Tone management: An environmental scan of current management practices across Canada.

BACKGROUND: Currently, there are no standardized approaches to care or evaluation for tone dysfunction in Canada. The study authors hypothesize that there is significant practice variation across the country. This environmental scan is aimed to describe the current practice for management of paediatric patients with hypertonia across Canada. METHODS: A web-based survey was developed by the authors with a multi-disciplinary approach and sent to representative paediatric rehabilitation sites in each province in Canada. Disciplines at the rehabilitation sites surveyed included all or some of the following disciplines: physiatry, neurology, neurosurgery, plastic surgery, orthopaedic surgery, physiotherapy and occupational therapy. All statistical analyses were performed using the R statistical software version 4.0. Fifteen rehabilitation sites were contacted, and 12 sites were used for the final analysis. RESULTS: Cerebral palsy was found to be the most common diagnosis for tone dysfunction, with 58% of sites diagnosing greater than 20 new patients per year. In 67% of sites, patients were seen within a formal multidisciplinary clinic to manage hypertonia. All 12 sites utilized oral baclofen and gabapentin, and 92% of sites utilized trihexyphenidyl. Botulinum toxin injections were offered at 50% of sites. Upper and lower extremity surgical procedures were offered in 83% of the sites. CONCLUSION: The information gained from this study provides some insight into the current practice across Canada for children with hypertonia. This study may assist in the development of a national, standardized strategy to tone management, potentially facilitating more equitable access to care for patients.

The Otto Aufranc Award: Does Hip Arthroscopy at the Time of Periacetabular Osteotomy Improve the Clinical Outcome for the Treatment of Hip Dysplasia? A Multicenter Randomized Clinical Trial.

BACKGROUND: A periacetabular osteotomy (PAO) is often sufficient to treat the symptoms and improve quality of life for symptomatic hip dysplasia. However, acetabular cartilage and labral pathologies are very commonly present, and there is a lack of evidence examining the benefits of adjunct arthroscopy to treat these. The goal of this study was to compare the clinical outcome of patients undergoing PAO with and without arthroscopy, with the primary end point being the International Hip Outcome Tool-33 at 1 year. METHODS: In a multicenter study, 203 patients who had symptomatic hip dysplasia were randomized: 97 patients undergoing an isolated PAO (mean age 27 years [range, 16 to 44]; mean body mass index of 25.1 [range, 18.3 to 37.2]; 86% women) and 91 patients undergoing PAO who had an arthroscopy (mean age 27 years [range, 16 to 49]; mean body mass index of 25.1 [17.5 to 25.1]; 90% women). RESULTS: At a mean follow-up of 2.3 years (range, 1 to 5), all patients exhibited improvements in their functional score, with no significant differences between PAO plus arthroscopy versus PAO alone at 12 months postsurgery on all scores: preoperative International Hip Outcome Tool-33 score of 31.2 (standard deviation [SD] 16.0) versus 36.4 (SD 15.9), and 12 months postoperative score of 72.4 (SD 23.4) versus 73.7 (SD 22.6). The preoperative Hip disability and Osteoarthritis Outcome pain score was 60.3 (SD 19.6) versus 66.1 (SD 20.0) and 12 months postoperative 88.2 (SD 15.8) versus 88.4 (SD 18.3). The mean preoperative physical health Patient-Reported Outcomes Measurement Information System score was 42.5 (SD 8.0) versus 44.2 (SD 8.8) and 12 months postoperative 48.7 (SD 8.5) versus 52.0 (SD 10.6). There were 4 patients with PAO without arthroscopy who required an arthroscopy later to resolve persistent symptoms, and 1 patient from the PAO plus arthroscopy group required an additional arthroscopy. CONCLUSIONS: This randomized controlled trial has failed to show any significant clinical benefit in performing hip arthroscopy at the time of the PAO at 1-year follow-up. Longer follow-up will be required to determine if hip arthroscopy provides added value to a PAO for symptomatic hip dysplasia.

Risk factors associated with prevalent vertebral fractures in Duchenne muscular dystrophy.

