Mitochondrial-Targeted Metal-Phenolic Nanoparticles to Attenuate Intervertebral Disc Degeneration: Alleviating Oxidative Stress and Mitochondrial Dysfunction.

As intervertebral disc degeneration (IVDD) proceeds, the dysfunctional mitochondria disrupt the viability of nucleus pulposus cells, initiating the degradation of the extracellular matrix. To date, there is a lack of effective therapies targeting the mitochondria of nucleus pulposus cells. Here, we synthesized polygallic acid-manganese (PGA-Mn) nanoparticles via self-assembly polymerization of gallic acid in an aqueous medium and introduced a mitochondrial targeting peptide (TP04) onto the nanoparticles using a Schiff base linkage, resulting in PGA-Mn-TP04 nanoparticles. With a size smaller than 50 nm, PGA-Mn-TP04 possesses pH-buffering capacity, avoiding lysosomal confinement and selectively accumulating within mitochondria through electrostatic interactions. The rapid electron exchange between manganese ions and gallic acid enhances the redox capability of PGA-Mn-TP04, effectively reducing mitochondrial damage caused by mitochondrial reactive oxygen species. Moreover, PGA-Mn-TP04 restores mitochondrial function by facilitating the fusion of mitochondria and minimizing their fission, thereby sustaining the vitality of nucleus pulposus cells. In the rat IVDD model, PGA-Mn-TP04 maintained intervertebral disc height and nucleus pulposus tissue hydration. It offers a nonoperative treatment approach for IVDD and other skeletal muscle diseases resulting from mitochondrial dysfunction, presenting an alternative to traditional surgical interventions.

Long-term outcomes in sacral agenesis.

INTRODUCTION: Sacral agenesis (SA) includes a range of clinical presentations of varying severity, with implications for function and quality of life (QoL). Diagnosis is often made perinatally, and prognostic discussions become an important aspect of parental counselling. This study engaged SA sufferers and their caregivers to obtain objective, long-term patient reported outcome data. METHOD: Patients with radiologically confirmed SA from a single tertiary spinal unit underwent retrospective medical record review. Patients were then contacted by telephone to complete QoL questionnaires including EQ-ED-5L for adults and EQ-ED-Y for < 16-year-olds. Additional information including Renshaw grade, employment, living situation and bladder function was also collected. RESULTS: Twenty-six patients with SA were identified. Mean age is 23.35 years (range 0.92-63.53), 13 M:17F. Renshaw grade ranged from 1 to 4. Sixty-eight percent had associated kyphoscoliotic deformities. The majority (70%) had either impaired or absent bladder control, and 80% need walking aids to mobilise. Twenty patients completed the questionnaire (10 adults and 10 < 16-year-olds). Mean EQ-ED-5L index for adults was +0.474 (range -0.1 to +0.089, 1 = best), with a lower mean value of +0.287 (range -0.54 to +1) for the < 16-year cohort. Those undergoing spinal fusion procedures had significantly lower scores (-0.08 v +0.44, p = 0.022). CONCLUSION: This study provides an objective record of the QoL of individuals with SA, illustrating a wide variety of outcomes, with differences between younger and older individuals which may reflect the results of a long-term adaptive process. The implications for individuals should be carefully tailored to the specific deformity and the likely underlying neurological deficits.

Systematic Review and Meta-analysis: Does Anterior-Posterior Spinal Fusion Still have a Role in Severe Thoracic Adolescent Idiopathic Scoliosis?

