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.

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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

We compared the prognostic value of the Catterall grouping, the Salter-Thompson grading, the arthrographic shape of the femoral head, and the Herring lateral pillar grouping during the fragmentation stage of Perthes’ disease in 73 patients with 81 affected hips. Radiographs were available for study from the onset of the disease until skeletal maturity. We used the Stulberg classification to assess outcome. The Herring grade and arthrographic sphericity proved to be the best predictors of final outcome. Combining these two values further increased the predictive value. All but one patient in Herring group A achieved an excellent outcome. In Herring group B, the age of the child and the sphericity of the femoral head influenced the end result. If the child was less than seven years old at the onset of symptoms the prognosis was invariably good and all spherical hips in group B had a good outcome with Stulberg grades 1 or 2. Moderately and severely deformed hips on arthrography resulted in Stulberg 3 and 4 hips. None of the hips in Herring group C had a normal appearance at maturity and the outcome was not significantly influenced by the age at onset or the arthrographic appearance

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. The term "observation hip" refers to a form of hip disease affecting children and adolescents, the most significant features being the transient nature of the symptoms and the absence of a bony or cartilaginous lesion on radiographic examination. 2. With a view to determining the possible sequelae of the disorder, twenty-three patients aged between two and fifteen years at the onset of the condition were studied fifteen to thirty years later. 3. Varying degrees of coxa magna, osteoarthritis or simple broadening of the femoral neck in the "observed" hip joint were found in the radiographs of twelve of the twenty-three patients studied. 4. This analysis suggests that the " observation hip" syndrome is the result ofan inflammatory process ofthejoint due to varied etiology, whether from injury or infection. The developmental and degenerative changes which may occur are a consequence of hypervascularisation of the bone. Thus changes may develop without necessarily producing the epiphysial necrosis characteristic of the first stage of ischaemia in Legg-Calvé-Perthes' disease. 5. The persistence of this stage of hypervascularity, and therefore the possible outcome of the transient synovitis, may be conditioned by the age at onset of the pathological process, the severity of the condition, and the duration of the symptoms and signs

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.

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Perthes’ disease (PD) often results in femoral head deformity and leg length discrepancy (LLD). Our objective was to analyze femoral morphology in PD patients at skeletal maturity to assess where the LLD originates, and evaluate the effect of contralateral epiphysiodesis for length equalization on proximal and subtrochanteric femoral lengths.

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The aim of this study was to assess whether it is possible to predict the mortality, and the extent and time of neurological recovery from the time of the onset of symptoms and MRI grade, in patients with the cerebral fat embolism syndrome (CFES). This has not previously been investigated.

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The aim of this study was to determine whether there is an increased prevalence of scoliosis in patients who have suffered from a haematopoietic malignancy in childhood.

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We aimed to address the question on whether there is a place for shoulder stabilization surgery in patients who had voluntary posterior instability starting in childhood and adolescence, and later becoming involuntary and uncontrollable.

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With novel promising therapies potentially limiting progression of Dupuytren’s disease (DD), better patient stratification is needed. We aimed to quantify DD development and progression after seven years in a population-based cohort, and to identify factors predictive of disease development or progression.

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To study the associations of lumbar developmental spinal stenosis (DSS) with low back pain (LBP), radicular leg pain, and disability.

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To identify a suite of the key physical, emotional, and social outcomes to be employed in clinical practice and research concerning Perthes' disease in children.

The residual shortening of the affected limbs in 55 patients treated by subtrochanteric varus derotation osteotomy was compared with that in 71 patients treated with weight-relieving calipers. When last examined, 43 of the former group and 47 of the latter had reached complete or near-complete skeletal maturity. The average follow-up was 9.1 years in the osteotomised patients and 5.25 years in the conservatively treated group. The average residual shortening (0.9 cm) was identical in both groups. In most patients the initial shortening caused by the osteotomy gradually corrected as, over a period of several years, the postosteotomy angle gradually became less varus. Any residual shortening depended principally on the severity of inhibition of endochondral ossification at the proximal femoral growth plate. Less residual shortening was seen in children who were under seven years of age at the onset of symptoms (under eight at operation) in whom the open-wedge technique of osteotomy was employed and who had good anatomical results

