Aims. The aims of this study were to describe the course of non-operatively managed, bilateral Perthes’ disease, and to determine specific prognostic factors for the radiographic and clinical outcome. . Patients and Methods. We identified 40 children with a mean age of 5.9 years (1.8 to 13.5), who were managed non-operatively for bilateral Perthes’ disease from our prospective, multicentre study of this condition, which included all children in Norway who were diagnosed with Perthes’ disease in the five-year period between 1996 and 2000. All children were followed up for five years. . The hips were classified according to the Catterall classification. A modified three-group Stulberg classification was used as an outcome measure, with a spherical femoral head being defined as a good outcome, an oval head as fair, and a flat femoral head as a poor outcome. . Results. Concurrent, simultaneous bilateral Perthes’ disease was seen in 23 children and 17 had the sequential onset of bilateral disease. The mean delay in onset for the second hip in the latter group was 1.9 years (0.3 to 5.5). . The five-year radiographic outcome was good in 30 (39%), fair in 25 (33%) and poor in 21 (28%) of the hips. The strongest predictors of poor outcome were > 50% necrosis of the femoral head, with odds ratio (OR) 19.6, and age at diagnosis > 6 years (OR 3.3). Other risk factors for poor outcome were the timing of the onset of disease, where children with the sequential onset of bilateral disease had a higher risk than those with the concurrent onset of bilateral disease (p = 0.021, chi-squared test). . Following a diagnosis of Perthes’ disease in one hip, there was a 5% chance of developing it in the contralateral hip. . Conclusion. These results imply that we need to distinguish between children with concurrent onset and those with sequential onset of bilateral Perthes’ disease, as the outcomes may be different. This has not been previously described. Children with concurrent onset of bilateral disease had a similar outcome to our previous series of those with unilateral disease, whereas children with sequential onset of bilateral disease had a worse prognosis. The increased risk of developing Perthes’ disease in the contralateral hip in those with unilateral disease is important information for the child and parents. . Cite this article: Bone Joint J 2016;98-B:569–75

Aims. We aimed to assess the cumulative risk of total hip arthroplasty (THA) from in situ fixation for slipped capital femoral epiphysis (SCFE) after a follow-up of almost 50 years. Methods. In this study, 138 patients with 172 affected hips treated with in situ fixation were evaluated retrospectively. A total of 97 patients (70%) were male and the mean age was 13.6 years (SD 2.1); 35 patients (25%) had a bilateral disease. The median follow-up time was 49 years (interquartile range 43 to 55). Basic demographic, stability, and surgical details were obtained from patient records. Preoperative radiographs (slip angle; SA) were measured, and data on THA was gathered from the Finnish National Arthroplasty Register. Results. The preoperative SA was a mean of 39° (SD 19°). At follow-up, 56 of the patients had undergone THA for a hip previously fixed in situ for SCFE (41%) and 64 of all affected hips had been replaced (37%). Kaplan-Meier analysis gave a median prosthesis-free postoperative survival of 55 years (95% confidence interval (CI) 45 to 64) for the affected hips. In a multivariate analysis, female patients had a two-fold risk for THA (hazard ratio (HR) 2.42 (95% CI 1.16 to 5.07)) and a greater preoperative SA increased the risk of THA (HR 1.03 for every increment of 1° (95% CI 1.01 to 1.05)), while patient age at surgery, slip laterality, stability of slip, or diagnostic delay did not have a statistically significant effect on the risk of THA. Conclusion. SCFE treated primarily with in situ fixation may lead to THA in more than 40% of affected hips at a near 50-year follow-up. This risk is approximately 15-times the reported lifetime risk in the Finnish general population. Female sex and increasing preoperative SA significantly predicted higher risk of THA. Cite this article: Bone Joint J 2023;105-B(12):1321–1326

