title: The Quality of Life in Adult Patients With Syndromic Craniosynostosis From Their Perspective.
abstract: BACKGROUND
  Clinical intuition may perceive those adults with syndromic craniosynostosis to have a lower quality of life (QOL) compared with the normative population. Classification of facial difference; standardization of cognitive capacity and selection of an appropriate QOL measurement tool provides a less intuitive and more evidence-based method of assessing QOL in this particular group of patients.
  
  
  METHODS
  Adults with syndromic craniosynostosis treated by the same surgeons underwent Whittaker Classification for facial difference by an independent observer. Neuropsychology screening ensured cognitive ability in patients for independent answering of a World Health Organization QOL postal questionnaire. Data analysis using descriptive and z test statistics allowed comparison to nonsyndromic adult United Kingdom data provided by the World Health Organization.
  
  
  RESULTS
  Forty adult patients met authors' inclusion criteria. Whittaker Classification of facial difference ranged from I (31 patients) to II (8 patients) and III (1 patient). Quality of life showed no correlation to facial difference. Quality of life was better in the physical, psychological, and environmental domains compared with the normative adult UK population. However, no statistical difference was found in the social domain. Female Apert syndrome patients had a worse QOL than males in the social domain.
  
  
  CONCLUSIONS
  The counterintuitive findings show that adult syndromic patients with similar cognitive capacity perceive their quality of life as being above that experienced in a normative UK nonsyndromic population with no correlation to the degree of facial difference.
authors.affiliation: Department of Craniofacial Surgery, Birmingham Children's Hospital, Birmingham, UK.
authors.forename: Mark S
authors.initials: MS
authors.lastname: Lloyd
authors.forename: Anuradha
authors.initials: A
authors.lastname: Venugopal
authors.forename: Johan
authors.initials: J
authors.lastname: Horton
authors.forename: Desiderio
authors.initials: D
authors.lastname: Rodrigues
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nishikawa
authors.forename: Nicholas
authors.initials: N
authors.lastname: White
authors.forename: Guirish
authors.initials: G
authors.lastname: Solanki
authors.forename: Peter
authors.initials: P
authors.lastname: Noons
authors.forename: Martin
authors.initials: M
authors.lastname: Evans
authors.forename: Stephen
authors.initials: S
authors.lastname: Dover
mesh_terms: D000168:Acrocephalosyndactylia / Q000503:physiopathology / Q000523:psychology; D000328:Adult; D003398:Craniosynostoses / Q000503:physiopathology / Q000523:psychology; D005260:Female; D006801:Humans; D008297:Male; D011788:Quality of Life; D011795:Surveys and Questionnaires; D006113:United Kingdom