We present a case of Morquio syndrome, which is an autosomal recessive condition that is classified as a lysosomal storage disorder. The syndrome results from abnormal accumulation of mucopolysaccharides due to deficiency of the enzyme N-acetyl-galactosamine-9-sulphate sulphatase. The disorder primarily presents with skeletal abnormalities, some of which can be seen in the images provided. The disease also causes extreme short stature. 

Some common radiological findings of Morquio syndrome include platyspondyly of the cervical and thoracic vertebrae, anterior beaking of the cervical vertebral bodies, thoracolumbar kyphosis, odontoid process hypoplasia, and coxa valga of the hip joints. The disease is progressive and often results in death secondary to spinal cord involvement. Instability of the atlantoaxial joint is almost invariably found in patients with this condition and has the potential to result in spinal cord compression or sudden death if not detected and treated early. The C1-C2 joint is commonly fused surgically to prevent atlantoaxial dislocation as seen in this patient. The thoracolumbar spine is often severely kyphotic and can result in spinal cord compression and paraplegia.

Case contributed by Dr. N. Larocque.