Spinal abnormalities in camptomelic dysplasia.

Significant spinal abnormalities were found in eight patients (average age of 6 years, 5 months) with camptomelic dysplasia. The late ossification of the midthoracic pedicles served as a clear diagnostic criterion for the syndrome. Scoliosis averaging 63 degrees was found in all seven nonquadriplegic cases. Thoracic hyperkyphosis averaging 126 degrees was seen in six (75%) of the patients, while cervical kyphosis averaging 66 degrees was noted in three (38%). Vertebral body hypoplasia appeared to be a major cause of deformity. This study clarifies that patients with camptomelic dysplasia are surviving longer than previously expected and therefore should have their spinal deformities treated aggressively.