The case histories and investigations for five adolescent girls with a presumed diagnosis of either primary acetabular protrusio or acute idiopathic chondrolysis are presented. The follow-up ranged from three to nine years. All were treated by extensive soft-tissue release but in no case did this improve movement of the affected hip and permanent stiffness was the inevitable result. The literature is reviewed and methods of treatment are discussed in the light of the CT findings

Bone-marrow oedema can occur both in isolation and in association with necrosis of bone, but it has not been shown whether each respond to the same methods of treatment. We treated 16 patients with isolated oedema and 17, in which it was associated with necrosis of the proximal femur, with the prostacyclin derivative iloprost, which has been shown to be effective in the idiopathic form. The Harris hip score, the range of movement, the extent of the oedema as measured by MRI, pain on a visual analogue scale and patient satisfaction were recorded before and subsequent to treatment. In both groups, we were able to show a significant improvement (p < 0.001) in these observations during the period of follow-up indicating that iloprost will produce clinical improvement in both circumstances

A study of two siblings with a severe infantile form of familial idiopathic hyperphosphatasia is reported. A girl aged one year was followed for two years while receiving intermittent treatment with porcine calcitonin. This induced a clinical remission, a reduction of both the high serum level of alkaline phosphatase and the raised urinary excretion of hydroxyproline, and a remarkable improvement in bone structure as seen radiologically. Her sister aged two months received porcine calcitonin for three weeks, during which clinical improvement, no change in the serum level of alkaline phosphatase and a marked decrease of the excretion of hydroxyproline were recorded

We have reviewed 54 hips in 46 patients from 2 to 14 years after a joint-preserving operation for idiopathic avascular necrosis of the femoral head. The choice between core decompression (17 hips), bone grafting (18), rotation osteotomy (15) or varus osteotomy (4) was determined by the stage and location of the area of necrosis. The overall success rate was unexpectedly low at 60%. Core decompression and bone grafting by our techniques gave poor long-term results, but those of rotation or varus osteotomies, performed with care for the correct indications, were better. The indications for each procedure are discussed: osteotomy is best when the area of necrosis is shallow and localised in the medial or anterior portion of the femoral head

Aims

Scoliosis is a lateral curvature of the spine with associated rotation, often causing distress due to appearance. For some curves, there is good evidence to support the use of a spinal brace, worn for 20 to 24 hours a day to minimize the curve, making it as straight as possible during growth, preventing progression. Compliance can be poor due to appearance and comfort. A night-time brace, worn for eight to 12 hours, can achieve higher levels of curve correction while patients are supine, and could be preferable for patients, but evidence of efficacy is limited. This is the protocol for a randomized controlled trial of ‘full-time bracing’ versus ‘night-time bracing’ in adolescent idiopathic scoliosis (AIS).

A radiological study of 50 patients with thoracic Scheuermann's disease revealed two types of lateral spinal curvature. A total of 43 lateral curves was present in 35 of the patients. Thirteen were apical at the same level as the Scheuermann's kyphosis and were due to vertebral-body wedging in the coronal plane; these curves had a mean Cobb angle of 15 degrees, occurred with equal prevalence in boys and girls and were directed equally to right and left. Thirty curves occurred in regions of compensatory lordosis (mean 5.6 degrees) situated above or, more commonly, below the Scheuermann's kyphosis. These scolioses had a mean Cobb angle of 16 degrees, were more often convex to the right than to the left and were significantly more prevalent in girls than in boys. The presence of these kyphoses and scolioses in the same spine, separated by only a few vertebrae, emphasises the importance of the sagittal plane in idiopathic spinal deformities and strongly suggests that idiopathic scoliosis and Scheuermann's disease share a common pathological process

The systemic use of steroids and habitual alcohol intake are two major causative factors in the development of idiopathic osteonecrosis of the femoral head (ONFH). To examine any interaction between oral corticosteroid use and alcohol intake on the risk of ONFH, we conducted a hospital-based case-control study of 71 cases with ONFH (mean age 45 years (20 to 79)) and 227 matched controls (mean age 47 years (18 to 79)). Alcohol intake was positively associated with ONFH among all subjects: the adjusted odds ratio (OR) of subjects with ≥ 3032 drink-years was 3.93 (95% confidence interval (CI) 1.18 to 13.1) compared with never-drinkers. When stratified by steroid use, the OR of such drinkers was 11.1 (95% CI 1.30 to 95.5) among those who had never used steroids, but 1.10 (95% CI 0.21 to 4.79) among those who had. When we assessed any interaction based on a two-by-two table of alcohol and steroid use, the OR of those non-drinkers who did use steroids was markedly elevated (OR 31.5) compared with users of neither. However, no further increase in OR was noted for the effect of using both (OR 31.6). We detected neither a multiplicative nor an additive interaction (p for multiplicative interaction 0.19; synergy index 0.95), suggesting that the added effect of alcohol may be trivial compared with the overwhelming effect of steroids in the development of ONFH.

