Aims. The aim of this study was to compare outcomes after growth-friendly treatment for early-onset scoliosis (EOS) between patients with skeletal dysplasias versus those with other syndromes. Methods. We retrospectively identified 20 patients with skeletal dysplasias and 292 with other syndromes (control group) who had completed surgical growth-friendly EOS treatment between 1 January 2000 and 31 December 2018. We compared radiological parameters, complications, and health-related quality of life (HRQoL) at mean follow-up of 8.6 years (SD 3.3) in the dysplasia group and 6.6 years (SD 2.6) in the control group. Results. Mean major curve correction per patient did not differ significantly between the dysplasia group (43%) and the control group (28%; p = 0.087). Mean annual spinal height increase was less in the dysplasia group (9.3 mm (SD 5.1) than in the control group (16 mm (SD 9.2); p < 0.001). Mean annual spinal growth adjusted to patient preoperative standing height during the distraction period was 11% in the dysplasia group and 14% in the control group (p = 0.070). The complication rate was 1.6 times higher (95% confidence interval (CI) 1.3 to 2.0) in the dysplasia group. The following complications were more frequent in the dysplasia group: neurological injury (rate ratio (RR) 5.1 (95% CI 2.3 to 11)), deep surgical site infection (RR 2.2 (95% CI 1.2 to 4.1)), implant-related complications (RR 2.0 (95% CI 1.5 to 2.7)), and unplanned revision (RR 1.8 (95% CI 1.3 to 2.5)). Final fusion did not provide additional spinal height compared with watchful waiting (p = 0.054). There were no significant differences in HRQoL scores between the groups. Conclusion. After growth-friendly EOS treatment, patients with skeletal dysplasias experienced a higher incidence of complications compared to those with other syndromes. Surgical growth-friendly treatment for skeletal dysplasia-associated EOS should be reserved for patients with severe, progressive deformities that are refractory to nonoperative treatment. Cite this article: Bone Joint J 2024;106-B(6):596–602

Aims. The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. Patients and Methods. A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation. Results. Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). Conclusion. Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563–1569

Aims. Different methods of anterior cruciate ligament (ACL) reconstruction have been described for skeletally immature patients before closure of the growth plates. However, the outcome and complications following this treatment remain unclear. The aim of this systematic review was to analyse the outcome and complications of different techniques which may be used for reconstruction of the ACL in these patients. Materials and Methods. We performed a systematic review of the literature according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. This involved a comprehensive search of PubMed, Medline, CINAHL, Cochrane, Embase and Google Scholar databases using the following combinations of keywords, “knee”, “anterior cruciate ligament”, “reconstruction”, “injury”, “children”, “adolescent”, “skeletally immature”, “open physis” and “surgery”. Results. A total of 53 studies met the inclusion criteria and were included for analysis. The overall rate of disturbance of growth after ACL reconstruction was 2.6%, with no statistical difference between transphyseal and physeal-sparing techniques. Physeal-sparing techniques had a lower rate of post-operative complications compared with transphyseal techniques (p = 0.0045). Outcomes assessed were Lysholm score, International Knee Documentation Committee (IKDC) score, the IKDC grade, the Tegner score and the KT-1000. Both techniques had similar clinical outcomes. Conclusions. This review reveals low rates of disturbance of growth after ACL reconstruction in skeletally immature patients. Although limited, the available evidence did not support any particular surgical technique when considering disturbance of growth or clinical outcome. Further randomised controlled trials are needed to investigate the efficacy of differing surgical techniques on outcomes in skeletally immature patients. Cite this article: Bone Joint J 2017;99-B:1053–60

Intermittently administered parathyroid hormone (PTH 1-34) has been shown to promote bone formation in both human and animal studies. The hormone and its analogues stimulate both bone formation and resorption, and as such at low doses are now in clinical use for the treatment of severe osteoporosis. By varying the duration of exposure, parathyroid hormone can modulate genes leading to increased bone formation within a so-called ‘anabolic window’. The osteogenic mechanisms involved are multiple, affecting the stimulation of osteoprogenitor cells, osteoblasts, osteocytes and the stem cell niche, and ultimately leading to increased osteoblast activation, reduced osteoblast apoptosis, upregulation of Wnt/β-catenin signalling, increased stem cell mobilisation, and mediation of the RANKL/OPG pathway. Ongoing investigation into their effect on bone formation through ‘coupled’ and ‘uncoupled’ mechanisms further underlines the impact of intermittent PTH on both cortical and cancellous bone. Given the principally catabolic actions of continuous PTH, this article reviews the skeletal actions of intermittent PTH 1-34 and the mechanisms underlying its effect. Cite this article: L. Osagie-Clouard, A. Sanghani, M. Coathup, T. Briggs, M. Bostrom, G. Blunn. Parathyroid hormone 1-34 and skeletal anabolic action: The use of parathyroid hormone in bone formation. Bone Joint Res 2017;6:14–21. DOI: 10.1302/2046-3758.61.BJR-2016-0085.R1

