Neurological Evaluation of Severe Congenital Kyphosis With Compressive Myelopathy in 39 Patients With a Minimum 1-Year Follow-Up

Abstract

Objective: Congenital angular kyphosis with compressive myelopathy is a rare yet underreported neurologic emergency for spine surgeons. The study aimed to report the neurological outcomes of 39 patients with compressive myelopathy in congenital kyphosis (CK) and provide magnetic resonance imaging (MRI) features in these patients, identifying the potential prognostic factors.

Methods: This is a retrospective cohort study. From January 2010 to December 2022, 39 patients were diagnosed with severe congenital angular kyphosis with compressive myelopathy and received surgical correction. The American Spinal Injury Association (ASIA) Impairment Scale grades at admission and final follow-up were recorded to measure neurological outcomes. A series of qualitative and quantitative parameters were evaluated on preoperative MRI by observers who were blinded to the clinical outcomes. According to the final status of neurological recovery, patients were divided into two groups and compared: Group E (patients with ASIA E) and Group N (patients with ASIA A, B, C, and D).

Results: At the final follow-up, 79.5% of patients (31 cases) had improved neurological function and myelopathy symptoms. The average Cobb angle of kyphosis decreased from 78.4° to 20.1° after correction surgery. The duration of myelopathy in Group N was significantly longer than that in Group E (p = 0.038). T1-weighted imaging (T1WI) signal hypointensity was presented in 35.9% (14/39) of patients, and T2-weighted imaging (T2WI) signal hyperintensity was 69.2% (27/39). On average, these patients had a maximum canal compromise (MCC) of 52.2%, maximum spinal cord compression (MSCC) of 47.0%, and maximum transverse area compression (MTAC) of 49.1%. Compared to Group E, Group N showed greater MCC (p = 0.023), MSCC (p = 0.008), and MTAC (p < 0.001) at baseline, and was more likely to present T1WI hypointensity and T2WI hyperintensity. The cut-off values of MTAC, MCC, and MSCC for fully neurological recovery were 47.1%, 46.7%, and 52.6%, respectively.

Conclusion: MRI indicators of poor prognosis in CK patients with compressive myelopathy included the presence of T1WI hypointensity, T2WI hyperintensity, and greater MCC, MSCC, and MTAC. It was suggested that early surgical intervention is critical to prevent irreversible cord injury, particularly in patients with MTAC > 47.1%.

Keywords

compressive myelopathy / congenital angular kyphosis / magnetic resonance imaging / neurological dysfunction

References

[1] M. J. McMaster and H. Singh, “Natural History of Congenital Kyphosis and Kyphoscoliosis. A Study of One Hundred and Twelve Patients,” Journal of Bone and Joint Surgery. American Volume 81 (1999): 1367-1383.

[2] J. Pizones, L. Moreno-Manzanaro, A. Vila-Casademunt, et al., “Adult Congenital Spine Deformity: Clinical Features and Motivations for Surgical Treatment,” International Journal of Spine Surgery 15 (2021): 1238-1245.

[3] M. J. McMaster, “Spinal Growth and Congenital Deformity of the Spine,” Spine 31 (2006): 2284-2287.

[4] Z. He, Y. Liu, F. Xue, H. Xiao, W. Yuan, and D. Chen, “Surgical Management of Congenital Cervical Kyphosis,” Orthopedics 35 (2012): e1396-e1401.

[5] R. B. Winter, J. H. Moe, and J. F. Wang, “Congenital Kyphosis. Its Natural History and Treatment as Observed in a Study of One Hundred and Thirty Patients,” Journal of Bone and Joint Surgery. American Volume 55 (1973): 223-256.

[6] S. Wang, G. Lin, Y. Yang, et al., “Outcomes of 360° Osteotomy in the Cervicothoracic Spine (C7-T1) for Congenital Cervicothoracic Kyphoscoliosis in Children,” Journal of Bone and Joint Surgery. American Volume 101 (2019): 1357-1365.

[7] Z. Zhang, H. Wang, and C. Liu, “Compressive Myelopathy in Severe Angular Kyphosis: A Series of Ten Patients,” European Spine Journal 25 (2016): 1897-1903.

[8] K. S. Song, B. S. Chang, J. S. Yeom, J. H. Lee, K. W. Park, and C. K. Lee, “Surgical Treatment of Severe Angular Kyphosis With Myelopathy: Anterior and Posterior Approach With Pedicle Screw Instrumentation,” Spine 33 (2008): 1229-1235.

[9] K. W. Chang, C. W. Cheng, H. C. Chen, and T. C. Chen, “Correction Hinge in the Compromised Cord for Severe and Rigid Angular Kyphosis With Neurologic Deficits,” Spine 34 (2009): 1040-1045.

[10] Z. Zhang, H. Wang, and W. Zheng, “Compressive Myelopathy in Congenital Kyphosis of the Upper Thoracic Spine: A Retrospective Study of 6 Cases,” Clinical Spine Surgery 30 (2017): E1098-e1103.