Background: Debate exists as to whether anterior-posterior spinal fusion (APSF), rather than posterior-only spinal fusion (PSF), provides benefit for treating severe thoracic adolescent idiopathic scoliosis (AIS). This systematic review and meta-analysis compare (1) Cobb angle correction, (2) complication and reoperation rate, (3) pulmonary function, (4) number of fused segments, and 5) patient-reported outcome measures (PROMs) in both groups. Methods: Electronic databases were searched to identify studies that met the following inclusion criteria: comparative studies (level 3 or above), severe thoracic curves (≥ 70°), age ≤ 16, AIS aetiology, Lenke 1-4 curves and follow-up ≥ 1 year for ≥ 95% of patient population. Literature was graded for quality and bias using GRADE and MINORS criteria. Results: Eight studies were included, defined by GRADE as low or moderate level evidence. Three studies showed superior curve correction in the APSF group; however, the meta-analysis showed no significant difference in curve correction between groups (95% CI - 3.45-12.96, P = 0.26). There were more complications in the APSF group, without statistical significance (95% CI 0.53-3.39, P = 0.54; I 2 = 0%, P = 0.78). There were no re-operations in either group. Two studies reported pulmonary function; one showed better function in the APSF group, the other better function in the PSF group. One study showed fewer fused segments in the APSF group, however, no significance was observed in the meta-analysis (95%CI - 1.65-0.31, P = 0.18). Three studies reported PROMs with no differences reported between groups. Conclusions: APSF and PSF have been found to have comparable results. The present evidence cannot support recommendations for guidelines on future practice with regards to effect on curve correction, complications, re-operations, pulmonary function or PROMs. Level of evidence: Level III, Systematic review of Level-III studies.

Can a culture of team psychological safety and MDT proforma improve team performance and patient outcomes in spinal MDTs?

PURPOSE: Spinal multidisciplinary teams (MDTs) are now standard of care for complex patient management in tertiary spinal units. This study investigates whether a scheduling proforma, and cultural change to the team that promotes psychological safety, can improve spinal MDT effectiveness for team members and patients. METHODS: Retrospective cohort study including 165 spinal MDT patients before and after intervention. The intervention was use of a scheduling proforma and team learning to promote a culture of psychological safety. Data on accident and emergency (A&E) attendances, unplanned emergency admissions and post-operative 30-day readmissions were collected. At the team level, data were collected from 16 MDT participants using the MDT Observational Assessment Rating Scale (MDT-OARS), which measures MDT effectiveness. RESULTS: Pre-intervention MDT-OARS was 28. Analysis of 80 patients demonstrated there were six A&E attendances, three unplanned emergency admissions and four post-operative 30-day re-admissions. Post-intervention MDT-OARS was 38 (p < 0.05). Analysis of 85 patients demonstrated there were three A&E attendances, one unplanned emergency admission and one post-operative re-admission. CONCLUSIONS: Team culture that promotes psychological safety, along with use of a scheduling proforma, can improve MDT effectiveness for participants in spinal MDTs.

Does Coronavirus Disease 2019 (COVID-19) Affect Perioperative Morbidity and Mortality for Patients Requiring Emergency Instrumented Spinal Surgery? A Single-Center Cohort Study.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic sent shockwaves through health services worldwide. Resources were reallocated. Patients with COVID-19 still required instrumented spinal surgery for emergencies. Clinical outcomes for these patients are not known. The objective of this study was to evaluate the effects of COVID-19 on perioperative morbidity and mortality for patients undergoing emergency instrumented spinal surgery and to determine risk factors for increased morbidity/mortality. METHODS: This retrospective cohort study included 11 patients who were negative for COVID-19 and 8 patients who were positive for COVID-19 who underwent emergency instrumented spinal surgery in 1 hospital in the United Kingdom during the pandemic peak. Data collection was performed through case note review. Patients in both treatment groups were comparable for age, sex, body mass index (BMI), comorbidities, surgical indication, and preoperative neurologic status. Predefined perioperative outcomes were recorded within a 30-day postoperative period. Univariable analysis was used to identify risk factors for increased morbidity. RESULTS: There were no mortalities in either treatment group. Four patients positive for COVID-19 (50%) developed a complication compared with 6 (55%) in the COVID-19-negative group (P > 0.05). The commonest complication in both groups was respiratory infection. Three patients positive for COVID-19 (37.5%) required intensive care unit admission, compared with 4 (36%) in the COVID-19-negative group (P > 0.05). The average time between surgery and discharge was 19 and 10 days in COVID-19-positive and -negative groups, respectively (P = 0.02). In the COVID-19 positive group, smoking, abnormal BMI, preoperative oxygen requirement, presence of fever, and oxygen saturations <95% correlated with increased risk of complications. CONCLUSIONS: Emergency instrumented spinal surgery in patients positive for COVID-19 was associated with increased length of hospital stay. There was no difference in occurrence of complications or intensive care unit admission. Risk factors for increased morbidity in patients with COVID-19 included smoking, abnormal BMI, preoperative oxygen requirement, fever and saturations <95%.

