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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 IVA: systematic evidence- and consensus-based guidance

Statement

Percentage consensus

Decompression of the spinal cord is recommended in patients with MPS IVA who have evidence of spinal cord compression based on clinical and radiographic findingsa

Evidence Grade: C (level 3/4 studies)

97%

Spinal stabilisation of the craniocervical junction with either cervical fusion or occipital-cervical fusion is recommended in patients with MPS IVA who have evidence of significant instability

Evidence Grade: D (limited published evidence)

97%

Correction of thoracolumbar kyphoscoliosis is recommended in patients with MPS IVA who present with progressive radiographic deformity, intractable pain and neurological deterioration

Evidence Grade: C (level 3/4 studies)

100%

  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 with MPS IVA who have evidence of spinal cord compression and risk of injury based on clinical and neuroimaging findings
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172