Duchenne muscular dystrophy (DMD) causes progressive skeletal, respiratory and cardiac muscle weakness in affected males. Most DMD patients develop scoliosis following loss of ambulation. This narrative review describes recommendations for the management of scoliosis in DMD patients using a review of the current literature evidence and a consensus review by the DMD Care UK Spinal Surgery Working Group. Advances in medical treatments have improved life expectancy for DMD patients. Spinal bracing is not effective in preventing the deterioration of scoliosis. Seating and wheelchair adaptations can provide postural support. The multidisciplinary assessment of patients with DMD requiring treatment for scoliosis is reviewed, with particular focus on bone, cardiac and respiratory health. The indications, surgical techniques, and type of spinal instrumentation for surgical management for progressively severe scoliosis with or without pelvic obliquity are discussed. Anaesthetic techniques, intraoperative neuromonitoring, perioperative care, and postoperative management in the ICU are discussed for the optimal management of DMD patients undergoing surgery to correct spinal deformity. Finally, regional and holistic functional assessments, patient satisfaction and long-term health, quality of life, and life expectancy for DMD patients undergoing treatment for spinal deformity are reviewed.
Congenital spinal deformities develop due to the presence of congenital anomalies that impact one or more vertebrae, leading to asymmetrical vertical growth of the spine, with the curvature worsening as skeletal development progresses. In Part 1 of this review, the aetiology, classification and associated anomalies related to the congenital spinal deformity are discussed. In Part 2 of this review, the evaluation of patients presenting with a congenital spinal deformity, necessary investigations, as well as both conservative and surgical treatment strategies are discussed. Accurate and timely identification of the specific type of congenital spinal deformity and the likelihood of progression of the curve will determine the initiation of appropriate treatment. Bracing has proven ineffective in managing congenital scoliosis other than when trying to control structural compensatory curves that develop at the levels above or below the congenital vertebral defect. Surgical interventions are designed to promote balanced spinal growth and prevent both severe deformities and neurological complications. Surgical management may include in situ fusion, growth arrest techniques, hemivertebra excision, posterior correction and instrumented spinal fusion, and growth-sparing techniques, each carrying varying risks of significant medical and neurological complications. In cases of severe deformities with risk of spinal cord compression, spinal osteotomies and vertebral column resection may be necessary.
Surgical management of adolescent idiopathic scoliosis [AIS] is a complex undertaking with the primary goals to correct the deformity, maintain sagittal balance, preserve pulmonary function, maximize postoperative function, and improve or at least not harm the function of the lumbar spine. The evolution of surgical techniques for AIS has been remarkable, transitioning from rudimentary methods of spinal correction to highly refined, biomechanically sound procedures. Modern techniques incorporate advanced three-dimensional correction strategies, often leveraging pedicle screw constructs, which provide superior rotational control of the vertebral column. A number of surgical techniques have been described in the literature, each having its own pros and cons. This narrative review provides a detailed analysis of the contemporary surgical techniques used in the treatment of patients with AIS.
Congenital spinal deformities arise from congenital anomalies that impact one or more vertebrae, leading to asymmetrical vertical growth of the spine, with the curvature worsening as skeletal development progresses. In Part 1 of this review, the aetiology, classification and anomalies associated with the congenital spinal deformity are discussed. Congenital vertebral anomalies manifest during the embryonic phase of spinal development and can be categorized into failures of formation, segmentation, or mixed anomalies. Timely identification of the specific vertebral anomaly is crucial for predicting prognosis of the deformity and initiating treatment prior to the onset of a severe curvature. Factors such as the anatomy, location, and number of abnormalities, along with their relation to surrounding spinal structures, influence the likelihood of deformity progression. In Part 2 of this review, the evaluation of patients presenting with congenital spinal deformity, necessary investigations, as well as conservative and surgical treatment strategies are discussed.
Biomechanics is the application of principles of engineering and computers to biological problems. Clinical biomechanics of the spine is the understanding of normal and pathological functions of the human vertebral column in response to mechanical insult, which may include traumatic, degenerative, pathological, or slowly applied loads to the spine. Spinal biomechanics involves the regional features of the vertebrae, intervertebral discs, ligaments, facet joints and muscles. The role of these structures individually and in concert in relation to spinal injuries is discussed. Regional spinal biomechanics, maintenance of spinal stability and assessment of spinal instability following injury are described. Finally, the biomechanical principles, individual structural components, mechanisms of injury and principles specific to the adult and paediatric spine are reviewed.