Aims. To explore the of age of onset distribution for Perthes’ disease of the hip, with particular reference to gender, laterality and conformity to the lognormal distribution. Patients and Methods. A total of 1082 patients were identified from the Liverpool Perthes’ Disease Register between 1976 and 2010, of which 992 had the date of diagnosis recorded. In total, 682 patients came from the geographical area exclusively served by Alder Hey Hospital, of which 673 had a date of diagnosis. Age of onset curves were analysed, with respect to the predefined subgroups. Results. The age of onset demonstrated a positive skew with a median of 5.8 years (interquartile range 4.6 to 7.5). Disease onset was a mean five months earlier in girls (p = 0.01) and one year earlier in those who went on to develop bilateral disease (p < 0.001). There was no difference in the age of onset between geographical districts with differing incidence rates. The entire dataset (n = 992) conformed to a lognormal distribution graphically and with the chi-squared test of normality (p = 0.10), but not using the Shapiro-Wilk test (p = 0.01). The distribution for the predefined geographical subgroup (n = 673) conformed well to a lognormal distribution (chi-squared p = 0.16, Shapiro-Wilk p = 0.08). Given the observed lognormal distribution it was assumed that Perthes’ disease followed on incubation period consistent with a point-source disease exposure. The incubation period was further examined using Hirayama’s method, which suggested that the disease exposure may act in the prenatal period. Conclusion. The age of onset in Perthes’ disease conforms to a lognormal distribution, which allows comparisons with infectious disease epidemiology. Earlier onset in girls and those who develop bilateral disease may offer clues to understanding the aetiological determinants of the disease. The analysis suggests that an antenatal aetiological determinant may be responsible for disease. Take home message: Perthes’ disease age of onset conforms to a lognormal model, which is most typical of infectious diseases. The shape of the distribution suggests that an aetiological trigger in the pre-natal period may be an important determinant of disease. Cite this article: Bone Joint J 2016;98-B:710–14

Aims. The aim of this study was to assess the prognostic value of the modified three-group Stulberg classification, which is based on the sphericity of the femoral head, in patients with Perthes’ disease. Methods. A total of 88 patients were followed from the time of diagnosis until a mean follow-up of 21 years. Anteroposterior pelvic and frog-leg lateral radiographs were obtained at diagnosis and at follow-up of one, five, and 21 years. At the five- and 21-year follow-up, the femoral heads were classified using a modified three-group Stulberg classification (round, ovoid, or flat femoral head). Further radiological endpoints at long-term follow-up were osteoarthritis (OA) of the hip and the requirement for total hip arthroplasty (THA). Results. There were 71 males (81%) and 17 females. A total of 13 patients had bilateral Perthes’ disease; thus 101 hips were analyzed. At five-year follow-up, 37 hips were round, 38 ovoid, and 26 flat. At that time, 66 hips (65%) were healed and 91 (90%) were skeletally immature. At long-term follow-up, when the mean age of the patients was 28 years (24 to 34), 20 hips had an unsatisfactory outcome (seven had OA and 13 had required THA). There was a strongly significant association between the modified Stulberg classification applied atfive-year follow-up and an unsatisfactory outcome at long-term follow-up (p < 0.001). Between the five- and 21-year follow-up, 67 hips (76%) stayed in their respective modified Stulberg group, indicating a strongly significant association between the Stulberg classifications at these follow-ups (p < 0.001). Conclusion. The modified Stulberg classification is a strong predictor of long-term radiological outcome in patients with Perthes’ disease. It can be applied at the healing stage, which is usually reached five years after the diagnosis is made and before skeletal maturity. Cite this article: Bone Joint J 2021;103-B(12):1815–1820

Aims

There is no consensus regarding optimum timing and frequency of ultrasound (US) for monitoring response to Pavlik harness (PH) treatment in developmental dysplasia of the hip (DDH). The purpose of our study was to determine if a limited-frequency hip US assessment had an adverse effect on treatment outcomes compared to traditional comprehensive US monitoring.

Aims

The aim of this study was to evaluate the epidemiology and treatment of Perthes’ disease of the hip.

Aims

To assess the long-term effect of distal trochanteric transfer (DTT) on the clinical and radiographic outcomes of patients with Legg-Calvé-Perthes’ disease (LCPD) following a varus derotational osteotomy (VDRO).

We present the results of treatment of developmental dysplasia of the hip in infancy with the Pavlik harness using a United Kingdom screening programme with ultrasound-guided supervision. Initially, 128 consecutive hips in 77 patients were reviewed over a 40-month period; 123 of these were finally included in the study. The mean age of the patients at the start of treatment was five weeks (1 to 12). All hips were examined clinically and monitored with ultrasound scanning. Failure of treatment was defined as an inability to maintain reduction with the harness. All hips diagnosed with dysplasia or subluxation but not dislocation were managed successfully in the harness. There were 43 dislocated hips, of which 39 were reducible, but six failed treatment in the harness. There were four dislocated but irreducible hips which all failed treatment in the harness. One hip appeared to be successfully treated in the harness but showed persistent radiological dysplasia at 12 and 24 months. Grade 1 avascular necrosis was identified radiologically in three patients at 12 months.

Children presenting with Perthes’ disease before their sixth birthday are considered to have a good prognosis. We describe 166 hips in children in this age group. The mean age at onset of the disease was 44 months (22 to 72). Mild forms (Catterall I and II) were treated conservatively and severe forms (Catterall III and IV) either conservatively or operatively. The aim of the former treatment was to restrict weight-bearing. Operative treatment consisted of innominate osteotomy and was indicated by a Conway type-B appearance on the bone scan. All the patients were followed to skeletal maturity with a mean follow-up of 11 years (8 to 15).

