PRENATAL DIAGNOSTICS OF OSTEOGENESIS IMPE RFECTA (CONTINUATION) - REPORT ON A CASE CLASSIFIED AS CLASSICAL LETHAL TYPE VROLIK

Report on prenatal sonographic diagnosis of osteogenesis imperfecta of the classical type ''letalis Vrolik'' in the case of a fetus in the 2 05h/21st week of pregnancy. The prenatal diagnosis of this kind of ost eogenesis imperfecta is based on the soft membranous cranium (Caput me mbranacium), the microthorax caused by the bell-shaped configuration o f the ribs and the diaphysis of the long bones which are too short for the age of pregnancy. These symptoms are first of all the consequence of a disturbed bone formation and mineralisation; its basis is a conn atal deficit of collagen synthesis. The sonographic differential diagn osis to the type ''Ekman Lobstein'' of osteogenesis imperfecta and tha natophoric dwarfism are discussed.