Exercise-based interventions targeting balance and falls in people with COPD: a systematic review and meta-analysis.

INTRODUCTION: This review quantifies the mean treatment effect of exercise-based interventions on balance and falls risk in people with COPD. METHODS: A structured search strategy (2000-2023) was applied to eight databases to identify studies evaluating the impact of exercise-based interventions (≥14 days in duration) on balance or falls in people with COPD. Pooled mean treatment effects (95% confidence intervals (CIs), 95% prediction intervals (PIs)) were calculated for outcomes reported in five or more studies. Inter-individual response variance and the promise of behaviour change techniques (BCTs) were explored. RESULTS: 34 studies (n=1712) were included. There were greater improvements in balance post intervention compared to controls for the Berg Balance Scale (BBS) (mean 2.51, 95% CI 0.22-4.80, 95% PI -4.60-9.63), Timed Up and Go (TUG) test (mean -1.12 s, 95% CI -1.69- -0.55 s, 95% PI -2.78-0.54 s), Single-Leg Stance (SLS) test (mean 3.25 s, 95% CI 2.72-3.77 s, 95% PI 2.64-3.86 s) and Activities-specific Balance Confidence (ABC) scale (mean 8.50%, 95% CI 2.41-14.58%, 95% PI -8.92-25.92%). Effect on falls remains unknown. Treatment effects were larger in male versus mixed-sex groups for the ABC scale and SLS test, and in balance training versus other exercise-based interventions for the BBS and TUG test. Falls history was not associated with changes in balance. Meta-analysis of individual response variance was not possible and study-level results were inconclusive. Eleven promising BCTs were identified (promise ratio ≥2). CONCLUSION: Evidence for the effect of exercise-based interventions eliciting clinically important improvements in balance for people with COPD is weak, but targeted balance training produces the greatest benefits. Future exercise interventions may benefit from inclusion of the identified promising BCTs.

Femur Fractures in 5 Individuals With Pantothenate Kinase-associated Neurodegeneration: The Role of Dystonia and Suggested Management.

BACKGROUND: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare, neurodegenerative disorder that manifests with progressive loss of ambulation and refractory dystonia, especially in the early-onset classic form. This leads to osteopenia and stress on long bones, which pose an increased risk of atraumatic femur fractures. The purpose of this study is to describe the unique challenges in managing femur fractures in PKAN and the effect of disease manifestations on surgical outcomes. METHODS: A retrospective case review was conducted on 5 patients (ages 10 to 20 y) with PKAN with a femur fracture requiring surgical intervention. Data regarding initial presentation, surgical treatment, complications, and outcomes were obtained. RESULTS: All patients were non-ambulatory, with 4 of 5 patients sustaining an atraumatic femur fracture in the setting of dystonia episode. One patient had an additional contralateral acetabular fracture. Postoperatively, 4 of the 5 patients sustained orthopaedic complications requiring surgical revision, with 3 of these secondary to dystonia. Overall, 4 required prolonged hospitalization in the setting of refractory dystonia. CONCLUSION: Femur fractures in PKAN present distinct challenges for successful outcomes. A rigid intramedullary rod with proximal and distal interlocking screws is most protective against surgical complications associated with refractory dystonia occurring during the postoperative period. Multidisciplinary planning for postoperative care is essential and may include aggressive sedation and pain management to decrease the risk of subsequent injuries or complications. LEVEL OF EVIDENCE: Level IV.

Amputation vs Reconstruction in Type IV Tibial Hemimelia: Functional Outcomes and Description of a Novel Surgical Technique.

