Background
Among the skeletal dysplasias, spinal deformity is seen most commonly in patients with achondroplasia.
Issues
1. Thoracolumbar kyphosis
Occurs in almost all patients prior to walking
- non rigid, likely due to hypotonia
- resolves with ambulation
Bracing may help to prevent clinically significant kyphosis
- persists in approximately 15% to 20% of patients
2. Spinal Stenosis
Pathology
- Short pedicles
- narrow interpedicular distance
- Stenosis may be present at any level from the foramen magnum down
- most commonly L1-S1
Symptoms
Over 50% of patients with achondroplasia have back pain
- other symptoms may include leg pain, claudication, paresthesias, weakness, and paraplegia.
- Bowel and bladder difficulties may occur with stenosis in the lower thoracic spine
Symptoms usually occur in early adulthood and are slowly progressive
- Occasionally, symptoms may arise in childhood
Surgical decompression
Wide, multilevel laminectomies
- Disc removal usually is not indicated
- unless radiculopathy is present
- Early surgical intervention leads to a better outcome
Postlaminectomy instability may occur
- fusion probably should be added in cases of multilevel decompression
3. TL kyphosis + stenosis
Anterior decompression and strut grafting should be added