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

Background: Current treatment for Perthes disease aims at preventing deformation of the femoral head during the active stage of the disease by obtaining containment of the femoral head. To effectively pre-empt femoral head deformation, one needs to know, when during the disease irreparable femoral head deformation occurs. This study was undertaken to attempt to clarify this.

Methods: Records and 2634 pairs of radiographs (AP and lateral) of 610 patients with Perthes’ disease were reviewed. The evolution of the disease was divided into seven stages (Stages Ia, Ib, IIa, IIb, IIIa, IIIb & IV) based on plain radiographic appearances. Intra-observer and inter-observer reproducibility of this new classification system was assessed. The duration of each stage of the disease was noted. The stage at which epiphyseal extrusion and widening of the metaphysis occurred and the stage at which metaphyseal and acetabular changes appeared were identified. The shape and the size of the femoral head, the extent of trochanteric overgrowth and the radius of the acetabulum were assessed in hips that had healed.

Results: The reproducibility of the new classification system of the evolution Of Perthes’ disease was good. The median duration of each stage varied between 95 and 326 days. Epiphyseal extrusion and metaphyseal widening was modest in Stages Ia, Ib and IIa but increased dramatically after Stage IIb. > 20% extrusion occurred in 70% of the hips by Stage IIIa. Metaphyseal changes were most frequently encountered in Stage IIb, while acetabular changes were most prevalent in Stage IIIa. At healing, only 24% of untreated patients had spherical femoral heads, while 52% had irregular femoral heads.

Conclusions: The new classification of the stages of evolution of Perthes’ disease helps to identify when crucial events occur during the course of the disease. The timing of epiphyseal extrusion, metaphyseal widening and the appearance of adverse metaphyseal and acetabular changes suggest that femoral head deformation occurs by Stage IIIa in untreated hips. Hence, if containment were to succeed, it should be achieved before this stage.

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

Rapidly progressive osteoarthritis of the hip (RPOH) is an unusual subset of osteoarthritis. It is characterized by rapid joint space loss, chondroly­sis, and sometimes marked femoral head and acetabular destruction as a late finding. The exact pathogenetic mechanism is unknown. Potential causes of RPOH include subchondral insufficiency fracture resulting from osteoporosis, increasing posterior pelvic tilt as a mechanical factor, and high serum levels of matrix metalloproteinase (MMP)-3 as biological factors. This study was aimed to identify some markers that associate with the destructive process of RPOH by analyzing the proposed pathological factors of the disease, MMP-3, pelvic tilt, and osteoporosis. Of female patients who visited our hospital with hip pain from 2012 through 2018, this study enrolled female patients with sufficient clinical records including the onset of hip pain, age and body mass index (BMI) at the onset, a series of radiographs during the period of >12 months from the onset of hip pain, and hematological data of MMP-3 and C-reactive protein (CRP). We found the hip joints of 31 patients meet the diagnostic criteria of RPOH, chondrolysis >two mm in one year, or 50% joint space narrowing in one year. Those patients were classified into two groups, 17 and 14 patients with and without subsequent femoral head destruction in one year shown by computed tomography, respectively. Serum MMP-3 and CRP were measured with blood samples within one year after the hip pain onset. The cortical thickness index (CTI) as an indicator of osteoporosis and pelvic tilt parameters were evaluated on the initial anteroposterior radiograph of the hip. These factors were statistically compared between the two groups. This study excluded male patients because RPOH occurs mainly in elderly females and the reference intervals of MMP-3 are different between males and females. There was no difference in age at onset or bone mass index between the RPOH patients with and without subsequent femoral head destruction. Serum levels of MMP-3 were significantly higher in the RPOH patients with the destruction (152.1 ± 108.9 ng/ml) than those without the destruction (66.8 ± 27.9 ng/ml) (P = 0.005 by Mann-Whitney test). We also found increased CRP in the patients with femoral head destruction (0.725 ± 1.44 mg/dl) compared with those without the destruction (0.178 ± 0.187 mg/dl) (P = 0.032 by Mann-Whitney test). No difference in the duration between the hip pain onset and the blood examination was found between the two groups. There was no significant difference in CTI or pelvic tilt between the two groups. The pathological condition that may increase serum MMP-3 and CRP could be involved in femoral head destruction after chondrolysis of the hip in patients with RPOH

