Rajavelu Rajesh, Shetty Ajoy Prasad, Viswanathan Vibhu Krishnan, Kanna Rishi Mukesh, Rajasekaran S
Ganga Hospital, 313, Mettupalayam Road, Coimbatore, 641043, India.
Spine Deform. 2022 Mar;10(2):401-410. doi: 10.1007/s43390-021-00427-y. Epub 2021 Oct 19.
Congenital spinal deformities (CSD) are uncommon; and usually present during early childhood. Rarely, patients have been reported to present with neglected CSD in association with myelopathy. The current study reports the largest series of patients with neglected CSD and major neuro-deficit; and discusses their long-term outcome.
We retrospectively analysed patients with CSD who presented with myelopathy and underwent surgical management between January 2008 and January 2018. Only patients who had complete clinico-radiological records and completed minimum 2-year follow-up were included. Neurology was graded according to American Spinal Injury Association Impairment, Nurick and modified Japanese Orthopaedic Association scores. Radiological details like deformity type, location, magnitude, and underlying anomaly were recorded. A comparative analysis of parameters between pre-operative and final follow-up periods was performed.
Thirty three (age = 21.1 ± 11.9 years) patients with CSD and myelopathy were included. Mean duration since myelopathy was 5.2 ± 6.2 months. Thirty (91%) patients presented with kyphosis or kyphoscoliosis; and 18 had upper thoracic-level lesion (12 and 3 with mid- or low-thoracic and lumbar lesions). 16 (48.5%) and 12 (36.4%) presented with types 1 and 3 anomalies, respectively. Mean pre-operative mJOA and Nurick grades were 8.8 ± 2.4 and 3.4 ± 0.7. Based on ASIA score, 2, 5, 21 and five patients presented with grades A, C, D and E, respectively. All patients underwent surgeries from posterior-only approach. Mean follow-up was 4.7 ± 2.6 years. In kyphoscoliosis group, coronal and sagittal Cobb improved by 23.8° and 25.9°, respectively. Mean deformity improved by 19.6° and 15.6° in scoliosis and kyphosis groups, respectively. Neurological status improved in 5, remained stable in 23 and deteriorated in five patients. Mean mJOA and Nurick grades at final follow-up was 8.3 ± 3.1and 3.4 ± 0.9.
Major neurological deficit is a rare complication of neglected CSD. In our series, such a presentation was observed in patients with kyphotic or kyphoscoliotic deformities, type 1 or 3 vertebral anomalies and proximal thoracic vertebral lesions. Surgery can be valuable in these patients, as it not only stabilises deformity, but also provides the best chance of preventing neuro-deterioration.
先天性脊柱畸形（CSD）并不常见，通常在儿童早期出现。很少有患者被报道因脊髓病而出现被忽视的CSD。本研究报告了最大系列的被忽视的CSD和严重神经功能缺损患者，并讨论了他们的长期预后。
我们回顾性分析了2008年1月至2018年1月期间因脊髓病接受手术治疗的CSD患者。仅纳入具有完整临床放射学记录且完成至少2年随访的患者。根据美国脊髓损伤协会损伤分级、Nurick分级和改良日本骨科协会评分对神经功能进行分级。记录畸形类型、位置、程度和潜在异常等放射学细节。对术前和最终随访期间的参数进行比较分析。
纳入33例（年龄=21.1±11.9岁）CSD合并脊髓病患者。脊髓病后的平均病程为5.2±6.2个月。30例（91%）患者表现为后凸或后凸侧弯；18例患者有上胸段病变（12例和3例分别为中胸段或下胸段及腰椎病变）。分别有16例（48.5%）和12例（36.4%）表现为1型和3型异常。术前平均改良日本骨科协会（mJOA）评分和Nurick分级分别为8.8±2.4和3.4±0.7。根据美国脊髓损伤协会（ASIA）评分，分别有2例、5例、21例和5例患者表现为A级、C级、D级和E级。所有患者均采用单纯后路手术。平均随访时间为4.7±2.6年。在后凸侧弯组，冠状面和矢状面Cobb角分别改善了23.8°和25.9°。侧弯组和后凸组的平均畸形分别改善了19.6°和15.6°。5例患者神经功能改善，23例保持稳定，5例恶化。最终随访时的平均mJOA评分和Nurick分级分别为8.3±3.1和3.4±0.9。
严重神经功能缺损是被忽视的CSD的罕见并发症。在我们的系列研究中，这种表现见于后凸或后凸侧弯畸形、1型或3型椎体异常以及上胸段椎体病变的患者。手术对这些患者可能有价值，因为它不仅能稳定畸形，还能提供预防神经功能恶化的最佳机会。
