空洞分流对Chiari Ⅰ畸形伴脊髓空洞症近远期疗效的影响

Effect of shunting syrinx on surgical outcome and follow-up result of Chiari I malformation with syringomyelia

目的比较ChiariⅠ畸形伴脊髓空洞症空洞分流与否对近远期疗效的影响,探讨脊髓空洞分流的必要性和临床意义,为临床选择手术方法提供依据。方法回顾性分析21例ChiariⅠ畸形伴脊髓空洞症手术治疗病例,观察8例经后颅寓减压+颈1、2或3椎板切开+硬脑膜修补(A组)与13例同时行脊髓空洞-蛛网膜下腔分流病人(B组)术后早期、随访半年以上临床症状与体征的变化,并采用Karnofsky行为能力评分法对两组病例远期疗效进行评定,同时比较术后空洞变化与临床症状的关系。结果A组术后早期症状改善5例(62.5%),稳定3例,无症状恶化者;随访4例,按照Karnofsky评分标准,临床症状改善3例,有效率75%(3/4)。B组术后早期症状改善6例,稳定5例,恶化1例,有效率46.3%(6/13);随访8例,临床症状改善7例,有效率87%(7/8)。随访MRI结果显示,B组空洞缩小程度明显优于A组,多数空洞缩小者,症状好转,少数病例症状无变化。结论后颅窝减压加颈1、2或3椎板切开及硬脑膜修补是治疗ChiariⅠ畸形伴脊髓空洞症的有效方法,加行空洞分流能有效缩小空洞体积,多数患者远期疗效优于未分流者,但部分病人存在无效或症状恶化可能。

Objective To probe into the effect of shunting syrinx on operational outcome of Chiari I malformation with syringomyelia, and discuss clinical significance of shunting syrinx and provide some clues for surgical procedure. Methods The clinical symptoms and signs were retrospectively analyzedin 21 cases with Chiari I malformation combined with syringomyelia, of which 8 cases underwent posterior fossa decompression with cervical laminectomy of C1-C2 or C3 plus duroplasty (group A), and 13 did plus syrinx-subarachnoid shunting (group B). All were investigated by Karnofsky performance scales and followed up for over 6 months. The changes of syrinx size and symtomes were compared. Results In group A, 5 cases (62.5%) were found improved in symptoms and signs, 3 cases stable, no patient deteriorated at the early stage following operation. 3 out of 4 cases followed up for over 6 months experienced improvement in symptoms, with improvement rate 75%. In group B, 6 cases were improved in symptoms, 5 cases stable, 1 deteriorated during early stage after operation, with improvement rate 46.3% (6/13). In the over 6 months' follow-up, 7 cases experienced improvement (87%). MRI showed the reduction of syrinx diameter in both groups, but with significant difference between them. Conclusion Posterior fossa decompression with laminectomy of cervical vertebra and duroplasty, as an operational procedure, is effective in the treatment of Chiari malformation combined with syrinx of spinal cord. Shunting syrinx can effectively reduce the size of syrinx. Most of patients who underwent syrinx shunting has better outcome than those with no shunting as far as far future. However this effect can be limited in some patients, even worsen their clinical symptoms and signs.