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 report the six-year results of a prospective, controlled demographic trial of developmental dysplasia of the hip (DDH) treated in the Pavlik harness using ultrasound supervision. Our aim was to assess the value of ultrasound and its role in monitoring reduction in the harness, in terms of progression or failure of reduction at an early state. From 1988 to 1994, a total of 221 patients with 370 ultrasonographically abnormal hips was treated in the Pavlik harness. This represents a treatment rate for the Southampton district of 5.1 per 1000 live births. Sixteen hips in 12 patients were not reduced in the harness and required surgical treatment; 95.7% were successfully reduced. One case of mild avascular necrosis (0.3%) was identified in those treated by harness alone. Of the 221 patients 87.8% remain under radiological review, with 3.2% of affected hips showing continued, mild acetabular dysplasia. We conclude that ultrasound monitoring has led to an acceptably low level of intervention, a high reduction rate and minimal iatrogenic complications. The trial is continuing

We reviewed 32 children after the treatment of simple bone cysts by intralesional injections of methyl-prednisolone acetate. The age of the child and the activity and size of the cyst did not significantly affect the radiological outcome. The earliest time at which the radiological response could be reliably determined was three months. After a median period of review of five years, four (13%) cysts had healed, 20 (62%) cysts were partially visible but sclerotic, four (12.5%) were still visible but opaque and four (12.5%) were clearly visible. The healed and partially visible but sclerotic cysts were classified as having satisfactory radiological healing. This was observed in 13 of 32 cysts (41%) after the first injection, in eight of 21 (38%) after the second injection, but in relatively few of the remaining cysts after subsequent injections. A satisfactory symptomatic outcome was achieved in all of the 18 children with humeral cysts and in the one child with a fibular cyst irrespective of the radiological outcome, but only in nine (67%) of the 13 children with femoral or tibial lesions, in whom the cysts were healed or sclerotic. The remaining four children had exertional bone pain and repeated fractures of their femoral or tibial cysts which were incompletely healed with sclerosis in one and opacities in three. We conclude that the healing response to intralesional corticosteroids is unpredictable and usually incomplete even after multiple injections. The failure rate in weight-bearing bones is too high