Spinal clearance and management of spinal cord injury in the trauma patient.

The British Orthopaedic Association's Standards for Trauma and Orthopaedics outline the essential clinical standards for spinal clearance and management of spinal cord injury in the acute trauma patient. From initial presentation in the hospital setting to long-term rehabilitation, the recommendations for clinical assessment, imaging, treatment priorities and the role of trauma networks are summarised.

A Hemodynamic Safety Checklist Can Improve Blood Pressure Monitoring in Patients with Acute Spinal Cord Injury.

OBJECTIVE: The American Association and Congress of Neurological Surgeons recommended mean arterial blood pressure (MAP) in patients with acute spinal cord injury (SCI) should be 85-90 mm Hg for the first 7 days. We evaluated whether hemodynamic management differed between a primary-receiving and tertiary hospital in the first 24 hours for patients with acute SCI and assessed whether use of a checklist could improve hemodynamic management. METHODS: Observational review was performed of 79 patients with acute SCI before and after introduction of a blood pressure monitoring checklist and staff educational program designed to improve tertiary center management. Hemodynamic management in the primary-receiving hospital was compared with the tertiary center before and after checklist introduction. RESULTS: At the primary-receiving center, mean number of documented MAP readings/hour was 2.2 and 3 before and after checklist introduction. The proportion having >50% of MAP recordings <80 mm Hg was 26% and 22%. The proportion having >50% of MAP recordings <70 mm Hg was 8.5% and 7%. At the tertiary center, mean number of MAP readings/hour was 1.3 and 2.7 before and after checklist introduction (P = 0.02). The proportion having >50% of MAP recordings <80 mm Hg decreased from 36.5% to 16% after checklist introduction (P = 0.05). The proportion having >50% of MAP recordings <70 mm Hg decreased from 9% to 5.5% (P = 0.6). Polytrauma, inotrope use, and head injury significantly correlated with low MAP recordings (P < 0.05). Polytrauma was an independent risk predictor for low MAP recordings (P < 0.05). CONCLUSIONS: Achieving MAP targets for patients with acute SCI is challenging. Checklist use and staff education were associated with improved hemodynamic management. Presence of polytrauma identified patients at particular risk.

Results of Early and Late Surgical Decompression and Stabilization for Acute Traumatic Cervical Spinal Cord Injury in Patients with Concomitant Chest Injuries.

