脊柱Gorham病合并重度侧后凸畸形的手术疗效及其对策  

The outcome and countermeasure of posterior correction surgery for severe kyphoscoliosis secondary to spinal Gorham disease

作　　者：杜长志 孙旭[1] 邱勇[1] 李松[1] 徐亮[1] 王牧一 王斌[1] 钱邦平[1] 朱泽章[1] Du Changzhi;Sun Xu;Qiu Yong;Li Song;Xu Liang;Wang Muyi;Wang Bin;Qian Bangping;Zhu Zezhang(Department of Spine Surgery,Nanjing Drum Tower Hospital,Medical School of Nanjing University,Nanjing 210008,China)

机构地区：[1]南京大学医学院附属鼓楼医院脊柱外科,210008

出　　处：《中华骨科杂志》2020年第23期1583-1591,共9页Chinese Journal of Orthopaedics

基　　金：国家自然科学基金(81772422)。

摘　　要：目的评估脊柱Gorham病合并重度侧后凸畸形的手术疗效,并探讨手术对策。方法2005年1月至2019年12月共确诊脊柱Gorham病合并脊柱畸形患者12例,其中4例接受矫形手术治疗。男3例,女1例;手术年龄的中位数为14.5岁(11.5岁,27.5岁);侧凸和后凸Cobb角的中位数分别为29°(21.5°,78.0°)和94°(78.0°,103.0°)。患者于halo轮椅悬吊重力牵引后均行一期脊柱后路矫形内固定融合术,置钉节段跨越病变椎体,术中行SchwabⅠ或Ⅱ级截骨术,术后接受二膦酸盐类药物治疗。在站立位全脊柱正、侧位X线片上测量脊柱侧凸、后凸Cobb角,冠状面平衡及矢状面平衡等参数,在CT和MRI上评估脊柱骨溶解侵袭情况。结果4例患者在牵引3个月(2.5个月,3.5个月)后,脊柱侧凸和后凸Cobb角中位数分别降至23.5°(15.5°,77.0°)和65°(57.0°,83.5°)。矫形术中2例行关节突切除术,2例行Ponte截骨术,手术时间中位数为5.5 h(5.1 h,5.9 h),术中出血量为3095 ml(2950 ml,3320 ml),融合节段为13.5个(12.5个,14.5个),置钉密度为47.8%(40.9%,57.3%)。术后脊柱侧凸和后凸Cobb角中位数分别为18°(10.5°,38.5°)和59°(42.0°,78.0°),矫正率分别为46.7%(33.1%,59.5%)和35%(12.3%,51.1%)。冠状面平衡中位数由牵引前的15.5 mm(9.0 mm,21.0 mm)降为6.5 mm(4.0 mm,9.0 mm),矢状面平衡由14 mm(-18.0 mm,27.5 mm)降至5.5 mm(-5.5 mm,12.5 mm)。随访时间中位数为2.8年(2.0年,3.5年),随访期间1例患者因断棒行翻修手术,未见骨溶解进展、神经损伤及乳糜胸等并发症发生。结论一期后路矫形术联合术前Halo牵引和术后抗骨质疏松治疗等可满意纠正脊柱Gorham病合并重度侧后凸畸形,但要警惕术后内固定失败等并发症。Objective To investigate the outcome of posterior correction surgery for severe kyphoscoliosis secondary to spinal Gorham disease, further to explore the countermeasure in such complicated condition.Methods From January 2005 to December 2019, a total of 12 consecutive patients were diagnosed with spinal Gorham disease. Four patients who had undergone correction surgery were reviewed retrospectively. There were 3 males and 1 female. The median age of surgery was 14.5 years (11.5 years, 27.5 years), with the median of Cobb angle of scoliosis and kyphosis 29° (21.5°, 78.0°) and 94° (78.0°, 103.0°), respectively. After Halo-gravity traction, one-stage posterior correction surgeryand Schwab grade I or II osteotomy, with pedicle screw fixation bridging the diseased vertebrae was performed. Drug therapy of bisphosphonate was recommended after surgery. The Cobb angle of scoliosis and kyphosis, coronal and sagittal balance were measured on the standing upright radiographs of the spine. CT and MRI were used to give precise evaluation of spinal and peripheral soft tissue involvement.Results After Halo-gravity traction of 3 months (2.5 months, 3.5 months), the median of Cobb angle of scoliosis decreased to 23.5° (15.5°, 77.0°) and kyphosis decreased to 65° (57°, 83.5°) respectively. Two patients underwent facetectomyand 2 received Ponte osteotomy. The median operative time and blood loss were 5.5 h (5.1 h, 5.9 h) and 3 095ml (2 950 ml, 3 320 ml), with the fusion segment of 13.5 (12.5, 14.5) and the fixation density of 47.8% (40.9%, 57.3%). After surgery, the median of Cobb angle of scoliosis and kyphosis decreased to 18° (10.5°, 38.5°) and 59° (42.0°, 78.0°). Compared to the values before traction, the median of correction rates of scoliosis and kyphosis after surgery were 46.7% (33.1%, 59.5%) and 35% (12.3%, 51.1%) respectively. Moreover, the median of coronal balance decreased from 15.5 mm (9.0 mm, 21.0 mm) to 6.5 mm (4.0 mm, 9.0 mm), while the median of sagittal balance decreased from 14 mm (-18.0 mm, 27

关 键 词：骨质溶解 原发性 脊柱侧凸 脊柱后凸 治疗结果 

分 类 号：R687.3[医药卫生—骨科学]