Davidoff Christopher L, Liu Shinuo, Wong Johnny H Y, Koustais Stavros, Rogers Jeffrey M, Stoodley Marcus A
Department of Clinical Medicine, Faculty of Medicine and Health Sciences, Macquarie University, New South Wales, Australia.
Department of Clinical Medicine, Faculty of Medicine and Health Sciences, Macquarie University, New South Wales, Australia.
World Neurosurg. 2017 Nov;107:565-573. doi: 10.1016/j.wneu.2017.08.064. Epub 2017 Aug 24.
Craniocervical junction arachnoiditis (CCJA) is an uncommon cause of syringomyelia. The pathophysiology of syrinx formation is uncertain, and the appropriate management unclear. A series of cases is reported to demonstrate variations in etiology, uniformity of functional cerebrospinal fluid obstruction at the foramen magnum, and results of surgical intervention.
We retrospectively analyzed the clinical and radiologic features of a consecutive series of patients treated for syringomyelia related to CCJA.
Eight patients (5 male, 28-66 years old) were treated from 2000 to 2016. Magnetic resonance imaging demonstrated cervicothoracic syringomyelia in all cases, with the rostral extension of the syrinx suggesting communication with the fourth ventricle in all but one case. There was reduction of foramen magnum cerebrospinal fluid space in all cases, cerebellar ectopia in 5 cases, and fourth ventricular entrapment in 3 cases. Treatment consisted of posterior fossa decompression with either a GoreTex or pericranial patch graft. Six patients had a fourth-ventricle spinal subarachnoid shunt. Two patients had titanium mesh cranioplasty. The immediate postoperative period was associated with reduction in syrinx cavity size and improvement in neurologic symptoms in all cases. At follow-up 10-60 months postoperatively, 3 patients exhibited recurrence of the syrinx and underwent successful reoperation at the craniocervical junction. One patient with persistence of the inferior component of the syrinx was treated with a syrinx-spinal subarachnoid shunt.
Most syrinx cavities associated with CCJA communicate with the fourth ventricle. Posterior fossa decompression and fourth ventricle to spinal subarachnoid space shunting appears a reasonable treatment for this form of syringomyelia.
颅颈交界区蛛网膜炎（CCJA）是脊髓空洞症的一种罕见病因。脊髓空洞形成的病理生理学尚不确定，且合适的治疗方法也不明确。本文报告一系列病例，以展示病因的差异、枕骨大孔处功能性脑脊液梗阻的一致性以及手术干预的结果。
我们回顾性分析了一系列因CCJA相关脊髓空洞症接受治疗的患者的临床和影像学特征。
2000年至2016年期间，共治疗了八名患者（5名男性，年龄28 - 66岁）。磁共振成像显示所有病例均有颈胸段脊髓空洞症，除1例病例外，其余病例脊髓空洞向上延伸提示与第四脑室相通。所有病例均存在枕骨大孔脑脊液间隙减小，5例有小脑扁桃体下疝，3例有第四脑室受压。治疗包括使用GoreTex或帽状腱膜修补片进行后颅窝减压。6例患者进行了第四脑室 - 脊髓蛛网膜下腔分流术。2例患者进行了钛网颅骨成形术。所有病例术后即刻脊髓空洞腔大小均减小，神经症状改善。术后10 - 60个月随访时，3例患者脊髓空洞复发，并在颅颈交界区成功进行了再次手术。1例脊髓空洞下部持续存在的患者接受了脊髓空洞 - 脊髓蛛网膜下腔分流术。
大多数与CCJA相关的脊髓空洞腔与第四脑室相通。后颅窝减压及第四脑室至脊髓蛛网膜下腔分流术似乎是这种形式脊髓空洞症的合理治疗方法。
