Survival of patients with Duchenne muscular dystrophy who underwent spinal deformity correction.

AIM: We investigated survival rates after scoliosis correction in individuals with Duchenne muscular dystrophy (DMD) and evaluated factors that can affect them. METHOD: This was a retrospective cohort study from 2000 to 2022 with a minimum 2-year postoperative follow-up. We reviewed the hospital records/spinal radiographs and analysed data with XLSTAT. Kaplan-Meier and multivariate Cox regression survival analysis was performed. RESULTS: Forty-three patients had a mean age at surgery of 14 years 5 months. Mean postoperative follow-up was 10 years 10 months. There was no operative or 30-day postoperative mortality in this group. Twenty-four patients died because of cardiorespiratory failure. Median survivorship was 14 years 2 months, with the longest observed survival being 22 years 6 months given the limitation of the length of postoperative follow-up. The degree of preoperative coronal imbalance and pelvic obliquity, as well as intraoperative blood loss, were factors that significantly affected survival. The impact of preoperative sagittal imbalance and extension of the fusion to the sacrum/pelvis trended towards significance. In contrast, age at surgery, preoperative/postoperative scoliosis, thoracic kyphosis, lumbar lordosis, scoliosis and pelvic obliquity flexibility or correction indices, postoperative coronal/sagittal balance, need of preoperative non-invasive ventilation, preoperative feeding disorders, development of surgical complications, and length of hospital and intensive care unit stay were exposure variables that did not affect postsurgical survival in patients with DMD. INTERPRETATION: Survival of up to two decades or more was possible among young patients with DMD after scoliosis correction. This was affected by factors that related to disease and deformity severity, as well as surgical morbidity. WHAT THIS PAPER ADDS: There was no operative or 30-day postoperative mortality in this group of patients with Duchenne muscular dystrophy (DMD) undergoing scoliosis correction. Survival probabilities at 5-year, 10-year, 15-year, and 20-year intervals post-surgery were 92%, 80%, 33%, and 12% respectively. Scoliosis surgery achieved good deformity correction and a balanced spine that was maintained at follow-up. Respiratory failure, severe pneumonia, and left ventricular failure were the leading causes of death in the study participants. Preoperative global coronal imbalance, pelvic obliquity, and intraoperative blood loss significantly predicted survival. Factors that affected survival after scoliosis surgery were associated with perioperative morbidity and disease or deformity severity in the DMD group.

Cyanotic Congenital Cardiac Disease and Scoliosis: Pre-Operative Assessment, Surgical Treatment, and Outcomes.

OBJECTIVE: Congenital heart disease (CHD) is associated with the development of scoliosis. Improvements in cardiac care have extended survival of children with cyanotic CHD which possess a need for correction of scoliosis. There is limited information on spinal care for these patients. We present 3 patients with CHD who underwent surgical correction of scoliosis. MATERIALS AND METHODS: We reviewed demographic and clinical data on patients with cyanotic CHD. RESULTS: Patient 1 underwent posterior spinal fusion T3-L3 at the age of 16 years. He had a double inlet left ventricle and was treated with completion of a Fontan circulation. Hypotensive anaesthesia was used but he lost 3,000 mL of blood. The operative time was 370 min and most of the blood loss occurred in the second half of the procedure. Patient 2 underwent posterior spinal fusion T5-T12 when aged 14 years. She had transposition of the great vessels corrected over multiple surgeries. Hypotensive anaesthesia was used, she had blood loss of 300 mL, and the surgical time was 282 min. Patient 3 underwent posterior spinal fusion extending from T5-T12 when he was 17 years old. He had a double inlet left ventricle and was treated with completion of a Fontan circulation. Hypotensive anaesthesia was used, he had blood loss of 1,021 mL, and a surgical time of 342 min. CONCLUSION: Scoliosis surgery in patients with complex cardiac disease may be indicated to treat progressive deformities which produce severe symptoms. A multidisciplinary approach including a spinal surgeon as well as a cardiologist, haematologist, respiratory paediatrician, and spinal anaesthetist can evaluate the general medical condition and weigh the benefits and risks of surgery. Deformity correction can be performed using a meticulous technique and has produced a series of satisfactory outcomes.