BACKGROUND: The benefits of early surgical decompression and stabilisation (within 24 hours of injury) for patients with acute traumatic spinal cord injury (SCI) is unclear. The study objective was to investigate the effects of early (<24 hours of injury) versus late (>24 hours of injury) decompressive and stabilisation surgery for traumatic cervical SCI in patients with concomitant chest injuries. METHODS: This was a retrospective study including adults aged 16 years or over with traumatic cervical SCI, Glasgow Coma Scale score >13, and concomitant chest injuries (e.g. hemopneumothoraces, flail chest and pulmonary contusions) necessitating intensive care unit (ICU) admission. Forty patients who met the inclusion criteria and underwent decompressive surgery within 24 hours were compared with 55 patients who underwent decompressive surgery after 24 hours. Primary outcomes were ordinal change in the ASIA Impairment Scale (AIS) at 6 months and duration of ICU stay. Secondary outcomes included complications occurring within 30 days. RESULTS: In the early surgery group, 21 patients (52.5%) showed no improvement in ASIA grade, 13 (32.5%) had a 1-grade improvement, and 6 (15%) had a 2-grade improvement. The median length of ICU stay was 14 days (range, 2-68). 42.5% of patients developed a complication and 45% required a tracheostomy. In the late surgery group, AIS grade improvement was as follows: 32 (58%) no improvement, 19 (34.5%) had a 1 grade improvement, 3 (5.5%) had a 2 grade improvement and 1 (2%) had a 3 grade improvement. Mean ICU stay was 23 days (4-68). 53% of patients developed a complication and 55% required a tracheostomy. There was one mortality in the late surgery group. CONCLUSIONS: For patients with acute traumatic cervical SCI and concomitant chest trauma, early surgical decompression and stabilisation was associated with reduced ICU stay and a lower complication rate. Neurological recovery was more likely in younger patients and those with an incomplete SCI.

A Review of the Surgical Management of Extrathoracic Solitary Fibrous Tumors.

OBJECTIVES: Extrathoracic solitary fibrous tumors (ESFTs) are rare low-to-intermediate grade spindle-cell neoplasms of pluripotent fibroblastic or myofibroblastic origin. This review explores prognostic factors in the management of ESFTs and provides guidance on optimal treatment regimens based on the current literature. PATIENTS AND METHODS: Electronic searches were performed using MEDLINE, Embase, and the Cochrane library to identify studies on prognostic factors in the management of ESFTs published between January 1970 and June 2016. The literature search and review process identified 100 articles that were included in this review article. This included both surgical and nonsurgical studies on the management of ESFTs. RESULTS: Surgical excision with wide resection margins forms the mainstay of treatment and provides optimal long-term oncological outcomes. Large tumor size (>5 to 10 cm diameter), inadequate resection margins, malignant histologic features, dedifferentiation, and tumor location within the abdomen/pelvis are associated with adverse oncological outcomes. Radiotherapy may be used for preoperative tumor shrinkage and/or as adjuvant therapy in patients with malignant disease or incomplete surgical margins. Chemotherapy with molecular-targeted therapies has produced promising results and the results of further phase 2 trials are awaited. CONCLUSIONS: Routine long-term follow-up is essential for benign and malignant disease to enable early detection and treatment of recurrent disease.

Anterior lumbar discectomy and fusion for acute cauda equina syndrome caused by recurrent disc prolapse: report of 3 cases.

There is a lack of information and consensus regarding the optimal treatment for recurrent disc herniation previously treated by posterior discectomy, and no reports have described an anterior approach for recurrent disc herniation causing cauda equina syndrome (CES). Revision posterior decompression, irrespective of the presence of CES, has been reported to be associated with significantly higher rates of dural tears, hematomas, and iatrogenic nerve root damage. The authors describe treatment and outcomes in 3 consecutive cases of patients who underwent anterior lumbar discectomy and fusion (ALDF) for CES caused by recurrent disc herniations that had been previously treated with posterior discectomy. All 3 patients were operated on within 12 hours of presentation and were treated with an anterior retroperitoneal lumbar approach. Follow-up ranged from 12 to 24 months. Complete retrieval of herniated disc material was achieved without encountering significant epidural scar tissue in all 3 cases. No perioperative infection or neurological injury occurred, and all 3 patients had neurological recovery with restoration of bladder and bowel function and improvement in back and leg pain. ALDF is one option to treat CES caused by recurrent lumbar disc prolapse previously treated with posterior discectomy. The main advantage is that it avoids dissection around epidural scar tissue, but the procedure is associated with other risks and further evaluation of its safety in larger series is required.

The 2016 Austrian-Swiss-German Fellowship.

