Long-term functional outcome in 167 patients with syndromic craniosynostosis; defining a syndrome-specific risk profile

Summary Objective Little is known about the long-term prevalence of elevated intracranial pressure (ICP), obstructive sleep apnoea (OSA), level of education, language and motor skills, impaired sight and hearing in craniosynostosis syndromes. The objective of this study was to define the prevalence...

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Bibliographic Details
Published in:	Journal of plastic, reconstructive & aesthetic surgery reconstructive & aesthetic surgery, 2010-10, Vol.63 (10), p.1635-1641
Main Authors:	de Jong, T, Bannink, N, Bredero-Boelhouwer, H.H, van Veelen, M.L.C, Bartels, M.C, Hoeve, L.J, Hoogeboom, A.J.M, Wolvius, E.B, Lequin, M.H, van der Meulen, J.J.N.M, van Adrichem, L.N.A, Vaandrager, J.M, Ongkosuwito, E.M, Joosten, K.F.M, Mathijssen, I.M.J
Format:	Article
Language:	eng
Subjects:	Adolescent Adult Biological and medical sciences Chi-Square Distribution Child Child, Preschool Craniosynostoses - complications Craniosynostoses - surgery Craniosynostosis Development Diseases of the osteoarticular system Female Hearing Hearing Disorders - epidemiology Hearing Disorders - etiology Hearing Disorders - surgery Humans ICP Infant Intracranial Hypertension - epidemiology Intracranial Hypertension - etiology Intracranial Hypertension - surgery Male Malformations and congenital and or hereditary diseases involving bones. Joint deformations Medical sciences OSA Plastic Surgery Prevalence Recovery of Function Retrospective Studies Sight Sleep Apnea, Obstructive - epidemiology Sleep Apnea, Obstructive - etiology Sleep Apnea, Obstructive - surgery Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases Syndrome Treatment Outcome Vision Disorders - epidemiology Vision Disorders - etiology Vision Disorders - surgery
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Summary:	Summary Objective Little is known about the long-term prevalence of elevated intracranial pressure (ICP), obstructive sleep apnoea (OSA), level of education, language and motor skills, impaired sight and hearing in craniosynostosis syndromes. The objective of this study was to define the prevalence per syndrome of elevated ICP, OSA, impaired sight and impaired hearing. Methods A retrospective study was undertaken on 167 consecutive patients diagnosed with Apert, Crouzon, Pfeiffer, Muenke or Saethre–Chotzen syndrome, aged 1–25 years and treated between 1983 and 2008. The mean age at time of referral and review was 1 years and 2 months and 10 years and 3 months, respectively. Results Patients with Apert and Crouzon/Pfeiffer syndromes had the highest prevalence of elevated ICP (33% and 53%, respectively) and OSA (31% and 27%, respectively), while Saethre–Chotzen syndrome was also associated with a fair risk for elevated ICP (21%). The prevalence of impaired sight (61%) and hearing (56%) was high in all syndromes. Conclusion Based on these data, a syndrome-specific risk profile with suggestions for screening and treatment is presented.
ISSN:	1748-6815 1878-0539