Introduction: The management of tibial hemimelia can be complex and involve either amputation or reconstruction. The decision made carries significant implications on patients and their families. This is a case series in the management of Type IV tibial hemimelia with a description of a novel surgical technique in the reconstructive arm of the pathway. Materials and methods: The study included four patients with bilateral tibial hemimelia have an amputation in one limb and reconstructive surgery on the other. The reconstruction involved a supratalar double osteotomy of the tibia and fibula, followed by a staged hindfoot osteotomy using a circular ring fixator. Functional outcomes are reported using the Special Interest Group in Amputee Medicine (SIGAM) and the short form 12 (SF-12) methods. Results: The mean age of patients in our cohort is 14 years (3-27 years) with mean age of surgery at 3 years. One case had an amputation following initial reconstructive surgery due to psychological distress and regressive behaviour. SIGAM functional outcome scores of F were recorded in three of four cases, with one patient performing at level B. On the reconstructive side, two of three patients reported a mean physical short form 12 (SF-12) score of 56.7 and a mental SF-12 score of 55.7. One patient reported a physical SF-12 score of 28.5 and a mental SF-12 score of 30.3. Discussion and conclusion: A reconstructive option provides a satisfactory functional outcome, comparable to the population mean, in the majority of patients in our cohort. Clinical significance: A staged supratalar double osteotomy followed later by a hindfoot osteotomy is effective in centralising the ankle and creates a plantigrade weight-bearing platform for ambulation in patients with Type IV tibial hemimelia. How to cite this article: Tang CH, Addar A, Fernandes JA. Amputation vs Reconstruction in Type IV Tibial Hemimelia: Functional Outcomes and Description of a Novel Surgical Technique. Strategies Trauma Limb Reconstr 2023;18(1):32-36.

How Much Does Paediatric Femoral Lengthening Cost? A Cost Comparison between Magnetic Lengthening Nails and External Fixators.

Aim: Motorised intramedullary lengthening nails are considered more expensive than external fixators for limb lengthening. This research aims to compare the cost of femoral lengthening in children using the PRECICE magnetic lengthening nail with external fixation. Methods: Retrospective analysis of 50 children who underwent femoral lengthening. One group included patients who were treated with PRECICE lengthening nails, the other group included patients who had lengthening with external fixation. Each group included 25 patients aged between 11 and 17 years. The patients in both groups were matched for age. Cost analysis was performed following micro-costing and analysis of the used resources during the different phases of the treatments. Results: Each group's mean patient age was 14.7 years. Lengthening nails were associated with longer operative times compared with external fixators, both for implantation and removal surgery (p-values of 0.007 and < 0.0001, respectively). Length of stay following the implantation surgery, frequency of radiographs and frequency of outpatient department appointments were all lower with lengthening nails. The overall cost of lengthening nails was £1393 more than external fixators, however, this difference was not statistically significant (p-value = 0.088). Conclusion: The difference in the mean costs between femoral lengthening with lengthening nails versus external fixators was not statistically significant. Further research to review the effectiveness of the devices and the quality of life during the lengthening process is crucial for robust health economic evaluation. How to cite this article: Hafez M, Nicolaou N, Offiah A, et al. How Much Does Paediatric Femoral Lengthening Cost? A Cost Comparison between Magnetic Lengthening Nails and External Fixators. Strategies Trauma Limb Reconstr 2023;18(1):16-20.

Quality of life of children during distraction osteogenesis: a comparison between intramedullary magnetic lengthening nails and external fixators.

BACKGROUND: Distraction osteogenesis is a very demanding process. For decades, external fixation was the only reliable option for gradual deformity correction. Recently, intramedullary magnetic nails have gained popularity. This research aimed to assess the quality of life in children during gradual deformity correction using intramedullary lengthening nails compared to external fixation. METHOD: Prospective analysis included children who had gradual lower limb deformity correction between 2017 and 2019. Group A included children who had magnetic lengthening nails; patients in group B had external fixation devices. Child health utility 9D (CHU- 9D) and EuroQol 5D youth (EQ- 5D-Y) were used to measure the quality of life at fixed points during the distraction osteogenesis process. The results were used to calculate the utility at each milestone and the overall quality of life adjusted years (QALYs). RESULTS: Thirty-four children were recruited, group A had 16 patients, whilst group B had 18 patients. The average ages were 16.0 years and 14.7 years for groups A and B, respectively. Group A patients reported significantly better utility compared to group B. This was observed during all stages of treatment (P = 0.00016). QALYs were better for group A (0.44) compared to group B (0.34) (P < 0.0001). CONCLUSION: The quality of life was generally better in group A compared to group B. In most patients, the health utility progressively improved throughout treatment. In the same way, QALYs were better with the lengthening nails compared to external fixators. The magnetic lengthening devices (PRECICE nails) which were used in this research were recently relabelled to restrict their applications in children; this study was conducted before these restrictions.

The (extended) achondroplasia foramen magnum score has good observer reliability.