1. A systematic collection of the clinical findings in fifty patients with pyrophosphate synovitis,among some 300 patients with obscure disorder of the knee, has been made over a period of eighteen months. The numbers of men and women were equal, the mean age being seventy years (range thirty-seven to ninety), and the mean age at the onset of symptoms fifty-nine years. 2. A difference in the clinical picture between the sexes was found. In men an acute synovitis predominated, in women chronic joint complaints. 3. A high incidence of accompanying disease was found, but none had a significant relationship to the arthropathy, although the high frequency (20 per cent) of synovitis following an acute severe illness of some other kind was striking. 4. No specific radiological sign except for calcifications in articular cartilage and menisci was found in these patients, and no relationship to osteoarthrosis could be established. 5. The great variability of symptoms and the surprisingly high incidence attracts attention to pyrophosphate synovitis as a cause of joint symptoms, especially in elderly patients

We analysed the short-term outcome after varus osteotomy for Perthes’ disease in 48 older children from south-west India, comparing them with 30 historical controls. The children were between 7 and 12 years of age at the onset of the disease. All had stage-I or stage-II disease, with half or more of the epiphysis involved. The operated children had an open-wedge subtrochanteric varus osteotomy with derotation or extension and a trochanteric epiphyseodesis. Weight-bearing was avoided until late stage III. The non-operated children had been treated symptomatically by conservative methods. At the time of healing, 62.5% of the operated group had spherical femoral heads compared with 20% of those treated non-operatively (p < 0.001). Of the operated children with Catterall group-IV involvement, 48% had good results as against 24% of the non-operated group (p < 0.05). The percentage increase in the radius of the affected femoral head compared with the normal side was significantly lower in children who had operations (14.68 v 25.65; p < 0.001). We have shown that the short-term results of early surgical containment in children over seven years of age are satisfactory

Aims

Spinal tuberculosis (TB) remains an important concern. Although spinal TB often has sequelae such as myelopathy after treatment, the predictive factors affecting such unfavourable outcomes are not yet established. We investigated the clinical manifestations and predictors of unfavourable treatment outcomes in patients with spinal TB.

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The purpose of this study was to investigate the potential for achieving local and systemic control after local recurrence of a chondrosarcoma of bone

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The aim of this study was to determine the influence of developmental spinal stenosis (DSS) on the risk of re-operation at an adjacent level.

We have reviewed 38 surgically treated cases of spontaneous posterior interosseous nerve palsy in 38 patients with a mean age of 43 years (13 to 68) in order to identify clinical factors associated with its prognosis. Interfascicular neurolysis was performed at a mean of 13 months (1 to 187) after the onset of symptoms. The mean follow-up was 21 months (5.5 to 221). Medical Research Council muscle power of more than grade 4 was considered to be a good result. A further 12 cases in ten patients were treated conservatively and assessed similarly.

Of the 30 cases treated surgically with available outcome data, the result of interfascicular neurolysis was significantly better in patients < 50 years old (younger group (18 nerves); good: 13 nerves (72%), poor: five nerves (28%)) than in cases > 50 years old (older group (12 nerves); good: one nerve (8%), poor: 11 nerves (92%)) (p < 0.001). A pre-operative period of less than seven months was also associated with a good result in the younger group (p = 0.01). The older group had a poor result regardless of the pre-operative delay.

Our recommended therapeutic approach therefore is to perform interfascicular neurolysis if the patient is < 50 years of age, and the pre-operative delay is < seven months. If the patient is > 50 years of age with no sign of recovery for seven months, or in the younger group with a pre-operative delay of more than a year, we advise interfascicular neurolysis together with tendon transfer as the primary surgical procedure.

Juvenile idiopathic arthritis (JIA) is a chronic disease of childhood; it causes joint damage which may require surgical intervention, often in the young adult. The aim of this study was to describe the long-term outcome and survival of hip replacement in a group of adult patients with JIA and to determine predictors of survival for the prosthesis. In this retrospective comparative study patients were identified from the database of a regional specialist adult JIA clinic. This documented a series of 47 hip replacements performed in 25 adult patients with JIA. Surgery was performed at a mean age of 27 years (11 to 47), with a mean follow-up of 19 years (2 to 36). The mean Western Ontario and McMaster Universities osteoarthritis index questionnaire (WOMAC) score at the last follow-up was 53 (19 to 96) and the mean Health Assessment Questionnaire score was 2.25 (0 to 3). The mean pain component of the WOMAC score (60 (20 to 100)) was significantly higher than the mean functional component score (46 (0 to 97)) (p = 0.02). Kaplan-Meier survival analysis revealed a survival probability of 46.6% (95% confidence interval 37.5 to 55.7) at 19 years, with a trend towards enhanced survival with the use of a cemented acetabular component and a cementless femoral component. This was not, however, statistically significant (acetabular component, p = 0.76, femoral component, p = 0.45). Cox’s proportional hazards regression analysis showed an implant survival rate of 54.9% at 19 years at the mean of covariates.

