Division of Neurosurgery, Department of Neurosciences, University of Torino, Turin, Italy.
Division of Neurosurgery, Department of Neurosciences, University of Torino, Turin, Italy.
World Neurosurg. 2019 Oct;130:499-505. doi: 10.1016/j.wneu.2019.07.004. Epub 2019 Jul 9.
Syringobulbia is an uncommon lesion that occurs in the central nervous system; it is often defined as a pathologic cavitation in the brainstem. The cases with partial blockage of the cerebrospinal fluid pathways at the level of the foramen magnum are more common and the most important group. The most common treatment of syringobulbia is craniovertebral decompression.
This paper reports a case of a symptomatic syringobulbia in which an urgent endoscopic endonasal approach to the craniovertebral junction (CVJ) was done to limit bulbo-medullary compression and rapid neurologic deterioration. A 69-year-old man was admitted to the hospital because of acute onset of dysphonia, dysphagia, imbalance, and vomiting. Magnetic resonance imaging revealed a cystic lesion in the brainstem, suggestive of a syringobulbia in Klippel Feil syndrome with CVJ stenosis.
This case report details the successful use of endoscopic endonasal anterior decompression to treat syringobulbia, and adds to the growing literature in support of the endonasal endoscopic approach as a safe and feasible means for decompressing the craniocervical junction, even in the setting of urgency. However, prudent patient selection, combined with sound clinical judgment, access to instrumentation, and intraoperative imaging cannot be overemphasized.
延髓空洞症是一种发生在中枢神经系统的罕见病变；通常被定义为脑干的病理性空洞。在颅颈交界区（CVJ）水平存在部分脑脊液通路阻塞的病例更为常见，也是最重要的一组。延髓空洞症的最常见治疗方法是颅颈减压。
本文报告了一例症状性延髓空洞症患者，该患者因延髓-脊髓压迫和快速神经功能恶化，行紧急经鼻内镜颅颈交界区（CVJ）手术。一名 69 岁男性因急性发声困难、吞咽困难、失衡和呕吐而入院。磁共振成像显示脑干囊性病变，提示 Klippel Feil 综合征伴 CVJ 狭窄的延髓空洞症。
本病例报告详细介绍了经鼻内镜前路减压术治疗延髓空洞症的成功应用，并为支持经鼻内镜入路作为一种安全可行的方法来减压颅颈交界区，增加了越来越多的文献支持，即使在紧急情况下也是如此。然而，谨慎的患者选择、结合良好的临床判断、器械的获得和术中成像都不能被过分强调。