BACKGROUND: Achondroplasia is the most common skeletal dysplasia. A significant complication is foramen magnum stenosis. When severe, compression of the spinal cord may result in sleep apnea, sudden respiratory arrest and death. To avoid complications, surgical decompression of the craniocervical junction is offered in at-risk cases. However, practice varies among centres. To standardize magnetic resonance (MR) reporting, the achondroplasia foramen magnum score was recently developed. The reliability of the score has not been assessed. OBJECTIVE: To assess the interobserver reliability of the achondroplasia foramen magnum score. MATERIALS AND METHODS: Base of skull imaging of children with achondroplasia under the care of Sheffield Children's Hospital was retrospectively and independently reviewed by four observers using the achondroplasia foramen magnum score. Two-way random-effects intraclass coefficient (ICC) was used to assess inter- and intra-observer reliability. RESULTS: Forty-nine eligible cases and five controls were included. Of these, 10 were scored normal, 17 had a median score of 1 (mild narrowing), 11 had a median score of 2 (effacement of cerebral spinal fluid), 10 had a score of 3 (compression of cord) and 6 had a median score of 4 (cord myelopathic change). Interobserver ICC was 0.72 (95% confidence interval = 0.62-0.81). Intra-observer ICC ranged from 0.60 to 0.86. Reasons for reader disagreement included flow void artefact, subtle T2 cord signal and myelopathic T2 cord change disproportionate to canal narrowing. CONCLUSION: The achondroplasia foramen magnum score has good interobserver reliability. Imaging features leading to interobserver disagreement have been identified. Further research is required to prospectively validate the score against clinical outcomes.

Femoral Lengthening in Children-A Comparison Between Magnetic Intramedullary Lengthening Nails and External Fixators.

BACKGROUND: Femoral lengthening can be achieved using external fixators or intramedullary lengthening nails. The purpose of this research was to compare the outcome of femoral lengthening in children using PRECICE magnetic lengthening nails with lengthening external fixators. METHODS: Retrospective analysis of 50 children who had femoral lengthening. Group A included patients who had lengthening with external fixation, patients in Group B had lengthening with PRECICE intramedullary lengthening nails. Each group included 25 patients. The sample strictly included children aged between 11 and 17 years. Patients in each group were matched according to age and indication for lengthening whether congenital or acquired conditions. The outcomes focused on the ability to achieve target length, healing index, residual malalignment, length of hospitalization following the osteotomy surgery, and encountered complications. RESULTS: Mean patient age was 14.7 years for each group. The length gain was 42±12 mm for Group A and 41.6±8 mm for Group B (P=0.84). Lengthening nails achieved the target length more accurately compared with external fixation (P=0.017). The healing index was significantly higher in group A with 53.2±19 days/cm compared with 40.2±14 days/cm in group B (P=0.03). Group A had significantly higher complications than group B (P<0.0001). There was no statistically significant difference in the final coronal malalignment between the 2 groups (P=0.2). The mean length of stay was 9.2±5.8 days for group A and 4.2±3.3 days for group B (P=0.0005). CONCLUSION: Magnetic lengthening nails are clinically effective for femoral lengthening in the pediatric population. Compared with external fixation, healing index and complications were more favorable with PRECICE nails. Further research is required to study the cost-effectiveness of this technique. LEVEL OF EVIDENCE: Level IV-case series.

Acetabular volume and femoral coverage change following Dega-like osteotomy in treatment of developmental dysplasia of the hip.

Although Dega's acetabuloplasty has become a commonly used osteotomy, there is still an ongoing debate regarding its impact on the volume and depth of the acetabulum. The purpose of our study was to assess the postoperative images of the transverse acetabular plane and version obtained from developmental dysplasia of the hip (DDH) patients after a modified Dega osteotomy. We performed a retrospective study of the morphologic indices obtained from the pelvic X-rays and computer tomography (CT) scans of patients with DDH, who were operated in our institution between July 2005 and October 2013, using the modified Dega osteotomy. Preoperative and postoperative pelvic X-rays were used to measure the acetabular index and the acetabular (ACM) angle. The postoperative CT scans were used to measure the acetabular anteversion angle (AAA), acetabular depth and depth of acetabular coverage [total acetabular index (TAI)]. The contralateral unaffected hips served as control. The postoperative acetabular indices and ACMs showed a significant improvement, with near-normal values measured 1 year after the surgery. Comparing the CT scan parameters (AAA, TAI and the depth of acetabulum) showed that TAI and the depth of acetabulum were not significantly different between the groups (TAI: P = 0.423; depth of acetabulum: P = 0.132), whereas AAA comparison implied a slight advantage of the DDH hips (17.6° DDH vs. 13.3° contralateral, P = 0.001). On the basis of images set of acetabular morphometric parameters, our study substantiates previous reports on an increase of acetabular volume and a significant improvement of femoral head coverage for patients with DDH who underwent Dega acetabuloplasty. Level of evidence: Level III - retrospective comparative study.