Background and objective Thoracolumbar spine trauma (TST) is frequently associated with spinal cord injury and other soft tissue and bony injuries. The management of such injuries requires an evidence-based approach. This study used the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument to assess the methodological quality of clinical guidelines for the management of TST published by the Congress of Neurological Surgeons (CNS). Methods All clinical guidelines on TST published by CNS until 2020 were assessed. Five appraisers from three international centers evaluated the quality of eligible clinical guidelines by using AGREE II. Mean AGREE II scores for each domain were determined. In higher-quality domains, the scores for individual items were analyzed. Results A total of 12 guidelines published by CNS on TST were assessed. Mean scores for all six domains were as follows: Scope and Purpose (75.2%), Stakeholder Involvement (45.4%), Rigor of Development (57.0%), Clarity of Presentation (58.7%), Applicability (16.9%), and Editorial Independence (64.1%). The mean score for the overall quality of all CNS guidelines was 52.9% [95% confidence interval (CI): 52.2-53.5%]. The overall agreement among appraisers was excellent [intra-class correlation coefficients (ICCs) for each guideline ranged from 0.903 to 0.963]. Conclusions CNS guidelines for the management of TST demonstrated acceptable quality across most domains; however, the domains of Applicability and Stakeholder Involvement could be further improved in future guideline updates. The assessors concluded that all guidelines could still be recommended for clinical practice with or without modifications.
Spinal cord injury (SCI) is a catastrophic neurological condition resulting in increased physical dependency, medical comorbidity, psychological distress and economic burden. New SCI affects 250,000–500,000 individuals globally each year. The pathophysiological mechanisms following SCI are incompletely understood, and the management of patients following SCI is challenging. Understanding the evolution of pathophysiological changes following SCI is fundamental to delivering and developing appropriate management strategies. Timely interventions following acute SCI can prevent secondary neurological deterioration and improve potential for neurological recovery. A bespoke, multidisciplinary and holistic approach to the management and rehabilitation of SCI patients can optimize physical and mental wellbeing and maximize patient's neurological recovery and functional capabilities. Current therapeutic options following SCI are limited and do not overcome the debilitating effects of SCI. Current knowledge of the pathophysiological mechanism following SCI, management strategies and novel therapies for adult and paediatric SCI patients are reviewed.
BACKGROUND Life expectancy in patients with Duchenne muscular dystrophy (DMD) has improved due to advances in medical care. DMD patients develop progressive spinal deformity after loss of ambulatory function and onset of wheelchair dependence for mobility. There is limited published data on the effect of spinal deformity correction on long-term functional outcomes, quality of life (QoL), and satisfaction in DMD patients. AIM To investigate the long-term functional outcomes following spinal deformity correction in DMD patients. METHODS This was a retrospective cohort study from 2000-2022. Data was collected from hospital records and radiographs. At follow-up, patients completed the muscular dystrophy spine questionnaire (MDSQ). Statistical analysis was performed by linear regression analysis and ANOVA to analyse clinical and radiographic factors significantly associated with MDSQ scores. RESULTS Forty- three patients were included with mean age 14.4 years at surgery. Spinopelvic fusion was performed in 41.9% of patients. Mean surgical time was 352.1 min and mean blood loss was 36% of estimated total blood volume. Mean hospital stay was 14.1 d. Postoperative complications occurred in 25.6% of patients. Mean preoperative scoliosis was 58 degrees, pelvic obliquity 16.4 degrees, thoracic kyphosis 55.8 degrees, lumbar lordosis 11.1 degrees, coronal balance 3.8 cm, and sagittal balance + 6.1 cm. Mean surgical correction of scoliosis was 79.2% and of pelvic obliquity was 80.8%. Mean follow-up was 10.9 years (range: 2-22.5). Twenty-four patients had died at follow-up. Sixteen patients completed the MDSQ at mean age 25.4 years (range 15.2-37.3). Two patients were bed-ridden and 7 were on ventilatory support. Mean MDSQ total score was 38.1. All 16 patients were satisfied with the results of spinal surgery and would choose surgery again if offered. Most patients ( 87.5%) reported no severe back pain at follow-up. Factors significantly associated with functional outcomes (MDSQ total score) included greater duration of post-operative follow-up, age, scoliosis postoperatively, correction of scoliosis, increased lumbar lordosis postoperatively, and greater age at loss of independent ambulation. CONCLUSION Spinal deformity correction in DMD patients leads to positive long-term effects on QoL and high patient satisfaction. These results support spinal deformity correction to improve long-term QoL in DMD patients.