Multimodal Intraoperative Spinal Cord Monitoring during Spinal Deformity Surgery: Efficacy, Diagnostic Characteristics, and Algorithm Development.

OBJECTIVE: This study aims to present the diagnostic characteristics of multimodal intraoperative monitoring (IOM) in spinal deformity surgery and to define and categorise the neuromonitoring events, as well as propose an algorithm of action. MATERIALS AND METHODS: We reviewed 1,155 consecutive patients (807 female, 348 male) who underwent deformity correction using standardised perioperative care, cortical/cervical somatosensory evoked potentials (SSEPs), and upper/lower limb transcranial electrical motor evoked potential (MEPs) by a single surgeon. The mean age at surgery was 13.8 years (range 10-23.3). We categorised IOM events as true, transient true, and false positive or negative. Diagnostic performance criteria were calculated. RESULTS: The most common diagnosis was adolescent idiopathic scoliosis in 717 (62%) patients. We identified 3 true positive monitoring events occurring in 2 patients (0.17%), 8 transient true positive (0.69%), and 8 transient false positive events (0.69%). There were no false negative events and no patient had postoperative neurological complications. The multimodal IOM technique had a sensitivity of 100%, specificity of 99.3%, positive predictive value of 55.6%, and negative predictive value of 100%. Sensitivity was 100% for MEPs and multimodal monitoring compared to 20% for cortical or cervical SSEPs. The frequency of true or transient true positive events was higher (p = 0.07) in Scheuermann's kyphosis (3/91 patients, 3.3%) compared to adolescent idiopathic scoliosis (6/717 patients, 0.84%). CONCLUSION: Multimodal IOM is highly sensitive and specific for spinal cord injury. This technique is reliable for the assessment of the condition of the spinal cord during major deformity surgery. We propose an algorithm of intraoperative action to allow close cooperation between the surgical, anaesthetic, and neurophysiology teams and to prevent neurological deficits.

Transcranial magnetic stimulation in the semi-quantitative, pre-operative assessment of patients undergoing spinal deformity surgery.

PURPOSE: To compare measurements of motor evoked potential latency stimulated either magnetically (mMEP) or electrically (eMEP) and central motor conduction time (CMCT) made pre-operatively in conscious patients using transcranial and intra-operatively using electrical cortical stimulation before and after successful instrumentation for the treatment of adolescent idiopathic scoliosis. METHODS: A group initially of 51 patients with adolescent idiopathic scoliosis aged 12-19 years was evaluated pre-operatively in the outpatients' department with transcranial magnetic stimulation. The neurophysiological data were then compared statistically with intra-operative responses elicited by transcranial electrical stimulation both before and after successful surgical intervention. MEPs were measured as the cortically evoked compound action potentials of Abductor hallucis. Minimum F-waves were measured using conventional nerve conduction methods and the lower motor neuron conduction time was calculated and this was subtracted from MEP latency to give CMCT. RESULTS: Pre-operative testing was well tolerated in our paediatric/adolescent patients. No neurological injury occurred in any patient in this series. There was no significant difference in the values of mMEP and eMEP latencies seen pre-operatively in conscious patients and intra-operatively in patients under anaesthetic. The calculated quantities mCMCT and eCMCT showed the same statistical correlations as the quantities mMEP and eMEP latency. CONCLUSIONS: The congruency of mMEP and eMEP and of mCMCT and eCMCT suggests that these measurements may be used comparatively and semi-quantitatively for the comparison of pre-, intra-, and post-operative spinal cord function in spinal deformity surgery.

Management of a periaortic spinal pedicle screw in a patient with adult presentation thoracic scoliosis.