Patients with Duchenne muscular dystrophy (DMD) have a high fracture burden due to progressive myopathy and steroid-induced osteoporosis. This study in males with DMD showed that markers of systemic glucocorticoid exposure including shorter stature, greater bone age delay, and lower lumbar spine bone mineral density were associated with spine fragility. INTRODUCTION: Fragility  fractures are frequent in DMD. The purpose of this study was to identify clinical factors associated with prevalent vertebral fractures (VF) in boys, teens/young adults with Duchenne muscular dystrophy (DMD). METHODS: This was a cross-sectional study of males aged 4-25 years with DMD. VF were evaluated using the modified Genant semi-quantitative method on T4-L4 lateral spine radiographs. Areal bone mineral density (aBMD) was measured at the lumbar spine (LS) and used to estimate volumetric BMD (vBMD). Clinical factors were analyzed for their association with the Spinal Deformity Index (SDI, the sum of the Genant grades). RESULTS: Sixty participants were enrolled (mean age 11.5 years, range 5.4-19.5). Nineteen participants (32%) had a total of 67 VF; 23/67 VF (34%) were moderate or severe. Participants with VF were shorter (mean height Z-score ± standard deviation: - 3.1 ± 1.4 vs. - 1.8 ± 1.4, p = 0.001), had longer glucocorticoid exposure (mean duration 6.0 ± 3.3 vs. 3.9 ± 3.3 years, p = 0.027), greater bone age (BA) delay (mean BA to chronological age difference - 3.2 ± 3.4 vs. - 1.3 ± 1.2 years, p = 0.035), and lower LSaBMD Z-scores (mean - 3.0 ± 1.0 vs. - 2.2 ± 1.2, p = 0.023). There was no difference in LSvBMD Z-scores. Multivariable Poisson regression showed that every 0.1 mg/kg/day increment in average glucocorticoid daily dose was associated with a 1.4-fold SDI increase (95% confidence interval: 1.1-1.7, p = 0.013). Greater BA delay (p < 0.001), higher weight Z-score (p = 0.004), decreased height Z-score (p = 0.025), and lower LSvBMD Z-score (p = 0.025) were also associated with SDI increase. CONCLUSION: Readily measurable clinical variables were associated with prevalent VF in males with glucocorticoid-treated DMD. These variables may be useful to identify candidates for primary osteoporosis prevention after glucocorticoid initiation.

Mitigating the Denosumab-Induced Rebound Phenomenon with Alternating Short- and Long-Acting Anti-resorptive Therapy in a Young Boy with Severe OI Type VI.

Osteogenesis imperfecta (OI) type VI, a recessively inherited form of OI caused by mutations in SERPINF1, is a severe form distinguished by osteomalacia on bone histomorphometry. We describe a boy with severe OI type VI who was initially treated with intravenous (IV) zoledronic acid (ZA) at 1.4 years of age; however, a year later he transitioned to denosumab 1 mg/kg sub-cutaneously every three months in an effort to decrease fracture rates. After two years on denosumab, he presented with symptomatic hypercalcemia due to the denosumab-induced, hyper-resorptive rebound phenomenon. Laboratory parameters at the time of the rebound were as follows: elevated serum ionized calcium (1.62 mmol/L, N 1.16-1.36), elevated serum creatinine due to hypercalcemia-induced muscle catabolism (83 µmol/L, N 9-55), and suppressed parathyroid hormone (PTH) (< 0.7 pmol/L, N 1.3-5.8). The hypercalcemia was responsive to low-dose IV pamidronate, with a rapid decline in serum ionized calcium, and otherwise normalization of the aforementioned parameters within 10 days. To benefit from the powerful, albeit short-term, anti-resorptive effect of denosumab without further rebound episodes, he was treated thereafter with denosumab 1 mg/kg alternating every three months with IV ZA 0.025 mg/kg. Five years later, he remained on dual alternating anti-resorptive therapy without further rebound episodes, and an overall improvement in his clinical status. This novel pharmacological approach of alternating short- and long-term anti-resorptive therapy every three months has not previously been described. Our report suggests this strategy may be an effective method for prevention of the rebound phenomenon in select children for whom denosumab may be beneficial.

The Toddler's Fracture.

OBJECTIVE: The tibial toddler's fracture is an important diagnosis in both emergency and urgent care, presenting as acute onset lower extremity pain or limping in a young child. Diagnosis and management may be challenging because of an extensive differential diagnosis. The objectives of this study were to provide an overview of the toddler's fracture and to guide clinicians by summarizing up to date literature for both diagnosis and management this common condition. METHODS: This study analyzed literature from the PubMed database from the years of 1964 to 2018. The main focus was on the diagnosis and management of the toddler's fracture. RESULTS: This review demonstrates that diagnosis is primarily made through history and physical examination, as radiographs are often negative at initial presentation. Treatment involves a short period of immobilization, which can be facilitated through the use of a cast, a splint, or no external support. Successful healing and a full return to normal activities and development are near universal. CONCLUSIONS: Although a stable fracture with an excellent prognosis, opportunities exist to improve toddler's fractures diagnosis and treatment protocols, to optimize clinical management.

