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Figure 2: A 4-year-old girl child suspected of Larsen syndrome, presented with progressive myelopathy and regression of motor milestones; (a) T2-weighted sagittal MRI was suggestive of compression of cervicomedullary junction with intensity changes with retroodontoid connective deposition. (b) Extension and (c) flexion sagittal CT images showing a reducible AAD. (d) Postoperative radiology showing reduction of AAD with C1-2 rod and screw fixation. In the postoperative period, the child had worsening myelopathy with difficulty in being weaned from ventilator. The child underwent a successful transoral decompression of connective tissue. (e) Intraoperative image showing a fibrocollagenous tissue compressing the thecal sac; (f) The exposed dura after removal of fibrocollagenous tissue; histopathology revealed a dense fibrocollagenous tissue with no evidence of granuloma/tumor/metastasis

Figure 2: A 4-year-old girl child suspected of Larsen syndrome, presented with progressive myelopathy and regression of motor milestones; (a) T2-weighted sagittal MRI was suggestive of compression of cervicomedullary junction with intensity changes with retroodontoid connective deposition. (b) Extension and (c) flexion sagittal CT images showing a reducible AAD. (d) Postoperative radiology showing reduction of AAD with C1-2 rod and screw fixation. In the postoperative period, the child had worsening myelopathy with difficulty in being weaned from ventilator. The child underwent a successful transoral decompression of connective tissue. (e) Intraoperative image showing a fibrocollagenous tissue compressing the thecal sac; (f) The exposed dura after removal of fibrocollagenous tissue; histopathology revealed a dense fibrocollagenous tissue with no evidence of granuloma/tumor/metastasis