Introduction: Vascular injury due to direct contact with malpositioned pedicle screws is perhaps the most devastating complication associated with screw instrumentation. While frank perforation of major vessels warrants urgent open or endovascular repair, the management of pedicle screws in close vicinity of large vessels is unclear. We present a systematic multi-disciplinary approach to manage a patient with a periaortic pedicle screw. Case Report: A 20-year-old female underwent posterior scoliosis correction and spinal fusion for an adult thoracic scoliosis with spinal instrumentation extending from T3-T12. In the immediate post-operative period, an episode of acute desaturation prompted a computed tomography pulmonary angiography (CTPA) to rule out pulmonary embolism. While CTPA demonstrated an enlarged right side of the heart (corresponding to a large atrial septal defect as evident on 2D-ECHO), an incidental lateral breech at the left T10 pedicle screw level was also identified with the screw tip in close proximity to the aorta. At this time, cardiac management was prioritized and a successful atrial septal defect repair procedure was performed. After recovery from the cardiac procedure, a plan was formulated with a multidisciplinary team to remove the pedicle screw. To determine the exact position of the screw tip in relation to the aorta, an angiogram and an intravascular ultrasound were done which showed no evidence of intra-luminal extension of the screw tip. In addition, a transesophageal ultrasound was performed and this ruled out any extra-luminal contact with the aorta. Subsequently, the spinal instrumentation was removed safely with the vascular surgeon and interventional radiologist being on standby. Conclusion: The authors suggest the removal of spinal pedicle screws in proximity to major vessels and describe an algorithm for this. Thorough pre-operative planning and detailed vascular imaging, as well as a multidisciplinary approach resulting in a clear surgical plan are critical to allow safe removal of such implants.

Minimally Invasive Surgery for Adolescent Idiopathic Scoliosis: A Systematic Review.

Background: Minimally invasive surgical (MIS) techniques have gained popularity as a safe and effective alternative to open surgery for degenerative, traumatic, and metastatic spinal pathologies. In adolescent idiopathic scoliosis, MIS techniques comprise anterior thoracoscopic surgery (ATS), posterior minimally invasive surgery (PMIS), and vertebral body tethering (VBT). In the current systematic review, the authors collected and analyzed data from the available literature on MIS techniques in AIS. Methods: The articles were shortlisted after a thorough electronic and manual database search through PubMed, EMBASE, and Google Scholar. Results: The authors included 43 studies for the review; 14 described the outcomes with ATS, 13 with PMIS, and 16 with VBT. Conclusions: While the efficacy of the ATS approach is well-established in terms of comparable coronal and sagittal correction to posterior spinal fusion, the current use of ATS for instrumented fusion has become less popular due to a steep learning curve, high pulmonary and vascular complication rates, implant failures, and increased non-union rates. PMIS is an effective alternative to the standard open posterior spinal fusion, with a steep learning curve and longer surgical time being potential disadvantages. The current evidence, albeit limited, suggests that VBT is an attractive procedure that merits consideration in terms of radiological correction and clinical outcomes, but it has a high complication and re-operation rate, while the most appropriate indications and long-term outcomes of this technique remain unclear.

Management Principles in a Case of True-positive Loss of Neuromonitoring Signals during Scoliosis Surgery: A Case Report.