Survival of the prosthesis was significantly poorer (p = 0.001) in patients who had been taking long-term corticosteroids and significantly better (p = 0.02) in patients on methotrexate.

The ideal treatment for traumatic anterior dislocation of the shoulder in the skeletally immature patient is controversial. The aim of this study is to evaluate the outcomes after either conservative and/or surgical treatment using the Latarjet technique. A retrospective series of 49 out of 80 patients were reviewed. We found no significant differences between either treatment method regarding functional scores and pain levels. Although not statistically significant, post-surgical patients showed better signs of shoulder stability than others who have a higher rate of recurrence. Further, 92% of the post-surgical group had returned to the same level of activity versus 52% in the non-surgically treated group. We found no contraindications to operate on a skeletally immature patient.

Cite this article: Bone Joint J 2014;96-B:354–9.

Perthes’ disease is an osteonecrosis of the juvenile hip, the aetiology of which is unknown. A number of comorbid associations have been suggested that may offer insights into aetiology, yet the strength and validity of these are unclear. This study explored such associations through a case control study using the United Kingdom General Practice Research database. Associations investigated were those previously suggested within the literature. A total of 619 cases of Perthes’ disease were included, as were 2544 controls. The risk of Perthes’ disease was significantly increased with the presence of congenital anomalies of the genitourinary and inguinal region, such as hypospadias (odds ratio (OR) 4.04 (95% confidence interval (CI) 1.41 to 11.58)), undescended testis (OR 1.83 (95% CI 1.12 to 3.00)) and inguinal herniae (OR 1.79 (95% CI 1.02 to 3.16)). Attention deficit hyperactivity disorder was not associated with Perthes’ disease (OR 1.01 (95% CI 0.48 to 2.12)), although a generalised behavioural disorder was (OR 1.55 (95% CI 1.10 to 2.17)). Asthma significantly increased the risk of Perthes’ disease (OR 1.44 (95% CI 1.17 to 1.76)), which remained after adjusting for oral/parenteral steroid use.

Perthes’ disease has a significant association with congenital genitourinary and inguinal anomalies, suggesting that intra-uterine factors may be critical to causation. Other comorbid associations may offer insight to support or refute theories of pathogenesis.

Previous studies have identified clinical and demographic risk factors for recurrence in the treatment of idiopathic clubfoot (congenital talipes equinovarus). Evertor muscle activity is not usually considered amongst them. This study aimed to evaluate whether recurrence could be predicted by demographic, clinical and gait parameters. From a series of 103 children with clubfeet, 67 had completed a follow-up of two years: 41 male and 26 female, 38 with idiopathic and 29 with non-idiopathic deformities. The mean age was 3.2 years (2.1 to 6.3). Primary correction was obtained in all 38 children (100%) with an idiopathic deformity, and in 26 of 29 patients (90%) with a non-idiopathic deformity. Overall, 60 children (90%) complied with the abduction brace regime. At a mean follow-up of 31.4 months (24 to 62), recurrence was noted in six children (15.8%) in the idiopathic and 14 children (48.3%) in the non-idiopathic group. Significant correlation was found between poor evertor activity and recurrence in both groups. No statistically significant relationship was found between the rate of recurrence and the severity of the initial deformity, the age at the time of treatment, the number of casts required or the compliance with the brace.

After correction of idiopathic and non-idiopathic clubfoot using the Ponseti method, only poor evertor muscle activity was statistically associated with recurrence. The identification of risk factors for recurrent deformity allows clinicians to anticipate problems and advocate early additional treatment to improve muscle balance around the ankle.

Cite this article: Bone Joint J 2014;96-B:1264–8.