Femoral lengthening in young patients: An evidence-based comparison between motorized lengthening nails and external fixation.

BACKGROUND: Femoral lengthening is a procedure of great importance in the treatment of congenital and acquired limb deficiencies. Technological advances have led to the latest designs of fully implantable motorized intramedullary lengthening nails. The use of these nails has increased over the last few years. AIM: To review and critically appraise the literature comparing the outcome of femoral lengthening in children using intramedullary motorized lengthening nails to external fixation. METHODS: Electronic databases (MEDLINE, CINAHL, EMBASE, Cochrane) were systematically searched in November 2019 for studies comparing the outcome of femoral lengthening in children using magnetic lengthening nails and external fixation. The outcomes included amount of gained length, healing index, complications and patient reported outcomes. RESULTS: Of the 452 identified studies, only two (retrospective and non-randomized) met the inclusion criteria. A total of 91 femora were included. In both studies, the age of patients treated with nails ranged from 15 to 21 years compared to 9 to 15 years for patients in the external fixation group. Both devices achieved the target length. Prevalence of adverse events was less in the nail (60%-73%) than in the external fixation (81%-100%) group. None of the studies presented patient reported outcomes. CONCLUSION: The clinical effectiveness of motorized nails is equivalent or superior to external fixation for femoral lengthening in young patients. The available literature is limited and does not provide evidence on patient quality of life or cost effectiveness of the interventions.

Deformity Correction, Surgical Stabilisation and Limb Length Equalisation in Patients with Fibrous Dysplasia: A 20-year Experience.

INTRODUCTION: Fibrous dysplasia (FD) of bone can be present with pain, deformity and pathological fractures. Management is both medical and surgical. Little literature exists on the surgical management of both monostotic and polyostotic FD. We present our experience of limb reconstruction surgery in this pathological group of bone disease. MATERIALS AND METHODS: A retrospective cohort of children who underwent limb reconstruction surgery at a single high-volume paediatric centre was identified from a prospective database. Case notes and radiographs were reviewed. Surgical techniques, outcomes and difficulties were explored. RESULTS: Twenty-one patients were identified aged between 7 and 13 at presentation to the limb reconstruction unit. Eleven were female, nine had McCune-Albright syndrome, seven had polyostotic FD and five had monostotic. Proximal femoral varus procurvatum deformity was the most common site requiring surgical intervention. The distal femur, tibia, humerus and forearm were also treated.Methods include deformity correction with intramedullary fixation including endo-exo-endo techniques, elastic nailing, guided growth, circular fixator technique and fixator-assisted plating. Correction of deformity and leg length discrepancies was common.The osteotomies went on to heal with no nonunions or delayed healing. We encountered secondary deformity at distal end of nails as the children grew as expected. These were managed with revision nailing techniques and in some cases external fixation. There was one implant failure, which did not require revision surgery. CONCLUSION: The surgical management of pathological bone disease is challenging. Corrective osteotomies with intramedullary fixation can be very successful if appropriate limb reconstruction principles are adhered to. Deformity correction, guided growth and lengthening can all be successfully achieved in bone affected by FD. Polyostotic FD can be present with secondary deformities, and these can be difficult to manage. HOW TO CITE THIS ARTICLE: Hampton MJ, Weston-Simmons S, Giles SN, et al. Deformity Correction, Surgical Stabilisation and Limb Length Equalisation in Patients with Fibrous Dysplasia: A 20-year Experience. Strategies Trauma Limb Reconstr 2021;16(1):41-45.

Mechanical Analysis of Explanted Telescopic Rods in the Management of Osteogenesis Imperfecta: A Multicenter Study.

