A family of 114 people in six generations, of whom 45 were affected by multiple epiphysial dysplasia, was described in this Journal in May 1960. We have been able to review them again 23 years later, and also to report on the type and incidence of the disease in more children and in a further generation. The relative severity of the disease in different branches of the family has been unchanged through the generations. Patients who were previously hopelessly crippled can today be rehabilitated by total hip replacement. Genetic counselling and birth control have reduced the relative and absolute numbers of affected children; this is tending to make the disease self-limiting

Our study describes the mid-term clinical results of the use of transtrochanteric valgus osteotomy (TVO) for the treatment of osteoarthritis of the hip secondary to acetabular dysplasia. The operation included valgus displacement at the level of the lesser trochanter, and lateral displacement of the greater trochanter by inserting a wedge of bone. We reviewed 70 hips. The mean age of the patients at operation was 44 years (14 to 59). Most (90%) had advanced osteoarthritis. The scores for pain and gait had improved significantly at a mean follow-up of 9.4 years. The rate of survival until an endpoint of a further operation during a follow-up of ten years was 82%. The survival rate was 95% in patients with unilateral involvement who were less than 50 years of age at operation. TVO is a useful form of treatment for advanced osteoarthritis of the hip, particularly in young patients with unilateral disease

The treatment of substantial proximal femoral bone loss in young patients with developmental dysplasia of the hip (DDH) is challenging. We retrospectively analysed the outcome of 28 patients (30 hips) with DDH who underwent revision total hip replacement (THR) in the presence of a deficient proximal femur, which was reconstructed with an allograft prosthetic composite. The mean follow-up was 15 years (8.5 to 25.5). The mean number of previous THRs was three (1 to 8). The mean age at primary THR and at the index reconstruction was 41 years (18 to 61) and 58.1 years (32 to 72), respectively. The indication for revision included mechanical loosening in 24 hips, infection in three and peri-prosthetic fracture in three. Six patients required removal and replacement of the allograft prosthetic composite, five for mechanical loosening and one for infection. The survivorship at ten, 15 and 20 years was 93% (95% confidence interval (CI) 91 to 100), 75.5% (95% CI 60 to 95) and 75.5% (95% CI 60 to 95), respectively, with 25, eight, and four patients at risk, respectively. Additionally, two junctional nonunions between the allograft and host femur required bone grafting and plating. An allograft prosthetic composite affords a good long-term outcome in the management of proximal femoral bone loss in revision THR in patients with DDH, while preserving distal host bone

Aims

Despite the presence of screening programmes, infants continue to present with late developmental dysplasia of the hip (DDH), the impact of which is significant. The aim of this study was to assess infants with late presenting dislocation of the hip despite universal clinical neonatal and selective ultrasound screening.

Aims

We report the clinical results of glenoid osteotomy in patients with atraumatic posteroinferior instability associated with glenoid dysplasia.

An attempt has been made to estimate the number of living people with skeletal dysplasias (osteochondrodysplasias) in Scotland, England and Wales, ascertained through five orthopaedic centres in different parts of Britain. Index patients and their affected relatives were sought and reassessed. Over the 30-year period between 1950 and 1979 inclusive a minimum prevalence was calculated (excluding stillbirths, perinatal deaths, and patients with chromosome anomalies, metabolic bone disease and short stature per se). The results indicate that there were in the community upwards of 10 000 individuals, at various ages over this period, with these largely genetic disorders. A more accurate estimate is of some 6000 of them requiring substantial orthopaedic care, and who were physically handicapped throughout life, about half of them severely so

Premature fusion of the triradiate cartilage was obtained surgically in 10 three-week-old rabbits, and compared with isolated fusion of the ilio-ischial and of the ilio-pubic limbs of the triradiate cartilage in two further groups of 10 rabbits. Complete fusion caused acetabular dysplasia five weeks after operation in all animals and hip dislocation at nine weeks in half of them; ilio-ischial fusion had a comparable effect. Ilio-pubic fusion had only a minimal effect on acetabular development. The posterior position of the ilio-ischial limb in the acetabulum and its predominance in the formation of the triradiate cartilage in quadrupeds may have contributed to its decisive effect on acetabular development