We reviewed 31 consecutive patients with Friedreich’s ataxia and scoliosis. There were 24 males and seven females with a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean curve of 51° (13° to 140°). A total of 12 patients had thoracic curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar. Two patients had long thoracolumbar collapsing scoliosis with pelvic obliquity and four had hyperkyphosis. Left-sided thoracic curves in nine patients (45%) and increased thoracic kyphosis differentiated these deformities from adolescent idiopathic scoliosis. There were 17 patients who underwent a posterior instrumented spinal fusion at mean age of 13.35 years, which achieved and maintained good correction of the deformity. Post-operative complications included one death due to cardiorespiratory failure, one revision to address nonunion and four patients with proximal junctional kyphosis who did not need extension of the fusion. There were no neurological complications and no wound infections. The rate of progression of the scoliosis in children kept under simple observation and those treated with bracing was less for lumbar curves during bracing and similar for thoracic curves. The scoliosis progressed in seven of nine children initially treated with a brace who later required surgery. Two patients presented after skeletal maturity with balanced curves not requiring correction. Three patients with severe deformities who would benefit from corrective surgery had significant cardiac co-morbidities.

We retrospectively identified 18 consecutive patients with synovial chrondromatosis of the shoulder who had arthroscopic treatment between 1989 and 2004. Of these, 15 were available for review at a mean follow-up of 5.3 years (2.3 to 16.5). There were seven patients with primary synovial chondromatosis, but for the remainder, the condition was a result of secondary causes. The mean Constant score showed that pain and activities of daily living were the most affected categories, being only 57% and 65% of the values of the normal side. Surgery resulted in a significant improvement in the mean Constant score in these domains from 8.9 (4 to 15) to 11.3 (2 to 15) and from 12.9 (5 to 20) to 18.7 (11 to 20), respectively (unpaired t-test, p = 0.04 and p < 0.0001, respectively). Movement and strength were not significantly affected. Osteoarthritis was present in eight patients at presentation and in 11 at the final review. Recurrence of the disease with new loose bodies occurred in two patients from the primary group at an interval of three and 12 years post-operatively. In nine patients, loose bodies were also present in the bicipital groove; seven of these underwent an open bicipital debridement and tenodesis.

We found that arthroscopic debridement of the glenohumeral joint and open debridement and tenodesis of the long head of biceps, when indicated, are safe and effective in relieving symptoms at medium-term review.

Between July 1986 and August 1996, we performed 32 total knee arthroplasties (TKA) on 32 patients with partially or completely ankylosed knees secondary to infection. Their mean age at surgery was 40 years (20 to 63) and the mean follow-up was ten years (5 to 13). The mean post-operative range of movement was 75.3° (30 to 115) in those with complete and 98.7° (60 to 130) in those with partial ankylosis. The mean Hospital for Special Surgery knee score increased from 57 to 86 points post-operatively. There were complications in four knees (12.5%), which included superficial infection (one), deep infection (one), supracondylar femoral fracture (one) and transient palsy of the common peroneal nerve (one). Although TKA in the ankylosed knee is technically demanding and has a considerable rate of complications, reasonable restoration of function can be obtained by careful selection of patients, meticulous surgical technique, and aggressive rehabilitation.

In a longitudinal case-control study, we followed 81 subjects with dysplasia of the hip and 136 control subjects without dysplasia for ten years assessing radiological evidence of degeneration of the hip at admission and follow-up. There were no cases of subluxation in the group with dysplasia. Neither subjects with dysplasia nor controls had radiological signs of ongoing degenerative disease at admission. The primary radiological discriminator of degeneration of the hip was a change in the minimum joint space width over time. There were no significant differences between these with dysplasia and controls in regard to age, body mass index or occupational exposure to daily repeated lifting at admission.

We found no significant differences in the reduction of the joint space width at follow-up between subjects with dysplasia and the control subjects nor in self-reported pain in the hip. The association of subluxation and/or associated acetabular labral tears with dysplasia of the hip may be a conditional factor for the development of premature osteoarthritis in mildly to moderately dysplastic hips.

We prospectively studied the clinical, arthroscopic and histological results of collagen-covered autologous chondrocyte implantation (ACI-C) in patients with symptomatic osteochondritis dissecans of the knee. The study included 37 patients who were evaluated at a mean follow-up of 4.08 years.

Clinical results showed a mean improvement in the modified Cincinnati score from 46.1 to 68.4. Excellent and good clinical results were seen in 82.1% of those with juvenile-onset osteochondritis dissecans but in only 44.4% of those with adult-onset disease.

