Dutch Craniofacial Center, Erasmus MC, Sophia Children's hospital, Department of plastic surgery, SK-1202, PO Box 2060, 3000 CB Rotterdam, the Netherlands.
J Plast Reconstr Aesthet Surg. 2010 Oct;63(10):1635-41. doi: 10.1016/j.bjps.2009.10.029. Epub 2009 Nov 12.
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.
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.
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.
Based on these data, a syndrome-specific risk profile with suggestions for screening and treatment is presented.
颅缝早闭综合征患者颅内压升高（ICP）、阻塞性睡眠呼吸暂停（OSA）、受教育程度、语言和运动技能、视力和听力受损的长期患病率知之甚少。本研究的目的是确定每个综合征 ICP 升高、OSA、视力和听力受损的患病率。
对 1983 年至 2008 年间诊断为 Apert、Crouzon、Pfeiffer、Muenke 或 Saethre-Chotzen 综合征的 167 例连续患者进行回顾性研究，年龄为 1-25 岁。转诊和复查时的平均年龄分别为 1 岁 2 个月和 10 岁 3 个月。
Apert 和 Crouzon/Pfeiffer 综合征患者 ICP 升高（分别为 33%和 53%）和 OSA（分别为 31%和 27%）的患病率最高，而 Saethre-Chotzen 综合征也与 ICP 升高的风险相当（21%）。所有综合征的视力受损（61%）和听力受损（56%）的患病率都很高。
根据这些数据，提出了一种基于综合征的风险特征，并提出了筛查和治疗建议。
