We performed Charnley total hip arthroplasties on 64 patients (71 hips) between 1976 and 1984 for moderate congenital acetabular dysplasia in which a superolateral cement thickness of less than 20 mm was expected when the cup was placed in the true acetabulum at an angle of 45°. Of these, 59 hips were examined 10 to 17 years after operation; 37 (group A) had been operated on between 1976 and 1982 using Charnley’s original technique of cementing the acetabulum and 22 (group B) between 1983 and 1984 using more modern techniques. In group A, aseptic loosening of the socket was observed in ten hips (27.0%) and the 17-year survival rate was 81.5%. In group B, loosening was noted in only one socket (4.5%) and the 13-year survival rate was 100%. The improved techniques produced significantly better long-term results in fixation of the cup in dysplastic hips without bone grafting

We describe our experience with vascularised bone grafting for the treatment of fibrous dysplasia of the upper limb in eight patients, five men and three women, aged between 17 and 36 years. The site was in the humerus in six and the radius in two. Persistent pain, progression of the lesion and pathological fracture with delayed union were the indications for surgical intervention. We used a vascularised fibular graft after curettage of the lesion. Function and radiological progress were serially monitored. Early radiological union of the graft occurred at periods ranging from 8 to 14 weeks. The mean period for reconstitution of the diameter of the bone was 14 months (12 to 18) predominantly through inductive formation of bone around the vascularised graft, which was a prominent feature in all patients. There were no recurrences and none of the grafts sustained a fracture or failed to unite. After operation function was excellent in three patients and good in five. Vascularised bone grafts provide a safe and reliable means of ensuring good continuity of bone with little risk of recurrence and failure

Although the technique of autogenous acetabular bone grafting has been widely used to augment containment of the acetabulum in total hip arthroplasty (THA) for developmental dysplasia, the role of this technique in improving long-term results remains controversial. We present the long-term results of cemented THA with acetabular bone grafting in 112 patients (133 hips) in order to clarify the factors which affect the outcome. The mean follow-up was for 12.3 years (8 to 24). Kaplan-Meier survivorship analysis predicted a rate of survival of the acetabular component at 15 years of 96% (95% confidence interval (CI) 92 to 99) with revision for aseptic loosening as the endpoint, and of 75% (95% CI 65 to 85) when radiological loosening was used. Parametric survivorship analysis using the Cox proportional-hazards model indicated that trochanteric nonunion, lateral placement of the socket, and delayed trabecular reorientation of the bone graft were risk factors for loosening of the acetabular component. Our findings have shown that autologous acetabular bone grafting is of value for long-term success provided that the risk factors are reduced

1. A family is described of which several members in four generations suffered epiphysial growth disturbances of an unusual kind in the capital epiphyses of the femur, with shortness of stature. 2. This disorder forms one of a group of epiphysial growth disturbances which embraces several recognised disorders, whose effects on the epiphyses vary in severity. They may occur sporadically or they may be inherited as recessive or dominant characters. 3. Differentiation depends on the behaviour of the joint line–in other words, whether the epiphysis starts to show defects through inability to sustain the stress of weight bearing. In the most serious cases the picture is that of Morquio-Brailsford's disease; when the changes are less severe the appearance may be that of bilateral Perthes' disease. Differential diagnosis is no longer possible in later life, because the ultimate condition of the defective femoral heads is the same. Only in the hereditary cases is one able, by studying the capital epiphyses of the hip in childhood, to differentiate between the changes like those of Perthes' disease on the one hand, and micro-epiphysial dysplasia without necrosis on the other

An area of fibrous dysplasia of bone may undergo rapid enlargement which may be due to either cystic degeneration or malignant transformation. These complications may be clinically and radiologically indistinguishable and, unless both are borne in mind, incorrect management may follow. Magnetic resonance imaging was used in one of our cases and was the only imaging modality to demonstrate the true nature of the condition

1. A case of fibrous dysplasia in a woman aged fifty, with unilateral skeletal lesions, cutaneous pigmentation and possibly accelerated skeletal growth, is described. 2. At necropsy, the bony changes were found to be complicated by many of the changes of lipoid granulomatosis. Disseminated plaques of demyelinisation were found in the central nervous system. 3. The implications of the case are discussed

Between 1986 and 1990, we carried out 55 rotational acetabular osteotomies in 54 patients with acetabular dysplasia. Five hips were lost to follow-up. Of the 50 remaining, the degenerative changes were classified according to the criteria of Tönnis as grade 0 in 23, grade 1 in 16 and grade 2 in 11. The mean age of the three men and 46 women at the time of operation was 31.8 years (13 to 53). The mean follow-up was 137 months (120 to 174). At the most recent follow-up, 48 patients had satisfactory relief from pain. There was a slight decrease in the range of movement, particularly of flexion, in 18 hips. Radiologically, all osteotomies had united satisfactorily. There was radiological evidence of improvement in degenerative changes in 13 hips (5 grade 1 and 8 grade 2). Ten deteriorated (5 grade 0, 3 grade 1, and 2 grade 2) and one required total hip arthroplasty ten years after osteotomy. The osteoarthritis in the two hips with an associated valgus osteotomy progressed. The changes in radiological indices such as the centre-edge angle, acetabular femoral head index, acetabular root obliquity and horizontal or vertical displacement of the femoral head showed no statistical difference (unpaired Student’s t-test) between the patients with radiological progression and those with and without improvement