Introduction: Multimodal intraoperative neuromonitoring (IOM) is essential in scoliosis surgery. This is affected by misplaced instrumentation, cord trauma, hemodynamic instability, and anesthesia. We present an irreversible loss of IOM without identifiable intra-operative cause to highlight its occurrence and discuss post-operative investigations and management. Case Report: A 14-year-old girl with adolescent idiopathic scoliosis, no co-morbidities, and normal spinal magnetic resonance imaging (MRI) underwent posterior spinal fusion. During screw placement, bilateral motor evoked potentials (MEPs) and right somatosensory evoked potentials (SSEPs) were lost in the legs. All screws were removed with no evidence of cortical breach. Left leg responses gradually improved, but there was no recovery of right leg SSEPs or MEPs. Subsequently, the procedure was abandoned. The patient had reduced right leg strength (3/5) and sensation with the left leg was normal. Immediate post-operative spinal MRI identified no abnormality. Computed tomography (CT) showed no cortical breach with satisfactory pedicle screw tracts. Repeat MRI (day 7) showed high T2-signal within the cord at T11 indicating ischemia. Gradual neurological recovery occurred and on day 15, repeat neurophysiology found reproducible SSEPs and MagStim MEPs. The patient underwent revision posterior fusion with single rod correction without complication and IOM was maintained. By day 24, the patient had 5/5 power and normal sensation in both legs. Good scoliosis correction was achieved and maintained at 3-year follow-up. Conclusion: This patient represents a vascular event affecting the lower spinal cord and highlights the role of sequential imaging and pre-operative neurophysiology including MagStim in deciding when to proceed with revision surgery while reducing risk using conservative techniques.

Long-Term Health-Related Quality of Life (QOL) after Paediatric Spinal Deformity Surgery and Comparison with the General Population.

QOL questionnaires assess patients' perception on surgical outcomes. We reviewed 1354 patients with spinal deformity. Four hundred and twenty-eight patients had >10 years of follow-up. The SRS-22r questionnaire was completed before surgery, at 6/12/24 months, 5-10 years and >10 years postoperatively. Patients with >10 years of follow-up completed the EQ-5D VAS/index and the VAS for back/leg pain. We used QOL data reporting in the general population of 20-29 and 30-39 years of age to compare against our patient cohort. Among the patients, 993 had AIS, 80 congenital scoliosis, 102 syndromic or secondary scoliosis, 105 Scheuermann kyphosis and 40 low-grade and 34 high-grade spondylolisthesis. SRS-22r total and domain scores improved from preoperative to follow-up in all diagnosis categories. At >10 years after surgery, patients with congenital scoliosis and Scheuermann kyphosis had better SRS-22r total/domain and EQ-5D (index/VAS) scores along with lower VAS back/leg pain scores compared to the other groups. Patients with congenital scoliosis and Scheuermann kyphosis had comparable SRS-22r total/domain, EQ-5D (index/VAS) and VAS back/leg pain scores to the general population in the 20-29 year category and better scores than the 30-39 year group. Patients with AIS, syndromic/secondary scoliosis and low/high-grade spondylolisthesis had reduced SRS-22r total/domain and EQ-5D (index/VAS) scores and higher VAS back/leg pain scores compared to the 20-29 year group but comparable scores to the 30-39 year group. Patients with spinal deformity reported improved QOL and high satisfaction after surgery which was maintained at >10 years of follow-up. Patients with congenital scoliosis and Scheuermann kyphosis had better QOL outcomes (comparable to the general population of similar age) as opposed to other types of scoliosis or lumbosacral spondylolisthesis.

Surgical and long-term functional outcomes of patients with Duchenne muscular dystrophy following spinal deformity correction.

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. Spino-pelvic 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°, pelvic obliquity 16.4°, thoracic kyphosis 55.8°, lumbar lordosis 11.1°, 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.

Effectiveness of the Boston Brace in the Treatment of Paediatric Scoliosis: A Longitudinal Study from 2010-2020 in a National Spinal Centre.

