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. Perthes’ disease (PD) is a childhood hip disorder that can affect the quality of life in adulthood due to femoral head deformity and osteoarthritis. There is very little data on how PD patients function as adults, especially from the patients’ perspective. The purpose of this study was to collect treatment history, demographic details, the University of California, Los Angeles activity score (UCLA), the 36-Item Short Form survey (SF-36) score, and the Hip disability and Osteoarthritis Outcome score (HOOS) of adults who had PD using a web-based survey method and to compare their outcomes to the outcomes from an age- and sex-matched normative population. Methods. The English REDCap-based survey was made available on a PD study group website. The survey included childhood and adult PD history, UCLA, SF-36, and HOOS. Of the 1,182 participants who completed the survey, the 921 participants who did not have a total hip arthroplasty are the focus of this study. The mean age at survey was 38 years (SD 12) and the mean duration from age at PD onset to survey participation was 30.8 years (SD 12.6). Results. In comparison to a normative population, the PD participants had significantly lower HOOS scores across all five scales (p < 0.001) for all age groups. Similarly, SF-36 scores of the participants were significantly lower (p < 0.001) for all scales except for age groups > 55 years. Overall, females, obese participants, those who reported no treatment in childhood, and those with age of onset > 11 years had significantly worse SF-36 and HOOS scores. Pairwise correlations showed a strong positive correlation within HOOS scales and between HOOS scales and SF-36 scales, indicating construct validity. Conclusion. Adult PD participants had significantly worse pain, physical, mental, and social health than an age- and sex-matched normative cohort. The study reveals a significant burden of disease on the adult participants of the survey, especially females. Cite this article: Bone Joint J 2022;104-B(12):1304–1312

Aims. To assess the characteristic clinical features, management, and outcome of patients who present to orthopaedic surgeons with functional dystonia affecting the foot and ankle. Methods. We carried out a retrospective search of our records from 2000 to 2019 of patients seen in our adult tertiary referral foot and ankle unit with a diagnosis of functional dystonia. Results. A total of 29 patients were seen. A majority were female (n = 25) and the mean age of onset of symptoms was 35.3 years (13 to 71). The mean delay between onset and diagnosis was 7.1 years (0.5 to 25.0). Onset was acute in 25 patients and insidious in four. Of the 29 patients, 26 had a fixed dystonia and three had a spasmodic dystonia. Pain was a major symptom in all patients, with a coexisting diagnosis of chronic regional pain syndrome (CRPS) made in nine patients. Of 20 patients treated with Botox, only one had a good response. None of the 12 patients who underwent a surgical intervention at our unit or elsewhere reported a subjective overall improvement. After a mean follow-up of 3.2 years (1 to 12), four patients had improved, 17 had remained the same, and eight reported a deterioration in their condition. Conclusion. Patients with functional dystonia typically presented with a rapid onset of fixed deformity after a minor injury/event and pain out of proportion to the deformity. Referral to a neurologist to rule out neurological pathology is advocated, and further management should be carried out in a movement disorder clinic. Response to treatment (including Botulinum toxin (Botox) injections) is generally poor. Surgery in this group of patients is not recommended and may worsen the condition. The overall prognosis remains poor. Cite this article: Bone Joint J 2021;103-B(6):1127–1132

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

Aims. In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years. Patients and Methods. This retrospective study of patients attending an urban scoliosis clinic identified 71 patients satisfying a criteria of: clinical diagnosis of idiopathic scoliosis; age of onset ten years and 11 months or less; MRI screening for intraspinal abnormalities. United Kingdom census data combined with patient referral data was used to calculate incidence. Results. Mean age at diagnosis was six years with 39 right-sided and 32 left-sided curves. Four patients (5.6%) were found to have intraspinal abnormalities on MRI. These consisted of: two combined Arnold-Chiari type 1 malformations with syrinx; one syrinx with a low lying conus; and one isolated syrinx. Overall annual incidence of early onset idiopathic scoliosis was one out of 182 000 (0.0006%). Conclusion. This study reports the lowest rates to date of intraspinal anomalies in patients with early onset idiopathic scoliosis, adding to knowledge regarding current incidences of these abnormalities as well as any geographical variation in the nature of the disease. Cite this article: Bone Joint J 2017;99-B:829–33