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 study identifies variations in presentation and demographics between structural and non-structural (muscle patterning) shoulder instability. We analysed 1020 unstable shoulders (855 patients) from our institution database. Demographic details, direction and aetiology were obtained from medical records. Anterior dislocations comprised 67%, posterior 31% and inferior 2% of all directions of instability and 75 shoulders had multidirectional instability. Structural causes were dominant in anterior instability (traumatic 39% and atraumatic 38%) and muscle patterning in posterior (81%) and inferior (90%) instability. Males accounted for 64% of all patients (73% of all structural patients and 53% of muscle patterning patients. Mean age at presentation was 25 years old (structural patients 28 years and muscle patterning patients 21 years old). There were 690 unilaterally unstable shoulders (57% right- and 43% left-sided); the dominant arm was affected in 58% overall, in 42% of all left-handers and only 33% of left-handers with muscle patterning. Bilateral shoulder instability occurred in 19% of all patients (12% of patients with structural instability and 28% of those with muscle patterning instability). For muscle patterning, the mean age at onset of symptoms was 14 years, and mean length of symptoms before presentation was 8 years. There was a trimodal distribution of age at onset of symptoms corresponding to peaks at 6, 14 and 20 years. In the group with onset of muscle patterning under 10 years old, there was a higher proportion of females (71% vs 47%), laxity (63% vs 29%) and bilaterality (54% vs 42%), and fewer presenting with pain (17% vs 50%). Muscle patterning instability is associated with a demographic and presentation profile which may help distinguish it from structural forms of instability. As age at presentation increased, pain increased and joint laxity decreased. Bilaterality did not appear to be associated with gender, the presence of laxity or pain

This study identifies variations in presentation and demographics for different forms of shoulder instability. We analysed 1020 unstable shoulders (855 patients) from a previously presented database. Demographic details, direction and aetiology were obtained from medical records. Anterior dislocations comprised 67%, posterior 31% and inferior 2% of all directions of instability and 75 shoulders had multidirectional instability. Structural causes were dominant in anterior instability (traumatic 39% and atraumatic 38%) and muscle patterning in posterior (81%) and inferior (90%) instability. Males accounted for 64% of all patients (73% of all structural patients and 53% of muscle patterning patients. Mean age at presentation was 25 years old (structural patients 28 years and muscle patterning patients 21 years old). There were 690 unilaterally unstable shoulders (57% right- and 43% left-sided); the dominant arm was affected in 58% overall, in 42% of all left-handers and only 33% of left-handers with muscle patterning. Bilateral shoulder instability occurred in 19% of all patients (12% of patients with structural instability and 28% of those with muscle patterning instability). For muscle patterning, the mean age at onset of symptoms was 14 years, and mean length of symptoms before presentation was 8 years. There was a trimodal distribution of age at onset of symptoms corresponding to peaks at 6, 14 and 20 years. In the group with onset of muscle patterning under 10 years old, there was a higher proportion of females (71% vs 47%), laxity (63% vs 29%) and bilaterality (54% vs 42%), and fewer presenting with pain (17% vs 50%). As age at presentation increased, pain increased and joint laxity decreased. Bilaterality did not appear to be associated with gender, the presence of laxity or pain. Muscle patterning instability is associated with a demographic and presentation profile which may help distinguish it from structural forms of instability

Purpose of the study: The purpose of this retrospective study was to analyze clinical datao n pigmented villon-odular synovitis (PVSN) of the knee as well as outcome after treatment in order to define the diagnostic stages, the surgical treatment, and follow-up modalities for this rare benign proliferative disease of the synovial which predominantly affects the knee joint. Material and methods: Between 1996 and 2004, 28 patients were managed in our department, 13 men and 15 women, diffuse PVNS in 20 and localized PVNS in 8. IN the localized forms, symptoms were similar to those observed in knees with intra-articular foreign bodies or a meniscal lesion (75%) was present for 14 months on average at the first consultation. Mean age at onset of therapeutic management was 40 years (range 20–62). Localized arthroscopic or open resection was performed. For the diffuse forms, symptoms had been present for 15 months on average at the first consultation. Patients sought medical care because of spontaneous hemarthrosis or diffuse knee pain with no specific signs. Mean age at onset of therapeutic management was 38 years (range 15–59). Bony lesions were observed in 20%. Synoviorthesis or surgical synovectomy were performed. Mean follow-up was 97 months (range 12–309). Outcome was analyzed separately for the localized and diffuse forms. Results: For the localized PVNS, there were no complications after surgical treatment but the relapse rate reached 12.5%. For diffuse PVNS, the cumulative rate of relapse was 50%, recurrence being noted on average 37 months after treatment. A stiff joint developed in 14% after open synovectomy. Surgical treatment was necessary in four cases (total arthroplasty in three) seen late after development of bony lesions; the clinical outcome was good with good gain in flexion. Discussion: MRI is essential for the topographic diagnosis and to guide surgery. For diffuse PVNS seen at an advanced stage or after several recurrences, adjuvant synoviorthesis can be useful 4 to 8 months after surgery. Conclusion: Appropriate treatment of PVNS of the knee depends on the presentation but usually involves a surgical procedure. The risk of recurrence for diffuse PVNS warrants annual MRI for four years