Arthroscopy at one year revealed International Cartilage Repair Society grades of 1 or 2 in 21 of 24 patients (87.5%). Of 23 biopsies, 11 (47.8%) showed either a hyaline-like or a mixture of hyaline-like and fibrocartilage, 12 (52.2%) showed fibrocartilage.

The age at the time of ACI-C determined the clinical outcome for juvenile-onset disease (p = 0.05), whereas the size of the defect was the major determinant of outcome in adult-onset disease (p = 0.01).

A total of 25 children (37 legs and 51 segments) with coronal plane deformities around the knee were treated with the extraperiosteal application of a flexible two-hole plate and screws. The mean age was 11.6 years (5.5 to 14.9), the median angle of deformity treated was 8.3° and mean time for correction was 16.1 months (7 to 37.3). There was a mean rate of correction of 0.7° per month in the femur (0.3° to 1.5°), 0.5° per month in the tibia (0.1° to 0.9°) and 1.2° per month (0.1° to 2.2°) if femur and tibia were treated concurrently. Correction was faster if the child was under 10 years of age (p = 0.05). The patients were reviewed between six and 32 months after plate removal. One child had a rebound deformity but no permanent physeal tethers were encountered.

The guided growth technique, as performed using a flexible titanium plate, is simple and safe for treating periarticular deformities of the leg.

Obesity is thought to be an aetiological factor for slipped capital femoral epiphysis (SCFE). We analysed changes in the incidence of SCFE in Scotland over the last two decades. During this period rates of childhood obesity have risen substantially and evidence for a relationship between these changes and the incidence of SCFE was sought.

We found that the incidence of SCFE increased from 3.78 per 100 000 children in 1981 to 9.66 per 100 000 in 2000 (R² = 0.715): a two and a half times increase over two decades. It was seen at a younger age, with a fall in the mean age at diagnosis from 13.4 to 12.6 years for boys (p = 0.007) and 12.2 to 11.6 for girls (p = 0.047). More children under eight years old were seen with SCFE in Scotland in the decade to 2000 than in the previous decade (p = 0.002, R² = 0.346).

A close correlation was observed between rising childhood obesity over the last 20 years in Scotland and an increasing incidence of SCFE.

The role of heritable thrombophilic risk factors in the pathogenesis of the Perthes’ disease is controversial. The clinical and radiological findings of Perthes’ disease may be indistinguishable from those of Gaucher’s disease, and the most common Jewish N370S Gaucher mutation is threefold greater in patients with Perthes’ disease. Familial osteonecrosis of the femoral head is associated with variant mutations of collagen type II (COL2A1 mutations). We therefore studied the potential role of genetic thrombophilia and the Gaucher and COL2A1 mutations in children with Perthes’ disease.

Genomic DNA of 119 children with radiologically-confirmed Perthes’ disease diagnosed between 1986 and 2005 was analysed for the thrombophilic polymorphisms Factor V Leiden, 677T-MTHFR and FIIG20210A. The results were compared with those of a group of 276 children without Perthes’ disease. DNA was also analysed for the Gaucher mutations N370S, G insertion (84GG), L444P, Intron 2 (IVS2+1G> A) and R496H. Enzymic assays confirmed the Gaucher disease status. Collagen (COL2A1) mutations of the 12q13 gene were also analysed. The prevalence of thrombophilic markers was similar among the 119 patients with Perthes’ disease and the 276 control subjects. The prevalence of the Gaucher mutation was consistent with Israeli population carriership data and did not confirm an earlier-claimed association with Perthes’ disease. All 199 patients were negative for the studied COL2A1 mutations.

We found no genetic association between Perthes’ disease and either Gaucher’s disease or COL2A1 mutations or increased genetic thrombophilia among our patients compared with the control group. A systematic review of case-control studies suggested that there was a positive association between Perthes’ disease and Factor V Leiden. The impact of this association upon the disease, although not consistent across the studies, remains unclear.

The treatment of developmental dysplasia of the hip diagnosed after the first year of life remains controversial. A series of 36 children (47 hips), aged between one and 4.9 years underwent gradual closed reduction using the Petit-Morel method. A pelvic osteotomy was required in 43 hips (91.5%). The patients whose hips did not require pelvic osteotomy were among the youngest. The mean age at final follow-up was 16.1 years (11.3 to 32). The mean follow-up was 14.3 years (10 to 30).

