[Scoliosis in patients with osteogenesis imperfecta: a federal nation-wide cross-sectional study].

OBJECTIVE

Quite different prevalence of scoliosis in patients with Osteogenesis imperfecta is reported, not at least owing to the difficult recruitment of patients. The present study aims to investigate Osteogenesis imperfecta-patients with scoliosis by means of a cross-section study.

PATIENTS AND METHODS

A total of 102 patients (46 males, 56 females) with an average age of 24.6 years (3-71 yrs.) were investigated in 1995 supported by the "Gesellschaft für Osteogenesis imperfecta Betroffene e.V.". Physical examination included pain and range of motion. Localisation and expanse of the curvature as well as attendant deformities of the spine were assessed by radiograph.

RESULTS

Scoliosis was observed in 76 patients (74.5%). Most of them showed thoracal or thoracolumbar localisation with 36 cases of right and 40 cases of left convexity. The curvature was mainly mild (56 pat. < 40 degrees), while 8 patients showed moderate (< 60 degrees), 7 patients severe (< 80 degrees) and 5 patients very severe deformity (> 80 degrees). Average expansion amounted to 6.7 vertebrae (3-12) with an average rotation of 2 (1-4). Attendant deformities were distributed as follows: codfish (59 pat.) or wedge-shaped vertebrae (42 pat.), platyspondylia (16 pat.), thoracal cyphosis (5 pat.), flat back (1 pat.) or lordosis (2 pat.) and lumbar lordosis (10 pat.) or vertical position (14 pat.). 69 scoliosis patients complained about pain with an average VAS-value of 4.3. They also showed moderate to severe impairment of the range of motion.

CONCLUSIONS

Our cross-section study with 102 Osteogenesis imperfecta-patients showed scoliotic deformity in 74.5% of cases. 69 patients suffered from remarkable pain and impaired range of motion, which was not only attributable to scoliosis alone, but also to concomitant spinal deformities.