Citations

Background: Vertebral body tethering and other non-fusion techniques for the treatment of pediatric idiopathic scoliosis are increasing in popularity. There is limited physician consensus on this topic as the result of a paucity of published data regarding which patients most benefit from non-fusion strategies. Thus, much of the decision-making is left to patients and parents, who must select a treatment based on their goals and values and the information available from health-care providers, the internet, and social media. We sought to understand patient and family preferences regarding the attributes of fusion versus non-fusion surgery that drive these choices. Methods: Patients and families were recruited from 7 pediatric spine centers and were asked to complete a survey-based choice experiment that had been jointly developed with the U.S. Food and Drug Administration (FDA) to evaluate patient preferences. Choices between experimentally designed alternatives were analyzed to estimate the relative importance of outcomes and requirements associated with the choice options (attributes). The attributes included appearance, confidence in the planned correction, spinal motion, device failure, reoperation, and recovery period. The inclusion criteria were (1) an age of 10 to 21 years and (2) a diagnosis of adolescent idiopathic scoliosis in patients who were considering, or who had already undergone, treatment with fusion or non-fusion surgery. Preference weights were estimated from the expected changes in choice given changes in the attributes. Results: A total of 344 respondents (124 patients, 92 parents, and 128 parent/patient dyads) completed the survey. One hundred and seventy-three patients were enrolled prior to surgery, and 171 were enrolled after surgery. Appearance and motion were found to be the most important drivers of choice. For the entire cohort, fusion was preferred over non-fusion. For patients who were considering surgery, the most important attributes were preservation of spinal motion and appearance. Conclusions: Patients and families seeking treatment for idiopathic scoliosis value appearance and preservation of spinal motion and, to a lesser extent, reoperation rates when considering fusion versus non-fusion surgery.

The digitization of medical records and expanding electronic health records has created an era of “Big Data” with an abundance of available information ranging from clinical notes to imaging studies. In the field of rheumatology, medical imaging is used to guide both diagnosis and treatment of a wide variety of rheumatic conditions. Although there is an abundance of data to analyze, traditional methods of image analysis are human resource intensive. Fortunately, the growth of artificial intelligence (AI) may be a solution to handle large datasets. In particular, computer vision is a field within AI that analyzes images and extracts information. Computer vision has impressive capabilities and can be applied to rheumatologic conditions, necessitating a need to understand how computer vision works. In this article, we provide an overview of AI in rheumatology and conclude with a five step process to plan and conduct research in the field of computer vision. The five steps include (1) project definition, (2) data handling, (3) model development, (4) performance evaluation, and (5) deployment into clinical care.

Neurofibromatosis type 1 is a genetic syndrome characterized by a wide variety of tumor and non-tumor manifestations. Bone-related issues, such as scoliosis, tibial dysplasia, and low bone mineral density, are a significant source of morbidity for this population with limited treatment options. Some of the challenges to developing such treatments include the lack of consensus regarding the optimal methods to assess bone health in neurofibromatosis type 1 and limited data regarding the natural history of these manifestations. In this review, the Functional Committee of the Response Evaluation in Neurofibromatosis and Schwannomatosis International Collaboration: (1) presents the available techniques for measuring overall bone health and metabolism in persons with neurofibromatosis type 1, (2) reviews data for use of each of these measures in the neurofibromatosis type 1 population, and (3) describes the strengths and limitations for each method as they might be used in clinical trials targeting neurofibromatosis type 1 bone manifestations. The Response Evaluation in Neurofibromatosis and Schwannomatosis International Collaboration supports the development of a prospective, longitudinal natural history study focusing on the bone-related manifestations and relevant biomarkers of neurofibromatosis type 1. In addition, we suggest that the neurofibromatosis type 1 research community consider adding the less burdensome measurements of bone health as exploratory endpoints in ongoing or planned clinical trials for other neurofibromatosis type 1 manifestations to expand knowledge in the field.

Posterior spinal fusion has long been established as an effective treatment for the surgical management of spine deformity. However, interest in nonfusion options continues to grow. Vertebral body tethering is a nonfusion alternative that allows for the preservation of growth and flexibility of the spine. The purpose of this investigation is to provide a practical and relevant review of the literature on the current evidence-based indications for vertebral body tethering. Early results and short-term outcomes show promise for the first generation of this technology. At this time, patients should expect less predictable deformity correction and higher revision rates. Long-term studies are necessary to establish the durability of early results. In addition, further studies should aim to refine preoperative evaluation and patient selection as well as defining the benefits of motion preservation and its long-term effects on spine health to ensure optimal patient outcomes.

Surgical treatment for Legg–Calve–Perthes disease (LCPD) is recommended for older children with moderate to severe disease. We sought to determine whether double osteotomies lead to improved radiologic outcomes compared to reported non-operative outcomes. Patients older than 6 years of age diagnosed with LCPD lateral pillar B or C who were treated with pelvic and femoral osteotomies were included. Radiologic outcomes and leg-length discrepancies were assessed using the Stulberg classification and were compared with the current literature. Fifteen hips in fourteen patients were treated with double osteotomy for LCPD, and seven had lateral pillar C disease (47%). The mean age at surgery was 8.6 years (range, 7.2–10.4) and the mean age at follow-up was 20.2 years (range, 14.2–35.6). At a mean 11.6-year follow-up (range: 6.3–25.2), double osteotomy resulted in 40% of patients having Stulberg I/II scores, 27% having Stulberg III scores, and 33% having Stulberg IV/V scores. The mean leg-length discrepancy was 1.4 cm in lateral pillar C patients compared to 0.8 cm in lateral pillar B patients. Four patients underwent additional surgeries, including two who required total hip arthroplasty. Double osteotomy as an alternative surgical procedure for the treatment of LCPD did not show improved outcomes when compared to historic non-operative cohorts.

