Abstract
Osteogenesis imperfecta is a systemic disease chracterized by blue sclera and multipl fractures due to defective collagen production. Sillance defined 4 types of osteogenesis imperfecta in 1981. Type II and III are equivalent to type II and III "congenita" and type I and IV to"tarda". Osteogenesis imperfecta type II is the fatal form vvith otosomal recessive inheritance. Two autopsy cases (1 intrauterine dealth, 1 perinatal) diagnosed at SSK Tepecik Teaching Hospital are presented with histologic and radiologic findings stressing the importance of recognition and description of these cases at autopsy.
Keywords:
Perinatal autopsy, osteopeny