BACKGROUND: Telescopic rods in the management of osteogenesis imperfecta fail frequently. This could be attributed to technical errors, rod design, and rod structure. We aimed to analyze the mechanical properties and tribology of explanted male and female components to identify effects of in vivo telescoping that may relate to observed patterns of successful telescoping or failure. METHODS: Recruitment took place at 3 of the 4 English centers for osteogenesis imperfecta. Twenty-five rods explanted for growth or failure during revision to a new rod were analyzed in terms of clinical indication and prerevision imaging to identify if there was a technical mode of failure. Laboratory analysis was performed using optical and scanning electrical microscopy, radiograph diffraction analysis, hardness test, bending test, and energy-dispersive x-ray spectroscopy. RESULTS: All implants tested were of high-grade stainless steel. Female components had inferior strength [mean Vickers hardness property (HV0.3) at 0.3 to 313 kg] in comparison to male components (HV0.3 406) due to different techniques of manufacture. Female rods also had a higher wear coefficient (7.89×10-12 m3/N/m3) than the male rods (6.46×10-12 m3/N/m3). Abrasive wear, shear deformation, scratches, and wear debris were identified in all rods. Male and female components displayed corrosion contributing to adhesive wear. Intraoperatively cut rods, particularly the female components, had irregular ends leading to more wear. CONCLUSIONS: Current manufacturing techniques result in inferior material strength in female components compared with males, which combined with wear patterns is likely to lead to implant failure. Intraoperative cutting of rods may increase risk of failure due to wear. Considering techniques to improve strength as well as design in new implants may lead to better outcomes. LEVELS OF EVIDENCE: Level IV-cross-sectional study.

Pelvic Support Hip Reconstruction with Internal Devices: An Alternative to Ilizarov Hip Reconstruction.

AIM AND OBJECTIVE: Ilizarov hip reconstruction (IHR) is a traditional method of salvaging chronic adolescent problem hips but faces practical issues from external fixators leading to reduced compliance. We present the same reconstruction procedure using only internal devices with a modification in the technique and review early results. MATERIALS AND METHODS: We retrospectively evaluated eight patients between 2014 and 2017 with chronic painful hips treated by two-stage reconstruction; stage I included femoral head resection and pelvic support osteotomy using double plating, whereas stage II comprised distal femoral osteotomy avoiding varus followed by the insertion of a retrograde magnetic nail for postoperative lengthening. Patients continued physiotherapy postoperatively while protecting from early weight-bearing. RESULTS: At a mean follow-up of 19 months (range, 6-36), all osteotomies healed with a bone healing index of 47 days/cm (range, 30-72). Pain improved from 8.3 (range, 7-9) to 2 (range, 0-6) while the limb length discrepancy got corrected from 4.3 cm (range, 3-5) to 1.4 cm (range, 0-2.5) at the final follow-up. Trendelenburg sign was eliminated in three patients and delayed in five patients. No examples of infection or permanent knee stiffness were noted. One patient had plate breakage due to mechanical fall, and another patient had 35 mm of lateral mechanical axis deviation (MAD) requiring corrective osteotomy. CONCLUSION: Pelvic support hip reconstruction with exclusive internal devices is a technique in evolution with encouraging early results. It avoids common complications of external fixators and facilitates quick rehabilitation of joints. Refraining from distal varus can effectively eliminate Trendelenburg gait, although with some degree of lateral MAD. Unlike external fixation where there is a possibility of gradual correction, this staged procedure of internal fixation is technically demanding with a learning curve. CLINICAL SIGNIFICANCE: Pelvic support hip reconstruction performed by internal implants is a viable alternative to Ilizarov hip reconstruction with potential benefits. HOW TO CITE THIS ARTICLE: Metikala S, Kurian BT, Madan SS, et al. Pelvic Support Hip Reconstruction with Internal Devices: An Alternative to Ilizarov Hip Reconstruction. Strategies Trauma Limb Reconstr 2020;15(1):34-40.

Pelvic Support Hip Reconstruction with Internal Devices: An Alternative to Ilizarov Hip Reconstruction.

