Xenos C, Sgouros S, Walsh R, Hockley A
Department of Paediatric Neurosurgery and Institute of Child Health, Birmingham Children's Hospital, Birmingham, UK.
Pediatr Neurosurg. 2000 Jun;32(6):295-307. doi: 10.1159/000028958.
Spinal cord lipomas are a common cause of cord tethering that can lead to progressive neurological defects. The role of prophylactic surgery for spinal lipomas has recently been questioned. Between 1985 and 1999, 59 children underwent a total of 69 surgical procedures at the Birmingham Children's Hospital in Birmingham, UK. The spinal lipomas were classified into: 18 terminal, 17 transitional, 6 dorsal and 18 filum lipomas - including 12 who had a typical thickened filum terminale. At the first operation, 19 patients (32%) were asymptomatic, and 40 patients (68%) presented with symptoms. Surgical indications in the asymptomatic group included the presence of a dermal sinus tract or syrinx. Prophylactic surgery was undertaken in selected cases. The mean total follow-up for the group since the first surgical procedure was 61.8 months (range: 7.0-203.0 months). In the asymptomatic group, 26% of the patients had late neurological deterioration. Of the 14 patients with asymptomatic conus lipomas, 3 (21%) developed sphincter dysfunction and motor problems at long-term follow-up. In the symptomatic group, 68% improved, 20% remained unchanged, and 12% had late neurological deterioration. None of the 18 patients with symptomatic filum lipoma deteriorated postoperatively. However, 39% had bladder dysfunction, 54% had neuro-orthopaedic deformity, and only 15% returned to overall normal function at latest follow-up. Of the 27 patients with symptomatic conus lipomas, 67% improved, 15% remained stable, and 18% had late neurological deterioration. However, 74% had bladder dysfunction, 67% had neuro-orthopaedic deformity, and 45% had motor problems at long-term follow-up. Spinal lipomas can cause progressive neurological deficits irrespective of spinal untethering surgery. This study demonstrates that filum and conus lipomas have similar clinical presentation, but differ in their outcome following surgery. Filum lipomas are 'benign', for which surgery is safe and effective. Conus lipomas are more difficult to manage. When asymptomatic, our results suggest that prophylactic surgery does provide some protection from future neurological deterioration. When symptomatic, conus lipoma surgery is effective in stopping further deterioration. Improvement in neurological function can occur, but few patients return to normal overall function, and pre-existing sphincter dysfunction is not significantly altered by surgery.
脊髓脂肪瘤是导致脊髓栓系的常见原因，可引起进行性神经功能缺损。脊髓脂肪瘤预防性手术的作用最近受到质疑。1985年至1999年间，英国伯明翰市伯明翰儿童医院的59名儿童共接受了69次手术。脊髓脂肪瘤分为：18例终丝型、17例过渡型、6例背侧型和18例终丝脂肪瘤型——其中12例有典型的终丝增厚。初次手术时，19例患者（32%）无症状，40例患者（68%）有症状。无症状组的手术指征包括存在皮样窦道或脊髓空洞症。在部分病例中进行了预防性手术。自首次手术以来，该组患者的平均总随访时间为61.8个月（范围：7.0 - 203.0个月）。在无症状组中，26%的患者出现晚期神经功能恶化。在14例无症状圆锥脂肪瘤患者中，3例（21%）在长期随访中出现括约肌功能障碍和运动问题。在有症状组中，68%的患者病情改善，20%无变化，12%出现晚期神经功能恶化。18例有症状的终丝脂肪瘤患者术后均未恶化。然而，39%的患者有膀胱功能障碍，54%有神经 - 骨科畸形，在最近一次随访时只有15%恢复到整体正常功能。在27例有症状的圆锥脂肪瘤患者中，67%的患者病情改善，15%病情稳定，18%出现晚期神经功能恶化。然而，74%的患者有膀胱功能障碍，67%有神经 - 骨科畸形，45%在长期随访中有运动问题。无论是否进行脊髓松解手术，脊髓脂肪瘤均可导致进行性神经功能缺损。本研究表明，终丝脂肪瘤和圆锥脂肪瘤临床表现相似，但术后结果不同。终丝脂肪瘤是“良性的”，手术安全有效。圆锥脂肪瘤更难处理。无症状时，我们的结果表明预防性手术确实能为未来神经功能恶化提供一定保护。有症状时，圆锥脂肪瘤手术可有效阻止病情进一步恶化。神经功能可有所改善，但很少有患者恢复到整体正常功能，且术前存在的括约肌功能障碍手术并未显著改变。
