It has been suggested that Perthes’ disease is more prevalent in urban areas, and that the risk increases with deprivation. We present the findings of a preliminary analysis of Perthes’ disease in Northern Ireland, which is shown to have one of the highest national annual rates of incidence in the world (11.6 per 100 000). Of the 313 children diagnosed over a seven-year period, 311 were allocated to the enumeration districts of the 1991 census, thus allowing the incidence to be calculated using both spatial and non-spatial aggregation. The cases were grouped according to the size of the settlement from highly urbanised to open countryside and by level of area deprivation. While the incidence of Perthes’ disease was found to be associated with indicators of the level of deprivation for areas, there was no evidence to suggest that there was an increased risk in urban areas; the highest rate was found in the most deprived rural category

The bicompartmental acetabulum is one of the morphological changes which may be seen in children with Legg-Calvé-Perthes’ disease. Three-dimensional CT and MRI were used to analyse the detailed morphology of the acetabulum with special reference to its inner surface, in 16 patients with Perthes’ disease and a bicompartmental acetabulum. The bicompartmental appearance was seen on the coronal plane image through the acetabular fossa. The lunate surface was seen to grow laterally resulting in an increased mediolateral thickness of the triradiate cartilage. On the horizontal plane images, the acetabular fossa had deepened and had a distinct prominence at its posterior border. The combination of these morphological changes resulted in a bicompartmental appearance on plain radiography. Acetabular bicompartmentalisation appears to be the result of an imbalance of growth between the cartilage-covered lunate surface and the cartilage-devoid acetabular fossa

Serum immunoglobulin concentrations in 41 children with Perthes' disease and 82 age and sex matched controls were measured by radial immunodiffusion. Significant increases in IgG and IgM were seen in children with Perthes' disease

Recent work has suggested that thrombophilia may be an aetiological factor in up to 50% of children with Perthes’ disease, and that up to 75% may have a coagulopathy. Our aim was to test these findings in the local population of children with Perthes’ disease and attempt to correlate them with the severity of the condition. In 64 children there were only eight (12%) with low levels of clotting proteins, as defined by normal paediatric ranges. Of these eight, only five could be said to show any thrombophilic tendency

Skeletal age was estimated by examination of radiographs of the carpus in 182 children suffering from Perthes' disease after the reliability of the Greulich and Pyle Atlas had been checked for a control group of British children. A striking tendency to delayed skeletal maturation was shown in the children with Perthes' disease. This trait was also found in ninety-three unaffected siblings of the patients. The velocity of skeletal ageing as the disease progressed was estimated. In some patients the carpal skeleton failed to mature at all for periods of up to three years and the term "skeletal standstill" is applied to this phenomenon. The significance of these findings is discussed and it is suggested that the maturation defect may have aetiological significance

We performed superselective angiography in 28 hips in 25 patients with Perthes’ disease in order to study the blood supply of the lateral epiphyseal arteries (LEAs). Interruption of the LEAs at their origin was observed in 19 hips (68%). Revascularisation in the form of numerous small arteries was seen in ten out of 11 hips in the initial stage of Perthes’ disease, in seven of eight in the fragmentation stage and in five of nine in the healing stage. Penetration of mature arteries into the depths of the epiphysis was seen in four of nine hips in the healing stage. Vascular penetration was absent in the weight-bearing portion of the femoral head below the acetabular roof. Interruption of the posterior column artery was seen where it passed through the capsule in seven hips when they lay either in internal rotation or in abduction with internal rotation. We suggest that in Perthes’ disease the blood supply of the LEAs is impaired at their origin and that revascularisation occurs from this site by ingrowth of small vessels into the femoral epiphysis. This process may be the result of recurrent ischaemic episodes

We treated 98 consecutive patients with Perthes’ disease by a unilateral brace in external rotation, flexion and abduction and a further consecutive 110 by a bilateral cast with the hips in internal rotation and abduction. During treatment in the unilateral brace, six (6.1%) hips on the opposite side developed evidence of Perthes’ disease and one developed this after the brace had been removed. In children managed in bilateral casts, no contralateral Perthes’ disease was seen. Adequate containment of the femoral head may prevent subsequent changes in the opposite hip