Aims

The gluteus minimus (GMin) and gluteus medius (GMed) have unique structural and functional segments that may be affected to varying degrees, by end-stage osteoarthritis (OA) and normal ageing. We used data from patients with end-stage OA and matched healthy controls to 1) quantify the atrophy of the GMin and GMed in the two groups and 2) describe the distinct patterns of the fatty infiltration in the different segments of the GMin and GMed in the two groups.

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

The atlas of Greulich and Pyle for skeletal maturity and epiphyseal closure is widely used in many countries to assess skeletal age and to plan orthopaedic surgery. The data used to compile the atlas were collected from institutionalised American children in the 1950s. In order to determine whether the atlas was relevant to subSaharan Africa, we compared skeletal age, according to the atlas, with chronological age in 139 skeletally immature Malawian children and young adults with an age range from 1 year 11 months to 28 years 5 months. The height and weight of each patient were also measured in order to calculate the body mass index. The skeletal age of 119 patients (85.6%) was lower than the chronological age. The mean difference was 20.0 ± 24.1 months (t-test, p = 0.0049), and the greatest difference 100 months. The atlas is thus inaccurate for this group of children. The body mass index in 131 patients was below the normal range of 20 to 25 kg/m. 2. . The reasons for the low skeletal age in this group of children are discussed. Poor nutrition and chronic diseases such as malaria and diarrhoea which are endemic in Malawi are likely to be contributing factors. We did not find any correlation between the reduction in body mass index in our patients and the degree of retardation of skeletal age

Patients with skeletal dysplasia are prone to developing advanced osteoarthritis of the knee requiring total knee replacement (TKR) at a younger age than the general population. TKR in this unique group of patients is a technically demanding procedure owing to the deformity, flexion contracture, generalised hypotonia and ligamentous laxity. We retrospectively reviewed the outcome of 11 TKRs performed in eight patients with skeletal dysplasia at our institution using the Stanmore Modular Individualised Lower Extremity System (SMILES) custom-made rotating-hinge TKR. There were three men and five women with mean age of 57 years (41 to 79). Patients were followed clinically and radiologically for a mean of seven years (3 to 11.5). The mean Knee Society clinical and function scores improved from 24 (14 to 36) and 20 (5 to 40) pre-operatively, respectively, to 68 (28 to 80) and 50 (22 to 74), respectively, at final follow-up. Four complications were recorded, including a patellar fracture following a fall, a tibial peri-prosthetic fracture, persistent anterior knee pain, and aseptic loosening of a femoral component requiring revision. Our results demonstrate that custom primary rotating-hinge TKR in patients with skeletal dysplasia is effective at relieving pain, with a satisfactory range of movement and improved function. It compensates for bony deformity and ligament deficiency and reduces the likelihood of corrective osteotomy. Patellofemoral joint complications are frequent and functional outcome is worse than with primary TKR in the general population

Skeletal scintigraphy, which has now been established as a useful and accurate method of detecting early skeletal metastases and assessing their response to treatment, has been investigated for its pathological basis. Histological examination of several hundred necropsy specimens, from sixty-eight patients who died from malignant disease, showed a significant increase of osteoid and immature woven bone in the presence of metastatic cancer. Tumour-cell suspensions of the VX2 carcinoma were injected into the medullary cavity or on to the periosteal surface of the ilia or tibiae of New Zealand white rabbits. A combination of bone destruction and new bone formation, similar to the autopsy material, was seen. There were at least two mechanisms for the new bone production. Initially, intramembranous ossification was seen in the fibrous stroma surrounding the tumour. Once the cortex was involved and cortical bone destruction had occurred, large amounts of woven bone resembling fracture callus were laid down. The new bone had a markedly increased avidity for boneseeking isotopes, indicating why skeletal scintigraphy was useful. A further twenty rabbits, in whose ilia the VX2 carcinoma was growing, were treated by local irradiation. When treatment was successful the tumour was destroyed, the production of new bone ceased, and the lesion lost its increased avidity for bone-seeking isotopes, indicating that skeletal scintigraphy could be used to assess the response of skeletal metastases to therapy

