Sahuquillo J, Poca M A
Servicio de Neurocirugía, Hospitales Universitarios Vall d'Hebron, Barcelona.
Neurologia. 1998 May;13(5):223-45.
Chiari type I malformation and so-called Chiari-I/syringomyelia complex continue to inspire controversy. Disagreement on these malformations concern both etiopathogenesis and treatment. Our still poor understanding of the natural course of the disease, the lack of consensus regarding therapeutic indications and even disagreement on appropriate surgery all contribute to cause disagreement. Significant progress has been made in our understanding of contributing etiopathogenetic factors in recent years. The most widely accepted hypothesis is that anomalous embryonic development characterized by paraxial mesodermal insufficiency would put volumetric constraints on postnatal development of the posterior fossa. We review historical and current controversies regarding Chiari type I malformation and classic theories on causative and contributing factors. We also discuss the latest surgical treatments that have been suggested, as well as associated anomalies--mainly syringomyelia, hydrocephalus and malformed cranial-cervical articulation. Finally, we propose a protocol useful for the diagnosis and treatment of Chiari type I malformation associated with dilation of the ventricular system.
Chiari I型畸形以及所谓的Chiari-I/脊髓空洞症复合体仍然存在争议。关于这些畸形的分歧涉及病因发病机制和治疗。我们对该疾病自然病程的了解仍然不足，在治疗指征方面缺乏共识，甚至在合适的手术方式上也存在分歧，所有这些都导致了争议。近年来，我们对促成病因发病机制的因素的理解取得了重大进展。最被广泛接受的假说是，以轴旁中胚层不足为特征的异常胚胎发育会对后颅窝的出生后发育造成体积限制。我们回顾了关于Chiari I型畸形的历史和当前争议以及关于病因和促成因素的经典理论。我们还讨论了已提出的最新手术治疗方法以及相关异常情况——主要是脊髓空洞症、脑积水和颅颈关节畸形。最后，我们提出了一个对诊断和治疗与脑室系统扩张相关的Chiari I型畸形有用的方案。
