Cleidocranial dysplasia (CCD) is inherited as an autosomal dominant disorder characterised by failure of membranous ossification. The condition is due to a mutation of the cbfa1 gene on chromosome 6 which has a role in the development of osteoblasts from the mesenchymal cells. In their growing years, these patients have an unusual shape of the femoral head reminiscent of a ‘chef’s hat’. In order to confirm the consistency of this sign, we have reviewed the radiographs of 28 patients with CCD. All except three had this appearance. The sign was also seen in patients with coxa vara associated with a variety of other conditions. The chef’s hat sign may occur secondary to the particular mechanical environment created by coxa vara as well as abnormal cellular function in patients with CCD. Although coxa vara has some influence on the shape of the femoral head, it is not entirely responsible for its morphology since it was present in only six of the 28 patients with CCD

We reviewed 22 children with cubitus varus who had been treated by a reverse V osteotomy and fixation by cross-pinning and wiring. The mean pre-operative humeral-elbow-wrist angle was −16.9° (−25° to +9°) and at the latest follow-up it was +7.3° (−2° to +14°). No child had a lateral prominence greater than 5 mm after correction. An excellent result was achieved in 20 children and a good result in two. We believe that this osteotomy has the advantages of better inherent stability, the avoidance of a prominent lateral condyle after correction and firm fixation allowing early movement.