Skeletal metastases from hepatocellular carcinoma are highly destructive vascular lesions which severely reduce the quality of life. Pre-existing liver cirrhosis presents unique challenges during the surgical management of such lesions. We carried out a retrospective study of 42 patients who had been managed surgically for skeletal metastases from hepatocellular carcinoma affecting the appendicular skeleton between January 2000 and December 2006. There were 38 men and four women with a mean age of 60.2 years (46 to 77). Surgery for a pathological fracture was undertaken in 30 patients and because of a high risk of fracture in 12. An intralesional surgical margin was achieved in 36 and a wide margin in six. Factors influencing survival were determined by univariate and multivariate analyses. The survival rates at one, two and three years after surgery were 42.2%, 25.8% and 19.8%, respectively. The median survival time was ten months (95% confidence interval 6.29 to 13.71). The number of skeletal metastases and the Child-Pugh grade were identified as independent prognostic factors by Cox regression analysis. The method of management of the hepatocellular carcinoma, its status in the liver, the surgical margin for skeletal metastases, the presence of a pathological fracture and adjuvant radiotherapy were not found to be significantly related to the survival of the patient, which was affected by hepatic function, as represented by the Child-Pugh grade

Femoral lengthening using the Intramedullary Skeletal Kinetic Distractor is a new technique. However, with intramedullary distraction the surgeon has less control over the lengthening process. Therefore, 33 femora lengthened with this device were assessed to evaluate the effect of operative variables under the surgeon’s control on the course of lengthening. The desired lengthening was achieved in 32 of 33 limbs. Problems encountered included difficulty in achieving length in eight femora (24%) and uncontrolled lengthening in seven (21%). Uncontrolled lengthening was more likely if the osteotomy was placed with less than 80 mm of the thick portion of the nail in the distal fragment (p = 0.052), and a failure to lengthen was more likely if there was over 125 mm in the distal fragment (p = 0.008). The latter problem was reduced with over-reaming by 2.5 mm to 3 mm. Previous intramedullary nailing also predisposed to uncontrolled lengthening (p = 0.042), and these patients required less reaming. Using the Intramedullary Skeletal Kinetic Distractor, good outcomes were obtained; problems were minimised by optimising the position of the osteotomy and the amount of over-reaming performed

The case histories of these Black Notley patients show that no serious harmful effects have been caused by pregnancies going to full term. Most of the patients had normal confinements; Caesarian sections were few and were usually done for obstetric reasons, not for fear of reactivation of the disease. This experience bears out a remark made to me by Marcel Galland. When asked if Caesarian sections were required for women who became pregnant after treatment at Berck for skeletal tuberculosis, he replied: "Jarnais! Tout passe!". Two patients in the series had artificial abortions, and one aborted spontaneously. Two of these three patients did not do as well as other patients whose pregnancies went to full term. Quite another question is whether the onset of skeletal tuberculosis sooii after pregnancy may be attributed to the pregnancy. In a group of women of child-bearing age, it would be difficult to distinguish between post hoc and propter hoc in considering this question. One patient of this series, H. Q. (Case 25), was admitted eight months pregnant with active tuberculosis of the knee; but pain in the joint had started fifteen months before admission, and the onset of disease no doubt preceded the pregnancy. Some of the patients admitted to Black Notley were mothers of young children hut there is no note of any pregnancy having preceded the onset of tuberculosis by a short interval. The follow-up reports of the married patients compare favourably with those of the unmarried. Further, it may be argued that pregnancy increases resistance to tuberculosis. Such a belief was current from the time of Hippocrates until the middle of the nineteenth century, and clinical evidence can be quoted in favour of such a view. In America, Lyman (1943) followed up 1,818 women who had been treated for tuberculosis in a sanatorium; many had married despite medical advice to the contrary. Lyman found that the late results of treatment were four times as good in the married patients as in the single women. Lyman says, "When we consider that the married group established this record in spite of the fact that 192 out of 315 gave histories of pregnancies (averaging 2·25 children each), it is clear that some factor not yet accounted for has exerted a profound influence in their cases.". I have speculated elsewhere as to what this factor may be (Wilkinson 1949). But it seems clear that increased sterol circulation in the body is associated with increased reticulo-endothelial activity (Fraser 1935). Thus reticulo-endothelial proliferation has been observed in the lesions of lupus following the administration of calciferol (Dowling, Gauvain and Macrae 1948). An increase of blood cholesterol is found during pregnancy. The sex hormones are sterols and might be expected to be raised in the marital state; it is perhaps significant that marital contacts form a group relatively immune to tuberculosis. There would appear to be good grounds for reassuring the married woman who has suffered from skeletal tuberculosis regarding the prospect of normal pregnancy. Many letters received from patients in a follow-up of this sort are poignant documents. There is no doubt that the majority of these women desire children as ardently as normal women, and that a safe confinement following skeletal tuberculosis is an excellent form of rehabilitation

