NEW FORM OF BONE DYSPLASIA WITH MULTIPLE FRACTURES ASSOCIATED WITH MONOSOMY-X

We report on the clinical, radiologic, and pathologic findings in a 20 -week-old fetus with monosomy X and severe hydrops associated with fet al dwarfism. The fetus presented with osteoporosis, bent bones, multip le fractures, and distinctive symmetric submetaphyseal transverse bone interruptions or pseudofractures. We excluded by radiologic and histo pathologic examination the diagnoses of osteogenesis imperfecta, hypop hosphatasia, campomelic dysplasia, achondrogenesis, hypochondrogenesis , and other types of bone dysplasia. To our knowledge, this is a previ ously undescribed bone dysplasia associated with monosomy X. This bone dysplasia may be inherited as an X-linked recessive disorder. (C) 199 6 Wiley-Liss, Inc.