The Austrian-Swiss-German (ASG) Traveling Fellowship, which began in 1979, is an annual exchange of surgeons between the German-speaking countries of Austria, Switzerland, and Germany and the English-speaking countries of the United States, England, and Canada. In 2016, 4 fellows were chosen to participate in the fellowship, including Eric Edmonds from the University of California, San Diego; Simon Mears from the University of Arkansas for Medical Sciences; Mathew Sewell from the James Cook University in Middlesbrough, England; and Andrea Veljkovic from the University of British Columbia in Vancouver.

Do Growing Rods for Idiopathic Early Onset Scoliosis Improve Activity and Participation for Children?

OBJECTIVE: To investigate whether growing rod surgery for children with progressive idiopathic early onset scoliosis (EOS) effects activity and participation, and investigate factors that may affect this. STUDY DESIGN: Multicenter retrospective cohort study using prospectively collected data on 60 children with idiopathic EOS and significant scoliosis (defined as a Cobb angle >40°). Thirty underwent brace treatment, and 30, growth rod surgery. Questionnaire and radiographic data were recorded at 1 year. The validated Activities Scale for Kids performance version (ASKp) questionnaire was used to measure activity and participation. RESULTS: In the brace group, Cobb angle increased from 60° to 68°. There was no change in ASKp score. In the operative group, Cobb angle decreased from 67° to 45°. ASKp decreased from 91 to 88 (P < .01). Presence of spinal pain correlated with greater reduction in activity and participation scores in both groups, as did occurrence of complications in the operative group (P < .05). Both treatments permitted growth of the immature spine. CONCLUSIONS: In children with significant idiopathic EOS (Cobb angle>40°), growth rod surgery was associated with a reduction in activity and participation and Cobb angle, whereas brace treatment was associated with an increase in Cobb angle and no change in activity and participation. Pain was the most important factor affecting activity and participation in both groups.

Recommendations for Lengthening of Magnetically Controlled Growing Rods in Children With Pacemakers.

PURPOSE OF THE STUDY: Pacemakers are currently identified as a contraindication for the use of magnetic growth rods (MGRs). This arises from concern that magnetic fields generated by the MGR external remote controller (ERC) during lengthening procedures may induce pacemaker dysfunction. We investigated (1) whether MGR lengthening affects pacemaker function, and (2) if the magnetic field of a pacemaker affects MGR lengthening. METHODS: MGRs were tested in conjunction with an magnetic resonance imaging-compatible pacemaker, which was connected to a virtual patient under continuous cardiac monitoring. To determine whether pacemaker function was affected during MGR lengthening, the electrocardiogram trace was monitored for arrhythmias, whereas an ERC was applied to lengthen the MGRs at varying distances from the pacemaker. To investigate if MGR lengthening was affected by the presence of a pacemaker, at the start and end of the experiment, the ability of the rods to fully elongate and shorten was tested to check for conservation of function. RESULTS: When the pacemaker was in normal mode, <16 cm away from the activated ERC during MGR lengthening, pacemaker function was affected by the ERC's magnetic forces. At this distance, prophylactically switching the pacemaker to tonic mode before lengthening prevented occurrence of inappropriate pacing discharges. No deleterious effect of the pacemaker's magnetic field on the MGR lengthening mechanism was identified. CONCLUSIONS: Magnetic resonance imaging-compatible pacemakers appear safe for concomitant use with MGRs, provided a pacemaker technician prophylactically switches the pacemaker to tonic function before outpatient lengthening procedures. CLINICAL RELEVANCE: This experiment was designed to provide the first safety information on MGR lengthening in children with pacemakers. Although currently a rare clinical scenario, with increasing use of MGRs, this clinical scenario may arise more frequently in the future.

Orthopaedic manifestations of congenital indifference to pain with anhidrosis (Hereditary Sensory and Autonomic Neuropathy type IV).