Background: Spinal conditions, such as scoliosis and spinal tumors, are prevalent in neurofibromatosis type 1 (NF1). Despite the recognized importance of their early detection and treatment, there remain knowledge gaps in how to approach these manifestations. The purpose of this study was to utilize the experience of a multidisciplinary committee of experts to establish consensus-based best practice guidelines (BPGs) for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric patients with NF1. Methods: Using the results of a prior systematic review, 10 key questions that required further assessment were first identified. A committee of 20 experts across medical specialties was then chosen based on their clinical experience with spinal deformity and tumors in NF1. These were 9 orthopaedic surgeons, 4 neuro-oncologists/oncologists, 3 neurosurgeons, 2 neurologists, 1 pulmonologist, and 1 clinical geneticist. An initial online survey on current practices and opinions was conducted, followed by 2 additional surveys via a formal consensus-based modified Delphi method. The final survey involved voting on agreement or disagreement with 35 recommendations. Items reaching consensus (≥70% agreement or disagreement) were included in the final BPGs. Results: Consensus was reached for 30 total recommendations on the management of spinal deformity and tumors in NF1. These were 11 recommendations on screening and surveillance, 16 on surgical intervention, and 3 on medical therapy. Five recommendations did not achieve consensus and were excluded from the BPGs. Conclusion: We present a set of consensus-based BPGs comprised of 30 recommendations for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric NF1.

Background: The Green-Anderson (GA) leg-length data remain the gold standard for the age-based assessment of leg lengths in children despite their methodologic weaknesses. We aimed to summarize current growth trends among a cross-sectional cohort of modern U.S. children using quantile regression methods and to compare the median femoral and tibial lengths of the modern U.S. children with those of the GA cohort. Methods: A retrospective review of scanograms and upright slot-scanning radiographs obtained in otherwise healthy children between 2008 and 2020 was completed. A search of a radiology registry revealed 3,508 unique patients between the ages of 2 and 18 years for whom a standard-of-care scanogram or slot-scanning radiograph had been made. All patients with systemic illness, genetic conditions, or generalized diseases that may affect height were excluded. Measurements from a single leg at a single time point per subject were included, and the latest available time point was used for children who had multiple scanograms made. Quantile regression analysis was used to fit the lengths of the tibia and femur and overall leg length separately for male patients and female patients. Results: Seven hundred patients (328 female and 372 male) met the inclusion criteria. On average, the reported 50th percentile tibial lengths from the GA study at each time point were shorter than the lengths in this study by 2.2 cm (range, 1.4 to 3.3 cm) for boys and 2 cm (range, 1.1 to 3.1 cm) for girls. The reported 50th percentile femoral lengths from the GA study at each time point were shorter than the lengths in this study by 1.8 cm (range, 1.1 to 2.5 cm) for boys and 1.7 cm (range, 0.8 to 2.3 cm) shorter for girls. Conclusions: This study developed new growth charts for femoral and tibial lengths in a modern U.S. population of children. The new femoral and tibial lengths at nearly all time points are 1 to 3 cm longer than traditional GA data. The use of GA data for epiphysiodesis could result in underestimation of expected childhood growth. Level of Evidence: Prognostic Level IV . See Instructions for Authors for a complete description of levels of evidence.

Long-term outcomes of surgical treatment for pediatric developmental dysplasia of the hip (DDH) are not well defined. The purpose of this study was to report long-term radiographic and clinical outcomes, survivorship free of total hip arthroplasty (THA), and predictors of subsequent THA following childhood treatment of DDH. This study was a single-institution retrospective review of hips treated for DDH with closed or open reduction at a minimum 10-year follow-up. 107 patients (119 hips) were included with a mean patient age of 3.3 years at childhood treatment. At mean 30.5 years follow-up, 24 hips had undergone THA (20%). Mean patient age at time of THA was 33.5 years. None of the hips treated with closed reduction alone required THA, whereas 8 hips treated with open reduction (25%) underwent THA. Hips with patient age > 4 years at the time of treatment had lower survivorship at 35 years follow-up (50% vs. 85%; p < 0.001). Additionally, femoral osteotomy (OR 2.0, p < 0.001), and previous treatment elsewhere (27% vs. 16%; p < 0.01) were associated with subsequent THA. Early referral and appropriate intervention may prove important, as age and prior treatment were predictive of subsequent THA.

Adolescent idiopathic scoliosis (AIS) can be treated with bracing or surgery, which may affect patient’s physical activity (PA). However, there are limited objective assessments of PA in patients with AIS. This study aims to compare the outcome of spinal bracing vs. surgery in patients with AIS based on a device that measured daily PA. In total 24 patients with AIS participated, including 12 patients treated with bracing and 12 with spinal surgery. Daily PA was measured throughout 4 consecutive days using four tri-axial accelerometers and patient-reported functional status was reported using the SRS-22 questionnaire. The participants were assessed both before the treatment and after treatment at a 12-month follow-up. Patients with AIS had no significant change in their PA levels at the 12-month follow-up after surgical correction. On the contrary, patients with AIS following a year-long bracing treatment had significantly reduced time spent active (P = 0.04) with an average reduction in walking steps by 2137 steps/day (P = 0.005). There was no significant difference in function, pain, self-image and mental health domains following both treatments, as reported by the SRS-22. There was a significant improvement in satisfaction for both treatment groups (P ≤ 0.02). Significantly reduced PA and increased sedentary time are reported in patients with AIS following bracing treatment. An objective PA assessment is recommended to track the effect of scoliosis treatment on PA. Patients with AIS should be actively encouraged to achieve and maintain their recommended daily PA levels irrespective of the type of treatment. Level of evidence: Level II.