[11] T. Shimomura, M. Sumi, K. Nishida, et al., “Prognostic Factors for Deterioration of Patients With Cervical Spondylotic Myelopathy After Nonsurgical Treatment,” Spine 32, no. 22 (2007): 2474-2479, https://doi.org/10.1097/BRS.0b013e3181573aee.

[12] M. G. Fehlings, S. C. Rao, C. H. Tator, et al., “The Optimal Radiologic Method for Assessing Spinal Canal Compromise and Cord Compression in Patients With Cervical Spinal Cord Injury. Part II: Results of a Multicenter Study,” Spine 24, no. 6 (1999): 605-613, https://doi.org/10.1097/00007632-199903150-00023.

[13] F. Kato, Y. Yukawa, K. Suda, M. Yamagata, and T. Ueta, “Normal Morphology, Age-Related Changes and Abnormal Findings of the Cervical Spine. Part II: Magnetic Resonance Imaging of Over 1,200 Asymptomatic Subjects,” European Spine Journal 21 (2012): 1499-1507.

[14] K. Fujiwara, K. Yonenobu, K. Hiroshima, S. Ebara, K. Yamashita, and K. Ono, “Morphometry of the Cervical Spinal Cord and Its Relation to Pathology in Cases With Compression Myelopathy,” Spine 13 (1988): 1212-1216.

[15] Y. Morio, K. Yamamoto, K. Kuranobu, M. Murata, and K. Tuda, “Does Increased Signal Intensity of the Spinal Cord on MR Images due to Cervical Myelopathy Predict Prognosis?,” Archives of Orthopaedic and Trauma Surgery 113 (1994): 254-259.

[16] Y. Zeng, Z. Chen, Q. Qi, et al., “Clinical and Radiographic Evaluation of Posterior Surgical Correction for the Treatment of Moderate to Severe Post-Tuberculosis Kyphosis in 36 Cases With a Minimum 2-Year Follow-Up,” Journal of Neurosurgery. Spine 16 (2012): 351-358.

[17] Y. Zeng, X. Qu, Z. Chen, et al., “Posterior Corrective Surgery for Moderate to Severe Focal Kyphosis in the Thoracolumbar Spine: 57 Cases With Minimum 3 Years Follow-Up,” European Spine Journal 26 (2017): 1833-1841.

[18] M. J. McMaster and H. Singh, “The Surgical Management of Congenital Kyphosis and Kyphoscoliosis,” Spine 26 (2001): 2146-2154.

[19] Y. Zeng, Z. Chen, Q. Qi, et al., “The Posterior Surgical Correction of Congenital Kyphosis and Kyphoscoliosis: 23 Cases With Minimum 2 Years Follow-Up,” European Spine Journal 22 (2013): 372-378.

[20] B. Grodzinski, R. Durham, O. Mowforth, D. Stubbs, M. R. N. Kotter, and B. M. Davies, “The Effect of Ageing on Presentation, Management and Outcomes in Degenerative Cervical Myelopathy: A Systematic Review,” Age and Ageing 50 (2021): 705-715.

[21] S. Kirshblum, B. Snider, F. Eren, and J. Guest, “Characterizing Natural Recovery After Traumatic Spinal Cord Injury,” Journal of Neurotrauma 38 (2021): 1267-1284.

[22] K. Shimizu, M. Nakamura, Y. Nishikawa, S. Hijikata, K. Chiba, and Y. Toyama, “Spinal Kyphosis Causes Demyelination and Neuronal Loss in the Spinal Cord: A New Model of Kyphotic Deformity Using Juvenile Japanese Small Game Fowls,” Spine 30 (2005): 2388-2392.

[23] S. Li, S. Mao, Y. Ma, et al., “Posterior 3-Column Osteotomy for Treatment of Congenital Kyphosis With Multiple Thoracolumbar/Lumbar Anterior Unsegmented Vertebrae: A Comparison Between Patients With Increasing Number of Anterior Unsegmented Vertebrae,” World Neurosurgery 159 (2022): e172-e183.

[24] X. Liu, S. Yuan, Y. Tian, L. Wang, Y. Zheng, and J. Li, “Expanded Eggshell Procedure Combined With Closing-Opening Technique (a Modified Vertebral Column Resection) for the Treatment of Thoracic and Thoracolumbar Angular Kyphosis,” Journal of Neurosurgery. Spine 23 (2015): 42-48.

[25] B. Garg, T. Bansal, and N. Mehta, “Three-Column Osteotomy by Single-Stage Posterior Approach in Congenital and Post-Tubercular Kyphosis: A Comparison of Outcomes,” Spine Deformity 10 (2022): 883-892.

[26] R. Xue, D. Liu, and Y. Shen, “Comparison of Posterior Unilateral Vertebral Column Resection Versus Posterior Vertebral Column Resection for Severe Thoracolumbar Angular Kyphosis as a Revision Surgical Modality: A Retrospective Cohort Study,” Clinical Spine Surgery 34 (2021): E303-e307.

