Osteogenesis imperfecta tarda. The morphology of rib biopsies

Abstract

Undecalcified bone biopsy specimens from 10 normal individuals and 8 osteogenesis imperfecta patients, 2 to 30 years of age, were analyzed using microradiography as well as ultraviolet, tungsten and polarized light microscope techniques. Biopsies of ribs most reliably reflected the disease process of osteogenesis imperfecta when compared to those biopsies of long bones in the lower extremities deformed enough to require corrective surgery. The rib biopsies consistently revealed that there is a rapid surface turnover of histologically well-organized but osteopenic bone. The high rate of bone turnover is similar to that seen in very young normals but in osteogenesis imperfecta turnover rate does not appear to decline with age. The percentage of surface involved in active new bone formation and in osteoclastic resorption is notably elevated. Excessive osteocyte osteolysis is also characteristic.