Arthrogryposis multiplex congenita is believed to be a specific clinical entity which is aetiologically unrelated to the "arthrogryposis-like" deformities of known neurological diseases such as myelomeningocele and myelodysplasia. The observation that the condition appeared to be three times as common in Melbourne, Australia, as in four centres in the United Kingdom (Wynne-Davies and Lloyd-Roberts 1976), prompted this survey of 132 patients: 73 from the United Kingdom, 34 from Australia and 25 from Wilmington, Delaware, USA. The survey aimed to established the same criteria for diagnosis in the three countries and to search for prenatal and genetic aetiological factors. It was shown that all centers treated more newborn children with this disorder during the 1960s than either before or after that period. All cases were sporadic and there was no family association with talipes equinovarus, congenital dislocation of the hip or hereditary neuromuscular disease. "Environmental" findings from all three centers were similar and it was concluded that arthrogryposis multiplex cogenita is a non-genetic disease of early pregnancy, associated with a variety of unfavourable intra-uterine factors. In addition, an unknown but possibly viral environmental agent may have been present to a significant extent only during recent decades and is now declining

Midline prolapse of a disc causing compression of the cauda equina is rare but needs urgent diagnosis and surgical treatment. The onset of bladder and rectal paralysis with saddle anaesthesia should be viewed with a high index of suspicion in a patient with backache and sciatica. Eight cases were seen over a period of five years, and they fell into three clinical groups. Group I patients presented with a sudden onset without any previous symptoms related to the back. Group II patients had a history of recurrent episodes of backache and sciatica, the latest episode resulting in involvement of the cauda equina. The group III patient was indistinguishable from one with a tumour as he presented with backache and sciatica slowly progressing to paralysis of the cauda equina. The prolapse was at the disc between L5 and S1 vertebrae in 50 per cent of the patients, most of whom did not have any limitation of straight leg raising. Urgent myelography and equally urgent removal of the disc within two weeks of the onset of the symptoms resulted in almost complete motor and bladder recovery within five months after the operation in most cases. However, recovery of sensation and sexual function was incomplete even four years after the operation

Osteomalacia may be a contributory factor in some patients in the development of fractures of the femoral neck and complicate the subsequent management. The level of serum alkaline phosphatase is often valuable in the diagnosis of metabolic bone disease but rises after any uncomplicated fracture, and since such a rise may limit the diagnostic usefulness of this measurement in detecting osteomalacia its extent was assessed in 106 patients. In the majority serum levels were normal on admission, rising after seven to nine days to reach a maximum within a month after fracture. Elevated levels on admission were found in patients with osteomalacia, liver damage or where there had been a delay of several weeks between injury and admission. In a small number of patients normal levels on admission subsequently reached very high values, usually in association with comminution or instability of the fracture. Elevated levels persisted for six to twelve weeks after fracture, the major influence upon the level at this time being the maximum value achieved rather than the presence of osteomalacia. If patients are to be screened for osteomalacia, the alkaline phosphatase must be measured within the first week after a fracture to avoid the distorting influences of the fracture itself

1. Forty-five patients with monarthritis of at least six months duration have been reviewed. Arthrotomy and synovial biopsy were carried out in every case. The period of follow-up varied from two to eight years from the time of biopsy. A re-examination of the biopsy material was made at the time of clinical assessment. 2. Twelve patients (27 per cent) were diagnosed as having definite or probable rheumatoid arthritis. Four patients (9 per cent) had psoriatic arthropathy and six (13 per cent) had a persistent monarthritis of undetermined type. A further four patients (9 per cent) had polyarthritis of undetermined type, ten (22 per cent) were diagnosed as having osteoarthritis and seven (16 per cent) were completely normal (self-limiting joint disease). 3. There was a moderately good relationship between biopsy findings suggestive of rheumatoid arthritis and the eventual clinical outcome. 4. Monarthritis of the wrist was followed in every case by the development of significant disease, either rheumatoid arthritis or psoriatic arthropathy. 5. Rheumatoid arthritis may remain monarticular for many years. 6. It is concluded that thorough investigation of patients with monarthritis is of considerable help in diagnosis and prognosis

1. The radiological features of skeletal hydatid disease are discussed. Osseous lesions occur in about 1 to 2 per cent of cases, bone being involved only after the embryos have passed the filters provided firstly by the liver and secondly by the lungs. At first, ill defined areas of translucency appear which are not diagnostic. In developed lesions, clear-cut destructive areas, with a surrounding sclerotic reaction, become visible. The cysts thin and expand the cortex and tend to spread throughout an affected bone. In advanced stages the cortex is ruptured, and exuberant hydatid cyst growth takes place in the adjacent soft tissue. Around this an ectocyst forms, which may later calcify, indicating death of the parasite. The progress of the disease is very slow. 2. Three cases of affection of the thoracic spine are described, and the differential diagnosis is considered, particularly from plasmacytoma and neurofibroma. Each case presented with cord pressure symptoms. Operative decompression relieved these totally in one case, incompletely in another, and not at all in the third and most advanced case. 3. With rapid and easy travel in the modern world hydatid disease is liable to be seen in areas where it is not endemic

Aims

The number of patients undergoing arthroscopic surgery of the hip has increased significantly during the past decade. It has now become an established technique for the treatment of many intra- and extra-articular conditions affecting the hip. However, it has a steep learning curve and is not without the risk of complications. The purpose of this systematic review was to determine the prevalence of complications during and following this procedure.

