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Patient-Reported Quality of Life in Highest-Functioning Apert and Crouzon Syndromes: A Comparative Study

Raposo-Amaral, Cassio Eduardo M.D., Ph.D.; Neto, José Garcia Junqueira M.D.; Denadai, Rafael M.D.; Raposo-Amaral, Cassio Menezes M.D., Ph.D.; Raposo-Amaral, Cesar Augusto M.D.

doi: 10.1097/01.prs.0000437260.31693.75
Pediatric/Craniofacial: Outcomes Article

Background: Crouzon and Apert syndromes are the most common syndromic forms of craniofacial dysostosis. Apert syndrome has a broad clinical spectrum, including complex craniofacial involvement, as well as limiting deformities of the hands, feet, and other joints that require multiple surgical procedures when compared with Crouzon syndrome, which is generally less severe. The authors hypothesized that the quality of life of Apert syndrome patients is inferior to that of Crouzon syndrome patients.

Methods: The quality of life of Apert (n = 8) and Crouzon (n = 12) syndrome patients was assessed using the World Health Organization Quality of Life-100 questionnaire. The Mann-Whitney test was used to compare the quality-of-life scores between Apert and Crouzon patients. Values were considered significant for a confidence interval of 95 percent (p < 0.05).

Results: Apert patients showed an overall higher (score > 60 percent) quality of life in most World Health Organization Quality of Life-100 facets (68 percent) and domains (83.33 percent), with significance (p < 0.05) in three facets (energy and fatigue, mobility, and environment in the home), compared with Crouzon patients.

Conclusion: Contrary to the authors’ initial hypothesis, both the highest-functioning Apert patients and the Crouzon patients presented a satisfactory quality of life, demonstrating that these syndromic patients had acquired the necessary repertoire to manage the adverse daily situations of their lives.

Campinas, Brazil

From the Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital, Campinas, Brazil.

Received for publication April 2, 2013; accepted August 29, 2013.

† Deceased.

Disclosure: The authors have no financial interest to declare in relation to the content of this article.

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Cassio Eduardo Raposo-Amaral, M.D., Ph.D., Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital, Av. Adolpho Lutz 100, Caixa Postal 6028, 13084-880 Campinas, São Paulo, Brazil, cassioraposo@hotmail.com.br

©2014American Society of Plastic Surgeons