[27] Y. Atici, M. B. Balioglu, D. Kargin, M. Mert, A. Albayrak, and M. A. Kaygusuz, “Analysis of Complications Following Posterior Vertebral Column Resection for the Treatment of Severe Angular Kyphosis Greater Than 100°,” Acta Orthopaedica et Traumatologica Turcica 51 (2017): 201-208.

[28] H. Ma, B. Shi, D. Liu, et al., “Posterior Corrective Surgery for Type II Congenital Kyphosis: SRS-Schwab Grade 4 Osteotomy or Vertebral Column Resection?,” Orthopaedic Surgery 16 (2024): 1710-1717.

[29] B. Shi, Q. Zhao, L. Xu, et al., “SRS-Schwab Grade 4 Osteotomy for Congenital Thoracolumbar Kyphosis: A Minimum of 2 Years Follow-Up Study,” Spine Journal 18 (2018): 2059-2064.

[30] A. Nouri, L. Tetreault, J. J. Zamorano, et al., “Role of Magnetic Resonance Imaging in Predicting Surgical Outcome in Patients With Cervical Spondylotic Myelopathy,” Spine 40, no. 3 (2015): 171-178, https://doi.org/10.1097/BRS.0000000000000678.

[31] B. Arvin, S. Kalsi-Ryan, D. Mercier, J. C. Furlan, E. M. Massicotte, and M. G. Fehlings, “Preoperative Magnetic Resonance Imaging Is Associated With Baseline Neurological Status and Can Predict Postoperative Recovery in Patients With Cervical Spondylotic Myelopathy,” Spine 38 (2013): 1170-1176.

[32] A. Karpova, R. Arun, D. W. Cadotte, et al., “Assessment of Spinal Cord Compression by Magnetic Resonance Imaging—Can It Predict Surgical Outcomes in Degenerative Compressive Myelopathy? A Systematic Review,” Spine 38, no. 16 (2013): 1409-1421, https://doi.org/10.1097/BRS.0b013e31829609a0.

[33] A. Nouri, A. R. Martin, S. Kato, H. Reihani-Kermani, L. E. Riehm, and M. G. Fehlings, “The Relationship Between MRI Signal Intensity Changes, Clinical Presentation, and Surgical Outcome in Degenerative Cervical Myelopathy: Analysis of a Global Cohort,” Spine 42 (2017): 1851-1858.

[34] T. Fischer, C. Stern, P. Freund, M. Schubert, and R. Sutter, “Wallerian Degeneration in Cervical Spinal Cord Tracts Is Commonly Seen in Routine T2-Weighted MRI After Traumatic Spinal Cord Injury and Is Associated With Impairment in a Retrospective Study,” European Radiology 31 (2021): 2923-2932.

[35] H. Yu, Z. Liu, M. Pang, et al., “Wallerian Degeneration Assessed by Multi-Modal Magnetic Resonance Imaging of Cervical Spinal Cord Is Associated With Neurological Impairment After Spinal Cord Injury,” Journal of Neurotrauma 41 (2024): 1240-1252.

[36] A. Karpova, R. Arun, S. Kalsi-Ryan, E. M. Massicotte, B. Kopjar, and M. G. Fehlings, “Do Quantitative Magnetic Resonance Imaging Parameters Correlate With the Clinical Presentation and Functional Outcomes After Surgery in Cervical Spondylotic Myelopathy? A Prospective Multicenter Study,” Spine 39 (2014): 1488-1497.

[37] P. Skeers, C. R. Battistuzzo, J. M. Clark, S. Bernard, B. J. C. Freeman, and P. E. Batchelor, “Acute Thoracolumbar Spinal Cord Injury: Relationship of Cord Compression to Neurological Outcome,” Journal of Bone and Joint Surgery. American Volume 100 (2018): 305-315.

[38] J. Lee, K. Satkunendrarajah, and M. G. Fehlings, “Development and Characterization of a Novel Rat Model of Cervical Spondylotic Myelopathy: The Impact of Chronic Cord Compression on Clinical, Neuroanatomical, and Neurophysiological Outcomes,” Journal of Neurotrauma 29 (2012): 1012-1027.

[39] J. Bakhsheshian, L. G. Lenke, F. M. Hassan, E. Lewerenz, J. L. Reyes, and S. L. Zuckerman, “Prospective Validation of the Spinal Cord Shape Classification System in the Prediction of Intraoperative Neuromonitoring Data Loss: Assessing the Risk of Spinal Cord Data Loss During Spinal Deformity Correction,” Journal of Bone and Joint Surgery. American Volume 106 (2024): 716-726.

[40] Z. Jin, J. Li, H. Xu, et al., “The Role of Spinal Cord Compression in Predicting Intraoperative Neurophysiological Monitoring Events in Patients With Kyphotic Deformity: A Magnetic Resonance Imaging-Based Study,” Neurospine 21 (2024): 701-711.

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2025 The Author(s). Orthopaedic Surgery published by Tianjin Hospital and John Wiley & Sons Australia, Ltd.