At the latest follow-up, 44 hips (93.6%) were graded as excellent or good according to the Severin classification. Closed reduction failed in only two hips (4.3%) which then required open reduction. Mild avascular necrosis was observed in one (2.1%).

The accuracy of the reduction and associated low complication rate justify the use of the Petit-Morel technique as the treatment of choice for developmental dysplasia of the hip in patients aged between one and five years.

It has been reported that there is an association between Perthes’ disease and poverty. We examined the demographic data of a group of 240 children (263 hips) who presented with Perthes’ disease in Greater Glasgow, where the mean deprivation scores are substantially greater than in the rest of Scotland, to see if this association applied and whether other clues to the aetiology of Perthes’ disease could be found. There were 197 boys and 43 girls; 39 (16.25%) had a family history of Perthes’ disease. Bone age in this series was heavily skewed towards the lower percentiles. The mean number of siblings was 1.9, with 31 (12.9%) being an only child. Maternal age at the birth of the first child showed no preponderance of older mothers. Maternal smoking during and after pregnancy was noted in 132 (55%), which compared with the 52% reported in the population of Greater Glasgow in general. Of the children in our series, 60 (25%) were in social class IV and V. However, this applies to more than half of the population of Greater Glasgow. There was no significant evidence of a preponderance of Perthes’ disease in the most deprived groups. The aetiology of Perthes’ disease is likely to be multifactorial and may include a genetic or deprivation influence resulting in delayed bone age.

We studied 15 patients with healed tuberculosis of the spine and a resultant kyphosis. We selected only those with no neurological deficit and performed a wedge resection of the vertebra using a transpedicular approach. The wedge was removed from the apex of the deformity. For those with a neurological deficit, we chose the conventional anterior debridement and decompression with 360° circumferential fusion. At a mean follow-up of 26.8 months (8 to 46) the outcome was good with an increase in the mean Oswestry Disability Index from 56.26 (48 to 62) pre-operatively to 11.2 (6 to 16) at the latest follow-up.

Between June 1991 and January 1995, 42 hydroxyapatite-coated CAD-CAM femoral components were inserted in 25 patients with inflammatory polyarthropathy, 21 of whom had juvenile idiopathic arthritis. Their mean age was 21 years (11 to 35). All the patients were reviewed clinically and radiologically at one, three and five years. At the final review at a mean of 11.2 years (8 to 13) 37 hips in 23 patients were available for assessment.

A total of four femoral components (9.5%) had failed, of which two were radiologically loose and two were revised. The four failed components were in patients aged 16 years or less at the time of surgery. Hydroxyapatite-coated customised femoral components give excellent medium- to long-term results in skeletally-mature young adults with inflammatory polyarthropathy. Patients aged less than 16 years at the time of surgery have a risk of 28.5% of failure of the femoral component at approximately ten years.

The purpose of this study was to determine the annual incidence of Perthes’ disease in Korea and compare this with other populations. A survey identified all newly diagnosed children with Perthes’ disease aged 14 years or younger in South Honam, Korea, between January 1999 and December 2001. A total of 84 children were included: 29 in 1999, 28 in 2000 and 27 in 2001. The mean annual incidence was 3.8 per 100 000. This is similar to that reported in other Asian countries, but higher than in black populations and lower than in Caucasians.

Arthritis of the hip in the young adult can be a disabling condition. Recent years have witnessed extensive research related to the management of this condition. This article reviews the current status with regard to aetiology, diagnosis and treatment of arthritis of the hip in the young adult.

We treated 50 patients with bony malignancy by en-bloc resection, extracorporeal irradiation with 50 Gy and re-implantation of the bone segment.

The mean survivor follow-up was 38 months (12 to 92) when 42 patients were alive and without disease. There were four recurrences. The functional results were good according to the Mankin score (17 excellent, 13 good, nine fair, three failures), the Musculoskeletal Tumour Society score (mean 77) and the Toronto Extremity Salvage score (mean 81). There was solid union, but bone resorption was seen in some cases. The dose of radiation was lethal to all cells and produced a dead autograft of perfect fit.

Extracorporeal irradiation is a useful technique for limb salvage when there is reasonable residual bone stock. It allows effective re-attachment of tendons and produces a lasting biological reconstruction. There should be no risk of local recurrence or of radiotherapy-induced malignancy in the replanted bone.