BACKGROUND: Congenital indifference to pain with anhidrosis (CIPA) is a rare hereditary neuropathy, which is associated with defective sensation to noxious stimuli and autonomic dysfunction. The objective of the study was to report on the orthopaedic manifestations of this condition and provide an evidence-based approach for management. METHODS: Retrospective review of 14 consecutive patients with CIPA referred to a single tertiary centre. Mean age of diagnosis was 2.5 years (range 0.5 to 11 years). RESULTS: Patients presented with a range of orthopaedic problems including fractures, infections, growth disturbance, joint subluxation and Charcot joints affecting the limbs and spine. Conservative treatment with closed reduction and cast immobilisation was satisfactory for stress fractures of the lower extremity and Charcot joints. Posterior instrumented correction of scoliosis was associated with a high-risk of infection requiring reoperation for debridement and removal of posterior instrumentation. Growth disturbance leading to leg-length discrepancies were managed with shoe raises and corrective osteotomies. Aspiration and cultures may be used to differentiate between acute fracture and infection. CONCLUSIONS: Preventative treatment strategies with appropriately padded shoe-wear, gait and posture modification, parental education regarding environmental thermoregulation, and behavioural support are essential for improving prognosis and reducing long-term complications.

Is balloon kyphoplasty safe and effective for cancer-related vertebral compression fractures with posterior vertebral body wall defects?

BACKGROUND AND OBJECTIVES: Balloon kyphoplasty (BKP) is a percutaneous treatment for cancer-related vertebral compression fractures (VCF). Posterior vertebral body wall (PVBW) involvement is considered a contraindication for BKP. This study assesses whether BKP is safe and effective for cancer-related VCFs involving the PVBW. METHODS: This study analyzed data on 158 patients with 228 cancer-related VCFs who underwent BKP. One hundred and twelve patients had VCFs with PVBW defects, and 46 had VCFs with no PVBW defect. Outcomes were assessed preoperatively and at 3 months. RESULTS: In the PVBW defect group, mean pain score decreased from 7.5 to 3.6 (P < 0.001), EQ5D increased from 0.39 to 0.48 and Oswestry Disability Index (ODI) decreased from 50 to 42. Cement leaks occurred in 31%. In the PVBW intact group, mean pain decreased from 7.3 to 3.3 (P < 0.001), EQ5D increased from 0.35 to 0.48 (P < 0.001), and ODI decreased from 53 to 50. Cement leaks occurred in 20%. No significant difference was observed in functional improvements between groups. Radiographically kyphotic angle and anterior and middle vertebral body heights were significantly worse in the PVBW defect group (P < 0.05). CONCLUSIONS: BKP can alleviate pain and improve QoL and function in patients with cancer-related VCFs with PVBW defects with no appreciable increase in risk. J. Surg. Oncol. 2016;113:835-842. © 2016 Wiley Periodicals, Inc.

Computerized tomography based 3D modeling of the clavicle.

UNLABELLED: Previous studies have suggested clavicular morphology is highly variable, particularly in the lateral retrocurved section. Current clavicle fracture plating systems require three dimensional intra-operative contouring to achieve adequate fit and necessitate variable soft tissue dissection placing fracture perfusion and muscular attachments at risk. The aim of this study was to search for a surgically relevant superficial shape pattern. This is a retrospective CT-based analysis of 174 non-pathological clavicles in 95 adults (45 females, 50 males). Using the principle of cylindrical parameterisation generated 3-D computer models, we identified an implant preferred pathway (IPP), defined as a continuous linear region where the least possible soft tissue disruption would be necessary for plate fixation. The IPP mean form was within 3.04 mm (SD ± 1.34) on all clavicles. Clavicle length, and not shape, was found to be the biggest variable (correlation between size and form co-ordinates r = 0.99, p < 0.05), accounting for 79% of overall variability. This length variation was mainly located in the medial antecurved section. Superior convexity and recurvatum were the main shape variables, however they only contributed 8% and 5% to the overall variation, respectively. Three IPP lengths were shown to match all clavicles when aligned at the acromial end first. In contrast to previous studies, we have shown that the IPP is fairly consistent with respect to the general shape with the exception of length variation which mainly affected the medial antecurved section. © 2015 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 34:1216-1223, 2016. CLINICAL RELEVANCE: Future pre-contoured fracture fixation systems should provide variable length plates with a constant lateral section retrocurve and a variable medial antecurve.