We treated 26 hips (24 consecutive patients) with residual dysplasia by a technique of incomplete triple pelvic osteotomy. The mean age of the patients was 21.6 years. The mean values for the pre-operative centre-edge angle of Wiberg, the refined centre-edge angle, the acetabular angle of Sharp, the modified acetabular angle and femoral head lateralisation were 7.7°, −3.1°, 49.3°, 53.2° and 17.2 mm, respectively. After a mean follow-up of 3.3 years they were 27.0°, 13.0°, 38.9°, 44.3° and 15.9 mm, respectively (p < 0.05). The osteoarthritic grading changed adversely in one hip. The mean pre-operative and latest Harris hip scores were 74.9 and 93.0, respectively (p < 0.05). This technique provides a stable osteotomy with maintenance of the posterior column which allows early mobilisation and minimal internal fixation. The technique is not complex and requires minimal blood transfusion. The use of an image intensifier is not necessary and harvesting of a subcristal bone graft avoids post-operative complications at the donor site

1. The results of Chiari pelvic osteotomy have been examined two to six years after operation in a group of eighteen patients with persisting acetabular dysplasia in the second and third decades. 2. The analysis suggests that for a good clinical result it is essential to create a relationship of the new outer acetabular lip to the original lip so that the CE angle is between 20 and 40 degrees and the roof angle is between 10 degrees below and 20 degrees above the horizontal. 3. This may be achieved by a pelvic osteotomy immediately above the joint capsule, angled 10 degrees upwards and inwards and displaced by 50 per cent of the pelvic thickness. 4. Details of the operative technique using a Smith-Petersen approach are presented

Seventeen patients with congenital spondylo-epiphysial dysplasia from six centres in Britain have been investigated and two variants delineated. There is wide clinical and radiological variability in each group with overlap between them, but 12 of the patients had very short stature and grossly disorganised hips with severe coxa vara, and the five remaining patients were less seriously affected with height only a little below the third percentile and only mild coxa vara. Both groups can be diagnosed at birth but the two cannot be differentiated on clinical and radiological grounds until after the age of three to four years when the developing severe coxa vara and difference in stature become apparent. All cases were sporadic with the exception of a concordant twin-pair

We have reviewed the outcome of 134 hips in 96 children with Graf type-III or type-IV dysplasia of the hip on ultrasound examination. We treated 28 affected hips in 22 children with the Craig splint, 43 hips in 30 children with the Pavlik harness, and 26 hips in 16 children with the von Rosen splint. A total of 37 affected hips in 28 children was not splinted. All children were less than three months of age at referral. Those treated with the von Rosen splint had a significantly better ultrasound appearance at 12 to 20 weeks of age and fewer radiological abnormalities than those not splinted or treated with the Pavlik harness. In the von Rosen group no hip required further treatment with an abduction plaster or operation compared with ten in the Pavlik harness group, three in the Craig splint group and eight in the group without splintage. Our results suggest that the von Rosen splint is more likely to improve the outcome of neonatal dysplasia of the hip and a definitive, large-scale randomised trial is therefore indicated

Three brothers with the X-linked variety of spondylo-epiphyseal dysplasia tarda are presented. Their clinical courses differed: one was relatively free of symptoms, the second had slowly progressive osteoarthritis of the hip, and the third had an acute, disabling arthritis necessitating total hip arthroplasty. The clinical features of this disorder are reviewed

The clinical features of nine new patients with dysplasia epiphysialis hemimelica are reported, with a long-term follow-up on a further seven patients who were described in the earliest case reports of this disease. Each of these 16 patients had only one leg involved, but 12 had multiple epiphyses affected. The distal femur, distal tibia and talus were the commonest sites and most patients presented with painless swelling or deformity. Wasting of the muscles of the affected leg was a common finding, and was occasionally disproportionate to the degree of disuse. One patient had the unique combination of involvement of the lateral and medial halves of different epiphyses in the same limb and another had unusual metaphysial changes. Diagnosis was often delayed despite typical radiographic appearances. There was no evidence for a genetic component in the aetiology nor was any common environmental factor identified. Treatment by local excision was generally effective for lesions in the vicinity of the knee, but some patients with involvement of the talus required arthrodesis around the ankle. The long-term prognosis appears to be good and so far only two of these patients have developed premature osteoarthritis

