Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston.
Department of Neurosurgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
World Neurosurg. 2022 Jul;163:24. doi: 10.1016/j.wneu.2022.03.132. Epub 2022 Apr 6.
Syringomyelia is often resistant to various treatment modalities. Chiari I malformations are associated with syringomyelia in approximately 69% of operative cases. Failure to resolve syringomyelia after Chiari decompression is common. The pathophysiology of Chiari-associated syringomyelia has been well studied, with Oldfield emphasizing the water-hammer mechanism, with treatment limited to bony decompression and duraplasty. On the other hand, capacious fourth ventricular drainage is thought to be essential for syrinx resolution. Persistence or progression of the syrinx after decompression is an indication for reoperation. Direct shunting of the syrinx is associated with high failure rates. The technique of shunting the fourth ventricle has been applied successfully in the pediatric population. We emphasize the need to ensure outflow from the fourth ventricle in Chiari decompressions associated with syringomyelia. In revisions to treat progressive syringomyelia after failed decompression, we undertake the following steps: 1) adequate lateral bony decompression, 2) lysis of scar/adhesions around the cisterna magna, 3) opening the fourth ventricle outlet by releasing any web/adhesions, 4) insertion of a shunt from the fourth ventricle to the cervical subarachnoid space, and 5) bipolar coagulation of the lateral tonsillar pia to maintain patency of cerebrospinal fluid pathways. We favor autologous fascia or pericranium for expansile duraplasty, as the use of nonautologous materials may cause excessive scarring. In this video, we demonstrate these tenets in 3 cases of Chiari-associated syringomyelia, 2 revisions and 1 primary case, with excellent resolution of the syrinx (Video 1). The patients consented to surgery and publication of images.
脊髓空洞症通常对各种治疗方法有抵抗力。Chiari I 畸形约占手术病例中脊髓空洞症的 69%。Chiari 减压后脊髓空洞症未得到解决的情况很常见。Chiari 相关脊髓空洞症的病理生理学已经得到了很好的研究，Oldfield 强调了水锤机制，治疗仅限于骨减压和硬脑膜成形术。另一方面，认为第四脑室宽大引流对于脊髓空洞症的解决至关重要。减压后脊髓空洞症持续存在或进展是再次手术的指征。分流术直接引流脊髓空洞症与高失败率相关。第四脑室分流术技术已成功应用于儿科人群。我们强调在 Chiari 减压相关脊髓空洞症中，需要确保第四脑室的流出。在治疗减压失败后进展性脊髓空洞症的翻修手术中，我们采取以下步骤：1）充分的侧骨减压，2）溶解枕大池周围的疤痕/粘连，3）通过释放任何网络/粘连打开第四脑室出口，4）将分流管从第四脑室插入颈蛛网膜下腔，5）双极电凝外侧扁桃体软脑膜以保持脑脊液通路通畅。我们赞成使用自体筋膜或颅骨膜进行可扩张硬脑膜成形术，因为使用非自体材料可能会导致过度瘢痕形成。在这个视频中，我们在 3 例 Chiari 相关脊髓空洞症、2 例翻修和 1 例原发性病例中演示了这些原则，脊髓空洞症得到了极好的解决（视频 1）。患者同意手术和发布图像。