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

We studied 21 patients with a spontaneous palsy of the anterior interosseous nerve. There were 11 men and 10 women with a mean age at onset of 39 years (17 to 65). Pain around the elbow or another region (forearm, shoulder, upper arm, systemic arthralgia) was present in 17 patients and typically lasted for two to three weeks. It had settled within six weeks in every case. In ten cases the palsy developed as the pain settled. A complete palsy of flexor pollicis longus and flexor digitorum profundus to the index finger was seen in 13 cases and an isolated palsy of flexor pollicis longus in five. All patients were treated without operation. The mean time to initial muscle contraction was nine months (2 to 18) in palsy of the flexor digitorum profundus to the index finger, and ten months (1 to 24) for a complete palsy of flexor pollicis longus. An improvement in muscle strength to British Medical Research Council grade 4 or better was seen in all 15 patients with a complete palsy of the flexor digitorum profundus and in 16 of 18 with a complete palsy of flexor pollicis longus. There was no significant correlation between the duration of pain and either the time to initial muscle contraction or final muscle strength. Prolonged pain was not always associated with a poor outcome but the age of the patient when the palsy developed was strongly correlated. Recovery occurred within 12 months in patients under the age of 40 years who achieved a final British Medical Research Council grade of 4 or better. Surgical decompression does not appear to be indicated for young patients with this condition

Aims

There is a lack of high-quality research investigating outcomes of Ponseti-treated idiopathic clubfeet and correlation with relapse. This study assessed clinical and quality of life (QoL) outcomes using a standardized core outcome set (COS), comparing children with and without relapse.

Aims

We performed a systematic literature review to define features of patients, treatment, and biological behaviour of multicentric giant cell tumour (GCT) of bone.

The evolution of an idiopathic scoliosis is determined by the site of the primary curve and by the age of onset. It is significant that thoracic primary curves are commonly severe and the early onset of this curve accentuates this feature. Early operation based on prognosis is practised but sufficient time has not yet elapsed to justify any conclusions

1. The natural history and prognosis of progressive infantile idiopathic scoliosis are reviewed and twenty-eight cases are reported. 2. Resolving infantile idiopathic scoliosis is described and seven cases are reported. 3. The length of the curve, the degree of rotation, the age of onset of deterioration, and the rate of progression are the important factors in determining the type and severity of the deformity. 4. Infantile idiopathic scoliosis is briefly compared with congenital scoliosis

1. One hundred patients with osteochondritis dissecans of the knee have been reviewed. Sixty-eight were male. Unilateral lesions were found in seventy-four. The average age at onset of symptoms was eighteen years. 2. The outstanding etiological feature was found to be direct injury to the joint surface (46 percent), repeated injuries sustained in first class athletics and field sports, and mechanical abnormalities of the knee. 3. Osteochondral fracture was found in seven cases. 4. The results of various types of treatment are described and a plan of management outlined

1. Fifty-two patients with Perthes' disease (affecting both hips in six instances) have been reviewed ten or more years after the beginning of treatment. 2. Judged radiographically, approximately one-third developed good, one-third fair and one-third poor femoral heads. 3. The clinical results paralleled the radiographic. Except with the worst shaped heads, function was excellent. 4. Certain constant early and late radiographic features are recorded. 5. Of the factors influencing prognosis, the age at onset of the disease and the sex of the patient appear to be important