Hip Joint Preservation Surgery: What Every Orthopaedic Provider Needs to Know.

The understanding of the native hip's mechanics, physiology, and pathology has dramatically improved over the recent 2 decades. This was facilitated by the introduction of open and arthroscopic procedures to the native hip aimed at improving the joint's function and longevity. Associations between abnormal hip mechanics and further development of osteoarthritis are now clear. As the knowledge of hip joint mechanics has improved, other conditions around the hip have become evident, which may lead to pain but not necessarily osteoarthritis. It is important for the orthopaedic surgeon to be up to date on how the hip preservation field has evolved and the steps to consider when a painful hip presents in clinic.

Peroneal Nerve Function Before and Following Surgical Excision of a Proximal Fibular Osteochondroma.

BACKGROUND: Osteochondromas occur most commonly in the distal femur, proximal tibia, and humerus. There are no large studies reviewing the outcome of treatment for patients with an osteochondroma involving the proximal fibula. The purpose of this study is to specifically understand the manifestations of a proximal fibular osteochondroma (PFO) on the preoperative peroneal nerve function, and how surgical management of the osteochondroma affects function immediately postoperatively and at long-term follow-up. METHODS: This is an institutional review board-approved retrospective review of a consecutive series of patients with a PFO treated operatively at a single institution. The medical record was carefully reviewed to identify demographic data, clinical data especially the status of the peroneal function at various time points. RESULTS: There were 25 patients with 31 affected extremities who underwent surgical excision of the PFO at an average age of 12.4 years (range, 3.0 to 17.9 y). There were 16 males and 9 females. The underlying diagnosis was isolated PFO in 2 (8%) patients and multiple hereditary exostosis in 23 (92%) patients. Preoperatively, 9 (29%) had a foot drop and 22 (71%) did not. Those with a preoperative foot drop underwent surgery at a younger age (9.1 vs. 13.8 y) (P<0.004) and postoperatively 5 (55.5%) had complete resolution, 3 (33.3%) had improvement, and 1 (11.1%) persisted requiring an ankle foot orthosis. Of the 22 who were normal preoperatively, 5 (22.7%) developed an immediate postoperative foot drop, 3 (60%) completely resolved, 1 (20%) improved, and 1 (20%) persisted and was found to have a transected nerve at exploration. In total, 23 of the 25 (92%) patients who had a PFO excision, had a normal or near-normal peroneal nerve function including those who had poor function preoperatively. CONCLUSIONS: Patients with a PFO have a preoperative peroneal nerve dysfunction 30% of the time and 23% of those who were normal preoperatively have postoperative dysfunction. Fortunately, nearly all patients have a complete recovery following excision of the osteochondroma. LEVEL OF EVIDENCE: Level IV.

Electronic Consultation Systems: Impact on Pediatric Orthopaedic Care.

BACKGROUND: The demand for pediatric orthopaedic surgery consultation has grown rapidly, leading to longer wait times for elective consultation in some regions. Some specialties are addressing this increased demand through electronic consultation services. We wanted to examine the impact of pediatric orthopaedic e-consultations in Canada's Eastern Ontario region. METHODS: We developed a cross-sectional study of all the cases directed to a pediatric orthopaedic surgery specialist using the Champlain Building Access to Specialists through eConsultation (BASE) eConsult service over a 2-year period and examined their impact on in-person referrals, time of e-consultation and primary care satisfaction as well as types of clinical questions that were asked. RESULTS: Electronic consultations avoided in-person appointments in 68% of the submitted cases. The median response by specialists received by the primary care providers (PCPs) was <20 hours. A total of 69% of consultations involve >1 type of clinical questions, most commonly about basic trauma/fracture care and management recommendations. Ninety-seven percent of the PCPs found the overall value for the care of the patients to be good or excellent. CONCLUSIONS: This cross-sectional study demonstrates the effective and timely use of eConsult in pediatric orthopaedic surgery. It also shows a significant reduction in the number of in-person consultations required and demonstrates a high satisfaction rate by PCPs using the service. CLINICAL RELEVANCE: In addition to the efficacy and time-sensitive care provided to the patients, the study shows that, professionally, 89% of PCPs found this service to be excellent or good. The broader implications of electronic consultation on overall quality of care, population health, and patient satisfaction requires further investigation.

