Abstract: Metatropic dysplasia
is a rare skeletal dysplasia
characterized by rapid collapse of the thoracolumbar spine into kyphoscoliosis. Other spinal anomalies associated with metatropic dysplasia
include odontoid hypoplasia and atlantoaxial instability leading to cervical myelopathy. Children with metatropic dysplasia
evaluated at our institution for spinal deformity showed evidence of cervical stenosis
with or without associated cord compression. Magnetic resonance imaging was found to demonstrate these changes. The association of cervical spinal stenosis and metatropic dysplasia
has not been previously described. This has significant treatment implications, because decompression over the stenotic segments should be considered in conjunction with spinal fusion for treatment of odontoid hypoplasia or atlantoaxial instability. A retrospective review of 13 cases of metatropic dysplasia
was performed. Despite the challenges provided by this patient population, the chance to halt or reverse neurological dysfunction and improve deformity necessitates prompt surgical intervention.