Bracing can reduce curve progression in order to prevent or delay scoliosis surgery in growing children. Brace treatment is effective in adolescent idiopathic scoliosis (AIS), but there is less evidence of its efficacy in early-onset or non-idiopathic scoliosis. We assessed the outcome of bracing at the end point of treatment, including the patients' perception of clinical results. We reviewed 480 patients treated using Boston brace from 2010-2020 (70% female); 249 patients completed bracing (52%) and 118 patients (47.4%) did not require surgery, with 83% having idiopathic scoliosis. Brace success was considered scoliosis below 50° at the end of bracing, with the patient skeletally mature. A total of 131 patients required scoliosis surgery after bracing (64% had idiopathic scoliosis; adolescents 57% and juveniles 43%). All patients had a minimum two-year follow-up after bracing or after scoliosis correction, with the quality of life assessment questionnaires. A total of 98 out of 182 patients with idiopathic scoliosis did not require surgery (54%). Thoracic scoliosis improved with bracing by a mean of 3.4° and thoracolumbar/lumbar scoliosis by a mean of 6.8°. A total of 85 patients with AIS (64%) but only 9 patients with JIS (20%) did not need surgery. In the AIS group, 97 patients had scoliosis of 20-40°; 71 of these patients (73.2%) did not require scoliosis correction at the end of bracing. In total, 84 patients with idiopathic scoliosis had surgery at a mean of 14 years (surgery was delayed by a mean of 3.2 years). In total, 20 of 67 patients with non-idiopathic scoliosis did not need surgery (30%). Thoracic scoliosis improved with bracing by a mean of 8.4° and thoracolumbar/lumbar scoliosis by a mean of 0.8°. A total of 47 patients with non-idiopathic scoliosis required surgery at a mean of 13.1 years (surgery was delayed by a mean of 5.2 years). Multivariate regression analysis showed that idiopathic scoliosis, AIS, closed triradiate cartilage, post-menarche status, higher Risser grade and smaller scoliosis angle at initial presentation predicted brace success. Patients reported good function and self-image, reduced pain and high satisfaction after treatment in both the bracing-only and the bracing followed by surgery groups.

Surgical correction of spinal deformity in patients with cerebral palsy using pedicle screw instrumentation.

STUDY DESIGN: Retrospective review of a prospectively collected single surgeon's series. OBJECTIVE: To investigate the efficacy of pedicle screw instrumentation in correcting spinal deformity in patients with quadriplegic cerebral palsy. In addition to assess quality-of-life and functional improvement after deformity correction as perceived by the parents of our patients. SUMMARY OF BACKGROUND DATA: All pedicle screw constructs have been commonly used to correct adolescent idiopathic scoliosis. There is limited information on their effectiveness in treating patients with cerebral palsy and neuromuscular scoliosis. METHODS: We reviewed the medical records and serial radiographs of 45 consecutive patients with quadriplegia who underwent spinal arthrodesis using pedicle screw/rod instrumentation and a standardized surgical technique. All patients were wheelchair bound with collapsing thoracolumbar scoliosis and pelvic obliquity. Twenty-eight patients had associated sagittal deformities. A telephone survey was performed by an independent investigator to assess parents' perception on surgical outcome. RESULTS: Thirty-eight patients underwent posterior-only and 7 staged anteroposterior spinal arthrodesis. Mean age at surgery was 13.4 years (range: 9 to 18.3 y) and mean postoperative follow-up was 3.5 years (range: 2.8 to 5 y). Pedicle screw instrumentation extended from T2/T3 to L5 with bilateral pelvic fixation using iliac bolts. Scoliosis was corrected from mean 82.5 to 21.4 degree (74.1%). Pelvic obliquity was corrected from mean 24 to 4 degree (83.3%). In posterior-only procedures, average blood loss was 0.8 blood volumes, intensive care unit stay 3.5 days, and hospital stay 17.6 days. In anteroposterior procedures, average blood loss was 0.9 blood volumes, intensive care unit stay 8.9 days, and hospital stay 27.4 days. Major complications included 1 deep infection and 1 reoperation to remove prominent implants but no deaths, no neurological deficit, and no detected pseudarthrosis. Parents' survey showed 100% satisfaction rate. CONCLUSIONS: Pedicle screw instrumentation can achieve excellent correction of spinal deformity in quadriplegic cerebral palsy with low complication and re-operation rates and high parent satisfaction.

Paediatric Spinal Deformity Surgery: Complications and Their Management.

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.