1. The prognosis of paralytic scoliosis has been studied by defining curve patterns and establishing the natural development as seen in fully grown patients who have not had surgical correction. 2. The prognosis, unlike that in idiopathic scoliosis, is related to the age of onset of the curvature and the degree of muscle imbalance rather than the site of the primary curve. 3. Paralysis of limb muscles is shown to be unrelated to the development of scoliosis. The intercostal muscles and the lateral abdominal flexors produce scoliosis when weaker on the convex side of the curve. Gravity and the other trunk muscles certainly play a part in the development of lumbar curves but their importance is difficult to assess

Of 899 patients with sickle-cell disease, aged between 6 and 28 years, who attended clinics in the Guinea Savannah of Nigeria in 1982 and 1983, 29 had symptoms of avascular necrosis of the femoral head. This group was studied in detail. Twenty-eight patients had haemoglobin-SS electrophoretic patterns and one had haemoglobin-SC. The male to female ratio was 1 to 1.6, and most of the patients were aged between 6 and 15 years at the onset of hip symptoms. These symptoms correlated with the radiographic lesions, but were not related to the age or sex of the patient. The radiographic lesions varied widely and were related to the age at onset of hip symptoms. A new radiological classification is proposed

We compared 63 hips (Catterall Groups 3 and 4) contained by femoral osteotomy with 85 untreated hips and found that 50.7 per cent of treated patients developed congruous spherical femoral heads in contrast to 14.1 per cent of those untreated. We have also considered certain other features relevant to the outcome. We suggest that the indications should not be modified on the grounds of early age of onset. Relief from weight-bearing does not appear to improve the results of containment. We have assessed the shortening which follows femoral osteotomy and conclude that this is only significant when there is growth disturbance at the capital epiphysis. These changes are at least as frequent in untreated patients

We have devised a combined pillar score (CPS) system, based on the lateral pillar (LP) and the posterior pillar (PP) classifications, together with the age at onset of Perthes’ disease, and examined its correlation with prognosis. The correlation coefficient of the Catterall classification, LP, PP, and CPS systems with the Stulberg system was 0.39, 0.52, 0.50, and 0.70, respectively. Overall 21 of the 22 hips (95.4%) with a CPS of 0 to 1 point had a good outcome and 12 of the 13 hips (92.3%) with a CPS of 3 points or more had a fair or poor outcome. None with a CPS of 2 points, had a poor outcome. The study shows that an accurate prediction of the prognosis is not possible with the LP classification alone for patients classified as belonging to group B (LP height 50% to 100% of contralateral height). The CPS system does allow accurate prediction of outcome

1. The results of posterior spinal fusion for paralytic scoliosis in 118 patients have been reviewed after growth had finished. The criteria for skeletal maturity were both clinical and radiological, with emphasis on ossification of the iliac apophyses. 2. The age of onset of anterior poliomyelitis and the age at which scoliosis was first noticed, as well as the extent of the muscle weakness and the curve patterns, all have a bearing on the severity of the deformity and the indication for operative treatment. 3. The method of treatment including operation is described and the complications detailed. The use of a tibial strut has now been abandoned and Harrington instrumentation has become routine. 4. There were five deaths in the series, three early and two late. 5. The difference in height, changes in respiratory function and eventual functional capacity have been analysed

1. The duration of Legg-Perthes' disease seems somewhat shortened by abduction weightbearing plaster treatnlent. The average age of onset of our sixty patients at the start of this treatment was seven years nine months. The patients were treated in abduction plasters for an average of nineteen months. 2. The contour of the femoral head in relation to the acetabulum is better preserved than after our previous methods of treatment. We have evaluated our cases by the method of Mose, by the epiphysial index of Eyre-Brook and the epiphysial quotient of Sjovall, and by the centre/edge angle of Wiberg. 3. There was no undue influence on the growing epiphysis and no evidence of ligamentous strain. 4. With this type of protected weight-bearing, the patients were kept active when both hips were affected. 5. No patient with unilateral disease showed Legg-Perthes' changes on the opposite side while under treatment or at follow-up. 6. The children were able to be at home and to attend school

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