Durham S, Sun P, Schut L
División de Neurocirugía, Children's Hospital of Philadelphia, Pennsylvania 19104, USA.
Rev Neurol. 1998 Aug;27(156):231-7.
The association of hindbrain herniation, better known as Chiari malformation, and cystic cavitation of the spinal cord or hydrosyringomyelia has been well described in the literature although there is little consensus regarding its etiology, pathophysiology or optimal treatment.
Despite a well-accepted and utilized classification system of both Chiari malformation and hydrosyringomyelia, there remains considerable disagreement regarding the proper management of these patients.
This article reviews several popular theories on the etiology and pathophysiology of this disorder, briefly discusses the clinical features and radiologic findings associated with Chiari malformation and hydrosyringomyelia, and reviews basic surgical techniques for decompression of the cranial-cervical junction and treatment of the hydrosyringomyelia.
延髓下疝畸形，更广为人知的是Chiari畸形，与脊髓空洞症或积水性脊髓空洞症之间的关联在文献中已有详尽描述，尽管在其病因、病理生理学或最佳治疗方法上几乎没有共识。
尽管Chiari畸形和积水性脊髓空洞症都有被广泛接受和使用的分类系统，但在这些患者的恰当管理方面仍存在相当大的分歧。
本文回顾了关于这种疾病病因和病理生理学的几种流行理论，简要讨论了与Chiari畸形和积水性脊髓空洞症相关的临床特征和影像学表现，并回顾了颅颈交界区减压和积水性脊髓空洞症治疗的基本外科技术。