AIM AND OBJECTIVE: Ilizarov hip reconstruction (IHR) is a traditional method of salvaging chronic adolescent problem hips but faces practical problems from external fixators leading to reduced compliance. We present the same reconstruction utilising only internal devices with a modification in technique and aim to review early results. MATERIALS AND METHODS: We retrospectively evaluated eight patients between 2014 and 2017 with chronic painful hips treated by a two-stage reconstruction; stage 1 included femoral head resection and pelvic support osteotomy using double plating, while stage 2 comprised distal femoral osteotomy avoiding varus followed by insertion of retrograde magnetic nail for postoperative lengthening. Patients continued physiotherapy postoperatively while protecting from early weight-bearing. RESULTS: At mean follow-up of 19 months (range 6-36), all osteotomies healed with bone healing index of 47 days/cm (range 30-72). Pain improved from 8.3 (range 7-9) to 2 (range 0-6), while limb length discrepancy got corrected from 4.3 cm (range 3-5) to 1.4 cm (range 0-2.5) at final follow-up. Trendelenburg sign was eliminated in three and delayed in five. No examples of infection or permanent knee stiffness were noted. One patient had plates breakage due to mechanical fall and one had 35 mm of lateral mechanical axis deviation requiring corrective osteotomy. CONCLUSION: Pelvic support hip reconstruction with exclusive internal devices is a technique in evolution with encouraging early results. It avoids common complications of external fixators and facilitates quick rehabilitation of joints. Refraining from distal varus can effectively eliminate Trendelenburg gait, albeit with some degree of lateral mechanical axis deviation. Unlike external fixation where there is a possibility of gradual correction, this staged procedure of internal fixation is technically demanding with a learning curve. CLINICAL SIGNIFICANCE: Pelvic support hip reconstruction performed by internal implants is a viable alternative to IHR with potential benefits. HOW TO CITE THIS ARTICLE: Metikala S, Kurian BT, Madan SS, et al. Pelvic Support Hip Reconstruction with Internal Devices: An Alternative to Ilizarov Hip Reconstruction. Strategies Trauma Limb Reconstr 2020;15(2):91-97.

Association of psychological flexibility with engagement in pulmonary rehabilitation following an acute exacerbation of chronic obstructive pulmonary disease.

This study aimed to investigate (a) the association between psychological flexibility and engagement in pulmonary rehabilitation within 8 weeks following hospitalisation for an acute exacerbation of chronic obstructive pulmonary disease (AECOPD) and (b) how psychological (in)flexibility presents in this context. A mixed-methods study was conducted. Psychological flexibility during an AECOPD was assessed using The Acceptance and Action Questionnaire-II (AAQ-II) (n = 41) and the Engaged Living Scale (ELS) (n = 40). Engagement in post-AECOPD pulmonary rehabilitation was then recorded. Twenty-three patients also participated in cognitive interviews. Psychological flexibility was associated with a greater chance of accepting a pulmonary rehabilitation referral following an AECOPD. Small numbers prohibited analysis on attendance or completion. An AAQ-II score of 11 translated to a 60 (37-82)% probability of accepting a referral to pulmonary rehabilitation and an ELS score of 73 was associated with a 68 (46-91)% probability of accepting. Four themes were extracted from interviews: (1) family values, (2) self as abnormal, (3) 'can't do anything' versus 'I do what I can' and (4) disability, and related emotions, as barriers to action. Randomised clinical trials are needed to evaluate interventions designed to increase psychological flexibility (i.e. acceptance and commitment therapy) to support acceptance of pulmonary rehabilitation post-AECOPD.

Correction of Bowleg Deformity in Achondroplasia through Combined Bony Realignment and Lateral Collateral Ligament Tightening.

INTRODUCTION: Achondroplasia is one of the most common osteochondrodysplasias with an incidence of 1 in 26,000 live births. Bowing of lower limbs can cause significant morbidity in this population. The use of the Ilizarov external fixator to tighten collateral ligaments of the knee in children has not been reported in the literature. We report the technique and early results of lateral collateral ligament (LCL) tightening with correction of genu varum in children with achondroplasia. MATERIALS AND METHODS: A retrospective review of children with achondroplasia presenting with bowleg deformity who were treated by corrective osteotomy and LCL tightening was conducted. Between 1998 and 2003, 12 patients (24 limb segments) underwent this procedure and were included in the study. All patients had grade III LCL laxity preoperatively. Pre- and postoperative anteroposterior standing mechanical axis radiographs were evaluated. The final outcome was graded using the grading system of Paley et al. RESULTS: All patients had bilateral corrections. The Ilizarov external fixator was used in 10 patients and the Orthofix limb reconstruction system in 2 patients. The bony realignment was achieved through monofocal or bifocal tibial osteotomies. The LCL was tightened in all limb segments using the Paley's type II strategy. The final result was graded as excellent in 20 limb segments and good in 4 limb segments. One patient developed transient common peroneal nerve palsy, four developed grade II pin site infections, and there was premature consolidation of the tibial regenerate in one patient. HOW TO CITE THIS ARTICLE: Kurian BT, Belthur MV, Jones S, et al. Correction of Bowleg Deformity in Achondroplasia through Combined Bony Realignment and Lateral Collateral Ligament Tightening. Strategies Trauma Limb Reconstr 2019;14(3):132-138.

