The majority of patients with chronic infantile and juvenile forms of spinal muscular atrophy survive to adult life. Forty-four patients have been reviewed at an average of 17 years after diagnosis. The subdivision of patients into four groups, based on the maximal physical function developed by the individual, correlates well with the onset and severity of secondary deformity of the limbs and spine. This information allows anticipation of the problems and plans for their treatment to be made from early childhood. After analysis of the orthotic and surgical treatment received by these patients, a specific programme of care is recommended for each of the functional groups

The articular cartilage of the patella was studied in 100 knees at necropsy. In twenty-one of these knees the cartilage changes were related to the trabecular architecture of the underlying bone. It would appear that the initiation and location of cartilage damage and its rate and degree of progression are related to the relative stiffness of the underlying cancellous bone. On the basis of our observations we suggest that the diagnosis "chondromalacia of the patella" should be reserved for patients with asymptomatic or transiently symptomatic fibrillation of the articular cartilage of the central medial patellar facet. Those patients with persistent patellofemoral pain should be considered to have some other syndrome

A number of problems in the treatment of acute osteomyelitis have remained unresolved in recent years. The clinical experience of ninety-three patients with proven acute haematogenous osteomyelitis is presented to help resolve these problems. Analysis of the clinical features, the operative, bacteriological and haematological findings is made and discussed in detail. Eighteen patients had continuing bone infection and recommendations are made as to how diagnosis and management might have been improved. Surgery is considered to be an essential part of the diagnostic and therapeutic management of this disease. A combination of cloxacillin and fusidic acid is recommended at the antibiotic treatment

1. Blood from forty-one healthy volunteers, seventy-one patients with fractures but without symptoms of fat embolism and seven patients with clinical evidence of fat embolism was examined microscopically for fat droplets and chemically for triglycerides. 2. Fat droplets, after Millipore filtration of the blood, were seen evenly distributed in all three groups. 3. There was no significant difference in triglyceride concentration between serum and filtrate in the three groups. 4. The results do not support the thesis that the Gurd test is a reliable aid in the diagnosis of fat embolism

1. Eighty-nine cases of Perthes' disease are reviewed. 2. The prognosis varies with the amount of the epiphysis involved. 3. It is possible to assess the amount of epiphysial involvement by a study of the early radiographs. Cases were allocated to four groups on this basis. 4. It is confirmed that both sex and age at the time of diagnosis influence the final prognosis. The reasons for this are discussed. 5. The concept of "the head at risk" is suggested and radiological signs described to diagnose such cases. 6. It is hoped that the classification suggested may in future act as a basis for comparisons of treatment

1. Nineteen patients with classical rheumatoid arthritis complicated by severe subluxation of the cervical spine are reported. 2. Thirteen patients had atlanto-axial subluxation. This was the only level ofinvolvement in ten. 3. The next most frequent level to be involved was C.4-5. This occurred in five patients. 4. Eleven patients required surgery for symptoms or signs of spinal cord compression or vertebral artery insufficiency. 5. Operations included six posterior fusions, three anterior fusions and two laminectomies. 6. The differential diagnosis, the radiological findings, the indications for surgery and the results of treatment are discussed

1. Two cases of costal chondritis are presented. The special features and treatment of this condition are described and the literature is reviewed. 2. Early diagnosis and energetic treatment of costal chondritis obviates serious morbidity. 3. Treatment with antibiotics is of value in curing the condition before cartilage necrosis has occurred and in controlling the spread of infection to neighbouring tissues. 4. Excision of all necrotic cartilage is essential for cure, if cartilage necrosis has occurred. 5. Secondary infection with organisms of low virulence, notably pseudomonas pyocyanea, is nowadays the commonest cause of chronicity

1. The occurrence of bilateral double-layer patellae in association with multiple epiphysial dysplasia is described in three siblings. 2. Twelve cases of bilateral double-layer patellae have been reported previously, and in all there was an accompanying skeletal dystrophy which, although not diagnosed as such, is likely to have been multiple epiphysial dysplasia. 3. It is suggested that although they occur only in a minority of cases, double-layer patellae when present are a feature of considerable diagnostic value in multiple epiphysial dysplasia. Lateral radiography of the knees may therefore be of assistance in the diagnosis of multiple epiphysial dysplasia

We followed, prospectively, 77 patients (90 hips) with early-stage, non-traumatic osteonecrosis of the femoral head for a mean period of nine years. At the time of final review, 56 (62%) were symptomatic. Using the Cox model, the initial radiological stage, the progression of staging, the reduction in size of the lesions and the percentage of necrotic volume on MRI were identified as risk factors. Using the Harris score of < 70 or surgery as the endpoint, the cumulative rates of survival were 60.0% at one year, 43.3% at two years, 38.9% at five years and 37.2% at ten years. Survival curves demonstrated that clinical deterioration could occur 90 months after the initial diagnosis, suggesting that asymptomatic patients should be followed carefully for several years. Radiological time-dependent reduction in size without progressive collapse may represent repair even when the collapse is minimal

Sickle-cell disease (SCD) is probably the commonest cause of avascular necrosis worldwide, and its prevalence appears to be rising in developed countries. Avascular necrosis of the humeral head is a common complication but has not been previously studied in detail. We have reviewed 138 patients with SCD for clinical, radiological and functional abnormalities of the shoulder, using a radiological classification designed for avascular necrosis of the shoulder. Radiographic lesions, frequently bilateral, were found in 28% and only 53% of patients had normal shoulder function. The management of this relatively common complication is difficult. Joint replacement is likely to fail and early diagnosis is important

