Morgan D F, Young R F
J Neurosurg. 1980 Apr;52(4):463-72. doi: 10.3171/jns.1980.52.4.0463.
Spinal neurological complications caused the admission of 17 patients with achondroplasia to the UCLA affiliated hospitals between 1955 and 1979. These patients constituted 41% of all achondroplastic patients admitted during that period. The spinal stenotic syndromes could be divided into three groups: Group I: thoracolumbar stenosis (10 patients); Group II: foramen magnum and upper cervical stenosis (five patients); and Group III: generalized spinal stenosis (two patients). Eleven patients underwent a total of 18 decompressive operative procedures for treatment of paraparesis, quadriparesis, sensory deficits, and sphincter dysfunction. Excellent results were obtained with patients in Group I and II, 77% of whom were ambulatory and continent postoperatively. Two patients in Group III fared less well, showing steady neurological deterioration despite multiple operative procedures. The spectrum of spinal neurological manifestations secondary to achondroplasia is reviewed. Problems with conventional radiological studies and the potential role of computerized tomographic analysis of such patients are discussed. Recommendations for surgical technique are made. Early recognition, prompt clinical evaluation, and safe and accurate radiological analysis of spinal neurological complications of achondroplasia will allow appropriate decompressive surgical procedures to be performed. Excellent results may be anticipated in the reversal and prevention of neurological deficit secondary to achondroplasia with such an approach.
1955年至1979年间，脊柱神经并发症导致17名软骨发育不全患者入住加州大学洛杉矶分校附属医院。这些患者占该时期所有入院软骨发育不全患者的41%。脊柱狭窄综合征可分为三组：第一组：胸腰椎狭窄（10例）；第二组：枕骨大孔和上颈椎狭窄（5例）；第三组：广泛性脊柱狭窄（2例）。11名患者共接受了18次减压手术，以治疗截瘫、四肢瘫、感觉障碍和括约肌功能障碍。第一组和第二组患者取得了良好的效果，其中77%的患者术后能够行走且大小便正常。第三组的2名患者情况较差，尽管接受了多次手术，但神经功能仍持续恶化。本文回顾了软骨发育不全继发的脊柱神经表现谱。讨论了传统放射学检查的问题以及计算机断层扫描分析在此类患者中的潜在作用。提出了手术技术建议。对软骨发育不全的脊柱神经并发症进行早期识别、及时临床评估以及安全准确的放射学分析，将有助于进行适当的减压手术。采用这种方法有望在逆转和预防软骨发育不全继发的神经功能缺损方面取得良好效果。
