We have observed congenital hypophosphataemic rickets in two sons of a marriage between first cousins, their mother being clinically and biochemically normal. Both patients are now approaching middle age. In addition to severe childhood rickets and lifelong hypophosphataemia, their disease is characterised by gross osteosclerosis with extraskeletal ossification, clinically persistent osteomalacia in one and spinal cord compression in the other. The genetics of this disease can be satisfactorily explained only on the basis of autosomal recessive inheritance, a mode which has only once before been reported in the literature. The severity of certain features, which would be expected in a homozygous state, may help our understanding of the more usual X-linked form

1. The case is reported of a woman aged sixty-five with widespread osteosclerosis as a feature of IgA myelomatosis. 2. Improvement was noted after treatment by cyclophosphamide and prednisolone. 3. Only two previously reported cases of myeloma with osteosclerosis of comparable extent have been found, but in forty-seven other cases in the literature some sclerosis has been noted. 4. The finding of sclerotic lesions should not rule out multiple myeloma

Dynamic muscle-tendon substitution for acute anterior cruciate deficiency in the dog was studied using the semimembranosus muscle-tendon. Nineteen mongrel dogs each had a semimembranosus transfer in one knee; as a control, the anterior cruciate ligament and the semimembranosus were released in the opposite knee. No postoperative immobilisation was used. The anterior drawer sign was assessed before and after operation and when the dogs were killed five months later. Dogs were excluded from the study if they developed infections or contractures of the hind legs. At five months, 11 dogs were available for study. The operated knees were examined histologically and evaluated using a reproducible index of arthritis based on: the macroscopic discoloration of the articular cartilage, the cellularity of the cartilage, the microscopic appearance of the articular surface, the loss of proteoglycans, the formation of osteophytes and the degree of subchondral osteosclerosis. There was no significant difference in the anterior drawer sign or the degree of arthritic changes between knees with a semimembranous transfer and the controls. Examination showed that a muscle-tendon transfer into the tibia was equivalent to transferring the muscle into the posterior capsule--the intra-articular tendon being weak but histologically viable. The transfer did not prevent the anterior drawer sign becoming positive nor the development of osteoarthritis. A second control group, in which three dogs had an arthrotomy and semimembranosus release in both their hind legs, showed that a semimembranosus release alone did not cause osteoarthritis

Aims

Local recurrence remains a challenging and common problem following curettage and joint-sparing surgery for giant cell tumour of bone (GCTB). We previously reported a 15% local recurrence rate at a median follow-up of 30 months in 20 patients with high-risk GCTB treated with neoadjuvant Denosumab. The aim of this study was to determine if this initial favourable outcome following the use of Denosumab was maintained with longer follow-up.

Between 2003 and 2007, 99 knees in 77 patients underwent opening wedge high tibial osteotomy. We evaluated the effect of initial stable fixation combined with an artificial bone substitute on the mid- to long-term outcome after medial opening-wedge high tibial osteotomy (HTO) for medial compartmental osteoarthritis or spontaneous osteonecrosis of the knee in 78 knees in 64 patients available for review at a minimum of five years (mean age 68 years; 49 to 82). The mean follow-up was 6.5 years (5 to 10). The mean Knee Society knee score and function score improved from 49.6 (sd 11.4, 26 to 72) and 56.6 (sd 15.6, 5 to 100) before surgery to 88.1 (sd 12.5, 14 to 100) and 89.4 (sd 15.6, 5 to 100) at final follow-up (p <  0.001) respectively. There were no significant differences between patients aged ≥ 70 and < 70 years. The mean standing femorotibial angle was corrected significantly from 181.7° (sd 2.7°, 175° to 185°) pre-operatively to 169.7° (sd 2.4°, 164° to 175°) at one year’s follow-up (p < 0.001) and 169.6° (sd 3.0°, 157° to 179°) at the final follow-up (p = 0.69 vs one year).

Opening-wedge HTO using a stable plate fixation system combined with a bone substitute is a reliable procedure that provides excellent results. Although this treatment might seem challenging for older patients, our results strongly suggest that the results are equally good.

Cite this article: Bone Joint J 2014;96-B:339–44.

We prospectively examined the physical and imaging findings, including MRI, of 23 patients with spontaneous osteonecrosis of the knee after obtaining informed consent to acquire tissue specimens at surgery. There were four men and 19 women, with a mean age of 67.5 years (58 to 77). Plain radiographs were designated as stages 1, 2, 3 or 4 according to the classification of Koshino. Five knees were classified as stage 1, five as stage 2, seven as stage 3 and six as stage 4. The histological specimens were stained with haematoxylin and eosin and tetrachrome.

In the early stages of the condition, a subchondral fracture was noted in the absence of any features of osteonecrosis, whereas in advanced stages, osteonecrotic lesions were confined to the area distal to the site of the fracture which showed impaired healing. In such cases, formation of cartilage and fibrous tissue, occurred indicating delayed or nonunion. These findings strongly suggest that the histopathology at each stage of spontaneous osteonecrosis is characterised by different types of repair reaction for subchondral fractures.

Systemic mastocytosis is a rare condition that often involves the bone marrow. We report the case of a patient with systemic mastocytosis who underwent total hip replacement. Technical difficulties encountered during the procedure included a narrow medullary canal and abnormally hard bone, later confirmed by laboratory measurements. Follow-up at five years showed a good clinical and radiological outcome.

The sternoclavicular joint is vulnerable to the same disease processes as other synovial joints, the most common of which are instability from injury, osteoarthritis, infection and rheumatoid disease. Patients may also present with other conditions, which are unique to the joint, or are manifestations of a systemic disease process. The surgeon should be aware of these possibilities when assessing a patient with a painful, swollen sternoclavicular joint.