We carried out the Bernese periacetabular osteotomy for the treatment of 13 dysplastic hips in 11 skeletally mature patients with an underlying neurological diagnosis. Seven hips had flaccid paralysis and six were spastic. The mean age at the time of surgery was 23 years and the mean length of follow-up was 6.4 years. Preoperatively, 11 hips had pain and two had progressive subluxation. Before operation the mean Tönnis angle was 33°, the mean centre-edge angle was −10°, and the mean extrusion index was 53%. Postoperatively, they were 8°, 25° and 15%, respectively. Pain was eliminated in 7 patients and reduced in four in those who had preoperative pain. One patient developed pain secondary to anterior impingement from excessive retroversion of the acetabulum. Four required a varus proximal femoral osteotomy at the time of the pelvic procedure and one a late varus proximal femoral osteotomy for progressive subluxation. Before operation no patient had arthritis. At the most recent follow-up one had early arthritis of the hip (Tönnis grade I) and one had advanced arthritis (Tönnis grade III). Our results suggest that the Bernese periacetabular osteotomy can be used successfully to treat neurogenic acetabular dysplasia in skeletally mature patients

1. An account is given of a family in which five members in three generations were affected by osteochondritis involving the hips, in three cases bilaterally. 2. One patient showed aseptic osseous necrosis of the epiphyses of the ankles and fingers. 3. The differential diagnosis between Perthes' disease and multiple epiphysial dysplasia is discussed, but it is not certain into which category these patients fall. 4. The assistance of a family history and skeletal survey in diagnosis is illustrated

1. Early recognition of femoro-pelvic instability in proximal femoral dysplasia is essential to rational management. 2. Certain radiological signs helpful in identifying such patients within the first year are described. 3. Early operation is recommended to prevent displacement of a pseudarthrosis and to encourage healing. Alternative methods are described for established defects. 4. Radiological signs are described which indicate that healing without loss of femoro-pelvic stability will occur. Such patients may be managed expectantly

A Salter innominate osteotomy is used to treat acetabular dysplasia, but reports of its effects on the position of the femoral head are few and conflicting. Lateral shift would increase the resultant forces acting on the joint and be detrimental. We studied 15 Salter innominate osteotomies and demonstrated that a correctly performed osteotomy does not significantly alter the distance from the centre of the femoral head to the midline of the body. Stereophotogrammetry was used in three patients to delineate the axis of rotation of the distal acetabular fragment and determine the locus of movement of the centre of the femoral head about it. Our results explain why the Salter osteotomy does not lateralise the femoral head

Between May 1992 and April 1997, there were 20 452 births in the Blackburn District. In the same period 1107 infants with hip ‘at-risk’ factors were screened prospectively by ultrasound. We recorded the presence of dislocation and dysplasia detected under the age of six months using Graf’s alpha angle. Early dislocation was present in 36 hips (34 dislocatable and 2 irreducible). Of the 36 unstable hips, 30 (83%) were referred as being Ortolani-positive or unstable; 25 (69%) of these had at least one of the risk factors. Only 11 (31%) were identified from the ‘at-risk’ screening programme alone (0.54 per 1000 live births). Eight cases of ‘late’ dislocation presented after the age of six months (0.39 per 1000 live births). The overall rate of dislocation was 2.2 per 1000 live births. Only 31% of the dislocated hips belonged to a major ‘at-risk’ group. Statistical analysis confirmed that the risk factors had a relatively poor predictive value if used as a screening test for dislocation. In infants referred for doubtful clinical instability, one dislocation was detected for every 11 infants screened (95% confidence interval (CI) 8 to 17) whereas in infants referred because of the presence of any of the major ‘at-risk’ factors the rate was one in 75 (95% CI 42 to 149). Routine ultrasound screening of the ‘at-risk’ groups on their own is of little value in significantly reducing the rate of ‘late’ dislocation in DDH, but screening clinically unstable hips alone or associated with ‘at-risk’ factors has a high rate of detection

In acetabular dysplasia, fixation of the acetabular component of a cemented total hip prosthesis may be insecure and superolateral bone grafts are often used to augment the acetabular roof. We used finite element analysis to study the mechanical importance of the lateral acetabular roof and found that the lateral acetabular rim plays an important role in the load transfer of the pelvic bone. When the superlateral rim was lacking, the load shifted to the posterosuperior rim and to the area of pubic support, and the stresses in all materials, especially in the cement and in the trabecular bone, increased greatly. At the cement-bone interface the tilting component of the shear stress increased threefold. In a model in which the dysplastic acetabulum was augmented by a rigidly fixed, load-transmitting bone graft, the stresses were considerably diminished

We evaluated the long-term results of a modified Spitzy shelf operation for secondary osteoarthritis in 119 hips with a mean follow-up of 23.8 years. The mean age of the patients at the time of surgery was 25 years. Preoperative osteoarthritic change, the age at operation and shelf height were important factors in determining the outcome. Of the 61 hips in the pre-stage (three) and the initial stage (58) of osteoarthritis, 53 (87%) had good results, compared with only 30 (51%) of 58 hips with advanced osteoarthritis. Of the latter, 72% of those aged less than 25 years had good results compared with only 40% of patients aged over 25 years. The shelf height in the group with good results was significantly lower than in those with poor results. This operation is a safe procedure and indicated for acetabular dysplasia or subluxation of the hip with early osteoarthritic change in patients aged less than 25 years