The development of the spinal deformities in osteogenesis imperfecta

Evaluations were made of the spinal deformities in 22 of 30 patients (osteogenesis imperfecta congenita, eight; osteogenesis imperfecta tarda I, 13; osteogenesis imperfecta tarda II, one) treated at Hamagumi Gakuen Children's Hospital from 1959 to 1980. Seven of the congenital type patients were in the severe curve group (greater than 50 degrees), four of whom showed more than 100 degrees of curvature. The spinal deformities in the congenital type progressed rapidly after five years of age and reached a maximum around 12 years of age. The scoliosis in the tarda type patients developed slowly, but then progressed rapidly after the curve exceeded 50 degrees. The complications of the spinal deformities included disability on ambulation, inactivity in daily living, and possibly respiratory dysfunction. Progression of the spinal curvature should be prevented by functional bracing or surgical treatment before severe complications arise.