All Pedicle Screw versus Hybrid Hook-Screw Instrumentation in the Treatment of Thoracic Adolescent Idiopathic Scoliosis (AIS): A Prospective Comparative Cohort Study.

Background: Posterior spinal correction and fusion remains the most common surgical treatment in AIS. Surgeons currently favour all pedicle screw (AS) correction techniques with alternative implants being less utilised. The purpose of this study was to assess whether a hybrid hook−screw (HS) construct could achieve similar outcomes. Methods: A single centre, prospective cohort study was conducted. Patients with moderate and severe thoracic AIS (Lenke 1) were included. Clinical and radiological results of a standardised hybrid HS technique were compared with those obtained with an AS construct. All patients had a minimum 2-year follow-up. Results: 160 patients were included in this series (80 patients/group). The HS group had significantly reduced surgical time, blood loss and implant density. Both techniques achieved ≥75% scoliosis correction. The HS group was superior in restoring thoracic kyphosis and global sagittal balance with an average 31% increase in kyphosis compared to 10% with the AS group (p < 0.001). There was significant improvement in SRS-22 scores at 2 years postoperative (p < 0.001) in both groups. There were no neurological or visceral complications related to instrumentation, no detected non-union and no reoperations. The HS implant cost was significantly lower than that of AS, with a mean instrumentation saving of almost £2000/patient. Conclusion: A standardised hybrid HS technique achieved excellent correction of thoracic scoliosis, high patient satisfaction and low complication rates in patients with thoracic AIS. These results were comparable to the AS group. The HS technique achieved better correction of thoracic kyphosis and sagittal balance than the AS technique, together with reduced surgical time, blood loss and implant cost.

Population-based normative data for the Scoliosis Research Society 22r, EQ-5D, and VAS questionnaires among individuals aged 20 to 69 years.

AIMS: To provide normative data that can assess spinal-related disability and the prevalence of back or leg pain among adults with no spinal conditions in the UK using validated questionnaires. METHODS: A total of 1,000 participants with equal sex distribution were included and categorized in five age groups: 20 to 29, 30 to 39, 40 to 49, 50 to 59, and 60 to 69 years. Individuals with spinal pathologies were excluded. Participants completed the Scoliosis Research Society-22 (SRS-22r), visual analogue scale (VAS) for back/leg pain, and the EuroQol five-dimension index (EQ-5D/VAS) questionnaires, and disclosed their age, sex, and occupation. They were also categorized in five professional groups: doctors, nurses, allied health professionals, office workers, and manual workers. RESULTS: The mean age of all participants was 43.8 years (20 to 69). There was no difference in the SRS-22r, EQ-5D, or VAS scores among male and female participants (p > 0.05). There was incremental decrease in SRS-22r total scores as the age increased. The mean EQ-5D index score (0.84) ranged little across the age groups (0.72 to 0.91) but reduced gradually with increasing age. There was difference between the SRS-22r total score (4.51), the individual domain scores, and the EQ-5D score (index: 0.94 and VAS: 89) for the doctors' group compared to all other occupational categories (p < 0.001). Doctors had a younger mean age of participants, which may explain their improved spinal health. There was no difference in the total or sub-domain SRS-22r and EQ-5D scores between the other four occupational groups. CONCLUSION: This study provides the first normative data for the SRS-22r, EQ-5D, and VAS for back/leg pain questionnaires among adults in the UK. We recorded an excellent correlation between the three assessment tools with individuals who reported less back and leg pain having better quality of life and greater function. The participants' age, rather than their sex or profession, appears to be the major determinant for spinal health and quality of life. Cite this article: Bone Jt Open 2022;3(2):130-134.

Current concepts in the treatment of neuromuscular scoliosis: clinical assessment, treatment options, and surgical outcomes.

The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients' quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction.

Adolescent idiopathic scoliosis : a review of aetiological theories of a multifactorial disease.

