Lewandrowski Kai Uwe, Rachlin Jacob R, Glazer Paul A
Department of Orthopedic Surgery, Beth Israel Deaconess Medical Center, 330 Brookline Ave., Boston, MA 02215, USA.
Spine J. 2004 Jan-Feb;4(1):116-9. doi: 10.1016/j.spinee.2003.08.028.
Diastematomyelia is uncommon and rarely presents in adulthood. This report draws attention to the fact that patients who underwent spinal fusion for deformity before the widespread use of computed tomography (CT) and magnetic resonance imaging (MRI) may have unrecognized spinal cord abnormalities. This should be considered if revision surgery is contemplated.
This case report focuses on the late presentation of lower-extremity weakness in a 44-year-old woman with a split cord malformation (diplomyelia), diastematomyelia and tethered cord syndrome.
The patient underwent instrumented posterior spinal fusion with a Harrington rod as a child for progressive thoracolumbar scoliosis. As an adult, she developed paraparesis after a traumatic event. The patient underwent decompressive laminectomy, subtotal resection of the old fusion mass and resection of the osseous septum. Postoperatively, an anterior spinal fluid leak in the lower thoracic region required repeated fascial grafting, resection of a pseudomeningocele and reverse left latissimus dorsi flap transfer. The leak was controlled, and the patient had near complete resolution of her paraparesis 1 year after her surgery.
The case described herein is unusual in that patients with diplomyelia and diastematomyelia rarely are symptomatic in adulthood. However, trauma may precipitate the onset of neurologic symptoms. This patient underwent spinal surgeries to address deformity, pain and progressive lower-extremity weakness. Preoperative CT and MRI studies showed a split cord malformation and diastematomyelia at L1-L2 with spinal stenosis and tethering of both hemicords.
Progressive weakness without any previous neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may develop in the adult with unrecognized diastemotomyelia. This case demonstrates that a thorough preoperative workup of patients with complex spinal deformities is imperative.
脊髓纵裂并不常见，在成年期很少出现。本报告提醒注意，在计算机断层扫描（CT）和磁共振成像（MRI）广泛应用之前接受脊柱融合治疗畸形的患者，可能存在未被识别的脊髓异常。如果考虑进行翻修手术，应考虑到这一点。
本病例报告聚焦于一名44岁患有脊髓纵裂畸形（双脊髓畸形）、脊髓纵裂和脊髓拴系综合征的女性下肢无力的迟发性表现。
该患者小时候因进行性胸腰椎侧弯接受了哈灵顿棒器械辅助后路脊柱融合术。成年后，她在一次创伤事件后出现了双下肢轻瘫。患者接受了减压性椎板切除术、旧融合块次全切除术和骨隔切除术。术后，下胸部区域出现脑脊液漏，需要反复进行筋膜移植、假性脊膜膨出切除术和左背阔肌翻转皮瓣转移术。漏液得到控制，患者术后1年双下肢轻瘫几乎完全缓解。
本文所述病例不同寻常之处在于，患有双脊髓畸形和脊髓纵裂的患者在成年期很少出现症状。然而，创伤可能促使神经症状发作。该患者接受了脊柱手术以解决畸形、疼痛和进行性下肢无力问题。术前CT和MRI检查显示L1-L2水平存在脊髓纵裂畸形和脊髓纵裂，伴有椎管狭窄和双侧半脊髓拴系。
未被识别的脊髓纵裂成年患者可能会出现渐进性无力，之前无任何神经功能缺损或潜在脊髓异常的神经皮肤体征。本病例表明，对复杂脊柱畸形患者进行全面的术前检查至关重要。