A Preliminary Study to Assess Whether Spinal Fusion for Scoliosis Improves Carer-assessed Quality of Life for Children With GMFCS Level IV or V Cerebral Palsy.

BACKGROUND: Scoliosis affects 50% of children with Gross Motor Function Classification System (GMFCS) level IV or V cerebral palsy (CP). In children with complex neurodisability following intervention, the WHO considers quality of life (QoL) should be assessed to aid decision-making and assess the effects. This study assesses whether scoliosis surgery improves carer-assessed QoL for children with severe CP. METHODS: Retrospective review of 33 children (16 male:17 female) with GMFCS level IV/V CP and significant scoliosis. Fifteen underwent observational treatment during childhood, and 18 underwent surgery. Questionnaire and radiographic data were recorded over a 2-year period. The carer-completed Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) questionnaire was used to assess QoL. RESULTS: In the observational group, Cobb angle and pelvic obliquity increased from 46 (40 to 60) and 8 degrees (0 to 28) to 62 (42 to 94) and 12 degrees (1 to 35). Mean CPCHILD score decreased from 50 (30 to 69) to 48 (27 to 69) (P<0.05). In the operative group, Cobb angle and pelvic obliquity decreased from 78 (52 to 125) and 14 degrees (1 to 35) to 44 (16 to 76) and 9 degrees (1 to 24). Mean CPCHILD score increased from 45 (20 to 60) to 58 (37 to 76) (P<0.05). Change in pain, and not presence of associated impairments, was the most significant factor affecting QoL changes for children in both groups. There was no difference in mobility, GMFCS level, feeding, or communication in either group before and after treatment. CONCLUSIONS: Nonoperative treatment for children with GMFCS level IV/V CP and a significant scoliosis was associated with a small decrease in carer-assessed QoL over 2 years. Spinal fusion was associated with an increase in QoL. Change in pain was the most significant factor affecting QoL changes, and is therefore an important factor to consider when deciding upon surgery. LEVEL OF EVIDENCE: Level III-therapeutic retrospective study.

A retrospective review to assess whether spinal fusion and scoliosis correction improved activity and participation for children with Angelman syndrome.

OBJECTIVE: This study investigates outcome of scoliosis treatment for 11 children with Angelman syndrome (AS), with particular focus on activity, participation and the musculoskeletal factors that may affect these outcomes. METHODS: Retrospective review of medical records, radiographs and questionnaires administered to caregivers of 11 children (8M:3F) with AS and scoliosis. Six underwent observational treatment during childhood and five underwent spinal fusion. The Activities Scale for Kids (ASKp) questionnaire was used to measure activity and participation. Questionnaire and radiographic data were recorded over a 2 year period. RESULTS: In the observational group, scoliosis increased from 31° to 46°. Mean ASKp decreased from 13.8 to 11.9 (p = 0.06). In the operative group, scoliosis decreased from 68° to 29°. Mean ASKp increased from 11.4 to 15.9 (p < 0.01). There was also a reduction in spinal-related pain and mean number of hospital admissions for chest infection. However, there was a 60% major complication rate. There was no difference in mobility, GMFCS level, feeding or communication in either group before or after treatment. CONCLUSION: In children with significant scoliosis and AS, spinal fusion was associated with a small improvement in activity and participation, reduction in pain and a decrease in frequency of severe chest infections. Non-operative treatment resulted in progression of scoliosis during childhood and decrease in activity.