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颅骨缝早闭中的颅盖骨生长

Skull vault growth in craniosynostosis.

作者信息

Sgouros Spyros

机构信息

Department of Craniofacial Surgery, Birmingham Children's Hospital, Birmingham, UK.

出版信息

Childs Nerv Syst. 2005 Oct;21(10):861-70. doi: 10.1007/s00381-004-1112-2. Epub 2005 Mar 25.

Abstract

BACKGROUND

Since its first description by Virchow, the principle of abnormal skull growth due to restriction of skull growth at the fused sutures, and the realisation by Moss that the sutures at the skull base are equally affected, have been the main intellectual driving forces behind the majority of cranial expansion procedures performed currently in children with craniosynostosis.

CURRENT OBSERVATIONS

Despite original impressions that craniosynostosis leads to craniostenosis, many studies have demonstrated that in the majority of patients with craniosynostosis there is normal skull volume in those over the age of 6 months. In Apert syndrome, skull volume is invariably larger than normal. Some studies have shown that intracranial pressure is independent of intracranial volume, and can exist in the presence of normal volume, or indeed after cranial expansion. These observations imply that cranial expansion procedures create a state of artificially increased skull volume, in the quest to improve appearance and function.

FUTURE ADVANCES

This creates a new angle of view through which skull growth abnormalities are seen. It is becoming clearer that in most patients with craniosynostosis, there is regional imbalance of skull growth, which co-exists with a variety of other equally important factors, such as genetic defects, raised intracranial pressure, venous hypertension, and other brain parenchymal anomalies such as hindbrain hernia or hydrocephalus. It is becoming increasingly obvious that the type of surgical treatment currently practised in most cases is conceptually incorrect. Recent modifications such as the use of springs or distraction do not escape from the underlying philosophy of cranial expansion. With that in mind, it is hoped that advances in the fields of genetics and molecular biology will provide treatments for the cause of craniosynostosis rather than the symptomatic relief that surgery offers currently.

CONCLUSION

Until then, there is a need to develop better ways of quantifying regional abnormalities of skull growth.

摘要

背景

自魏尔啸首次描述以来,因颅骨融合缝线处生长受限导致颅骨异常生长的原理,以及莫斯认识到颅底缝线同样受到影响,一直是目前大多数针对儿童颅缝早闭症所进行的颅骨扩张手术背后的主要理论驱动力。

当前观察结果

尽管最初认为颅缝早闭会导致颅骨狭窄,但许多研究表明,在大多数6个月以上的颅缝早闭症患者中,颅骨体积是正常的。在Apert综合征中,颅骨体积总是大于正常。一些研究表明,颅内压与颅内体积无关,在颅内体积正常时或颅骨扩张后都可能存在。这些观察结果意味着,颅骨扩张手术制造了一种人为增加颅骨体积的状态,旨在改善外观和功能。

未来进展

这为观察颅骨生长异常创造了一个新视角。越来越清楚的是,在大多数颅缝早闭症患者中,存在颅骨生长的区域不平衡,同时还存在多种其他同样重要的因素,如基因缺陷、颅内压升高、静脉高压以及其他脑实质异常,如后脑疝或脑积水。越来越明显的是,目前大多数情况下实施的手术治疗方式在概念上是错误的。最近的改进措施,如使用弹簧或牵张技术,并未摆脱颅骨扩张的基本理念。考虑到这一点,希望遗传学和分子生物学领域的进展将为颅缝早闭症的病因提供治疗方法,而不是目前手术所提供的症状缓解。

结论

在此之前,需要开发更好的方法来量化颅骨生长的区域异常。

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