Adolescent idiopathic scoliosis (AIS), defined by an age at presentation of 11 to 18 years, has a prevalence of 0.47% and accounts for approximately 90% of all cases of idiopathic scoliosis. Despite decades of research, the exact aetiology of AIS remains unknown. It is becoming evident that it is the result of a complex interplay of genetic, internal, and environmental factors. It has been hypothesized that genetic variants act as the initial trigger that allow epigenetic factors to propagate AIS, which could also explain the wide phenotypic variation in the presentation of the disorder. A better understanding of the underlying aetiological mechanisms could help to establish the diagnosis earlier and allow a more accurate prediction of deformity progression. This, in turn, would prompt imaging and therapeutic intervention at the appropriate time, thereby achieving the best clinical outcome for this group of patients. Cite this article: Bone Joint J 2022;104-B(8):915-921.

The impact of the COVID-19 pandemic on spinal surgery.

The impact of the coronavirus disease (COVID-19) pandemic on healthcare services around the world has been unprecedented. Surgical specialities, in particular, had to respond rapidly and adapt to continue to meet the needs of their patients in this ever-evolving and uncertain situation. With the cancellation of elective surgery and outpatient clinics, the majority of work carried out by spinal surgeons was obliterated. It was imperative emergency spinal care continued throughout the pandemic, with the creation of guidelines to assist spinal surgeons manage patients appropriately. Alongside assisting on the frontline, spinal surgeons had to triage referrals to ensure urgent care was still provided, undertake outpatient clinics virtually and ensure educational opportunities were available for colleagues. Paediatric spinal surgery was affected by the pandemic; although the virus did not significantly impact children to the same extent, a notable consequence of the lockdown restrictions was a reduction in GP referrals into the specialist service. In the event of any future pandemics, national spinal organizations have created guidelines to assist in the prioritization and care of spinal pathologies. While a 'return to normality' is a long way off, the impact of the past year will undoubtedly change spinal surgeons and their practice forever.

The surgical treatment of spinal deformity in children with non-ambulatory myelomeningocele.

AIMS: To report the outcome of spinal deformity correction through anterior spinal fusion in wheelchair-bound patients with myelomeningocele. METHODS: We reviewed 12 consecutive patients (7M:5F; mean age 12.4 years (9.2 to 16.8)) including demographic details, spinopelvic parameters, surgical correction, and perioperative data. We assessed the impact of surgery on patient outcomes using the Spina Bifida Spine Questionnaire and a qualitative questionnaire. RESULTS: The mean follow-up was 5.4 years (2 to 14.9). Nine patients had kyphoscoliosis, two lordoscoliosis, and one kyphosis. All patients had a thoracolumbar deformity. Mean scoliosis corrected from 89.6° (47° to 151°) to 46.5° (17° to 85°; p < 0.001). Mean kyphosis corrected from 79.5° (40° to 135°) to 49° (36° to 65°; p < 0.001). Mean pelvic obliquity corrected from 19.5° (8° to 46°) to 9.8° (0° to 20°; p < 0.001). Coronal and sagittal balance restored to normal. Complication rate was 58.3% (seven patients) with no neurological deficits, implant failure, or revision surgery. The degree of preoperative spinal deformity, especially kyphosis and lordosis, correlated with increased blood loss and prolonged hospital/intensive care unit stay. The patients reported improvement in function, physical appearance, and pain after surgery. The parents reported decrease in need for everyday care. CONCLUSION: Anterior spinal fusion achieved satisfactory deformity correction with high perioperative complication rates, but no long-term sequelae among children with high level myelomeningocele. This resulted in physical and functional improvement and high reported satisfaction. Cite this article: Bone Joint J 2021;103-B(6):1133-1141.

The surgical treatment of severe Scheuermann's kyphosis.

