先天性脊柱侧凸合并椎管内占位的诊断和治疗

Diagnosis and treatment of congenital scoliosis associated with intraspinal mass

中文关键词:  先天性脊柱侧凸  椎管内占位  诊断  手术

英文关键词:Congenital scoliosis  Intraspinal mass  Diagnosis  Treatment

中文摘要:

  【摘要】 目的：探讨先天性脊柱侧凸合并椎管内占位的发病率、临床表现、影像学特征及手术治疗效果。方法：回顾性分析2007年1月~ 2016年6月于西京医院骨科接受手术治疗的495例先天性脊柱侧凸患者的临床资料，其中7例合并椎管内占位。男5例，女2例；年龄11~31岁，平均19.14±7.52岁。MRI检查示7例全部位于胸段椎管内。5例位于髓内，1例位于髓外硬膜内，1例髓内及髓外硬膜内均有侵及。根据患者临床表现及影像学特征，诊断为先天性脊柱侧凸合并椎管内占位。7例患者均行一期后路脊柱侧凸矫形及椎管内占位切除术。随访观察治疗效果，疗效评价指标包括侧凸矫正率、手术并发症、椎管内占位复发情况。结果：术后随访27~99个月，平均49.7±32.9个月。先天性脊柱侧凸合并椎管内占位患者占本单位先天性脊柱侧凸患者的1.41%。术前行MRI检查，椎管内占位均准确定位；4例定性诊断与术后病理诊断一致。7例均行术后病理学检查，示畸胎瘤2例，表皮样囊肿2例，皮样囊肿1例，支气管源性囊肿1例，毛细胞型星形细胞瘤1例。术后冠状面主弯矫正率（55.05±18.75）%，术后矢状面主弯矫正率为（41.53±19.43）%。末次随访未见明显矫形丢失。术后有1例患者出现暂时性神经损害，半年后恢复；1例患者术后出现脑脊液漏，引流7d后治愈。1例患者于术后5年随访时发现椎管内占位复发，再次行椎管内占位切除术。结论：先天性脊柱侧凸合并椎管内占位发病率低；临床表现、影像学检查及病理学检查对其诊断均有重要价值。一期手术治疗先天性脊柱侧凸合并椎管内占位是安全有效的。

英文摘要:

  【Abstract】 Objectives: To analyze the clinical characteristics of congenital scoliosis associated with intraspinal mass, and to investigate the incidence, clinical manifestations, imaging characteristics and surgical outcomes. Methods: The clinical data of congenital scoliosis patients associated with intraspinal mass who underwent surgical treatment from January 2007 to June 2016 in Department of Orthopedics, Xijing Hospital were retrospectively reviewed. There were 2 females and 5 males with an average age of 19.14±7.52 years (range, 11-31 years). MRI showed the intraspinal masses were all located at thoracic spine. Five of the intraspinal masses were intramedullary, one was intradural extramedullary and one was both of the intramedullary and intradural extramedullary. All of the patients were treated with one-stage intraspinal mass resection and scoliosis correction. Clinical evaluation included scoliosis correction rate, incidence of complications and recurrence rate. Results: Patients were observed for a minimum of 24 months after initial surgical treatment with an average follow-up of 49.71±32.90 months(range, 27-99 months). Congenital scoliosis associated with intraspinal mass accounted for 1.41% of congenital scoliosis. MRI could show the location of all intraspinal mass and four patients had the same pathological　results. Postoperative pathological　examination showed two cases of epidermoid cyst, one case of dermoid cyst, one case of bronchogenic cyst, two cases of teratoma and one case of pilomyxoid astrocytoma. The major coronal curve correction rate was (55.05±18.75)% and the major sgittal curve correction rate was (41.53±19.43)%. There was no obvious loss of correction at final follow-up. There were one patient with transient neurological deterioration and one patient with cerebrospinal fluid leakage. One patient recurred at the 5-year follow-up in this study. There was no paralysis or permanent nerve damage. Conclusions: The incidence of congenital scoliosis associated with intraspinal mass is low. Diagnosis mainly depends on clinical manifestations, imaging characteristics and pathological examination. Simultaneous intraspinal mass resection and scoliosis correction appears to be safe and effective.