纵向可延长钛金属肋骨假体技术治疗先天性脊柱侧弯合并胸廓畸形

目的 报道我们采用纵向可延长钛金属肋骨假体(VEPTR)技术治疗先天性脊柱侧弯合并胸廓畸形的初步体会.方法 5例先天性脊柱侧弯合并胸廓畸形的患儿,男3例,女3例,平均年龄8.3岁(4.7～12.2岁);均自出生就发现脊柱畸形并呈进行性加重.除1例曾经接受过弧度段脊柱张力钢丝捆绑治疗外,其余病例均无过去手术史;所有病例体格矮小、躯体平衡失调并倒向凸侧;肺功能检测明显低下,其中最大肺活量(VCMAX)和最大自主通气量(MVV)分别只有同年龄、同身高正常预测值的24.8%～48.1%(平均32.8%)和20.8%～54.4%(平均34.5%);胸段脊柱混合型分节、分化不良,3例存在凹侧肋骨融合畸形;术前Cobb's角平均为77.6°(63°～106°);CT及CT三维重建测量,脊柱旋转(SR)平均为19.6°(11.4°～26.8°),胸廓旋转(TR)平均为61.4°(34.2°～477.2°),后半胸廓对称度(PHSR)平均为2.4(1.2～3.6);MRI脊髓扫描未发现异常.手术在神经监护仪下进行,所有病例均I期完成手术,4例进行了肋骨开放截骨,术中4例胸膜壁层破裂,所有病例胸膜脏层均保持完整.所有病例均各安置了肋骨-肋骨、肋骨-脊柱钛肋各一组.结果 所有病例均获得随访,平均随访时间14个月(12～18个月),所有病例均已完成了2次延长.术后所有病例躯体平衡获得明显改善,凹侧胸廓扩大,骨盆倾斜平衡恢复,脊柱高度增加,身高平均增长4.4 cm(3～7 cm).所有患儿肺功能检查均较术前有不同程度提高,VCMAX和MVV平均增加35 7%(10.4%～78.4%)和51.7%(8.2%～84.9%).呼吸系统易患感染、活动后气急等现象明显改善.脊柱畸形均有所矫正,Cobb's角测量平均57°(44°～69°),家长对患儿外观改变均表示满意.结论 VEPTR技术对于治疗先天性脊柱侧弯合并胸廓畸形所致的胸腔功能不全综合症显示了一定的效果,肺功能改善,脊柱畸形控制,脊柱生长继续,躯体平衡恢复.其中长期功效和并发症尚待观察.

Application of VEPTR in the treatment of patients with congenital scoliosis associated with thoracic deformity

Objective To explore the experience in the treatment of congenital scoliosis associat ed with thoracic deformity by using VEPTR technique.Methods Five cases suffering congenital scoliosis associated with thoracic deformity were included in the study.There were three males and two females with the average age of 8.3 years(ranging from 4.7 to 12.2 years).All cases had spinal deformity since born and got progressive deterioration.Of the 5 patients,4 had no operation history,while one had accepted sublaminar wire binding in the curve segment of spine.All cases appeared runty and unahie to keep balance.The patients' bodies were prone to fall down to the convex side of spine.All cases had obviously poor pulmonary function.Compared with the normal children with the same age and stature,average VCMAX of the 5 patients was only 32.8%(24.8%～48.1%)and MVV was 34.51%(20.8%～54.4%).Thoracic spine had mixed unsegmental deformities and maldifferenciation.Three cases had the malformation of costodesis in the concavity side of spine.Average Cobb's angle of pa tients before operations was 77.6°(63°～106°).Spine rotation(SR),thoracic rotation(TR)and posture hemithoracic symmetry ratio(PHSR)bv CT scan measurement were respectively 19.6°(11.4°～26.8°);61.4°(34.2°～77.2°)and 2.4°(1.2°～3.4°).No abnorrnal findings were noted bv the MRI scanning.Operations were performed under cord monitoring and all patients underwent one-stage operation.Three cases who had costodesis underwent opening-wedge thoracostomy.Parietal pleura in 4 cases ruptured during operations,but visceral pleura remained perfect.Each patient had one rib-to rib and one rib-to-spine titanium rib.Results All cases were followed up for 12 to 18 months with the average period of 14 months.Twice instrumental lengthenings were performed on the 5 patients,who got obvious improvement of body balance,expending of thorax and lengthening of spine.Average height of patients increased after surgery by 4.40 cm (3～7 cm).Spine deformity was corrected,and the average Cobb's angle after surgery was 57°(44～69°).All cases got improvement in the pulmonary functions.One year later after surgery,VCMAX had increased 35.70%(10.4% to 78.4%) and MVV increased 51.66%(8.2%to 84.9%).The incidence of respiratory infection and dyspnea after mild activities was reduced significantly.Conclusions VEPTR is effective treatment for patients with congenital scoliosis associated with thoracic deformity.Improvement of pulmonary functions,control spine deformity,ability of continuing spine growth and recovery of body balance are its main advantages.But it's middle or long term efficacy and complications still need further observation.