1. The findings in a femoral head obtained at necropsy on a boy aged nine suffering from Perthes' disease are described. 2. The findings revealed that there had been avascular necrosis of the epiphysis followed by revascularisation and healing, and there was evidence to suggest a second episode of infarction. 3. The findings provide strong support for the suggestion that Perthes' disease is the result not of one but of more than one episode of major infarction

We evaluated radiological hip remodelling after shelf acetabuloplasty and sought to identify prognostic factors in 25 patients with a mean age of 8.9 years (7.0 to 12.3) who had unilateral Perthes’ disease with reducible subluxation of the hip in the fragmentation stage. At a mean follow-up of 6.7 years (3.2 to 9.0), satisfactory remodelling was observed in 18 hips (72%). The type of labrum in hip abduction, as determined by intra-operative dynamic arthrography, was found to be a statistically significant prognostic factor (p = 0.012). Shelf acetabuloplasty as containment surgery seems to be best indicated for hips in which there is not marked collapse of the epiphysis and in which the extruded epiphyseal segment slips easily underneath the labrum on abduction, without imposing undue pressure on the lateral edge of the acetabulum

The nuclide bone-scan will reliably diagnose Perthes' disease with a sensitivity of 0.98 and a specificity of 0.95. The comparable figures for radiographic sensitivity and specificity are respectively 0.92 and 0.78. In addition, it is possible on the scan to recognise the onset of revascularisation of the femoral capital epiphysis some months before there are radiographic signs of new bone formation. Scintigraphy also suggests that in some cases of transient synovitis there may bae a period of reversible ischaemia of the capital epiphysis, which may have relevance to the pathogenesis of Perthes' disease

We studied, clinically and radiologically, the growth and remodelling of 21 hips after valgus femoral osteotomy with both rotational and sagittal correction for hinge abduction in 21 patients (mean age, 9.7 years) with Perthes’ disease. The exact type of osteotomy performed was based on the pre-operative clinical and radiological assessment and the results of intra-operative dynamic arthrography. The mean IOWA hip score was 66 (34 to 76) before surgery and 92 (80 to 100) at a mean follow-up of 7.1 years (3.0 to 15.0). Radiological measurements revealed favourable remodelling of the femoral head and improved hip joint mechanics. Valgus osteotomy, with both rotational and sagittal correction, can improve symptoms, function and remodelling of the hip in patients with Perthes’ disease

Seventeen patients, with an average age of nine years 11 months, underwent 18 Chiari osteotomies for the treatment of painful subluxation of the hips following Perthes' disease. The average follow-up period was four years three months. The 13 patients reviewed clinically all did well and none complained of pain or instability. The radiographs were examined in all 17 cases. The average centre-edge angle and percentage femoral head cover were definitely improved by the operation. No significant medical displacement of the femoral head was achieved. The clinical success may result from improved femoral head coverage and diminished eccentricity. Chiari's osteotomy is recommended for adolescent patients with painful subluxation of the hip as a consequence of Perthes' disease

The aims of this survey were to establish the familial incidence of Perthes' disease, to note any associated developmental anomalies and to collect information on preceding trauma or synovitis, on the pregnancy and birth, and on various sociological factors. Height and weight measurements were obtained for 217 patients, and comparisons made with those of their parents, unaffected sibs and (local) controls. Results showed an extremely low frequency of Perthes' disease among relatives, with no obvious pattern of inheritance. As genetic factors were not apparent, environmental and sociological causes were sought. The disease occurred particularly in children who were third-born or later in the family, and had older than average parents. Many came from low-income families and one in ten had been a breech birth, shown other malposition or had had a version late in pregnancy. Many children were already undersized at the time of developing Perthes' disease and remained short than average throughout life. Neither their parents nor sibs were shorter than normal, indicating that the patients' short stature was not familial. The child who is going to develop Perthes' disease is already constitutionally and socially at a disadvantage, and during the perinatal period and the first few years of life is perhaps more susceptible to trauma than is a normal child

1. Intra-osseous venographs have been obtained in twenty-eight hips affected by Perthes disease and in twenty normal hips after the injection of opaque medium into the femoral neck. 2. In the normal hips the contrast medium drained rapidly into the local veins; none flowed distally into the diaphysis. 3. In the initial and in the fragmentation stages of Perthes' disease some contrast medium always flowed into the diaphysis and the flow into the local veins was greatly reduced. 4. In the restitution stage the venographs approached normal. 5. The implications of these findings are discussed

