摘要目的 评估脊柱-肋骨撑开术治疗先天性脊柱侧凸(congenitalscoliosis, CS)合并胸廓功能不全综合征(thoracic insufficiency syndrome, TIS)的矫形效果及肺功能变化。方法 回顾性分析2003-03至2012-03于北京儿童医院行脊柱-肋骨撑开术治疗合并TIS的CS患儿25例,测量并比较初次术前,术后及末次随访时主弯Cobb角、顶椎偏移、胸椎高度、肺的可容性空间比(space available for lung, SAL);测量并比较5岁以上患儿初次术前及末次随访时肺功能结果。结果 25例共行203次手术,平均8.1次/例。初次术前、术后,末次随访时主弯角分别为75.5°±21.1°、54.0°±15.5°、53.2°±16.0°,顶椎偏移分别为(42.9±14.5)mm 、(19.7±8.3)mm、(20.6±9.2)mm,末次随访均较术前有明显改善(P<0.05)。SAL术前为73.3%,术后82.5%,末次随访88.6%。最大肺活量及用力肺活量在末次随访时较初次术前明显改善(0.99 vs 0.87,0.98 vs 0.84, P<0.05)。结论 在治疗CS合并TIS时,使用脊柱-肋骨撑开术,不仅能够有效地控制脊柱畸形的进展,保留胸廓和脊柱的生长潜能,而且可改善胸廓畸形及肺功能。
Abstract：Objective To explore the efficacy of spine-rib distraction on congenital scoliosis (CS) associated with thoracic insufficiency syndrome (TIS). Methods Medical records of twenty-five CS patients (6 boys and 19 girls) with TIS who had undergone spine-rib distraction in our hospital between March 2003 and March 2012 were reviewed. Spine-to-rib distraction devices were implanted during the initial surgery. During the regular post-operative follow-ups, expansion surgeries were scheduled at an interval of 6 to 12 months. Measurements of primary curve magnitude, apical vertebral translation (AVT), thoracic height (T1-T12) and space available for the lung (SAL) were performed on radiographs preoperatively, postoperatively, and during the last follow-up, and were compared through paired t tests. Results Of preoperativepulmonary function tests were collected and compared with those of the last follow-up. ResultsTwenty-five patients had an average follow-up of 86.3 months (ranging from 61 to 122 months). A total of 183 expansion surgeries were performed, an average of 7.3 surgeries per patient. Preoperatively, postoperatively and during the last follow-up, the Cobb angle of the primary curve was 75.5±21.1°, 54.0±15.5°, and 53.2 ± 16.0°respectively, while AVT was 42.9±14.5 mm, 19.7±8.3 mm, and 20.6±9.2 mm respectively. According to the last follow-up, T1-T12 height improved significantly (15.8±2.1 to 21.4±3.0 cm), so did SAL (73.3% to 88.6%). VCmax and FVC measured during the last follow-up were increased significantly compared to preoperative ones(0.87 to 0.99; 0.84 to 0.98,P<0.05). Conclusions Spine-rib distraction technique proves to be an effective treatment for preventing curve progression in CS patients with TIS while ensuring the growth of the spine, symmetry of the chest, and improvement of pulmonary function.
Campbell R M, Smith M D, Mayes T C, et al . The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis [J]. J Bone Joint Surg (Am), 2003, 85: 399-408.
[2]
Campbell R M, Smith M D, Hell-Vocke A K. Expansion thoracoplasty : the surgical technique of opening-wedge thoracostomy: surgical technique [J]. J Bone Joint Surg (Am), 2004,86(1):51-64.
Campbell R M, Smith M D. Thoracic insufficiency syndrome and exotic scoliosis [J]. J Bone Joint Surg (Am), 2007,89(Suppl 1):108-122.
[5]
B.A. Akbarnia et al . The Growing Spine: Management of Spinal Disorders in Young Children[M]. Springer-Verlag, Berlin Heidelberg, 2016.
[6]
Mayer O H, Redding G. Early changes in pulmonary function after vertical expandable prosthetic titanium rib insertion in children with thoracic insufficiency syndrome [J]. J Pediatr Orthop,2009,29(1):35-38.
Campbell R M,Adcox B M,Smith M D, et al . The effect of mid-thoracic VEPTR opening wedge thoracotomy on cervical tiltassociated with congenital thoracic scoliosis in patients withthoracic insufficiency syndrome [J]. Spine(Phila Pa 1976),2007, 32:217l-2177.
[10]
Hasler C C,Mehrkens A,Hefli. Efficacy and safety of VEPTR instrumentation for progressive spine deformities in young children without rib fusions [J]. Eur Spine J,2010,19:400-408.
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