Treatment for Clubfoot: Stockinette Versus Webril Cast Padding in the Ponseti Method.

BACKGROUND: The Ponseti method of serial manipulation and casting revolutionized the treatment of one of the most common congenital orthopaedic conditions-clubfoot-resulting in less surgical morbidity and better functional results. Several studies have examined aspects of nonoperative treatment but none have explored the effect of different types of undercast padding. METHODS: The authors performed a randomized controlled trial comparing cotton Stockinette and Webril undercast padding using the Ponseti method. They included 56 patients under the age of 6 months who had an untreated idiopathic clubfoot. For treatment, the padding was randomized to double-layer Stockinette (29 patients) or standard 2- to 3-layer Webril (27 patients). The primary analysis consisted of a 2-sample t test comparing the mean number of casts required for correction in each group. Secondary outcomes included initial and final standardized clubfoot scores (Pirani and Dimeglio scores) and adverse events. RESULTS: Baseline demographics were not statistically different between groups, with respect to mean age, sex, and side being treated, nor were the baseline Dimeglio and Pirani scores. The primary outcome analysis revealed an equivalent number of casts (about 4) required before tenotomy. The secondary analysis on complications did not show any significant difference, with 10 events documented in the Webril group and 9 in the Stockinette group (P=1.00). At the end of the study, the mean Dimeglio (3.9±2.1 vs. 3.9±1.8, P=0.99) and Pirani (2.1±0.8 vs. 2.2±0.8, P=0.70) scores were not significantly different. Subjectively, parents and doctors indicated a substantially more positive experience for the Stockinette group. CONCLUSIONS: Webril and Stockinette paddings provide equivalent clubfoot deformity correction while applying a similar number of casts before tenotomy. Although minor skin complications were observed, these were not significantly different between groups.

Femoroacetabular Impingement: What the Surgeon Wants to Know.

Femoroacetabular impingement (FAI) is increasingly recognized as a risk factor for early hip degeneration in young active patients. The diagnosis depends on clinical examination and proper imaging that should be able to identify abnormal and sometimes subtle morphological changes. Labral tears and cartilage lesions rarely occur without underlying bone abnormalities. Surgical approaches to treat FAI are increasing significantly worldwide, even without a clearly defined consensus of what should be accepted as the standard imaging diagnosis for FAI morphology.Hip abnormalities encompass many variations related to the shape, size, and spatial orientation of both sides of the joint and can be difficult to characterize if adequate imaging is not available.This article presents a comprehensive review about the information orthopaedic surgeons need to know from radiologists to plan the most rational approach to a painful hip resulting from a mechanical abnormality.

A Contemporary Definition of Hip Dysplasia and Structural Instability: Toward a Comprehensive Classification for Acetabular Dysplasia.

Hip dysplasia has long been known to be a risk factor for pain and degenerative changes in the hip joint. The diagnosis of dysplasia has historically been based on assessments of acetabular anatomy on the anteroposterior pelvic radiograph, most commonly the lateral center-edge angle. Recent advances in imaging of the dysplastic hip with computerized tomography scans have demonstrated that hip dysplasia is in fact a 3-dimensional (D) deformity of the acetabulum and that multiple patterns of hip instability exist that may not be completely assessed on 2D imaging. A more thorough understanding of acetabular anatomy permits an evolution away from vague terms such as "borderline dysplasia." A 3D assessment of the acetabulum and the resultant patterns of instability may be more appropriate since this would allow more accurate treatment to correct the structural instability with acetabular reorientation. With this information, we propose a diagnostic framework that groups symptomatic dysplastic hips into one of 3 categories based on the primary direction of instability: (1) anterior, (2) posterior, and (3) global. This framework may aid the clinician in developing a differential diagnosis for the assessment of hip pain and suspected instability, and for planning an appropriate surgical management.

Predictors of True Scaphoid Fractures in Children.

