Spinal anomalies in achondroplasia

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Spinal deformity

  • thoracolumbar kyphosis
  • excessive lordosis

Spinal canal stenosis

  • Due to
    • Reduce spinal canal diameter
      • Short pedicles making the AP length smaller than the lateral length
    • Due to thickening of the connective structures such as the ligamentum flavum
    • Ligamentous laxity may also result in increased movement of the bony structures and increase the possibility of compression of the cord
  • spinal narrowing becomes potentially more problematic at the cervical regions and at the cervical and lumbar cord enlargements
  • Presents as CES/Neurogenic claudication
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Management

  • Lumbar stenosis
    • nonoperative
      • weight loss, physical therapy, corticosteroid injections
        • indications
          • first line of treatment and frequently effective
    • operative
      • multilevel laminectomy and fusion
        • indications
          • spinal stenosis with severe symptoms
          • nonoperative management has failed
  • Lumbar hyperlordosis
    • nonoperative
      • observation
        • treatment typically not required
  • Thoracolumbar kyphosis
    • nonoperative
      • Observation
        • 90% improve
      • bracing
        • if persistent vertebral wedging after age 3 years
        • may be poorly tolerated
    • Operative
      • Anterior strut corpectomy with posterior fusion or isolated posterior fusion
        • indications
          • bracing has failed
          • kyphosis of > 45-60°