Surgical correction of paediatric spinal deformity is associated with risks, adverse events, and complications that must be preoperatively discussed with patients and their families to inform treatment decisions, expectations, and long-term outcomes. The incidence of complications varies in relation to the underlying aetiology of spinal deformity and surgical procedure. Intraoperative complications include bleeding, neurological injury, and those related to positioning. Postoperative complications include persistent pain, surgical site infection, venous thromboembolism, pulmonary complications, superior mesenteric artery syndrome, and also pseudarthrosis and implant failure, proximal junctional kyphosis, crankshaft phenomenon, and adding-on deformity, which may necessitate revision surgery. Interventions included in enhanced recovery after surgery protocols may reduce the incidence of complications. Complications must be diagnosed, investigated and managed expeditiously to prevent further deterioration and to ensure optimal outcomes. This review summarises the complications associated with paediatric spinal deformity surgery and their management.
Background: Optimal management of severe and complex injuries to the medial knee ligaments remains uncertain. This systematic review evaluates outcomes from randomised controlled trials investigating interventions for treating injuries of medial ligaments of the knee. Methods: Randomised controlled clinical trials evaluating interventions for treating injuries of the medial knee ligaments were identified through searching EMBASE, Medline, Cochrane, WHO, and clinicaltrials.org. Comparisons were made between different nonoperative interventions, surgical versus non-operative interventions, different surgical interventions, and different postoperative interventions. Primary outcome measures included patient reported outcome measures (PROM) of knee function, pain, and treatment failure. Results: 412 trial records were identified; 259 were non-redundant and distinct studies, of which eight were included for analysis. No differences between operative and nonoperative management strategies were found for either primary outcome: Lysholm and IKDC scores. Compared to non-operative interventions, operative interventions resulted in reduced quadriceps strength at eight weeks (mean difference (MD) 29 (% knee extension strength deficit compared with unaffected knee); 95%CI 3.89-54.11) and were associated with greater incidence of minor complications (relative risk (RR) 25; 95%CI: 1.51-421, and RR 25; 95%CI: 1.50-416.58, respectively). Quadriceps strength between 16 and 52 weeks was similar in non-operative and operative groups (MD 20; 95%CI -1.92-41.92). Functional rehabilitation was associated with reduced valgus laxity at six weeks compared to bracing ((MD) - 1.40; 95%CI: - 2.21-0.59). Conclusions: Non-operative interventions provided similar functional results to operative interventions for grade I-III injuries of the medial collateral ligaments. Non-operative intervention was associated with improved quadriceps strength during rehabilitation, and fewer complications, compared to operative interventions. (C) 2020 Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. Published by Elsevier Ltd. All rights reserved.
Long-term cross-sectional study. To investigate the long-term effects of untreated Scheuermann’s kyphosis on quality of life, and its relationship to radiographic parameters of spinal deformity. Previous studies reported reduced self-image, increased pain and impaired physical status. Little is known of the long-term impact of sagittal plane deformity in untreated SK. One hundred and thirteen consecutive untreated patients with SK were identified from a national service database prior to 2000, when surgery was not offered at this unit. 81 of these patients were available for evaluation; 66 (81%) consented to questionnaire and clinical evaluation, and 47 (58%) consented to additional radiological evaluation. Health-related quality of life (HRQoL) was compared to normative population values. Mean age was 45.1 years (31–65), and mean follow-up was 27 years (16–36). 57 patients had thoracic kyphosis and 9 had thoracolumbar deformity. SRS-22 and SF-36 scores were lower, and ODI was greater in patients with untreated SK compared to normative population values. Kyphosis progressed from mean 66° at skeletal maturity to 78° (p < 0.001) after mean follow-up of 27 years. Long-term progression of untreated SK was 0.45°/year (n = 47). Multilinear regression showed good correlation between increasing SVA and worse ODI scores (r = 0.59; p = 0.001). Increasing SVA also correlated with worse function, pain and mental health scores reported by SRS-22, and with worse physical function and bodily pain scores reported by SF-36. Increasing CL correlated with worse SF-36 physical function scores. Increasing cSVA and increasing TK correlated with worse SRS-22 self-image scores. SRS-22 and SF-36 scores were lower, and ODI was greater in patients with untreated SK compared to normative data. Long-term progression of untreated SK was 0.45°/year (n = 47). Increasing SVA correlated with worse SF-36 physical function, SRS-22 function, SRS-22 pain and higher ODI scores. Total kyphosis (TK) and cSVA were independent predictors of low SRS self-image. III.