Purpose. To investigate associations between sagittal thoracolumbar spine shape with sex and measures of adiposity throughout adulthood. Methods. Thoracolumbar spine shape was characterised using statistical shape modelling on lateral dual-energy x-ray absorptiometry images, recorded for vertebral fracture analysis, of the spine from 1529 participants of the MRC National Survey of Health and Development, acquired at age 60–64 years. Associations between spine shape modes (SM) and 1) sex, 2) contemporaneous measures of overall and central adiposity (indicated by body mass index and waist circumference, respectively), 3) changes in total and central adiposity during earlier stages of adulthood and age at onset of overweight, were investigated. Results. Four of the first eight spine modes (SM) describing lumbar spine shape differed by sex; on average, women had more lordotic spines than men with relatively smaller but caudally increasing anterior-posterior (a-p) vertebral diameters. Greater BMI and waist circumference and earlier onset of overweight were associated with uneven (or snaking) spinal curvatures (SM2) and larger a-p vertebral diameters (SM3). Central adiposity was also associated with larger caudal disc heights (SM4) in women, especially increases between 36–43 years. Conclusions. Sagittal spine shapes differed by sex and associations with overall and central adiposity also differed. Overweight and greater central adiposity earlier in adulthood were particularly important, and were associated with a straighter but more unevenly curved spine with larger vertebrae and caudal discs heights, possibly explained by a chronic effect of increased mechanical loading on the spine. Conflicts of interest: None. Funding received from MRC

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

Introduction:. Paley et al has developed a multiplier method for calculating both leg length and total height. In the development of this algorithm, they evaluated the effect of factors including bone age and sex. They established that sex had a significant impact, but adjusting for bone age did not improve accuracy. Bone age and menarche have been shown to improve other height prediction models. Purpose:. We used a large prospective cohort to evaluate if the multiplier is independent of physiological age using menarche as a proxy. Methods:. Using the ALSPAC dataset we determined the accuracy of the Paley multiplier for predicting total height and leg length, and assed weather if the date of first menses increased the accuracy of the multiplier. Female patients over the age of 8, with documented final height and final sub-ishial leg length over the age of 15 and a date of first menses were evaluated. Predicted final height was compared with actual final height at all data points. Results:. There were 28332 data points in 3062 girls prior to skeletal maturity in the total height cohort and 8395 data points in 2300 girls in the leg length cohort. When age was corrected using the difference in age at onset of menarche from average, the accuracy of multiplier decreased for both measurements. When a correction of 50% was used, there was an improvement in the accuracy of multiplier predictions, reducing the average error by up to 24%. Conclusions:. Previous studies have failed to demonstrate that the accuracy of the multiplier is improved when adjusted for bone age. We have used the date of first menses as a proxy for bone age and established that making a 50% correction for physiological age improves the accuracy of this method. Significance:. This will potentially allow more accurate prediction of leg length discrepancy, and total height in girls with early menarche

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.

Aims

Perthes’ disease is an uncommon hip disorder with limited data on the long-term outcomes in adulthood. We partnered with community-based foundations and utilized web-based survey methodology to develop the Adult Perthes Survey, which includes demographics, childhood and adult Perthes’ disease history, the University of California Los Angeles (UCLA) Activity Scale item, Short Form-36, the Hip disability and Osteoarthritis Outcome Score, and a body pain diagram. Here we investigate the following questions: 1) what is the feasibility of obtaining > 1,000 survey responses from adults who had Perthes’ disease using a web-based platform?; and 2) what are the baseline characteristics and demographic composition of our sample?

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

Aims

Avascular femoral head necrosis in the context of gymnastics is a rare but serious complication, appearing similar to Perthes’ disease but occurring later during adolescence. Based on 3D CT animations, we propose repetitive impact between the main supplying vessels on the posterolateral femoral neck and the posterior acetabular wall in hyperextension and external rotation as a possible cause of direct vascular damage, and subsequent femoral head necrosis in three adolescent female gymnasts we are reporting on.