Developmental coxa vara associated with spondylometaphyseal dysplasia (DCV/SMD): "SMD-corner fracture type" (DCV/SMD-CF) demonstrated in most reported cases

Background. This paper reports three children with short stature: developme ntal coxa vara unilateral in the first case and bilateral in the other two; somewhat squared and '"ovoid" vertebral bodies in the first patient, and n ormal to slightly tall vertebral bodies in the third; metaphyseal changes i n some long tubular bones including bone fragments similar to the corner fr actures seen in child abuse in all three patients. Materials and methods. The first and second patients were sisters; their mo ther, also quite short, had surgical procedures in early life for bilateral "coxa vara"; their brother, also of short: stature, had bilateral coxa val ga with otherwise normal femoral beads and necks, and mild metaphyseal chan ges associated with two minute ''corner fractures" in the proximal metaphys is of the left tibia. Results. A review of reported cases of developmental coxa vara associated w ith spondylometaphyseal dysplasia revealed that simulated corner fractures were present in most instances.