Background: Primary total knee arthroplasty (TKA) is associated with high patient satisfaction. However, controversy remains regarding the safety and efficacy of conducting simultaneous bilateral (simBTKA) versus staged bilateral TKA (staBTKA). The objective of this systematic review and meta-analysis was to evaluate the current evidence for simBTKA versus staBTKA and compare clinical outcomes including mortality, complications and length of stay (LOS). Methods: A search was performed of PubMed, MEDLINE, EMBASE and Cochrane central databases between January 2000 and March 2020. Search terms included "simultaneous," "staged," and "bilateral TKA." Inclusion criteria comprised studies comparing outcomes of simBTKA versus staBTKA. Quality of included studies was assessed and meta-analyses of pooled data was conducted. Results: 29 articles published between 2001 and 2020 were included in qualitative synthesis from 927 potentially relevant titles, comprising 257,284 patients. 104,207 patients underwent simBTKA and 153,632 patients underwent staBTKA. simBTKA was associated with significantly increased 90-day mortality rate (P<.00001, OR 2.24, 95% CI 1.79-2.81), increased incidence of pulmonary embolism (P <.00001, OR 1.69, 95% CI 1.51-1.89), venous thrombosis (P <.00001, OR 1.33, 95% CI 1.23-1.43), and neurological complications (P=.002, OR 1.42, 95% CI 1.13-1.77). Incidence of superficial and deep infection was significantly increased with staBTKA (P=.02, P <.00001 respectively). Revision rate within one year was equivocal. Mean LOS was 2.1 days shorter for simBTKA. Conclusion: SimBTKA was associated with decreased incidence of infection and LOS but increased incidence of 90-day mortality, venous thromboembolism and neurological complications. Revision rates were equivocal. Patients should be selected and counseled based on the risks respective to each strategy. (C) 2021 Elsevier Inc. All rights reserved.
Adult spinal deformity (ASD) comprises a range of aetiologies and pathologies affecting the lumbar, thoracic or thoracolumbar region during the ageing process. Management of ASD is an increasing health-care burden due to its prevalence in patients aged over 65 years and increasing age of populations in developed countries. Non-operative management of ASD has limited benefit, but may be appropriate for patients with a mild spinal deformity, who can cope with their level of symptoms, or those patients too frail for major spinal surgery. Surgical management of ASD is a major undertaking, associated with significant risks. Minimally invasive surgical (MIS) techniques are rapidly evolving and further research is required to determine the most suitable ASD patient profiles and clinical parameters for treatment by MIS, hybrid, or open techniques. Understanding the resources available for preoperative risk stratification, patient optimization and outcomes assessment following surgery can help to personalize risk-benefit discussions with patients and to develop pragmatic patient-specific surgical strategies for patients with ASD to obtain successful outcomes and reduce the risk of complications. This narrative review discusses patient assessment, treatment outcomes, and surgical strategies to manage patients with ASD.
