Arshad Ayesha, Tsirikos Athanasios I
Scottish National Spine Deformity Centre, Royal Hospital for Children and Young People, Edinburgh, UK.
Dev Med Child Neurol. 2024 Feb;66(2):187-194. doi: 10.1111/dmcn.15711. Epub 2023 Jul 21.
We investigated survival rates after scoliosis correction in individuals with Duchenne muscular dystrophy (DMD) and evaluated factors that can affect them.
This was a retrospective cohort study from 2000 to 2022 with a minimum 2-year postoperative follow-up. We reviewed the hospital records/spinal radiographs and analysed data with XLSTAT. Kaplan-Meier and multivariate Cox regression survival analysis was performed.
Forty-three patients had a mean age at surgery of 14 years 5 months. Mean postoperative follow-up was 10 years 10 months. There was no operative or 30-day postoperative mortality in this group. Twenty-four patients died because of cardiorespiratory failure. Median survivorship was 14 years 2 months, with the longest observed survival being 22 years 6 months given the limitation of the length of postoperative follow-up. The degree of preoperative coronal imbalance and pelvic obliquity, as well as intraoperative blood loss, were factors that significantly affected survival. The impact of preoperative sagittal imbalance and extension of the fusion to the sacrum/pelvis trended towards significance. In contrast, age at surgery, preoperative/postoperative scoliosis, thoracic kyphosis, lumbar lordosis, scoliosis and pelvic obliquity flexibility or correction indices, postoperative coronal/sagittal balance, need of preoperative non-invasive ventilation, preoperative feeding disorders, development of surgical complications, and length of hospital and intensive care unit stay were exposure variables that did not affect postsurgical survival in patients with DMD.
Survival of up to two decades or more was possible among young patients with DMD after scoliosis correction. This was affected by factors that related to disease and deformity severity, as well as surgical morbidity.
There was no operative or 30-day postoperative mortality in this group of patients with Duchenne muscular dystrophy (DMD) undergoing scoliosis correction. Survival probabilities at 5-year, 10-year, 15-year, and 20-year intervals post-surgery were 92%, 80%, 33%, and 12% respectively. Scoliosis surgery achieved good deformity correction and a balanced spine that was maintained at follow-up. Respiratory failure, severe pneumonia, and left ventricular failure were the leading causes of death in the study participants. Preoperative global coronal imbalance, pelvic obliquity, and intraoperative blood loss significantly predicted survival. Factors that affected survival after scoliosis surgery were associated with perioperative morbidity and disease or deformity severity in the DMD group.
我们研究了杜氏肌营养不良症（DMD）患者脊柱侧弯矫正后的生存率，并评估了可能影响生存率的因素。
这是一项回顾性队列研究，研究时间为2000年至2022年，术后随访至少2年。我们查阅了医院记录/脊柱X光片，并使用XLSTAT分析数据。进行了Kaplan-Meier和多变量Cox回归生存分析。
43例患者手术时的平均年龄为14岁5个月。术后平均随访时间为10年10个月。该组患者无手术或术后30天内死亡情况。24例患者因心肺功能衰竭死亡。中位生存期为14年2个月，鉴于术后随访时间的限制，观察到的最长生存期为22年6个月。术前冠状面失衡和骨盆倾斜程度以及术中失血量是显著影响生存的因素。术前矢状面失衡以及融合至骶骨/骨盆的范围对生存的影响有显著趋势。相比之下，手术年龄、术前/术后脊柱侧弯、胸椎后凸、腰椎前凸、脊柱侧弯和骨盆倾斜的灵活性或矫正指数、术后冠状面/矢状面平衡、术前是否需要无创通气、术前喂养障碍、手术并发症的发生以及住院和重症监护病房的住院时间等暴露变量对DMD患者术后生存无影响。
DMD年轻患者脊柱侧弯矫正后存活长达二十年或更长时间是可能的。这受到与疾病和畸形严重程度以及手术并发症相关的因素影响。
在接受脊柱侧弯矫正的杜氏肌营养不良症（DMD）患者组中，无手术或术后30天内死亡情况。术后5年、10年、15年和20年的生存概率分别为92%、80%、33%和12%。脊柱侧弯手术实现了良好的畸形矫正和脊柱平衡，且在随访中得以维持。呼吸衰竭、重症肺炎和左心室衰竭是研究参与者的主要死亡原因。术前整体冠状面失衡、骨盆倾斜和术中失血量显著预测生存情况。脊柱侧弯手术后影响生存的因素与DMD组的围手术期并发症以及疾病或畸形严重程度相关。
