Department of Neurosurgery, The University of Tokyo, Tokyo, Japan.
Department of Neurosurgery, The University of Tokyo, Tokyo, Japan.
World Neurosurg. 2022 Nov;167:e127-e136. doi: 10.1016/j.wneu.2022.07.118. Epub 2022 Aug 6.
Syringomyelia often accompanies spinal hemangioblastoma (SHB). It often shows progression to the medulla oblongata, dubbed as "syringobulbia", which presents critical symptoms such as dysphagia and respiratory compromise. Appropriate management of chronological syringomyelia progression toward syringobulbia is not set in stone. This study aims to unravel the clinical and chronological behavior of syringobulbia and its management.
A single-institution case series study of 5 patients operated for SHB with syringobulbia was conducted. Serial preoperative magnetic resonance imaging scans were analyzed in further details, especially focusing on the chronological progression of syringomyelia. A literature review was performed to describe clinical/imaging characteristics.
Chronological imaging analyses revealed that despite the relatively steady progression of syringomyelia over years, it accelerated when developing syringobulbia. Intramedullary signal change ("presyringomyelia") was observed in the area where syringomyelia subsequently occurred. Literature review yielded another 15 cases of SHB with syringobulbia, totaling 20 cases. Bulbar dysfunction was seen in 4 cases (20%). Gross total resection was performed in all cases except 1, which underwent just syringotomy. Rapid postoperative symptom improvement was observed in all cases, as well as immediate imaging resolution of syringomyelia.
The symptoms associated with syringobulbia often become life-threatening. Notably, its resolution may be near-synchronous to surgical resection of the spinal lesion. The speed of progression of syringomyelia is usually steady, but it may accelerate when extending to syringobulbia. Regular imaging follow-up is thus highly recommended to determine the best timing of intervention when presyringomyelia and syringomyelia are ascending toward the medulla oblongata.
脊髓血管母细胞瘤（SHB）常伴有脊髓空洞症。它常向延髓发展，称为“延髓空洞症”，表现为吞咽困难和呼吸功能障碍等危急症状。对延髓空洞症进行适当的管理尚未达成共识。本研究旨在阐明延髓空洞症的临床和时间进展及其管理。
对 5 例因 SHB 伴延髓空洞症而行手术治疗的患者进行单机构病例系列研究。对连续的术前磁共振成像扫描进行了更详细的分析，特别是对脊髓空洞症的时间进展进行了分析。还进行了文献复习，以描述临床/影像学特征。
时间成像分析显示，尽管脊髓空洞症多年来相对稳定地进展，但当发展为延髓空洞症时，其进展速度会加快。在随后发生脊髓空洞症的区域观察到了脊髓内信号变化（“脊髓空洞前期”）。文献复习还发现了另外 15 例 SHB 伴延髓空洞症，总计 20 例。其中 4 例（20%）出现延髓功能障碍。所有病例均行全切除，除 1 例外仅行脊髓空洞切开术。所有病例术后症状均迅速改善，脊髓空洞症立即得到影像学缓解。
延髓空洞症相关症状常危及生命。值得注意的是，脊髓空洞症的缓解可能与脊髓病变的手术切除同步。脊髓空洞症的进展速度通常是稳定的，但当向延髓空洞症延伸时可能会加速。因此，强烈建议定期进行影像学随访，以确定在脊髓空洞前期和脊髓空洞症向延髓上升时进行干预的最佳时机。