Early-onset scoliosis is defined as a spinal curvature greater than 10 degrees in children prior to 10 years of age. Untreated EOS may lead to progressively severe spinal deformity, impaired pulmonary development, restrictive lung disease, and both increased morbidity and mortality. Limitations of established conservative treatments include inability to correct severe deformity, as well as challenges with compliance when casting and bracing is applied. In addition, surgical treatment in the form of traditional growing rods requires regular surgical lengthenings and is associated with complications inherent with repeated invasive procedures and exposure to general anesthesia. MAGEC is an evolving magnetically controlled growing rod system for the treatment of EOS. After initial implantation, lengthening is achieved non-invasively by using magnetic external remote control. MAGEC offers the potential to control moderate and severe EOS, while avoiding repeated surgical procedures and associated complications. In this review, we examine the results from clinical, radiological and explant studies following the use of MAGEC, in the context of other established and emerging treatments for EOS.
Scottish National Spine Deformity Center, Royal Hospital for Sick Children, University of Edinburgh, Edinburgh, UK Abstract: Early-onset scoliosis is defined as a spinal curvature greater than 10° in children prior to 10 years of age. Untreated EOS may lead to progressively severe spinal deformity, impaired pulmonary development, restrictive lung disease, and both increased morbidity and mortality. Limitations of established conservative treatments include inability to correct severe deformity, as well as challenges with compliance when casting and bracing is applied. In addition, surgical treatment in the form of traditional growing rods requires regular surgical lengthenings and is associated with complications inherent with repeated invasive procedures and exposure to general anesthesia. MAGEC is an evolving magnetically controlled growing rod system for the treatment of EOS. After initial implantation, lengthening is achieved non-invasively by using magnetic external remote control. MAGEC offers the potential to control moderate and severe EOS, while avoiding repeated surgical procedures and associated complications. In this review, we examine the results from clinical, radiological and explant studies following the use of MAGEC, in the context of other established and emerging treatments for EOS.
Background: The incidence of total hip and total knee arthroplasty (THA/TKA) is 3.1- and 1.7-fold higher in patients with Paget's disease of bone (PDB) compared to age-matched controls. No large studies or joint registry reports exist describing outcomes following THA or TKA in PDB patients. Methods: The study objectives were to investigate the outcomes following THA or TKA in PDB patients using national registry data. Data were requested from the Scottish Arthroplasty Project for all PDB patients undergoing THA or TKA in Scotland from 1996 to 2013. Results: Between 1996 - 2013, 144 patients underwent primary THA and 43 patients underwent primary TKA for PDB in Scotland. Following primary THA, the most common surgical complications within one year were haematoma (1.4%), and surgical-site infection (1.4%). The overall incidence of dislocation was 2.8%. Revision THA was performed in 2.8% of patients. THA implant survival was 96.3% (CI:92.8-99.8) at 10-years, and patient survival was 50.0% (CI:39.6-60.4) at 10-years. Following TKA, only one revision surgery occurred within one year (2.3%). Revision TKA was performed in 4.7% of patients, across the whole study period. TKA implant survival was 94.5% (CI:87.1-100) at 10-years; patient survival was 38.3% (CI:16.7-59.9) at 10-years. Compared with published literature and registry data, implant longevity and patient survival are comparable between PDB patients and the general population. Conclusion: This is the largest reported series of outcomes following primary THA/TKA in PDB patients. PDB patients are not at increased risk of surgical complications following primary THA or TKA compared with the general population. Crown Copyright (C) 2020 Published by Elsevier Ltd on behalf of Royal College of Surgeons of Edinburgh (Scottish charity number SC005317) and Royal College of Surgeons in Ireland. All rights reserved.