AIMS: To report the surgical outcome of patients with severe Scheuermann's kyphosis treated using a consistent technique and perioperative management. METHODS: We reviewed 88 consecutive patients with a severe Scheuermann's kyphosis who had undergone posterior spinal fusion with closing wedge osteotomies and hybrid instrumentation. There were 55 males and 33 females with a mean age of 15.9 years (12.0 to 24.7) at the time of surgery. We recorded their demographics, spinopelvic parameters, surgical correction, and perioperative data, and assessed the impact of surgical complications on outcome using the Scoliosis Research Society (SRS)-22 questionnaire. RESULTS: The mean follow-up was 8.4 years (2 to 14.9). There were 85 patients (96.6%) with a thoracic deformity. Posterior spinal fusion with closing-wedge osteotomies and hybrid instrumentation was used in 86 patients; two patients underwent combined anterior and posterior spinal fusion. The mean kyphosis was corrected from 94.5° to 47.5° (p < 0.001). Coronal and sagittal balance returned to normal. The rate of complications was 12.5%: there were no neurological deficits, implant failure, or revision surgery. SRS-22 scores improved from a mean 3.6 (1.3 to 4.1) to 4.6 (4.2 to 5.0) at two years (p < 0.001) with a high rate of patient satisfaction. Non-smokers and patients with lower preoperative SRS-22 scores showed greater improvement in their quality of life. Sagittal pelvic balance did not change after correction of the kyphosis and correlated with lumbar lordosis but not with thoracic or thoracolumbar kyphosis. CONCLUSION: Posterior spinal fusion using hybrid instrumentation, closing-wedge osteotomies, and iliac bone grafting achieves satisfactory correction of a severe kyphosis resulting in improvements in physical and mental health and a high degree of patient-reported satisfaction. Cite this article: Bone Joint J 2021;103-B(1):148-156.

Health-related quality of life and sagittal balance at two to 25 years after posterior transfixation for high-grade dysplastic spondylolisthesis.

AIMS: High-grade dysplastic spondylolisthesis is a disabling disorder for which many different operative techniques have been described. The aim of this study is to evaluate Scoliosis Research Society 22-item (SRS-22r) scores, global balance, and regional spino-pelvic alignment from two to 25 years after surgery for high-grade dysplastic spondylolisthesis using an all-posterior partial reduction, transfixation technique. METHODS: SRS-22r and full-spine lateral radiographs were collected for the 28 young patients (age 13.4 years (SD 2.6) who underwent surgery for high-grade dysplastic spondylolisthesis in our centre (Scottish National Spinal Deformity Service) between 1995 and 2018. The mean follow-up was nine years (2 to 25), and one patient was lost to follow-up. The standard surgical technique was an all-posterior, partial reduction, and S1 to L5 transfixation screw technique without direct decompression. Parameters for segmental (slip percentage, Dubousset's lumbosacral angle) and regional alignment (pelvic tilt, sacral slope, L5 incidence, lumbar lordosis, and thoracic kyphosis) and global balance (T1 spino-pelvic inclination) were measured. SRS-22r scores were compared between patients with a balanced and unbalanced pelvis at final follow-up. RESULTS: SRS-22r domain and total scores improved significantly from preoperative to final follow-up, except for the mental health domain that remained the same. Slip percentage improved from 75% (SD 15) to 48% (SD 19) and lumbosacral angle from 70° (SD 11) to 101° (SD 11). Preoperatively, 35% had global imbalance, and at follow-up all were balanced. Preoperatively, 63% had an unbalanced pelvis, and at final follow-up this was 32%. SRS-22r scores were not different in patients with a balanced or unbalanced pelvis. However, postoperative pelvic imbalance as measured by L5 incidence was associated with lower SRS-22r self-image and total scores (p = 0.029). CONCLUSION: In young patients with HGDS, partial reduction and transfixation improves local lumbosacral alignment, restores pelvic, and global balance and provides satisfactory long-term clinical outcomes. Higher SRS-22r self-image and total scores were observed in the patients that had a balanced pelvis (L5I < 60°) at two to 25 years follow-up. Cite this article: Bone Jt Open 2021;2(3):163-173.