1. A case is reported of a Jamaican boy of fifteen months with typical and florid manifestations of dysplasia epiphysialis hemimelica associated with a number of atypical features not previously recorded. 2. The progress of the disorder over a limited period of two years is described and the development of the atypical features is discussed

After late reduction of the hip in children with developmental dysplasia the acetabular contour slowly improves and it is difficult to know if and when acetabular reconstruction is required. We studied the radiographs of 19 patients with unilateral dislocated or subluxated hips which had been reduced between the ages of one and two years. Preoperatively, all the affected hips showed acetabular dysplasia. After reduction they steadily improved for three years by which time none was dysplastic as measured by the acetabular index. After the age of ten years, when assessed by the more sensitive centre-edge angle, two were found to be dysplastic. It was not possible to predict these from early radiographs. We conclude that the decision to reconstruct an acetabulum should not be taken until three years after reduction, and that a few hips which appear to be developing satisfactorily at that time will, nevertheless, become dysplastic

1. Monostotic fibrous dysplasia in the long bones occurs most frequently in adolescence. In the jaws it is found mainly in early adult life. It presents later in the ribs, probably because it is often asymptomatic in this site. 2. The disease is equally distributed in both sexes. 3. Reactivation may occasionally occur in later life and in pregnancy. 4. Successful surgical treatment is by no means always easily achieved, and requires, in addition to the problems of fracture fixation and the correction of deformity, careful consideration of the age of the patient, the activity of the lesion and the extent to which it involves the cortical bone

We carried out a morphometric analysis of acetabular dysplasia in patients with cerebral palsy requiring hip surgery using three-dimensional CT. We evaluated 54 hips in 27 patients. The contralateral normal hips of ten patients with unilateral Perthes’ disease were used as a control group. The acetabular defects were assessed qualitatively as anterior, posterior or global. Quantitative assessments were made using three-directional acetabular indices (anterosuperior, superolateral and posterosuperior) and measured by multiplanar reformation, from which we calculated the acetabular volume. In the qualitative study, posterior defects were most common in the subluxation group whereas global defects predominated in the dislocation group. In the quantitative study, all acetabular indices in both the subluxation and dislocation groups were higher than those in the control group and the superolateral indices showed a tendency to elevation in the dislocation group. The acetabular volume was largest in the control group, smallest in the dislocation group, and intermediate between the two in the subluxation group

Aims

A borderline dysplastic hip can behave as either stable or unstable and this makes surgical decision making challenging. While an unstable hip may be best treated by acetabular reorientation, stable hips can be treated arthroscopically. Several imaging parameters can help to identify the appropriate treatment, including the Femoro-Epiphyseal Acetabular Roof (FEAR) index, measured on plain radiographs. The aim of this study was to assess the reliability and the sensitivity of FEAR index on MRI compared with its radiological measurement.

We reviewed 12 patients with primary glenoid dysplasia. Ten were assessed clinically and two from case notes and radiographs. We identified two groups according to the age at onset of symptoms. The first (seven patients) consisted of boys and younger men, all of whom developed symptoms before the age of 40 years. All four children were free from pain, whereas the three adults in this group had varying degrees of this. Four patients had symptoms of instability. The second group consisted of older men (five patients) all of whom had noted the onset of symptoms, in the form of pain and stiffness, after the age of 40 years. All five had radiological evidence of osteoarthritis. Although the four children in our study had minimal symptoms, all eight adults had ongoing shoulder pain and dysfunction, despite a specific rehabilitation programme. Four patients required surgery; one had posterior stabilisation for instability and three arthroplasties of the shoulder for osteoarthritis

The Fairbank Collection in the Radiology Department of the Royal National Orthopaedic Hospital contains details of 15 patients in the section labelled "Metaphysial Dysplasia and Dysostosis". This material has been reviewed, long-term follow-up of the patients carried out and the diagnosis revised, where indicated, according to current concepts. Of the 15 patients, all children, seven had recognisable bone dysplasias. The remaining eight, all listed under the heading "metaphysial fragility", had the characteristic radiographic features of "battered" babies

1. A case of metaphysial dysplasia observed for eleven years is reported. 2. Only one case with similar features has previously been reported. 3. The literature on the subject is reviewed

Aims

The aim of this study was to review the value of accepting referrals for children with ‘clicky hips’ in a selective screening programme for hip dysplasia.

