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Table 9 Guidance statements for decompression of the spinal cord, spinal stabilisation and thoracolumbar kyphoscoliosis

From: Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance

Statement Percentage consensus
Decompression of the spinal cord is recommended in patients with MPS VI who have evidence of spinal cord compression based on clinical and radiographica findings
Evidence Grade: D (limited published evidence)
97%
Spinal stabilisation of the craniocervical junction with either cervical fusion or occipital-cervical fusion is recommended in patients with MPS VI who have evidence of significant instability
Evidence Grade: D (expert clinical opinion)
100%
Correction of thoracolumbar kyphoscoliosis is recommended in patients with MPS VI who present with progressive radiographic changes, intractable pain and clinical deterioration as defined by gait, lung function and changes in the degree of kyphosis
Evidence Grade: D (limited published evidence)
97%
  1. Post-consensus comments by the SC to be taken into consideration
  2. aThe SC would like to clarify that neuroimaging is a required radiologic procedure to define compression. MRI is best to image the brain and spinal cord for this indication. Decompression of the spinal cord is recommended in patients who have evidence of spinal cord compression and risk of injury based on clinical and neuroimaging findings