Scoliosis in patients with osteogenesis imperfecta: A nation-wide cross-section study

Objective: Quite different prevalence of scoliosis in patients with Osteoge nesis imperfecta is reported, not at least owing to the difficult recruitme nt of patients. The present study aims to investigate Osteogenesis imperfec ta-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 "Gese llschaft fur Osteogenesis imperfecta Betroffene e.V.": Physical examination included pain and range of motion. Localisation and expanse of the curvatu re as well as attendant deformities of the spine were assessed by radiograp h. Results: Scoliosis was observed in 76 patients (74.5%). Most of them sho wed thoracal or thoracolumbar localisation with 36 cases of right and 40 ca ses 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 expan sion 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 wed ge-shaped vertebrae (42 pat.), platyspondylia (16 pat.), thoracal cyphosis (5 pat.), flat back (1 pat.) or lordosis (2 pat.) and lumbar lordosis (10 p at.) 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 wi th 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 con comitant spinal deformities.