We examined 50 patients with diastrophic dysplasia both clinically and radiologically. Two legally aborted fetuses were dissected. The mean age of the patients was 16.2 years (newborn to 38) and the mean follow-up was 11.4 years (3 months to 34 years). The fetal hips and MRI of newborn infants showed congruity and no significant joint deformity. Flexion contracture of the hip became evident later in 93% and was progressive. The radiological appearance of the proximal femoral ossific nuclei was delayed and in 17% of males and 28% of females the ossific nuclei had not appeared by the age of 12 years. Radiological measurements differed considerably from reference values and were related to the rapid and progressive restriction of rotational movement and the increase in flexion contracture. The typical findings were flattening and inferomedial bulking of the femoral head and a double-hump deformation. The changes in the hip led to secondary osteoarthritis before early middle age. We describe the clinical and radiological measurements which define the early degeneration of the joint

Aims

To monitor the performance of services for developmental dysplasia of the hip (DDH) in Northern Ireland and identify potential improvements to enhance quality of service and plan for the future.

1. A condition of fibrous dysplasia of the jaws occurring in four brothers and a sister has been under observation since 1931. 2. Three of the five patients have been operated upon for correction of grotesque deformity, with satisfactory results. 3. The pathological nature of the disorder is discussed

In order to clarify how intra-articular lesions influence the survival of a periacetabular osteotomy in patients with dysplasia of the hip, we performed an observational study of 121 patients (121 hips) who underwent a transposition osteotomy of the acetabulum combined with an arthroscopy. Their mean age was 40.2 years (13 to 64) and the mean follow-up was 9.9 years (2 to 18). Labral and cartilage degeneration tended to originate from the anterosuperior part of the acetabulum, followed by the femoral side. In all, eight hips (6.6%) had post-operative progression to Kellgren–Lawrence grade 4 changes, and these hips were associated with the following factors: moderate osteoarthritis, decreased width of the joint space, joint incongruity, and advanced intra-articular lesions (subchondral bone exposure in the cartilage and a full-thickness labral tear). Multivariate analysis indicated subchondral bone exposure on the femoral head as an independent risk factor for progression of osteoarthritis (p = 0.003). In hips with early stage osteoarthritis, femoral subchondral bone exposure was a risk factor for progression of the grade of osteoarthritis. . Although the outcome of transposition osteotomy of the acetabulum was satisfactory, post-operative progression of osteoarthritis occurred at a high rate in hips with advanced intra-articular lesions, particularly in those where the degenerative process had reached the point of femoral subchondral bone exposure

Craniodiaphyseal dysplasia (CDD) is a rare sclerosing bone disorder, the severity of which depends on its phenotypic expression. Hyperostosis can cause progressive foraminal stenosis leading to palsy of cranial nerves, epilepsy and mental retardation. We report the only case of CDD in an adult, with stenosis of the cervical canal leading to quadriparesis as a late complication of hyperostosis, and describe the problems associated with its treatment. Although the syndrome is rare, its pathophysiological and therapeutic considerations may be applicable to the management of stenosis of the spinal canal in other hyperostotic bone disorders

1. A family of six generations, consisting of 114 members affected by epiphysial dysplasia of the lower extremities, is described. 2. Twenty-two males and twenty-three females were affected, while thirty-two males and thirty females were unaffected. Seven members of the family could not be traced. 3. Variations from Fairbank's description of dysplasia epiphysialis multiplex are discussed. In the family here reported the lower limbs only were affected and the severity of the disease was very variable. 4. Long term follow-up studies are presented

Reorientation of the acetabulum may be required in adolescents and young adults with developmental dysplasia of the hip. We have carried out a retrospective review of 51 hips after triple osteotomy with an average follow-up of ten years (8 to 15). Forty-eight hips (94%) were available for review and of these 39 (81%) were improved compared with before operation, 29 (60%) scoring good or excellent. Radiographic assessment showed improvement of the average centre-edge angle by 19°, the acetabular index by 12° and the anterior centre-edge angle by 26°. The degree of osteoarthritis progressed by one grade in ten hips (21%) over a period of ten years. The satisfactory long-term clinical and radiographic results have encouraged us to continue this treatment for symptomatic acetabular dysplasia in these patients

We report 40 cases in one family of an autosomal dominant bone dysplasia, which, though similar in some aspects to Paget's disease, seems unique in some features and in its natural history. The disease shows both general and focal skeletal changes, the latter being mainly in the limbs with an onset from the second decade. Progressive osteoclastic resorption is accompanied by medullary expansion which leads to pain, severe deformity and a tendency to pathological fracture. The serum alkaline phosphatase and urinary hydroxyproline are variably elevated, while other biochemical indices are normal. Most patients had an associated deafness of early onset and loss of dentition. No previous description of this disease has been found in the literature

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