initials: P
authors.lastname: Tarnow
authors.forename: Bengt
authors.initials: B
authors.lastname: Mohlin
authors.forename: Karl-Erik
authors.initials: KE
authors.lastname: Kahnberg
authors.forename: Catharina
authors.initials: C
authors.lastname: Hagberg
mesh_terms: D000168:Acrocephalosyndactylia / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016001:Confidence Intervals; D003394:Craniofacial Dysostosis / Q000473:pathology / Q000601:surgery; D005147:Facial Bones / Q000002:abnormalities / Q000601:surgery; D019066:Facies; D005260:Female; D034381:Hearing Loss; D006801:Humans; D007223:Infant; D008607:Intellectual Disability; D008046:Lip / Q000002:abnormalities; D008297:Male; D008310:Malocclusion / Q000601:surgery; D056948:Orthognathic Surgical Procedures; D021362:Palate, Hard / Q000002:abnormalities; D012744:Sex Ratio