BACKGROUND: The scaphoid is the most commonly fractured carpal bone in children. True scaphoid fractures have proven to be difficult to diagnose, as they may not be apparent on initial imaging. Children with clinical suspicion of a scaphoid fracture may be treated with continued immobilization, even in the absence of radiographic evidence of a fracture. The purpose of this study is to identify predictors of true scaphoid fractures in children to help guide management. METHODS: This study is a retrospective cohort study of children presenting to a tertiary pediatric hospital with hand or wrist injuries. Patients were grouped based on the presence of a true scaphoid fractures (confirmed on imaging) or those with clinical suspicion of a scaphoid fracture alone (no radiographic evidence of fracture). Demographic and clinical characteristics were compared with univariate and multivariate statistics to identify fracture predictors. RESULTS: One hundred and thirty patients were included in the study: 57 in the true scaphoid fracture group and 73 in the clinical scaphoid fracture group. Patients with a true scaphoid fracture were older than those with a clinical scaphoid fracture (median age [interquartile range], 14.2 [13.0-15.4] vs 12.9 [11.9-14.4], P = .01). Men were more likely to sustain a true scaphoid fracture (65.0% vs 35.0%, P = .01). Older age and male sex were shown to be independent predictors of true scaphoid fractures (odds ratio [95% confidence interval], 1.25 [1.03-1.50] and 2.93 [1.39-6.17], respectively). CONCLUSIONS: In the pediatric population, older age and male children may be at increased risk of true scaphoid fractures. This may help guide decisions surrounding further imaging and treatment.

Determining the factors associated with a prolonged length of stay for adolescent idiopathic scoliosis patients undergoing posterior spinal instrumentation and fusion in a single-payer health care system: are these factors different than those in the United States?

INTRODUCTION: Rapid recovery pathways (RRP) for adolescent idiopathic scoliosis patients undergoing posterior spinal instrumentation and fusion (PSIF) have been shown to be successful in reducing hospital length of stay (LOS). Although the majority of patients are discharged within 3 days, some patients require longer hospital admission. Previous studies in the United States have identified predictors of prolonged LOS for this patient population. The goal of this project was to determine if these predictors are the same for Canadian scoliosis patients and to identify those features which are different under this single-payer system. METHODS: A RRP for scoliosis surgery was implemented in March 2015 at a single, tertiary referral children's hospital in Canada. Previously identified features, along with numerous other patient factors, were collected. Spearman correlations were used to determine the factors most associated with hospital LOS and those factors were used in a multivariable regression model. RESULTS: A total of 161 patients were included in the analysis. Of the previously identified patient factors, only receiving a peri-operative transfusion was found to be significant (ρ = 0.24; p = 0.002). None of the other pre-identified variables were found to be significantly correlated with LOS. Variables not previously examined that were found to be significantly correlated with hospital LOS included ASA status (ρ = 0.19, p = 0.046), fusion involving both the thoracic and lumbar spine (ρ = 0.18, p = 0.025), and receiving celecoxib on post-operative day 1 (ρ = - 0.16; p = 0.038). The features that had the greatest association with LOS through multivariable regression was receiving a blood transfusion (B = 0.48; 95%CI 0.096-0.89; p = 0.017). CONCLUSIONS: In this study, we found that many of the features found to be significantly correlated with prolonged hospital LOS in the United States are not transferable to the Canadian healthcare system. This is important for the Canadian, and other surgeons in a single-payer system, in order to identify pre-operative or immediate post-operative factors that may extend patient LOS following PSIF and plan resources accordingly. LEVEL OF EVIDENCE: III; therapeutic.

Burosumab for the treatment of cutaneous-skeletal hypophosphatemia syndrome.

Cutaneous-skeletal hypophosphatemia syndrome (CSHS) is a rare bone disorder featuring fibroblast growth factor-23 (FGF23)-mediated hypophosphatemic rickets. We report a 2-year, 10-month-old girl with CSHS treated with burosumab, a novel human monoclonal antibody targeting FGF23. This approach was associated with rickets healing, improvement in growth and lower limb deformity, and clinically significant benefit to her functional mobility and motor development. This case report provides evidence for the effective use of FGF23-neutralizing antibody therapy beyond the classic FGF23-mediated disorders of X-linked hypophosphatemia and tumor-induced osteomalacia.

Risk Factors Associated with Incident Vertebral Fractures in Steroid-treated Males with Duchenne Muscular Dystrophy.