1. Cancellous bone grafting of the head of the femur in Perthes' disease accelerates reossification and thus shortens the period of treatment. 2. There is no evidence from this series of cases that the final shape of the head is affected for better or for worse by the operation of cancellous chip grafting, by the rate of regeneration of the head after operation or by the stage in the disease process at which the operation is done. 3. No relationship has been found between the shape of the head at the time of operation and its final shape. 4. A larger series of cases is needed to determine the value of bone grafting in Perthes' disease of the hip

Perthes' disease involving the whole of the femoral head in 36 children was treated by innominate osteotomy. Radiographs of all cases were reviewed to see the effect of the osteotomy on the shape, the degree of acetabular cover and any subluxation of the diseased femoral head. All femoral heads which were circular before operation remained so, and over half of the previously deformed heads became circular after the osteotomy. The improved acetabular cover provided by the osteotomy resulted in a CE angle of 25 degrees or more in 92% of hips. Possible subluxation of the femoral head was studied by inspecting Shenton's line. If this was intact before operation it remained so; of the 14 which were broken before operation, 11 were restored to normal after osteotomy. We conclude that innominate osteotomy is a worthwhile procedure for Perthes' disease involving the whole of the femoral head

Valgus extension osteotomy (VGEO) is a salvage procedure for ‘hinge abduction’ in Perthes’ disease. The indications for its use are pain and fixed deformity. Our study shows the clinical results at maturity of VGEO carried out in 48 children (51 hips) and the factors which influence subsequent remodelling of the hip. After a mean follow-up of ten years, total hip replacement has been carried out in four patients and arthrodesis in one. The average Iowa Hip Score in the remainder was 86 (54 to 100). Favourable remodelling of the femoral head was seen in 12 hips. This was associated with three factors at surgery; younger age (p = 0.009), the phase of reossification (p = 0.05) and an open triradiate cartilage (p = 0.0007). Our study has shown that, in the short term, VGEO relieves pain and corrects deformity; as growth proceeds it may produce useful remodelling in this worst affected subgroup of children with Perthes’ disease

It has recently been postulated that thrombophilia may have a role in the aetiology of Perthes’ disease. The published reports, however, remain conflicting. In this study a retrospective analysis of the coagulation parameters was made in 47 patients with Perthes’ disease and the results compared with the clinical data. Five patients with Factor V Leiden mutation were found (10.6%) and surprisingly four of them had a homozygous pattern. These four patients showed the most severe form of the disease, Catterall group IV, with flattening of the entire epiphysis, involvement of the metaphysis, shortening and broadening of the femoral neck, trochanteric overgrowth and developed mushroom-shaped aspherical laterally displaced femoral heads in dysplastic acetabula. We would like to suggest that the homozygous form of Factor V Leiden mutation has some role in the clinical course of Perthes’ disease and particularly its most severe form

We performed a paired study of mature patients with Perthes’ disease to compare the radiological results after treatment between conservatively- and surgically-treated groups. One patient was selected from each group to create the pairs for this study. Each pair was strictly matched for gender, body mass index, age at onset, stage at the first visit, necrotic area and radiological at-risk signs and each was assessed by comparing the values of six radiological measurements. Eighteen pairs (36 hips) fitted the criteria. The radiological measurements which showed a statistically better result in the surgical groups were Mose’s method, the acetabular-head index and leg-length discrepancy. There were no statistical differences in the slope of the acetabular roof and the articulotrochanteric distance. Four hips in the conservative group were in Stulberg class II, five in class III and nine in class IV. In the surgical group, 13 were in Stulberg class II, four in class III and one in class IV. We conclude that surgical treatment improved the sphericity of the femoral head and provided greater acetabular cover, but did not reform the acetabular roof. It was noteworthy that a greater leg-length discrepancy and a smaller articulotrochanteric distance were not seen in the surgical group. Our study suggests that surgical treatment is preferable in patients with severe Perthes’ disease

1 . Thirty-four cases of Perthes' disease followed into adult life were reviewed twenty-five to forty years (average thirty years) after diagnosis. 2. Rather more than one-third of the patients developed hips which were good, an equal number were considered fair and about one-quarter were poor. 3. Four out of five patients were fully active and free from pain but only two out of five had hips which were radiologically good. 4. Clinical and/or radiological deterioration had seldom occurred in the last twelve years. 5. A good result in childhood is likely to be maintained with no pain and good function up to the age of forty years and perhaps longer