小儿脊髓纵裂及其合并畸形的治疗

Management of split cord malformations associated with other deformities in children

[目的]探讨小儿脊髓纵裂及其合并畸形的治疗方法。[方法]回顾性分析近10 a获随访经手术治疗的脊髓纵裂患儿48例,男29例,女19例;年龄7 d^14岁。均因腰背部异常、双下肢和(或)二便功能障碍做全脊柱X线、MR I、CT等检查。手术先切断终丝松解栓系,再打开硬膜直视下切除纤维束或骨软骨嵴,合并先天性脊柱侧弯者一期或二期半脊椎后路切除矫形。[结果]48例患儿均有脊髓低位,伴发脊髓栓系综合征34例,脊柱侧弯及后凸26例,脊髓脊膜膨出14例,脊髓空洞15例,椎管内脂肪瘤12例。依照脊髓纵裂Pang分型:Ⅰ型27例,Ⅱ型18例,混合型3例。位于颈段1例,胸段6例,胸腰段1例,腰段38例,骶段2例。术后改善28例,恢复正常3例,无效3例。后路半脊椎切除术矫治先天性脊柱侧弯效果满意。[结论]脊髓纵裂极易合并脊髓栓系及先天性脊柱侧弯,患儿应常规行脊柱X线、MR I、CT等检查,以提高脊髓纵裂、脊髓栓系及其他脊柱畸形的诊断率。早期切断终丝松解栓系,直视下切除纤维性、骨或软骨性间隔是治疗脊髓纵裂的必要手段。同时一期或二期后路半脊椎切除是早期治疗先天性脊柱侧弯的有效方法。

[ Objective] To analyze clinical features, evaluate diagnostic procedures and discuss the different forms of therapy for each case of split cord malformation （SCM）. [ Method] A series of 48 patients had a follow-up period of 0. 5 - 9 years, 29 males and 19 females, aged 7 days to 14 years, were evaluated retrospectively. They suffered from back midline abnormalities, neurological deficits of lower limbs and bladder dysfunction. At first they were cut ilium terminale and then excised the fibrous or bony spur finally treated scoliosis. The radiologieal procedures used for the diagnosis of SCM were spinal X-ray, ultrasound, CT scan and MRI. [ Result] In all of the 48 patients with tethered cord, there were 28 cases with dysplasia of lower limbs and feet, 13 cases with urination and defecation dysfunction, 14 cases with only dorsal-lumbar abnormalities, 26 cases with scoliosis and kyphosis, 14 with myelomeningocele, 15 with syringomyelia and 12 with intraspinal lipemas. There were 27 cases suffering from type Ⅰ SCM, 18 type Ⅱ SCM and 3 composite type SCM according to Pang. The most frequent location of the spur was at the lumbar level （38 cases）, the less frequent in thoracic regions （6 cases） and exceptionally rare below S1 （2 cases）, in the cervical regions （ 1 case）, the thoralumbar （ 1 case）. After operation, 28 cases were improved, 3 cases were cured and 3 cases were inefficacy. [ Conclusion ] The most frequent syndrome of SCM patients is lower limbs dysplasia and dorsal-lumbar abnormalities. The patients are most suffered from tethered cord and spinal deformities. To improve diagnosis of SCM, tethered cord and other deformities, the patients should be routinely evaluated by MRI and X-ray of the whole spine, sometimes could be evaluated by ultrasound and CT scan. SCM cases should be early treated by first cutting filium terminal and then excising fibrous or bony spur and hemivertebra.