Assessment of skeletal age is important in children’s orthopaedics. We compared two simplified methods used in the assessment of skeletal age. Both methods have been described previously with one based on the appearance of the epiphysis at the olecranon and the other on the digital epiphyses. We also investigated the influence of assessor experience on applying these two methods. Our investigation was based on the anteroposterior left hand and lateral elbow radiographs of 44 boys (mean: 14.4; 12.4 to 16.1 ) and 78 girls (mean: 13.0; 11.1 to14.9) obtained during the pubertal growth spurt. A total of nine observers examined the radiographs with the observers assigned to three groups based on their experience (experienced, intermediate and novice). These raters were required to determined skeletal ages twice at six-week intervals. The correlation between the two methods was determined per assessment and per observer groups. Interclass correlation coefficients (ICC) evaluated the reproducibility of the two methods. The overall correlation between the two methods was r = 0.83 for boys and r = 0.84 for girls. The correlation was equal between first and second assessment, and between the observer groups (r ≥ 0.82). There was an equally strong ICC for the assessment effect (ICC ≤ 0.4%) and observer effect (ICC ≤ 3%) for each method. There was no significant (p < 0.05) difference between the levels of experience. The two methods are equally reliable in assessing skeletal maturity. The olecranon method offers detailed information during the pubertal growth spurt, while the digital method is as accurate but less detailed, making it more useful after the pubertal growth spurt once the olecranon has ossified. Cite this article: Bone Joint J 2014;3:1556–60

Aims. We wished to examine the effectiveness of tibial lengthening using a two ring Ilizarov frame in skeletally immature patients. This is a potentially biomechanically unstable construct which risks the loss of axial control. Patients and Methods. We retrospectively reviewed a consecutive series of 24 boys and 26 girls, with a mean age of 8.6 years (4 to 14), who underwent 52 tibial lengthening procedures with a mean follow-up of 4.3 years (4.0 to 16.9). Tibial alignment was measured before and after treatment using joint orientation lines from the knee and a calculation of the oblique plane axis. Results. The mean percentage length gain was 20% (13 to 31) with a mean bone-healing index (BHI) of 34 days per centimetre (20 to 54). Age at surgery and location of the osteotomy site showed a strong relationship as the location of the osteotomy migrated more proximally with age. However, no significant correlation was found between BHI and the position of the osteotomy. The coronal and sagittal joint orientation of the lengthened tibias were within the normal range in 67% and 92% of cases, respectively. The oblique plane angulation calculation revealed a statistically significant change pre- to post-operatively in both magnitude (p < 0.05) and direction (p < 0.01), but without clinical consequence. The majority of complications were minor (n = 40 in 27 patients; 1.48 complications per segment lengthened) with no residual disability reported. Conclusion. Two-ring tibial lengthening is effective in maintaining segmental alignment, efficient in callous production and relatively comfortable for the patient with few significant complications. Cite this article: Bone Joint J 2016;98-B:1276–82

Between 1992 and 1999, we treated 350 patients with skeletal metastases. A multivariable analysis of the patients was conducted using the Cox proportional hazards model. We identified five significant prognostic factors for survival, namely, the site of the primary lesion, the performance status (Eastern Cooperative Oncology Group status 3 or 4), the presence of visceral or cerebral metastases, any previous chemotherapy, and multiple skeletal metastases. The score for each significant factor was derived from the corresponding estimated regression coefficients (natural logarithm of the hazard ratio). The prognostic score was calculated by adding all the scores for individual factors. The rate of survival was 31% at six months and 11% at one year for the patients with a prognostic score of 6 or more. By contrast, patients with a prognostic score of 2 or less had a rate of survival of 98% at six months and 89% at one year. This scoring system can be used to determine the optimal treatment for patients with pathological fractures or epidural compression