Aims Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service. Methods Descriptive study of prospectively collected data. Clinical data of all patients undergoing surgery for spinal deformity between 2005 and 2018 was collected, compared to the demographics of the national population, and analyzed by underlying aetiology. Results Our cohort comprised 2,205 patients; this represents an incidence of 14 per 100,000 individuals among the national population aged between zero and 18 years. There was an increase in mean annual incidence of spinal deformity surgery across the study period from 9.6 (7.2 to 11.7) per 100,000 individuals in 2005 to 2008, to 17.9 (16.1 to 21.5) per 100,000 individuals in 2015 to 2018 (p = 0.001). The most common cause of spinal deformity was idiopathic scoliosis accounting for 56.7% of patients. There was an increase in mean incidence of surgery for adolescent idiopathic scoliosis (AIS) (from 4.4 (3.1 to 5.9) to 9.8 (9.1 to 10.8) per 100,000 individuals; p < 0.001), juvenile idiopathic scoliosis (JIS) (from 0.2 (0.1 to 0.4) to one (0.5 to 1.3) per 100,000 individuals; p = 0.009), syndromic scoliosis (from 0.7 (0.3 to 0.9) to 1.7 (1.2 to 2.4) per 100,000 individuals; p = 0.044), Scheuermann's kyphosis (SK) (from 0.2 (0 to 0.7) to 1.2 (1.1 to 1.3) per 100,000 individuals; p = 0.001), and scoliosis with intraspinal abnormalities (from 0.04 (0 to 0.08) to 0.6 (0.5 to 0.8) per 100,000 individuals; p = 0.008) across the study period. There was an increase in mean number of posterior spinal fusions performed each year from mean 84.5 (51 to 108) in 2005 to 2008 to 182.5 (170 to 210) in 2015 to 2018 (p < 0.001) and a reduction in mean number of growing rod procedures from 45.5 (18 to 66) in 2005 to 2008 to 16.8 (11 to 24) in 2015 to 2018 (p = 0.046). Conclusion The incidence of patients with spinal deformity undergoing surgery increased from 2005 to 2018. This was largely attributable to an increase in surgical patients with adolescent idiopathic scoliosis. Paediatric spinal deformity was increasingly treated by posterior spinal fusion, coinciding with a decrease in the number of growing rod procedures. These results can be used to plan paediatric spinal deformity services but also evaluate preventative strategies and research, including population screening.
This narrative review will summarise a clinical approach to the investigation of back pain in children and adolescent patients, including a discussion of the epidemiology, presentation, investigation and clinical management of back pain in children and adolescents. This will assist the prompt and accurate diagnosis of spinal disorders that require significant medical intervention. Existing evidence suggests a relatively high incidence of non-specific back pain among young people; 27-48% of presentations of back pain in children and adolescents are attributed to non-specific back pain. Low back pain among schoolchildren is often linked to psychosocial factors and only occasionally requires medical attention, as pain is benign and self-limiting. Nonetheless, those young patients who seek medical assistance exhibit a higher incidence of organic conditions underlying the major symptom of spinal pain. A cautious and comprehensive strategy - including a detailed history, examination, radiographic imaging and diagnostic laboratory studies - should be employed, which must be accurate, reliable, consistent and reproducible in identifying spinal pathologies. A specific diagnosis can be reached in 52-73% of the cases. For cases in which a specific diagnosis cannot be made, re-evaluation after a period of observation is recommended. At this later stage, minor symptoms unrelated to underlying pathology will resolve spontaneously, whereas serious pathologies will advance and become easily identified.
Blood loss is anticipated with all surgical procedures. Within the field of elective orthopaedic surgery it is especially important to reduce bleeding to maintain a patient's haemodynamic balance and ensure a clear surgical field. Complex reconstructive arthroplasty and spinal procedures can be complicated by significant intraoperative blood loss (IBL) and result in the need for allogenic blood transfusion. Patients with higher rates of IBL have increased blood transfusion requirements, resulting in a higher risk of mortality and significant postoperative morbidity. The aim of this review is to outline strategies to manage IBL for both paediatric and adult patients undergoing major elective orthopaedic surgery, and to evaluate the available evidence regarding their efficacy in managing IBL and reducing the need for perioperative allogenic transfusion.
Lowest instrumented vertebra (LIV) selection is critical to preventing complications following posterior spinal arthrodesis (PSA) for thoracolumbar/lumbar adolescent idiopathic scoliosis (TL/L AIS), but evidence guiding LIV selection is limited.This study aimed to investigate the efficacy of PSA using novel unilateral convex segmental pedicle screw instrumentation (UCS) in correcting TL/L AIS, to identify radiographic parameters correlating with distal extension of PSA, and to develop a predictive equation for distal fusion extension using these parameters.We reviewed data (demographic, clinical, radiographic, and SRS-22 questionnaires) preoperatively to 2-years' follow-up for TL/L AIS patients treated by PSA using UCS between 2006 to 2011. 53 patients were included and divided into 2 groups: Group-1 (n=36) patients had PSA between Cobb-to-Cobb levels; Group-2 (n=17) patients required distal fusion extension.A mean curve correction of 80% was achieved. Mean postoperative LIV angle, TL/L apical vertebra tr...