先天性脊柱侧凸合并脊髓分裂症的诊治探讨

Evaluation and treatment of congenital scoliosis with split cord malformation

目的探讨先天性脊柱侧凸合并脊髓分裂症的诊断和手术治疗方法及效果。方法1999年5月至2004年6月共收治先天性脊柱侧凸患者353例,术前经脊髓造影和(或)CTM检查发现58例合并脊髓分裂症。按照脊髓分裂畸形(SCM)的分类方法分为双管型(SCMⅠ型)和单管型(SCMⅡ型)。对其中的56例患者进行了手术治疗,除1例合并蛛网膜囊肿的患者行椎管内探查手术外,其他患者在脊柱矫形前未切除分裂脊髓间的间隔(骨性、软骨性或纤维性)。分别对SCMⅠ型和SCMⅡ型患者的临床表现、影像学改变、手术治疗的方法和效果进行回顾性分析。结果SCMⅠ型11例,占19%,SCMⅡ型47例,占81%。17例术前合并神经系统的异常表现,其中SCMⅠ型2例、SCMⅡ型15例。接受手术的56例患者中,SCMⅠ型11例,术前Cobb角(60±25)°;SCMⅡ型45例,术前Cobb角(67±21)°。术后SCMI型Cobb角(29±13)°,矫形率(51±17)%;SCMⅡ型Cobb角(39±19)°,矫形率(41±24)%。以上两组之间的差异无统计学意义。术后所有患者未出现新的神经损伤症状或原有神经症状加重。26例(45%)患者获得了平均17.3个月(4～59个月)的随访,矫形丢失(6±10)°,SCMⅠ型的7例患者丢失(5±8)°,SCMⅡ型的19例患者丢失(6±11)°,两组之间的差异无统计学意义。术后随访时患者神经症状稳定。结论先天性脊柱侧凸常合并椎管内异常病变,手术治疗前应常规行椎管内的影像学检查(脊髓造影、CTM或MRI检查)。并不是所有合并SCM的先天性脊柱侧凸患者在脊柱矫形前都需要处理椎管内的病变。对于SCMⅠ型的先天性脊柱侧凸患者,如果术前无脊髓栓系的临床表现并且影像学显示骨性间隔的上下方存在分裂的脊髓,侧凸矫形前可不必处理椎管内的骨嵴。对于合并SCMⅡ型的先天性脊柱侧凸患者,如果术前患者无进行性加重的神经系统表现,位于单管内分裂的脊髓可以像正常脊髓一样耐受脊柱矫形。

Objective To discuss the diagnosis and surgical treatment of congenital scoliosis (CS) with split cord malformation (SCM). Methods From May 1999 to June 2004, 353 cases of CS were admitted, and 58 cases were diagnosed CS combined with SCM via myelography and(or) CTM. According to Pang's classification for SCM, patients were divided into type Ⅰ SCM and type Ⅱ SCM. Two cases gave up operation, and surgeries were performed in 56 patients. Except that one case with arachnoid cyst underwent intraspinal exploration, all other 55 cases underwent spinal correction without dealing with the intraspinal abnormalities. Clinical manifestation, radiological findings, operative methods and results were retrospectively analyzed in this paper. Results There were 11 patients (19%) with type Ⅰ SCM and 47 patients (81%) with type Ⅱ SCM. In the 56 cases who had surgical treatment, the Cobb angle in the patients with type Ⅰ SCM (11 cases) was (60±25)°, and in type Ⅱ SCM (45 cases) was (67±21)°pre-operation. The Cobb angle in type I SCM was (29±13)°and in type Ⅱ SCM was (39±19)°post-operation, with the correction rate (51±17)% and (41±24)% respectively. No statistic differences were found between both types. And there were no new neurological deficits in both groups after the spinal correction operation. After average 17.3 months (4-59 months) follow-up, the correction loss was (6±10)°, (5±8)°in 7 patients with type Ⅰ SCM and (6±10)°in 19 patients with type Ⅱ SCM. There was no statistic difference in correction loss between both types. In the follow-up, the neurological symptoms and signs of the patients were stable. Conclusion Considering the incidence of intraspinal anomalies in patients with congenital scoliosis, intraspinal examination via myelography, CTM or MRI should be performed prior to spinal correction surgery. For congenital scoliosis with type I SCM, the bone spur need not be excised before spinal correction if there is no signs of spinal cord tethered and the bone spur locate in the middle of the split cord where there are much space to accommodate it. For congenital scoliosis with type II SCM, if there are no progressive neurological manifestations, the split cord in the single tubular can tolerate the manipulation of spinal correction as the normal spinal cord.