an autosomal dominant disorder characterized by small face, large ears, protruding eyes, micrognathia, malaligned teeth, short arms, narrow thorax, bowed extremities and scoliosis. This syndrome, which is also termed osteodysplasty, is seen only in female patients; it appears to be lethal in males. On radiographs, an undulating contour and multiple constrictions of the medullary cavities are observed, with a ribbon-like appearance of the ribs. Deformity of the scapulae, flared iliac wings, coxa valga, long femoral necks and subtrochanteric narrowing also are seen. Other features include sclerosis of the base of the skull and a thin and small mandible. The vertebral bodies show an increased height and anterior concavity, sometimes with enlargement of the spinal canal in the lumbar region. In some patients, scoliosis or kyphoscoliosis may occur.