Geroderma osteodysplasticum: Histological features and the role of panel-based exome sequencing in diagnosis.

Geroderma osteodysplasticum (GO) has clinical and histological features that overlap with other causes of wrinkly skin. Here we present the case of a child diagnosed with GO following exome sequencing of a panel of genes covering the wide differential diagnosis. The histological features of the overlapping conditions are presented, highlighting the utility of panel testing for conditions of this type. This is relevant to many genetic conditions and can influence ongoing management as exemplified by this case.

Managing Developmental Dysplasia of the Hip.

Developmental dysplasia of the hip (DDH) involves a spectrum of hip disorders that affect hip anatomy and development and can range from mild anatomical deformity with a reduced but subluxatable hip to a frankly dislocated hip. It was previously known as congenital dislocation of the hip (CDH) but this name is no longer used due to the fact that the hip may be anatomically abnormal whilst not being dislocated. The key aim of clinical management of DDH is early diagnosis and referral as this can often mean less invasive treatment is possible, and outcomes are significantly improved if treatment is initiated at an early stage and certainly before 6 wk of age.

The Limping Child - What a Pediatrician Should Know?

Diagnosis of a limping child can be challenging for the clinician given the vast array of conditions that present in this manner. This review article discusses the history, examination and subsequent differential diagnoses that should be considered along with the most appropriate investigations for each diagnosis. The latest evidence regarding choice of investigation to make the clinician's task less onerous is also discussed.

The use of recombinant morphogenic protein-2(rhBMP-2) in children undergoing revision surgery for persistent non-union.

The purpose of the study was to evaluate the safety and efficacy with the use of BMP-2 for treating persistent non-unions in children with underlying complex conditions. Between October 2006 and November 2010 in our unit, 15 patients were treated with rhBMP-2 to enhance bone union. There were nine females and six males with a mean age of 9.5 years (range 4-15) at time of surgery. Seventy-five per cent of the patients required revision of internal fixation with insertion of rhBMP-2 to the non-union site, and the reminder had freshening of the non-union site with rhBMP-2 application. Patients had undergone a mean of 2 (1-5) operations prior to implantation of rhBMP-2. All the patients in the study group were available for review with mean follow-up of 44 months (range 21-70). The mean time to union was 16 weeks (range 10-28 weeks). No adverse events related to BMP-2 application were noted in our study group. Healing occurred clinically and radiographically in 16 of the 17 sites. Our study demonstrates that BMP-2 enhances healing of the persistent non-unions without any adverse events.

Zoledronate Attenuates Accumulation of DNA Damage in Mesenchymal Stem Cells and Protects Their Function.

Mesenchymal stem cells (MSCs) undergo a decline in function following ex vivo expansion and exposure to irradiation. This has been associated with accumulation of DNA damage and has important implications for tissue engineering approaches or in patients receiving radiotherapy. Therefore, interventions, which limit accumulation of DNA damage in MSC, are of clinical significance. We were intrigued by findings showing that zoledronate (ZOL), an anti-resorptive nitrogen containing bisphosphonate, significantly extended survival in patients affected by osteoporosis. The effect was too large to be simply due to the prevention of fractures. Moreover, in combination with statins, it extended the lifespan in a mouse model of Hutchinson Gilford Progeria Syndrome. Therefore, we asked whether ZOL was able to extend the lifespan of human MSC and whether this was due to reduced accumulation of DNA damage, one of the important mechanisms of aging. Here, we show that this was the case both following expansion and irradiation, preserving their ability to proliferate and differentiate in vitro. In addition, administration of ZOL before irradiation protected the survival of mesenchymal progenitors in mice. Through mechanistic studies, we were able to show that inhibition of mTOR signaling, a pathway involved in longevity and cancer, was responsible for these effects. Our data open up new opportunities to protect MSC from the side effects of radiotherapy in cancer patients and during ex vivo expansion for regenerative medicine approaches. Given that ZOL is already in clinical use with a good safety profile, these opportunities can be readily translated for patient benefit.