Health-related quality of life in children and adolescents with syndromic craniosynostosis

Bannink, Natalja; Maliepaard, Marianne; Raat, Hein; Joosten, Koen F.M.; Mathijssen, Irene M.J.

doi:10.1016/j.bjps.2010.01.036

« Previous Next »Journal of Plastic, Reconstructive & Aesthetic Surgery
Volume 63, Issue 12 , Pages 1972-1981, December 2010

Health-related quality of life in children and adolescents with syndromic craniosynostosis☆

Natalja Bannink
- Dutch Craniofacial Center, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, the Netherlands
- Corresponding author. Erasmus MC- Sophia Children's Hospital, Department of Plastic Surgery, Sk-1202, P.O. box 2060, 3000 CB Rotterdam, The Netherlands. Tel.: +31107036818; fax: +31107036844.
Marianne Maliepaard
- Dutch Craniofacial Center, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, the Netherlands
Hein Raat
- Department of Public Health, Erasmus Medical Center, Rotterdam, the Netherlands
Koen F.M. Joosten
- Dutch Craniofacial Center, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, the Netherlands
Irene M.J. Mathijssen
- Dutch Craniofacial Center, Erasmus Medical Center Sophia Children's Hospital, Rotterdam, the Netherlands

Received 16 November 2009; accepted 23 January 2010. published online 11 March 2010.

Summary

Syndromic craniosynostosis is a congenital disorder characterised by premature fusion of calvarial sutures combined with other anomalies. The facial appearance is different and patients may show physical impairment, mental or developmental disabilities, elevated intracranial pressure and obstructive sleep apnoea. The impact of this condition on daily functioning has not been studied before. The aim of this study is to assess the health-related quality of life in children and adolescents with syndromic or complex craniosynostosis and to determine the impact of these syndromes on parents.

A prospective study was performed in 111 children. Health-related quality of life was measured by international standardised quality-of-life questionnaires, the Infant Toddler Quality of Life Questionnaire (ITQoL), Child Health Questionnaire Parental Form 50 (CHQ-PF50), Child Health Questionnaire Child Form 87 (CHQ-CF87) and Short-Form Health Survey (SF-36). For comparison, we used Dutch population norms of health-related quality-of-life-scores.

Parents' scores for patients with syndromic or complex craniosynostosis were significantly lower than those for the norm population. Apert syndrome had the largest impact on the different domains. Scores on the CHQ-PF50 scales for ‘physical functioning’, ‘parental impact emotional’ and ‘family activities’ for these patients were significantly lower than scores for patients with other syndromes, possibly due to the complexity of the syndrome, which includes complex syndactyly, cognitive impairment and behaviour problems. Parents reported a reduced health-related quality of life for themselves, mostly psychosocial with clearly significantly lower general health perceptions.

In conclusion, syndromic craniosynostosis has a large impact on the health-related quality of life of these children and their parents, both physical and psychosocial.

Keywords: Quality of life, Syndromic craniosynostosis, Children