Scoliosis in patients with diastrophic dysplasia - A new classification

Study Design. Scoliosis in patients with diastrophic dysplasia was analyzed . Objectives. To study the natural history of scoliosis and to classify the p atients with different types of scoliosis. Summary of Background Data. Typical findings in diastrophic dysplasia are s hort-limbed short stature, multiple joint contractures, early degeneration of joints, and spinal deformities. The largest studies have reported scolio sis in 37% to 88% of the patients with this rare skeletal dysplasia. The na tural history of the deformity is unknown. Methods. Of the 130 unselected patients, 98 (75%) who were older than 16 ye ars and/or had undergone surgery at the time of the last radiograph were in cluded in the final analysis. These 98 patients included 37 males and 61 fe males. Their ages at the first radiograph ranged from newborn to 78 years ( average, 21 years). The mean follow-up period was 20 years (range, 2-41 yea rs) for 80 patients. Standard standing anteroposterior and lateral radiogra phs were taken. The degrees of scoliosis, kyphosis, and lordosis were measu red according to the Cobb method. Classification of the scoliosis was based on the patient's age at onset of scoliosis, the rate of progression, the m agnitude of the scoliosis at the end of growth, and the curve pattern. Results. Of the 98 patients in this study, 86 (88%) had scoliosis. This dif ference was highly significant statistically (P < 0.001), as compared with the normal population. The frequency of scoliosis was 90% among females and 84% among males. Scoliosis can be divided further into three subtypes: ear ly progressive (11 patients), idiopathic-like (41 patients), and mild nonpr ogressive (33 patients). One patient had a congenital scoliosis. Conclusions. Scoliosis is very common in patients with diastrophic dysplasi a. The natural history of scoliosis varies from severe deformity with rapid progression to mild deformity without any progression. The authors suggest that the classification described in this report offers a tool for the pre dicting natural history of scoliosis in diastrophic dysplasia, and for adju sting the timing of surgery in individual patients.