1. Joseph I. Krajbich MD, FRCS(C) Shriners Hospital Portland, Oregon
Coronal Plane Spinal Stenosis in Metatropic Dysplasia 1 Previously Unreported Manifestation of the Condition
Joseph I. Krajbich MD, FRCS(C)
Shriners Hospital
Portland, Oregon

2. Metatropic dysplasia (MTD)
Rare genetic disorder
Very short stature, short limbs, joint and spinal abnormalities
High incidence of severe platospondyly, platybasia with odontoid hypoplasia and C1-2 instability
High incidence of kyphosis or scoliosis or a combination of the two in thoracic or thoracolumbar spine

3. Study design Review of two index patients with diagnosis of MTD.
Physical exam
Imaging studies – x ray, CT, MRI

4. Result
Both patients diagnosed in our skeletal dysplasia clinic and referred for cervical spine instability management.
In addition to cervical spine instability and thoracic kyphosis imaging studies demonstrated severe coronal plane stenosis in thoracic and thoracolumbar spine

5. Results
Both patient demonstrated subtle neurological changes which in one patient failed to resolve with cervical spine stabilization
Both patients required cervical spine stabilization

6. Platyspondyly with kyphosis and coronal plane stenosis

7. Stenotic segment
Adjacent normal segment

8. Stenotic segment
Adjacent nonstenotic segment

9. Conclusion
Report in two cases of MTD manifesting with thoracic coronal plane stenosis, previously not reported in the literature
In one patient the condition required decompression to address worsening neurological deficit

10. Conclusion
Patients with MDT should have spinal imaging in all three planes to role out coronal plane spinal stenosis