摘要目的:探讨脊髓脊膜膨出（myelomeningocele，MMC）修补术后迟发性脊柱畸形的影像学特征与矫形策略。方法:回顾性分析2006年1月至2019年12月收治的MMC修补术后迟发性脊柱畸形的患者23例，男16例、女7例，年龄（15.4±5.9）岁（范围6~28岁），均在幼儿期（0~4岁）接受MMC切除修补术。对脊髓脊膜膨出的合并症、脊柱畸形的影像学特征（侧凸Cobb角、冠状面平衡、局部后凸角）、矫形方法、临床疗效及并发症的发生率进行统计学分析。采用脊柱侧凸研究学会-22项问卷（Scoliosis Research Society，SRS-22）评分和Oswestry功能障碍指数（Oswestry disability index，ODI）评估患者的生活质量。结果:所有患者均获得随访，随访时间（2.4±0.8）年（范围1~4年）。23例患者中MMC发生于上胸段3例、胸段1例、胸腰段13例，腰骶段6例。16例患者脊柱侧凸或后凸的顶点与MMC病损在同一节段内。MMC位于胸腰段的13例患者中12例伴有侧凸畸形、9例伴有后凸畸形，MMC位于腰骶段的6例患者中3例伴有骨盆倾斜。椎体畸形包括椎弓根间距增宽21例、椎管扩大19例、棘突缺如17例、分节不良17例、半椎体畸形9例。髓内病变包括脊髓纵裂6例、脊髓拴系9例。总体植入物密度57.2%±17.0%（范围16.6%~100%）。末次随访时侧凸Cobb角为40.9°±19.1°，小于术前的71.5°±28.2°，差异有统计学意义（ P<0.001）；局部后凸角为26.7°±12.9°，小于术前的40.4°±21.5°，差异有统计学意义（ P<0.001）；冠状面平衡为（16.1±13.6） mm，小于术前的（28.5± 23.7） mm，差异有统计学意义（ P<0.001）。末次随访时SRS-22总分为（18.7±0.7）分，高于术前的（17.7±0.9）分，差异有统计学意义（ t=-9.74， P<0.001）；术后ODI为25.5%±6.2%，小于术前的44.8%±10.1%，差异有统计学意义（ t=13.66， P<0.001）。4例患者发生硬脊膜破裂，其中2例发生脑脊液漏；1例术后发生胸腔积液；2例患者出现螺钉位置不良。末次随访时3例患者出现断棒，1例出现深部感染。 结论:MMC患者在幼儿期接受切除修补术后约70%在成年期发生以病损部位为顶椎的迟发性脊柱畸形。后路矫形通常可获得满意的临床疗效，如顶椎区椎体后份发育不良造成置钉困难，可采用前路椎体钉、椎板钩固定及经S 2骶髂螺钉等方法增加植入物密度。

abstractsObjective:To investigate the imaging features and surgical strategies of late-onset spinal deformity after myelomeningocele (MMC) repair.Methods:A total of 23 patients with late-onset spinal deformity after MMC repair from January 2006 to December 2019 were retrospectively analyzed, including 16 males and 7 females, aged 15.4±5.9 years (range, 6-28 years). All patients underwent MMC resection and repair in infancy (0-4 years). The complications of MMC, imaging characteristics of spinal deformity (Cobb angle of scoliosis, coronal balance, regional kyphosis), surgical methods, clinical outcomes and incidence of complications were analyzed. The Scoliosis Research Society-22 (SRS-22) score and Oswestry disability index (ODI) were used to evaluate the quality of life.Results:All patients were followed up for 2.4±0.8 years (range, 1-4 years). Among 23 patients, MMC occurred in the upper thoracic segment in 3 cases, thoracic segment in 1 case, thoracolumbar segment in 13 cases, and lumbosacral segment in 6 cases. 16 patients had scoliosis or kyphosis with the apex of the spine in the same segment as the MMC lesion. Among 13 patients with MMC located in thoracolumbar segment, 12 patients had scoliosis and 9 patients had kyphosis. Among 6 patients with MMC located in lumbosacral segment, 3 patients had pelvic tilt. Vertebral deformities included widening of pedicle space in 21 cases, enlargement of spinal canal in 19 cases, absence of spinous process in 17 cases, malsegmentation in 17 cases, and hemivertebra deformity in 9 cases. Intramedullary lesions included split cord in 6 cases and tethered cord in 9 cases. The overall implant density was 57.2%±17% (range, 16.6%-100%). At the last follow-up, the Cobb angle of scoliosis was 40.9°±19.1°, which was significantly smaller than 71.5°±28.2° before operation ( P<0.001). The local kyphosis angle was 26.7°±12.9°, which was significantly lower than that before operation (40.4°±21.5°), the difference was statistically significant ( P<0.001).The coronal balance was 16.1±13.6 mm, which was smaller than that before operation 28.5± 23.7 mm, the difference was statistically significant ( P<0.001). The total score of SRS-22 was 18.7±0.7, which was higher than that before operation 17.7±0.9, and the difference was statistically significant ( t=-9.74, P<0.001); ODI was 25.5%±6.2% after operation, which was significantly lower than that before operation (44.8%±10.1%), the difference was statistically significant ( t=13.66, P<0.001). Dural rupture occurred in 4 patients, including postoperative cerebrospinal fluid leakage in 2 cases; postoperative pleural effusion in 1 patient; and screw malposition in 2 patients. Three patients had broken rods and one had deep infection at final follow-up. Conclusion:About 70% of MMC patients who underwent resection and repair in early childhood developed late-onset spinal deformity in adulthood with the lesion at the parietal vertebrae. Posterior correction can obtain satisfactory clinical results. If the posterior element of the apical vertebral body is hypoplastic, the implant density can be increased by anterior vertebral screw, lamina hook fixation, and S 2 sacroiliac screw.