Figure 2 .

A) AP spine showing platyspondyly. Lateral spine showing kyphosis, narrow chest. The ribs are narrow posteriorly, but widened and irregular anteriorly and markedly sclerotic. There is a gibbous deformity. B) Short sacro-iliac notches. Hypoplasia of the bodies of iliac bones. The shape of the iliac bone is unique, a notch between the shortened iliac body and prominent crescent shaped iliac wing resembles a halberd. Shortened tubular bones with flared metaphyses. C) The chondroosseous junction is smooth at the knee, but there are cartilaginous remnants in the distal femora. Carpal ossification is significantly retarded. D) The middle and distal phalanges are short. Degenerative joint disease is found in the proximal interphalangeal joints. Hypoplastic/dysplastic carpal bones. Short metacarpals and phalanges with flared ends.