Coagulase-negative staphylococci produce an exocellular glycolipid antigen which has potential as a serological marker of infection in bone. The value of this newly detected antigen was investigated by enzyme-linked immunosorbent assay (ELISA) in 15 patients with culture-proven infection of prostheses caused by Gram-positive bacteria. The antigen was purified by gel-permeation chromatography from the culture supernatants of coagulase-negative staphylococci grown in a chemically defined medium. There were significant differences (p < 0.0001) between the serum IgG and IgM levels in patients with infection due to Gram-positive staphylococci and those of a control group of 32 patients with no infection. The ELISA test, which has potential for the diagnosis of infection, may be valuable in distinguishing between staphylococcal infection around prostheses and aseptic loosening

In an eight-year period we treated 51 cases of vascular injury associated with fractures and/or dislocations or soft-tissue injuries of the limbs. We relied on a clinical diagnosis and immediate exploration of blood vessels rather than the time-consuming procedure of arteriography. All patients were operated on by the orthopaedic residents on duty and not by vascular surgeons. Only 17 (33%) were repaired within six hours of injury. Limb viability with good function was obtained in 38. Complications included six deaths, four amputations, two renal failures and delayed occlusion in one case

We report a case of systemic intraosseous lipomatosis involving the proximal femur, both ends of the tibia, and the tarsal and metatarsal bones. The lesions progressed during a five-year follow-up with a pathological fracture of the tibial plateau. CT scans were characteristic and helpful in diagnosis but MR imaging added little information. Intraosseous lipomatosis is a hamartomatous malformation due to hyperplasia of adipose tissue, and is fundamentally different from solitary benign intraosseous lipoma. Management involves reconstruction of any pathological fracture. Large progressive lesions should be treated by curettage and grafting in an attempt to prevent such fractures

Thirty-six patients with reflex sympathetic dystrophy primarily affecting the knee were reviewed. Injuries or operation about the patellofemoral joint triggered its onset in 64% of patients. Co-existent mechanical derangement of the knee was present in 64% of patients. Those patients who underwent sympathetic blockade or sympathectomy within one year of onset of symptoms had significantly better pain and function scores than those in whom intervention was later. Early diagnosis remains the key to successful management. Surgery for co-existent mechanical derangement in the affected knee should not be performed until the syndrome is controlled

This prospective study was performed to determine the true incidence of deep vein thrombosis of the lower limb in children who had undergone halo-femoral traction to correct scoliosis before operation. Bilateral ascending phlebography of the lower limbs was performed on 54 children two days before spinal fusion and Harrington rod instrumentation. Two patients developed clinical evidence of thrombosis whilst on traction. This diagnosis was confirmed by phlebography. The other 52 children had no clinical evidence of thrombosis and their venograms were normal. The incidence of thrombosis was 3.7 per cent and clinically silent thrombosis did not occur

The incidence of nerve injuries in primary shoulder dislocation and humeral neck fracture is uncertain. We made a prospective study of 101 patients, using clinical examination and extensive electrophysiological assessment when there was suspicion of nerve damage. We found electrophysiological evidence of nerve injury in 45%, most involving the axillary, suprascapular, radial and musculocutaneous nerves. There were significantly more nerve injuries in older patients and those with a haematoma. Most patients recovered partially or completely in less than four months, and only eight had persistent motor loss. Early diagnosis and physiotherapy are recommended

1. Strain or rupture of the anterior marginal attachments of the meniscus was observed in approximately 10 per cent of a group of patients operated upon for internal derangement. 2. In about half of these a tear of the body of the meniscus was found and it is probable that this tear may have been the cause of the symptoms. In the remainder no injury to the body of the meniscus was found. 3. Recognition may be difficult and delay in diagnosis may be the cause of degenerative joint changes. 4. The condition should be looked for at operation when the meniscus appears to be intact or when the only lesion appears to be an area of chondromalacia on the weight-bearing surface of the femoral condyle

The follow-up of these seventy-three cases suggested the following conclusions:. 1. The clinical signs alone are not always sufficient to make the diagnosis of herniated nucleus pulposus with sufficient accuracy. 2. When positive, myelography was reliable as a means of localising the hernia. 3. In doubtful cases in which conservative treatment failed a clearly positive myelography turned the scale for a well-indicated operation. 4. In doubtful cases a clearly negative myelography has often rightly been a contra-indication for laminectomy. 5. No serious complication from the use of water-soluble contrast medium for myelography has been encountered

1. A defect of the middle of one tibial crest is described in young healthy ballet dancers. 2. The clinical and radiological characteristics have been studied in four cases, and the histological appearances in two. 3. The defect is concluded to be an incomplete fatigue fracture—a fatigue infraction. 4. A fifth, uncertain, case has been added tentatively, as presenting a possible serious complication, namely acute fracture from muscle action. 5. The diagnosis, treatment and prognosis are mentioned, with emphasis on the importance of adequate clinical and radiological examination in cases of obscure mid-tibial pain, tenderness or swelling

In 30 patients in whom osteomyelitis was suspected fine-needle bone biopsies (FNBB) were taken at the same time as bone was aspirated for bacteriological examination. The diagnosis of osteomyelitis was eventually confirmed in 15 patients; the other 15 had myositis (3), arthritis (3), trauma (2), microgeodic phalangeal syndrome (2), haematoma in a non-ossifying fibroma (1), and Ewing's sarcoma (1). In three patients no pathology was found. The temperature, WBC and ESR at presentation did not help to distinguish osteomyelitis from other conditions. FNBB, however, proved to be a useful additional investigation with a sensitivity for osteomyelitis of 87% and a specificity of 93%