Borius P-Y, Cintas P, Lagarrigue J
Service de neurochirurgie, hôpital Rangueil, CHU de Toulouse, 1, avenue Jean-Poulhès, TSA 50032, 31059 Toulouse cedex 9, France.
Neurochirurgie. 2010 Oct;56(5):386-90. doi: 10.1016/j.neuchi.2009.12.006. Epub 2010 Jan 25.
The ventriculus terminalis (VT) is a virtual cavity of the conus medullaris that appears during embryonic life. The occurrence of its dilatation is very rare in adulthood. Only 27 cases have been documented so far. We present the case of a 47-year-old woman who presented with hypoesthesia and dysesthesia of the lower limb, distal paresis, and urinary dysfunction evolving for the past 5 years. Electrophysiology confirmed radiculopathy. MRI revealed a cystic dilatation of the VT. Surgery consisted in laminectomy with myelotomy and fenestration. Histology showed a simple cubic ependymal epithelium. Progression has been favorable concerning the motor deficit and sphincter dysfunction but neuropathic pain has persisted for 6 months. The data are similar to those found in the literature. The sex ratio is 6:21, the mean age is 45. Symptoms appear on average over 2 years with lower limb pain (71%), sphincter dysfunction (71%), lumbago (64%), motor deficits (57%), and sensory disorders (47%). Imaging demonstrated cystic dilatation in the conus medullaris filled with cerebrospinal fluid without contrast enhancement. The histology analysis showed a simple ependymal epithelium with no tumoral process. The fenestration of the cyst allows complete recovery of symptoms in 52% of cases and partial recovery in 43%. The VT seems to be a whole nosologic entity in itself. Myelotomy with fenestration provides good results on motor and sphincter symptoms, whereas the benefit in terms of pain is subject to discussion.
终室是脊髓圆锥的一个虚拟腔隙,在胚胎期出现。其扩张在成年期非常罕见。迄今为止,仅有27例病例被记录。我们报告一例47岁女性病例,该患者在过去5年中出现下肢感觉减退和感觉异常、远端轻瘫及排尿功能障碍。电生理学检查证实为神经根病。磁共振成像显示终室呈囊性扩张。手术包括椎板切除术、脊髓切开术和开窗术。组织学检查显示为单层立方室管膜上皮。运动功能缺损和括约肌功能障碍方面病情进展良好,但神经性疼痛持续了6个月。这些数据与文献报道的相似。男女比例为6:21,平均年龄为45岁。症状平均出现2年,其中下肢疼痛(71%)、括约肌功能障碍(71%)、腰痛(64%)、运动功能缺损(57%)和感觉障碍(47%)。影像学检查显示脊髓圆锥内囊性扩张,充满脑脊液,无强化。组织学分析显示为单层室管膜上皮,无肿瘤性病变。囊肿开窗术在52%的病例中可使症状完全缓解,43%的病例部分缓解。终室本身似乎是一个完整的疾病实体。脊髓切开术加开窗术对运动和括约肌症状疗效良好,而在疼痛方面的益处有待探讨。