Aims

The aim of this study was to compare the efficacy of a corticosteroid injection for the treatment of carpal tunnel syndrome (CTS) in patients with and without Raynaud’s phenomenon.

Postoperative radiculopathy is a complication of posterior cervical decompression associated with tethering of the nerve root. We reviewed retrospectively 287 consecutive patients with cervical compression myelopathy who had been treated by multilevel cervical laminectomy and identified 37 (12.9%) with postoperative radiculopathy. There were 27 men and ten women with a mean age of 56 years at the time of operation. The diagnosis was either cervical spondylosis (25 patients) or ossification of the posterior longitudinal ligament (12 patients). Radiculopathy was observed from four hours to six days after surgery. The most frequent pattern of paralysis was involvement of the C5 and C6 roots of the motor-dominant type. The mean time for recovery was 5.4 months (two weeks to three years). The results at follow-up showed that the rate of motor recovery was negatively related to the duration of complete recovery of postoperative radiculopathy (γ = −0.832, p < 0.01) and that patients with spondylotic myelopathy had a significantly better rate of clinical recovery than those with ossification of the posterior longitudinal ligament (t = 2.960, p < 0.01). Postoperative radiculopathy may be prevented by carrying out an anterior decompression in conjunction with spinal fusion, which will achieve stabilisation and directly remove compression of the cord at multiple levels

We made a retrospective study of 149 children and adolescents with moderate spondylolisthesis (slip less than or equal to 30%), 77 treated by fusion and 72 conservatively at an average follow-up of 13.3 years. Both groups were fully comparable with regard to age at diagnosis, sex distribution (46% girls), and mean slip. The patients who were treated operatively had more pain before treatment and showed more initial progression of the slip. They had better clinical results and less pain at latest review, but the total progression of the slip over the whole follow-up showed no statistical differences between the two groups. Patients with a pseudarthrosis after attempted fusion had had a longer period of postoperative pain, but at the latest review had no more pain than those with sound fusion. None of those treated conservatively came to fusion later and the long-term results in 18 patients who had refused the advised operation were no worse than those for other conservatively treated patients. Our results suggest that a moderate grade of spondylolisthesis in adolescents usually has a benign course. It seems that spontaneous segmental stabilisation occurs as a result of degeneration of the disc at the level of the slip

1. A clinical, radiological and histological description of a patient with fibrogenesis imperfecta ossium is given. We think that this is the first case in which diagnosis has been made during the life of the patient. 2. The disease is characterised by a defect in the formation of the collagen fibres of the bone matrix. There is also a failure of normal calcification of the matrix, giving rise to the appearance of wide "osteoid" seams. When examined with the polarising microscope and when stained with Gomori's reticulin stain the collagen fibres can be seen to be grossly deficient and abnormal. 3. The patient presented at the age of fifty-four years with bone pain and multiple fractures. The only biochemical abnormality detected in the plasma was an elevated alkaline phosphatase. He was also in negative calcium balance. 4. Treatment with vitamin D. 2. , later changed to dihydrotachysterol, appears to have produced clinical, biochemical and radiological improvement. It appears that a direct action of the vitamin on the abnormal bone collagen must be postulated, in addition to its known actions on the calcifying mechanisms. 5. An unusual feature of the case was the slow development of a total unresponsiveness to large doses of vitamin D. 2. , in spite of a markedly elevated level of vitamin D in the plasma. There was later a response to a much smaller dose of dihydrotachysterol, which is being maintained to date

The foregoing suggestions may be summarised in the following recommendations for the treatment of osteogenic sarcoma. 1. Deep x-ray therapy in high dosage, followed by local resection, should be given serious trial especially: 1) in the upper limb; 2) in the group with atypical clinical or radiographic signs, or histology resembling that of inflammatory lesions; 3) with Grade I histology; and 4) in the young. 2. Deep x-ray therapy followed at once by amputation should be used for osteogenic sarcoma if : 1) local resection would leave a lower limb more unstable than an artificial leg ; or 2) if response to x-rays is poor. 3. Deep x-ray therapy alone should be used: 1) if the patient is unsuitable for, or refuses, any operation ; and 2) palliatively, if metastases are present or the tumour is too advanced, or the patient is not fit for radical treatment. 4. Amputation alone should be used palliatively, for pain or fungation, when x-ray therapy has failed to relieve, or is not readily obtainable. 5. Biopsy and histological grading must be performed in every case. A histological diagnosis is most important. Coley (1949) and MacDonald and Budd (1943) support this view. 6. The records of every possible case should be sent to and discussed by a group with special experience of these tumours

Aims

Preserving growth following limb-salvage surgery of the upper limb in children remains a challenge. Vascularized autografts may provide rapid biological incorporation with the potential for growth and longevity. In this study, we aimed to describe the outcomes following proximal humeral reconstruction with a vascularized fibular epiphyseal transfer in children with a primary sarcoma of bone. We also aimed to quantify the hypertrophy of the graft and the annual growth, and to determine the functional outcomes of the neoglenofibular joint.