PURPOSE: Prevention of fractures is an unmet need in glucocorticoid (GC)-treated Duchenne muscular dystrophy. This study explored factors associated with incident vertebral fractures (VFs) to inform future fracture prevention efforts. METHODS: VFs were evaluated prospectively at study baseline and 12 months on lateral spine radiographs in participants aged 4 to 25 years with Duchenne muscular dystrophy. Clinical factors were analyzed for their association with the change in Spinal Deformity Index (sum of the Genant-defined VF grades from T4 to L4) between baseline and 12 months. RESULTS: Thirty-eight males were evaluated (mean ± SD age at baseline 11.0 ± 3.6 years; mean ± SD GC duration at baseline 4.1 ± 3.1 years; 74% ambulatory). Nine of 38 participants (24%) had 17 incident VFs, of which 3/17 VFs (18%) were moderate/severe. Participants with 12-month incident VF had lower mean ± SD baseline lumbar spine areal bone mineral density Z-scores (-2.9 ± 1.0 vs -1.9 ± 1.1; P = .049) and lower total body less head areal bone mineral density Z-scores (-3.1 ± 1.2 vs -1.6 ± 1.7; P = .036). Multivariable linear regression showed that at least 1 VF at baseline (P < .001), a higher number of antecedent non-VF (P < .001), and greater bone age delay at baseline (P = .027) were significant predictors of an increase in the Spinal Deformity Index from baseline to 12 months. CONCLUSION: The observation that ≥ 1 prevalent VF and/or non-VF were the strongest predictors of incident VFs at 12 months supports the need for prevention of first fractures in this high-risk setting. Bone age delay, a marker of GC exposure, may assist in the prioritization of patients in efforts to prevent first fractures.

Vertebral Body Reshaping after Fractures: An Important Index of Recovery in Glucocorticoid-Treated Children.

PURPOSE: In this 6-year study we identified factors associated with spontaneous vertebral body reshaping in glucocorticoid (GC)-treated children with leukemia, rheumatic disorders, and nephrotic syndrome. METHODS: Subjects were 79 children (mean age 7.4 years) who had vertebral fracture (VF) evaluation on lateral spine radiographs at least 1 year after VF detection. VF were graded using the modified Genant semiquantitative method and fracture burden for individuals was quantified using the spinal deformity index (SDI; sum of grades from T4 to L4). RESULTS: Sixty-five children (82.3%) underwent complete vertebral body reshaping (median time from VF detection to complete reshaping 1.3 years by Cox proportional hazard modeling). Of 237 VF, the majority (83.1%) ultimately reshaped, with 87.2% reshaping in the thoracic region vs 70.7% in the lumbar region (P = .004). Cox models showed that (1) every g/m2 increase in GC exposure in the first year after VF detection was associated with a 19% decline in the probability of reshaping; (2) each unit increase in the SDI at the time of VF detection was associated with a 19% decline in the probability of reshaping [hazard ratio (HR) = 0.81; 95% confidence interval (CI) = 0.71, 0.92; P = .001]; (3) each additional VF present at the time of VF detection reduced reshaping by 25% (HR = 0.75; 95% CI = 0.62, 0.90; P = .002); and (4) each higher grade of VF severity decreased reshaping by 65% (HR = 0.35; 95% CI = 0.21, 0.57; P < .001). CONCLUSION: After experiencing a VF, children with higher GC exposure, higher SDI, more severe fractures, or lumbar VF were at increased risk for persistent vertebral deformity.

One dose of tranexamic acid is safe and effective in revision knee arthroplasty.

Revision total knee arthroplasty (TKA) has been associated with an increased risk of perioperative blood loss. Tranexamic acid (TXA) has been proven to be safe and effective in preventing blood loss in primary TKA. The purpose of this study was to evaluate the effect of TXA on blood loss and transfusion rates in revision TKA. We performed a retrospective comparative study on 424 patients who had undergone revision TKA between January 2006 and March 2010. A total of 178 patients did not receive TXA while 246 patients received one intraoperative dose of 20mg/kg of TXA given prior to tourniquet release. There was a significant reduction in hemoglobin loss (42±16g/L vs 38±15g/L, P=0.005), transfusion rates (30.3% vs 16.7%, P=0.001) and average amount transfused (1.1±1.9units vs 0.5±1.1units, P=0.001) in the TXA group. There was no significant difference in recorded major adverse events with the administration of TXA.

The effect of vertebral body tethering on spine range of motion in adolescent idiopathic scoliosis: a pilot study.

