Three cases are reported in which an osteosarcoma developed in relation to an enchondroma in a long bone. Two of the cases were in the proximal femur whilst one occurred in the proximal humerus, both recognised sites for old calcified enchondromas or "cartilage rests". The ages of the patients at presentation were 55, 63 and 84 years and all were women. Two patients died with pulmonary metastases within six months of the onset of clinical symptoms. Despite their intimate relationship to the enchondromas, none of the osteosarcomas could be shown histologically to have arisen from tumour cartilage. It appears probable that these are cases in which independently arising tumours have merged to form a so-called "collision" tumour, but the possibility that they could have been derived by dedifferentiation of a previously benign neoplasm cannot be discounted.
Hyperphosphatasia, or hereditary bone dysplasia with hyperphosphatasaemia, is a rare genetic disorder which is characterised by failure to transform woven into lamellar bone. Clinical, radiological and histological features establish the diagnosis, fractures, deformities, diffuse sclerosis on radiographs and high serum alkaline phosphatase being characteristic. We report the case of a 27-year-old man with follow-up at the same hospital for 20 years. Attempts at treatment with calcitonin and disocium etidronate (EHDP) failed, but stapling of the growth plates at the knee was successfully performed. Transverse "brittle" fractures of the humerus, lower leg and ribs healed normally, but internal fixation and late bone grafting were required for a subtrochanteric stress fracture of the femur at the age of 24 years. At present the patient has no clinical problems and leads a normal life.
Torsion and subsequent ischaemia is a well-recognised cause of symptoms and morbidity in general surgery. We present three cases of solitary pigmented villonodular tumours of the knee which were found to have undergone torsion. We believe these to be the first intra-articular tumours in which torsion has been reported.
Two cases of aneurysmal bone cyst in the pelvis which healed spontaneously are described. Spontaneous regression of aneurysmal bone cysts has not previously been well documented. Our experience with these two cases suggests that radical intervention is not always necessary.
The pathogenesis of slipped upper femoral epiphysis is unknown but the condition has been linked with various endocrine disorders. Nine patients with slipped epiphyses in association with primary juvenile hypothyroidism are presented. In all patients, slipping occurred or symptoms developed in the affected hip before the hypothyroidism was diagnosed. A generalised pathology was suggested by the absence of trauma (8 patients), by bilateral slipping (6 patients), and by obesity and short stature in all patients. All cases had delayed skeletal maturation and characteristic metaphysial changes were seen on their radiographs. The clinical diagnosis of juvenile hypothyroidism can be difficult but it merits consideration in patients who have a slipped upper femoral epiphysis in association with short stature, obesity, delay in skeletal maturity, or any one of these.
Six patients with severe haemophilic arthropathy of the elbow have been treated by limited excision arthroplasty using a sheet of silicone rubber as an interposition membrane. There were no complications. The symptoms and signs before and after operation have been assessed using the grading system advocated recently by the World Federation of Haemophilia. In all cases there was relief of pain and increased movement. Moreover, the subsequent incidence of spontaneous haemorrhage into the elbow has been reduced with considerable cost benefit.
The operative and anaesthesic technique for 44 patients undergoing posterior spinal fusion with Harrington rod instrumentation for idiopathic scoliosis is described. There were two groups of 21 and 23 patients, matched for diagnosis and status before operation. The management of both groups was similar but in one group anaesthesia with induced hypotension was employed, using a mixture of sodium nitroprusside and trimetaphan. The mean blood loss at operation and after operation in this group was significantly lower than in the other group, with a consequent reduction in the transfusion requirement. No adverse sequelae were observed. All patients showed a drop in haemoglobin concentration after operation, despite clinically adequate blood transfusion.
One hundred and twenty-five patients with 194 feet affected by congenital talipes equinovarus were treated by the senior author during the period 1959 to 1980. Of these, 70 patients presented either at birth or in the early neonatal period, and 55 were seen later, having been referred from other centres. Seventy-five patients were subsequently reviewed by two of us; the remaining 50 were assessed from records and research files. Patients seen within four weeks of birth were termed "early", the remainder "late". Of the early group of 70 patients, 44 (with 68 affected feet) were reviewed and 26 (with 41 affected feet) were assessed from records. Excellent or good results were achieved in 94 per cent of feet treated conservatively and in 82 per cent of feet which required pantalar release. Of the 55 late referrals 32 patients (with 55 affected feet) were reviewed and 23 (with 30 affected feet) were assessed from records. Satisfactory results were slightly less frequent, but were achieved in 75 per cent of cases. There was no statistical correlation between early soft-tissue release and a good final outcome, but there was a positive statistical correlation between good clinical results and a high talocalcaneal index. Osseous correction (a laterally based wedge tarsectomy or a triple arthrodesis) was necessary at a later date in four feet (four per cent) of those who presented early and in 13 feet (15 per cent) of late referrals.
Two cases of coccydynia are presented. Their definitive treatment was excision of the coccyx and the pericoccygeal tissues. The histology in both cases revealed a glomus tumour of the coccygeal body and the symptoms were completely relieved after operation. Glomus tumours of the coccygeal body may be the cause of some cases of coccydynia.