Cite this article: Bone Joint J 2013;95-B:320–5.

The evolution of an idiopathic scoliosis is determined by the site of the primary curve and by the age of onset. It is significant that thoracic primary curves are commonly severe and the early onset of this curve accentuates this feature. Early operation based on prognosis is practised but sufficient time has not yet elapsed to justify any conclusions.

An attempt has been made to determine the aetiological factors in infantile idiopathic scoliosis from a clinical, genetic and epidemiological survey of 134 infants, ninety-seven of whom developed a curve in the first six months of life. Plagiocephaly was present in all cases; mental retardation occurred in 13 per cent of males with progressive scoliosis; congenital dislocation of the hip occurred in 3.5 per cent of cases and congenital heart disease in 2.5 per cent; and inguinal hernia was found in 7.4 per cent of males. Approximately 3 per cent of parents and 3 per cent of sibs had the same deformity, thirty times the general population frequency for the Edinburgh area. Other positive findings included an excess of breech presentations and of premature, low birthweight males, and a preponderance of curves developing in the winter months. Infants with progressive scoliosis tended to have older mothers and to come from poorer families. Only three children, all with resolving scoliosis, habitually lay prone in early infancy, in marked contrast to North American infants where this posture is usual. The almost complete absence of infantile idiopathic scoliosis in North America is noted and it is thought that the two facts may be related. The aetiology is likely to be multifactorial, with a genetic tendency to the deformity which is either "triggered off" or prevented by external factors.

1. Idiopathic necrosis of the femoral head is generally considered to be a rare disease but it appears to be rather frequent in France in view of the fact that 139 cases were recorded in the orthopaedic clinic of Hôpital Cochin between 1959 and 1963. Ninety cases treated by operation have been analysed in this paper. Men are nearly exclusively affected between the ages of eighteen and seventy, with the highest incidence between thirty and fifty years of age. Both hips are affected in 52 per cent of cases.

2. The etiology is unknown, but steroid therapy was noted in 36 per cent of the cases and some history of slight injury in 30 per cent. The sudden onset of pain in half the cases suggests the obliteration of one of the blood vessels supplying the femoral head.

3. Radiographs are often normal at the time of onset of the symptoms but later they show increased density of the head localised to the antero-superior aspect, and later still collapse of this weight-bearing region. The extent of the lesion appears to be determined from the very beginning rather than to be progressive. The superior joint space is never reduced and may in fact be widened.

4. Pathological examination of the head and neck confirms necrosis of the cancellous bone and the integrity of the overlying cartilage, but shows deep to the necrotic region a highly reactive zone characterised by hypervascularity and raised metabolism. These features have been demonstrated by injection of the blood vessels and also by the uptake of phosphorus 32 and by the succino-deshydrogenase test.

5. In six cases microscopic vascular lesions were found in the antero-lateral pedicle of the femoral head.

6. The high degree of activity of the tissue deep to the necrotic zone gives some hope for revascularisation of the necrotic segment. For this reason protection from pressure may be the way to prevent dramatic collapse of the head. Rest, medical treatment and freedom from weight bearing, however, do not achieve adequate protection. Varus or rotation osteotomy of the femoral neck not only gives relief from pain but appears to prevent collapse of the femoral head.

7. When destruction of the head has already taken place good results may be expected from the insertion of a metallic prosthesis, provided the acetabulum is sound. The results are less favourable when the acetabulum has been altered by secondary arthritic change, and arthrodesis may have to be considered if the disease is unilateral or when a prosthesis has been successfully inserted on the other side.

1. One hundred babies under the age of one year with untreated structural idiopathic scoliosis have been studied and the outcome assessed.

2. Ninety-two recovered spontaneously. Five developed progressive curves of bad prognosis and three developed secondary structural (double primary) scoliosis.

3. It is suggested that intrauterine moulding may cause the deformity.

1. Resolving infantile scoliosis is transient and unimportant; progressive infantile idiopathic scoliosis can be catastrophic.

2. To be able to differentiate the two at an early stage is a considerable advance. This is important for many reasons, but particularly for parents who are anxious for the future of an infant with a small curve which looks so innocent but which can be so malignant.

3. With the new observations reported by Mehta on the difference of the angles between the apical vertebra and its two ribs, and on the radiological relationship of these rib heads to the vertebral body, the prognosis is now almost wholly reliable.

4. Our former clinical impression that all cases with compensatory curves are progressive has now been verified.

5. At last an early distinction between progressive and resolving scoliosis can be made with confidence.

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.