We reviewed 116 patients who underwent 118 arthroscopic ankle arthrodeses. The mean age at operation was 57 years, 2 months (20 to 86 years). The indication for operation was post-traumatic osteoarthritis in 67, primary osteoarthritis in 36, inflammatory arthropathy in 13 and avascular necrosis in two. The mean follow-up was 65 months (18 to 144). Nine patients (10 ankles) died before final review and three were lost to follow-up, leaving 104 patients (105 ankles) who were assessed by a standard telephone interview. The pre-operative talocrural deformity was between 22° valgus and 28° varus, 94 cases were within 10° varus/valgus. The mean time to union was 12 weeks (6 to 20). Nonunion occurred in nine cases (7.6%). Other complications included 22 cases requiring removal of a screw for prominence, three superficial infections, two deep vein thromboses/pulmonary emboli, one revision of fixation, one stress fracture and one deep infection. Six patients had a subtalar fusion at a mean of 48 months after ankle fusion. There were 48 patients with excellent, 35 with good, 10 with fair and 11 with poor clinical results.

Replacement of the shoulder in juvenile idiopathic arthritis is not often performed and there have been no published series to date.

We present nine glenohumeral hemiarthroplasties in eight patients with systemic or polyarticular juvenile idiopathic arthritis. The mean follow-up was six years (59 to 89 months). The mean age at the time of surgery was 32 years. Surgery took place at a mean of 27 years after diagnosis. The results indicated excellent relief from pain. There was restoration of useful function which deteriorated with time, in part because of progression of the systemic disease in this severely affected group. No patient has required revision to date and there has been no radiological evidence of loosening or osteolysis around the implants.

We discuss the pathoanatomical challenges unique to this group. There was very little space for a prosthetic joint and, in some cases, bony deformity and the small size necessitated the use of custom-made implants.

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.

Over an eight-month period we prospectively enrolled 122 patients who underwent arthroscopic surgery of the hip for femoroacetabular impingement and met the inclusion criteria for this study. Patients with bilateral hip arthroscopy, avascular necrosis and previous hip surgery were excluded. Ten patients refused to participate leaving 112 in the study. There were 62 women and 50 men. The mean age of the patients was 40.6 yrs (95% confidence interval (CI) 37.7 to 43.5). At arthroscopy, 23 patients underwent osteoplasty only for cam impingement, three underwent rim trimming only for pincer impingement, and 86 underwent both procedures for mixed-type impingement. The mean follow-up was 2.3 years (2.0 to 2.9). The mean modified Harris hip score (HHS) improved from 58 to 84 (mean difference = 24 (95% CI 19 to 28)) and the median patient satisfaction was 9 (1 to 10). Ten patients underwent total hip replacement at a mean of 16 months (8 to 26) after arthroscopy.

The predictors of a better outcome were the pre-operative modified HHS (p = 0.018), joint space narrowing ≥ 2 mm (p = 0.005), and repair of labral pathology instead of debridement (p = 0.032).

Hip arthroscopy for femoroacetabular impingement, accompanied by suitable rehabilitation, gives a good short-term outcome and high patient satisfaction.

Vertebral haemangiomas are usually asymptomatic and discovered fortuitously during imaging. A small proportion may develop variable degrees of pain and neurological deficit. We prospectively studied six patients who underwent eight surgical procedures on 11 vertebral bodies. There were 11 balloon kyphoplasties, six lumbar and five thoracic. The mean follow-up was 22.3 months (12 to 36). The indications for operation were pain in four patients, severe back pain with Frankel grade C paraplegia from cord compression caused by soft-tissue extension from a thoracic vertebral haemangioma in one patient, and acute bleeding causing Frankel grade B paraplegia from an asymptomatic vascular haemangioma in one patient. In four patients the exhibited aggressive vascular features, and two showed lipomatous, non-aggressive, characteristics. One patient who underwent a unilateral balloon kyphoplasty developed a recurrence of symptoms from the non-treated side of the vertebral body which was managed by a further similar procedure.

Balloon kyphoplasty was carried out successfully and safely in all patients; four became asymptomatic and two showed considerable improvement. Neurological recovery occurred in all cases but bleeding was greater than normal. To avoid recurrence, complete obliteration of the lesion with bone cement is indicated. For acute bleeding balloon kyphoplasty should be combined with emergency decompressive laminectomy. For intraspinal extension with serious neurological deficit, a combination of balloon kyphoplasty with intralesional alcohol injection is effective.