In this series of nine patients with diastrophic dwarfism, multiple
problems with the spine were noted. Scoliosis was present in all patients and
usually became rapidly progressive in the preadolescent years. The scoliosis
had a strong tendency to become severe and rigid, with a large rotatory
component. Early brace treatment appeared to have merit. Cervical kyphosis was
noted in four patients; it resolved in three patients, but progressed in one
to a 130-degree deformity, quadriplegia, and death. All patients in this
series had spina bifida occulta in the cervical spine. Six of the nine
patients demonstrated a narrowed interpedicular distance below the third
lumbar vertebra, but none had spinal stenosis.