The end results were evaluated radiologically using the classifications of Stulberg and Mose. A total of 50 hips were Catterall grade-I or grade-II, 65 Catterall grade-III and 51 Catterall grade-IV. All hips with mild disease had a good result at skeletal maturity. Of the hips with severe disease 78 (67.3%) had good (Stulberg I and II), 26 (22.4%) fair (Stulberg III) and 12 (10.3%) poor results (Stulberg IV and V). Of the Catterall grade-III hips 38 were treated conservatively of which 31 (81.6%) had a good result, six (15.8%) a fair and one (2.6%) a poor result. Operative treatment was carried out on 27 Catterall grade-III hips, of which 21 (77.8%) had a good, four (14.8%) a fair and two (7.4%) a poor result. By comparison conservative treatment of 19 Catterall grade-IV hips led to ten (52.7%) good, seven (36.8%) fair and two (10.5%) poor results. Operative treatment was carried out on 32 Catterall grade-IV hips, of which 16 (50.0%) had a good, nine (28.1%) a fair and seven (21.9%) a poor result.

We confirm that the prognosis in Perthes’ disease is generally good when the age at onset is less than six years. In severe disease there is no significant difference in outcome after conservative or operative treatment (p > 0.05). Catterall grade-III hips had a better outcome according to the Stulberg and Mose criteria than Catterall grade-IV hips, regardless of the method of treatment.

This nationwide prospective study was designed to determine prognostic factors and evaluate the outcome of different treatments of Perthes’ disease.

A total of 28 hospitals in Norway were instructed to report all new cases of Perthes’ disease over a period of five years and 425 patients were reported and followed for five years. Of these, 368 with unilateral disease were included in the present study. The hips were classified radiologically according to a modified two-group Catterall classification and the lateral pillar classification. A total of 358 patients (97%) attended the five-year follow-up, when a modified three-group Stulberg classification was used as a radiological outcome measure. For patients over six years of age at diagnosis and with more than 50% necrosis of the femoral head (152 patients), the surgeons at the different hospitals had chosen one of three methods of treatment: physiotherapy (55 patients), the Scottish Rite abduction orthosis (26), and proximal femoral varus osteotomy (71). Of these hips, 146 (96%) were available for the five-year follow-up.

The strongest predictor of outcome was femoral head involvement of more or less than 50% (odds ratio (OR) = 7.76, 95% confidence interval (CI) 2.82 to 21.37), followed by age at diagnosis (OR = 0.98, 95% CI 0.92 to 0.99) and the lateral pillar classification (OR = 0.62, 95% CI 0.40 to 0.98). In children over six years at diagnosis with more than 50% of femoral head necrosis, proximal femoral varus osteotomy gave a significantly better outcome than orthosis (p = 0.001) or physiotherapy (p = 0.001). There was no significant difference between the physiotherapy and orthosis groups (p = 0.36), and we found no difference in outcome after any of the treatments in children under six years (p = 0.73).

We recommend proximal femoral varus osteotomy in children aged six years and over at the time of diagnosis with hips having more than 50% femoral head necrosis. The abduction orthosis should be abandoned in Perthes’ disease.

A nationwide study of Perthes’ disease in Norway was undertaken over a five-year period from January 1996. There were 425 patients registered, which represents a mean annual incidence of 9.2 per 100 000 in subjects under 15 years of age, and an occurrence rate of 1:714 for the country as a whole. There were marked regional variations. The lowest incidence was found in the northern region (5.4 per 100 000 per year) and the highest in the central and western regions (10.8 and 11.3 per 100 000 per year, respectively). There was a trend towards a higher incidence in urban (9.5 per 100 000 per year) compared with rural areas (8.9 per 100 000 per year). The mean age at onset was 5.8 years (1.3 to 15.2) and the male:female ratio was 3.3:1.

We compared 402 patients with a matched control group of non-affected children (n = 1 025 952) from the Norwegian Medical Birth Registry and analysed maternal data (age at delivery, parity, duration of pregnancy), birth length and weight, birth presentation, head circumference, ponderal index and the presence of congenital anomalies. Children with Perthes’ disease were significantly shorter at birth and had an increased frequency of congenital anomalies.

Applying Sartwell’s log-normal model of incubation periods to the distribution of age at onset of Perthes’ disease showed a good fit to the log-normal curve. Our findings point toward a single cause, either genetic or environmental, acting prenatally in the aetiology of Perthes’ disease.

We describe a patient with cerebral palsy, of normal intelligence, who could not walk but who by the age of 16 had been successfully managed with a staged bilateral total hip arthroplasty using a constrained liner.