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

This study evaluated the effect of limb lengthening on longitudinal growth in patients with achondroplasia. Growth of the lower extremity was assessed retrospectively by serial radiographs in 35 skeletally immature patients with achondroplasia who underwent bilateral limb lengthening (Group 1), and in 12 skeletally immature patients with achondroplasia who did not (Group 2). In Group 1, 23 patients underwent only tibial lengthening (Group 1a) and 12 patients underwent tibial and femoral lengthening sequentially (Group 1b). The mean lengthening in the tibia was 9.2 cm (59.5%) in Group 1a, and 9.0 cm (58.2%) in the tibia and 10.2 cm (54.3%) in the femur in Group 1b. The mean follow-up was 9.3 years (8.6 to 10.3). The final mean total length of lower extremity in Group 1a was 526.6 mm (501.3 to 552.9) at the time of skeletal maturity and 610.1 mm (577.6 to 638.6) in Group 1b, compared with 457.0 mm (411.7 to 502.3) in Group 2. However, the mean actual length, representing the length solely grown from the physis without the length of distraction, showed that there was a significant disturbance of growth after limb lengthening. In Group 1a, a mean decrease of 22.4 mm (21.3 to 23.1) (4.9%) was observed in the actual limb length when compared with Group 2, and a greater mean decrease of 38.9 mm (37.2 to 40.8) (8.5%) was observed in Group 1b when compared with Group 2 at skeletal maturity. In Group 1, the mean actual limb length was 16.5 mm (15.8 to 17.2) (3.6%) shorter in Group 1b when compared with Group 1a at the time of skeletal maturity. Premature physeal closure was seen mostly in the proximal tibia and the distal femur with relative preservation of proximal femur and distal tibia. We suggest that significant disturbance of growth can occur after extensive limb lengthening in patients with achondroplasia, and therefore, this should be included in pre-operative counselling of these patients and their parents

Aims

The epiphyseal approach to a chondroblastoma of the intercondylar notch of a child’s distal femur does not provide adequate exposure, thereby necessitating the removal of a substantial amount of unaffected bone to expose the lesion. In this study, we compared the functional outcomes, local recurrence, and surgical complications of treating a chondroblastoma of the distal femoral epiphysis by either an intercondylar or an epiphyseal approach.

We have previously shown that cytological diagnosis based on fine-needle aspiration biopsy (FNAB) is a safe and efficient method for the discrimination between benign, primary malignant and metastatic bony lesions. We have now studied metastatic lesions to assess the diagnostic accuracy and to ascertain whether FNAB allows identification of the primary lesion. Between 1990 and 1997, 447 patients were referred for diagnosis of skeletal lesions of unknown type. Of these 119 proved to have metastatic disease, either myeloma or lymphoma. Nine were excluded leaving 110 consecutive patients with metastatic carcinoma (80), myeloma (16) or lymphoma (14). FNAB gave a correct diagnosis in 102 of the 110 patients (93%). In eight it was inconclusive. It correctly diagnosed 15 of 16 patients with myeloma, 12 of 14 with lymphoma, and 75 of 80 with metastatic carcinoma. Furthermore, the site and type of malignancy were correctly suggested in two-thirds of patients with metastatic carcinoma. Overall, only seven open biopsies were carried out. We conclude that time-consuming and costly investigations can be reduced by choosing FNAB as the initial diagnostic method for skeletal lesions of unknown origin. The choice of radiological examinations, laboratory tests and surgical biopsies can be determined by using FNAB

We reviewed 277 patients with soft-tissue sarcoma (STS) treated between 1975 and 1995 to study the incidence, distribution, time of appearance, and radiological findings of skeletal metastases. Of these, 28 (10.1%) had metastases within a mean period of 18.6 months after admission. The incidence of skeletal metastases differed among the histological subtypes of sarcoma; alveolar soft-part sarcoma, dedifferentiated liposarcoma, angiosarcoma, and rhabdomyosarcoma tended to show higher incidences. The regional bones close to the primary tumour were affected in 13 (46.4%) of the 28 patients, and the axial bones in 18 (64.3%). Radiologically, the metastatic bony lesions predominantly showed osteolytic changes, and there were pathological fractures in 21 of 44 lesions