We studied the morphological changes in the proximal femur in 42 patients (42 hips) who had had varus derotation osteotomy of the upper femur for residual acetabular dysplasia and congenital subluxation of the hip and who did not show deformity of the head of the femur. In 19 patients the alignment of the whole leg was examined. The femoral neck-shaft angle (FNSA) at the final examination was unrelated to that immediately after operation or to the state of the acetabulum at that time. The postoperative FNSA was not related to the final result but the CE angle obtained at surgery influenced the outcome. The femoral length did not differ significantly between the unaffected and affected sides. Significant differences were found in the femorotibial angle (176.6 degrees v 174.5 degrees) and in the point of intersection between the mechanical axis and knee (65.0% v 57.2%)

The term developmental dysplasia of the hip (DDH) describes a spectrum of disorders that results in abnormal development of the hip joint. If not treated successfully in childhood, these patients may go on to develop hip symptoms and/or secondary osteoarthritis in adulthood. In this review we describe the altered anatomy encountered in adults with DDH along with the management options, and the challenges associated with hip arthroscopy, osteotomies and arthroplasty for the treatment of DDH in young adults.

Cite this article: Bone Joint J 2013;95-B:732–7.

We report the long-term results of 51 pelvic osteotomies in 43 patients with a mean follow-up of 15 years (13 to 20). The mean age of the patients was 28 years (14 to 46). At review three patients were lost to follow-up, and six had received a total hip arthroplasty. Of 48 hips, 42 (88%) were preserved, with good to excellent clinical results in 27 (64%). Pre-operatively, 41 (80%) of the treated hips had shown no sign of osteoarthritis. Thirty-one (65%) hips showed no progression of osteoarthritis after follow-up for 15 years. Significant negative factors for good long-term results were the presence of osteoarthritic changes and a fair or poor clinical score pre-operatively. Pelvic reorientation osteotomy for symptomatic hip dysplasia can give satisfactory and reproducible long-term clinical results.

We evaluated the long-term results of 116 total hip replacements with a superolateral shelfplasty in 102 patients with osteoarthritis secondary to developmental dysplasia of the hip. After a mean follow-up of 19.5 years (11.5 to 26.0), 14 acetabular components (12%) had been revised. The cumulative survival at 20 years was 78%, with revision for loosening of the acetabular component as the end-point. All grafts were well integrated and showed remodelling. In six grafts some resorption had occurred under the heads of the screws where the graft was not supporting the socket. Apart from these 14 revisions, seven acetabular components had possible radiological signs of loosening at a mean follow-up of 14.5 years, one had signs of probable loosening, and five had signs of definite loosening. These results indicate that this technique of bone grafting for acetabular reconstruction in hip dysplasia is a durable solution for cemented acetabular components.

A simple method of measuring the degree of acetabular development in the radiograph of the adult pelvis is described and arguments for its validity are advanced. This measurement is referred to as the acetabular angle. The normal values for this angle are between 33 and 38 degrees. Angles below 32 degrees are uncommon and probably of no clinical significance, whereas angles from 39 to 42 degrees are in the upper limit of normality. An angle of 47 degrees is shown in a hip with congenital subluxation. The prognosis for hip joints with acetabular angles between 42 and 47 degrees is under investigation.

We have reviewed 54 patients who had undergone 61 total hip replacements using bulk femoral autografts to augment a congenitally dysplastic acetabulum. There were 52 women and two men with a mean age of 42.4 years (29 to 76) at the time of the index operation. A variety of different prostheses was used: 28 (45.9%) were cemented and 33 (54.1%) uncemented. The graft technique remained unchanged throughout the series.

Follow-up was at a mean of 8.3 years (3 to 20). The Hospital for Special Surgery hip score improved from a mean of 10.7 (4 to 18) pre-operatively to a mean of 35 (28 to 38) at follow-up.

The position of the acetabular component was anatomical in 37 hips (60.7%), displaced less than 1 cm in 20 (32.7%) and displaced more than 1 cm in four (6.6%). Its cover was between 50% and 75% in 34 hips (55.7%) and less than 50% in 25 (41%). In two cases (3.3%), it was more than 75%.

There was no graft resorption in 36 hips (59%), mild resorption in 21 (34%) and severe resorption in four (6%).

Six hips (9.8%) were revised for aseptic loosening. The overall rate of loosening and revision was 14.8%. Overall survival at 8.3 years was 93.4%.

The only significant factor which predicted failure was the implantation of the acetabular component more than 1 cm from the anatomical centre of rotation of the hip.