PURPOSE: Posterior spinal fusion and instrumentation (PSF) and vertebral body tethering (VBT) are corrective surgical techniques used in treating adolescent idiopathic scoliosis (AIS). Comparing the preservation of spine range of motion (ROM) following PSF and VBT for treatment of AIS has yet to be explored. The purpose of this work was to retrospectively compare global spine ROM in adolescents (9-18 years of age) without spine deformity, adolescents with untreated AIS, adolescents having undergone PSF, and adolescents having undergone VBT to gain insight on the effect of VBT on spine motion. METHODS: Twenty participants were recruited into four groups including Control (n = 6), untreated AIS (n = 5), post-operative PSF (n = 4) and post-operative VBT (n = 5). Three-dimensional kinematics of the spine were collected and analyzed using an intersegmental spine model during constrained forward flexion, right-left lateral bending, and right-left axial twist movements. RESULTS: The PSF group displayed significantly lower spine ROM than the two non-operative groups during thoracic and total left axial twist (p ≤ 0.048), whereas thoracic and total ROM during right-left lateral bending is almost equally lower in the PSF (p ≤ 0.03) and VBT (p ≤ 0.01) groups when compared to the Control and AIS groups. CONCLUSION: These results suggest some preservation of spine motion in the transverse plane following VBT. This study provides initial evidence of some potential preservation of spine ROM following VBT; however, further prospective investigation of VBT is needed to assess and confirm these hypotheses.

Reliability of radiographic assessment of growth modulation from anterior vertebral body tethering surgery in pediatric scoliosis.

PURPOSE: To assess the reliability of vertebral height and angular measurements for anterior vertebral body tethering (AVBT). METHODS: Eight observers measured PA radiographs of 15 idiopathic scoliosis patients treated with AVBT, pre-operative and 4-year follow-up. Vertebral wedging, disc wedging, convex vertebral body heights, and concave vertebral body heights of the 3 apical vertebrae were measured. For each observer, there were a total of 90 measurements for vertebral body height and 75 measurements for all wedging types At least 14 days elapsed between first and second round measurements. RESULTS: From the pre-operative to the 4-year follow-up time-point, the total wedging angle over the 3 peri-apical levels fell from 30 ± 7° to 16 ± 6° (p < 0.001) and the difference between the convex and concave vertebral heights decreased from 9 ± 4 to 6 ± 3 mm (p < 0.001). Interobserver agreement for pre-operative vertebral body heights was good [ICC = 0.80; 95% CI (0.74-0.85)]. At 4-year follow-up there was a moderate agreement [ICC = 0.67 (0.59-0.74)]. There was a poor interobserver agreement for pre-operative wedging angle measurements [ICC = 0.41 (0.32-0.52)] and 4-year follow-up [ICC = 0.45 (0.36-0.56)]. The Limits of Agreement with the Mean (LOAM) for pre-op heights was ± 2.4 mm, similar to the follow-up ± 2.6 mm. When raters are averaged in random groups of two the agreement limits decrease to ± 1.8 mm pre-op and ± 1.6 mm at follow-up. Similarly for wedging angles, LOAM values among the 8 observers of ± 4.6° pre-op and ± 4.2° dropped to ± 2.7° for both pre-op and follow-up when random groups of two raters were averaged together. Intraobserver agreement ranged from good to excellent per individual (ICC = 0.84-0.94) for pre-operative vertebral body heights, but this decreased at 4-year follow-up (ICC = 0.52-0.88). Intraobserver agreement was low overall for wedging (pre-operative ICC = 0.41-0.71; 4-year follow-up ICC = 0.41-0.76). CONCLUSION: Based on 8 individual observers, interobserver agreement ranged from good (pre-operative vertebral body heights) to moderate (4-year follow-up vertebral body heights) to poor (all wedging angles). To improve the reliability of the measurement of wedging angles, we recommend averaging the measurements of at least two observers.

Canakinumab in addition to phosphate-binding and phosphaturia-inducing therapy were effective in achieving remission in a child with a large familial calcinotic tumour.

We describe the clinical evolution of a patient with tumoral calcinosis due to a pathogenic variant in the GALNT3 gene presented with a large mass overlying her left hip associated complicated by inflammatory flares. Therapy (sevelamer, acetazolamide, and probenecid) was unsuccessful in preventing tumour surgeries, therefore, interleukin-1ß monoclonal antibody therapy was added; this was successful in the prevention of tumour re-growth. This case highlights the importance of assessing and treating the inflammatory aspect of calcinotic tumour.