Subluxation of the hip is common in patients with intermediate spinal muscular atrophy. This retrospective study aimed to investigate the influence of surgery on pain and function, as well as the natural history of subluxed hips which were treated conservatively. Thirty patients were assessed clinically and radiologically. Of the nine who underwent surgery only one reported satisfaction and four had recurrent subluxation. Of the 21 patients who had no surgery, 18 had subluxation at the latest follow-up, but only one reported pain in the hip. We conclude that surgery for subluxation of the hip in these patients is not justified.
A consecutive series of 85 patients with Duchenne’s muscular dystrophy who underwent spinal fusion over a period of 16 years was followed up with regard to the progression of the scoliosis and pelvic obliquity. Of 74 patients with adequate radiographic follow-up, 55 were instrumented with the Luque single-unit rod system and 19 with the Isola pedicle screw system; seven were instrumented to L3/4, 42 to L5, 15 to S1 and 10 to the pelvis with intrailiac rods. The mean period of follow-up was 49 months (SD 22) before and 47 months (SD 24) after operation. There was one peri-operative death and three cases of failure of hardware. The mean improvement in the Cobb angle was 26° and in pelvic obliquity, 9.2°. Fusion to L3/4 achieved a poorer correction of both curves while intrapelvic rods, achieved and maintained the best correction of pelvic obliquity. Fusion to S1 did not provide any benefit over more proximal fusion excluding the sacrum, with regard to correction and maintenance of both angles. The Isola system appeared to provide and maintain a slightly better correction of the Cobb angle.
Previous studies have shown that the activity of the cytostatic drug methotrexate (MTX) embedded in acrylic cement is not affected by thermal changes in the cement. MTX is slowly released from the cement for several months and remains biologically active throughout this period. Our aim was to determine whether MTX embedded in cement would control the local growth of a tumour. In 15 rabbits we injected 0.1 ml of VX2 tumour suspension into the proximal tibia. At 3, 5, 7, 10 and 14 days three animals were killed and the tibiae removed and examined histologically. With increasing growth of the VX2 carcinoma there was increased bone destruction and a rise in the numbers of osteoclasts, but after 14 days the numbers of osteoclasts had decreased. We then injected VX2 into the tibiae of another 45 rabbits. After 5 days most of the tumour was curetted out and the defect filled with cement containing either 0 g, 0.1 g, 0.5 g, 1.0 g or 2.0 g MTX/40 g cement. The rabbits were divided into three groups and killed at 3, 7 or 10 days after implantation of cement. The number of osteoclasts and the amount of bone destruction were measured in each tibia. In all three groups bone destruction and osteoclast proliferation were markedly decreased with higher doses of MTX, but bone destruction was not eliminated. Our findings show that in the higher doses used, which were not toxic to the animal, MTX-embedded cement may be of value in minimising the amount of tumour-induced osteolysis and may be a useful adjunct in the surgical management of pathological fractures.
Skeletal scintigraphy, which has now been established as a useful and accurate method of detecting early skeletal metastases and assessing their response to treatment, has been investigated for its pathological basis. Histological examination of several hundred necropsy specimens, from sixty-eight patients who died from malignant disease, showed a significant increase of osteoid and immature woven bone in the presence of metastatic cancer. Tumour-cell suspensions of the VX2 carcinoma were injected into the medullary cavity or on to the periosteal surface of the ilia or tibiae of New Zealand white rabbits. A combination of bone destruction and new bone formation, similar to the autopsy material, was seen. There were at least two mechanisms for the new bone production. Initially, intramembranous ossification was seen in the fibrous stroma surrounding the tumour. Once the cortex was involved and cortical bone destruction had occurred, large amounts of woven bone resembling fracture callus were laid down. The new bone had a markedly increased avidity for boneseeking isotopes, indicating why skeletal scintigraphy was useful. A further twenty rabbits, in whose ilia the VX2 carcinoma was growing, were treated by local irradiation. When treatment was successful the tumour was destroyed, the production of new bone ceased, and the lesion lost its increased avidity for bone-seeking isotopes, indicating that skeletal scintigraphy could be used to assess the response of skeletal metastases to therapy.
1. Two patients with multicentric extra-abdominal desmoid tumours are reported. Multicentric foci have not been described previously. 2. Both cases conform to the usual clinical, macroscopic and microscopic criteria of desmoid tumours in all other regards. 3. Their occurrence in a single limb bud suggests a congenital propensity and leads to the advocacy of radical surgery in such cases.
1. A case of the scapho-capitate fracture syndrome is described. 2. At operation the proximal fragment of the capitate bone was found to have rotated on a transverse and not on a vertical axis.