Five patients severely affected by haemophilia, in whom six knees showed advanced haemophilic arthropathy, have been studied. The patients presented with painful limitation of movement and increasingly frequent episodes of spontaneous bleeding in the affected knees. These all showed secondary degenerative changes and varus deformity. A Corrective osteotomy of the proximal tibia was performed under full haematological cover without complications. Follow-up ranged from 6 to 36 months, with a mean of 18 months. At follow-up all patients were free of pain and had retained their range of movement. In the three patients with a longer follow-up there was radiological improvement. The most significant feature was that there was almost complete cessation of bleeding episodes into the knee in all patients and no progression of the arthropathy. The cost benefit implications of this corrective procedure are discussed.
A study was made of the mechanics of blood-bearing in a series of patients treated with a cast-brace for fracture of the distal femur. Knee hinges incorporating strain-gauges, a simple force-plate on the floor and a standardised weight-bearing test were used to record axial loads through the cast-brace itself and through the fracture during the phases of healing. The cast-brace carried loads of only 10 to 20 per cent of body weight and functioned mainly as an antibuckling hinged tube. Patterns of weight-bearing recovery showed that the fracture itself limited loads to safe levels. A measure of the recovery of strength at the fracture was determined and termed the "fracture load index". Graphs obtained in this way demonstrated four biomechanical phases of bony union which correlated well with the stages of clinical healing. The clinical application of these results have led to improvements in the design of braces and the use of a cylinder cast-brace for fractures of the distal half of the femoral shaft and of a new type of brace with a hinge at the hip attached to the thigh cast for fractures of the proximal shaft. A simple clinical test is described by which it is possible to monitor the healing of fractures in cast-braces.
Six patients are described with idiopathic osteoporosis which began between the ages of 4 and 16 years. In four children the disorder was mild with pain in the back, vertebral collapse, qualitatively normal iliac bone biopsies, variable calcium balance and spontaneous recovery. The two remaining patients had progressive bone disease with deformity. One with a previously normal skeleton developed changes similar to those of osteogenesis imperfecta; in the other patient, who rapidly developed structural collapse associated with severe metaphysial osteoporosis, treatment was ineffective and the histological appearances of the bone suggested osteoblastic failure. Quantitative bone histology in four patients showed no evidence of excessive active resorption; and the ratio of Type III to Type I collagen in the skin was normal, in contrast to the findings in osteogenesis imperfecta. The significance of this study in relation to previous accounts is reviewed.
The correction of angular deformities of long bones by incomplete osteotomy, followed three weeks later by manual osteoclasis, overcomes the problem of secondary displacement sometimes seen after correction by complete osteotomy and makes internal fixation unnecessary. This paper presents an experience of twenty-six operations in eighteen patients. In all cases the deformity was corrected with excellent cosmetic and functional results. Complete bony union was achieved and there were no problems with displacement at the osteotomy site. Four cases are described in detail to illustrate use of the technique in different clinical situations.
A case is described in which a large area of unstable skin overlying a tibial fracture was replaced by a single stage skin flap transfer, using microvascular surgical techniques. The shortening of treatment time and the improvement in the local blood supply compared to that provided by orthodox skin flaps are noted. The improved blood supply at the fracture site may have encouraged bony union.
Paralytic clawing of the hallux may occur in children with congenital disorders of the neuraxis. Tenodesis of flexor hallucis longus allows correction of this deformity in early childhood. Seventeen of these procedures have been reviewed and good results were obtained in fifteen.
The clinical features of eight patients with myositis ossificans progressiva are described and the effects of treatment with the diphosphonate EHDP, together with surgical removal of ectopic bone, are assessed. Early correct diagnosis remains unusual, mainly because the significance of the short great toes is unrecognised, and because myositis may be mistaken for bruising, sarcoma or mumps. The diphosphonate disodium etidronate (EDHP) was given to all patients in an attempt to suppress calcification of new lesions; in five of them ectopic bone was removed during the treatment. EHDP sometimes delayed the mineralisation of newly formed bone matrix after surgical removal but this delay could not be predicted. The variable effect of EHDP may depend particularly on the amount absorbed and on the activity of new bone formation.
We describe unexpected adverse psychological and social results in three adult men with severe Little's disease who were made more mobile by successful surgery.
In a clinical, radiological and biochemical study of forty-two patients from Oxford with osteogenesis imperfecta, it was found that patients could be divided simply into mild, moderate and severe groups according to deformity of long bones. In the severe group (seventeen patients) a family history of affected members was uncommon and fractures began earlier and were more frequent than in the mild group (twenty-two patients); sixteen patients in the severe group had scoliosis and eleven had white sclerae; no patients in the mild group had white sclerae or scoliosis. Radiological examination of the femur showed only minor modelling defects in patients in the mild group, whereas in the severe group five distinct appearances of bone (thin, thick, cystic and buttressed bones, and those with hyperplastic callus) were seen. The polymeric (structural) collagen from skin was unstable to depolymerisation in patients in the severe group, but normal in amount, whereas the reverse was found in the mild group. This division according to long bone deformity may provide a basis for future research more useful than previous classifications.
1. The results of internal fixation are described in 470 tibial shaft fractures. 2. The immediate internal fixation of compound fractures was followed by so high an incidence of serious complications that the use of this method is not recommended. The immediate internal fixation of fresh closed fractures was also followed by many complications. 3. Delayed rigid internal fixation proved satisfactory for difficult fractures in which an acceptable reduction could not be obtained by closed means ; skeletal traction was of value in such fractures until the skin was soundly healed. 4. Delayed internal fixation reduced both the healing time and the complications.
