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

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

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

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

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

Platyspondyly with kyphosis and coronal plane stenosis

Stenotic segment Adjacent normal segment

Stenotic segment Adjacent nonstenotic segment

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

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