Perioperative management and outcomes for posterior spinal fusion in patients with Friedreich ataxia: A single-center, retrospective study.

BACKGROUND: Friedreich ataxia is a rare genetic disorder associated with progressive mitochondrial dysfunction leading to widespread sequelae including ataxia, muscle weakness, hypertrophic cardiomyopathy, diabetes mellitus, and neuromuscular scoliosis. Children with Friedreich ataxia are at high risk for periprocedural complications during posterior spinal fusion due to their comorbidities. AIM: To describe our single-center perioperative management of patients with Friedreich ataxia undergoing posterior spinal fusion. METHODS: Adolescent patients with Friedreich ataxia presenting for spinal deformity surgery between 2007 and 2023 were included in this retrospective case series performed at the Children's Hospital of Philadelphia. Perioperative outcomes were reviewed along with preoperative characteristics, intraoperative anesthetic management, and postoperative medical management. RESULTS: Seventeen patients were included in the final analysis. The mean age was 15 ± 2 years old and 47% were female. Preoperatively, 35% were wheelchair dependent, 100% had mild-to-moderate hypertrophic cardiomyopathy with preserved systolic function and no left ventricular outflow tract obstruction, 29% were on cardiac medications, and 29% were on pain medications. Intraoperatively, 53% had transesophageal echocardiography monitoring; 12% had changes in volume status on echo but no changes in function. Numerous combinations of total intravenous anesthetic agents were used, most commonly propofol, remifentanil, and ketamine. Baseline neuromonitoring signals were poor in four patients and one patient lost signals, resulting in 4 (24%) wake-up tests. The majority (75%) were extubated in the operating room. Postoperative complications were high (88%) and ranged from minor complications like nausea/vomiting (18%) to major complications like hypotension/tachycardia (29%) and need for extracorporeal membrane oxygenation support in one patient (6%). CONCLUSIONS: Patients with Friedreich ataxia are at high risk for perioperative complications when undergoing posterior spinal fusion and coordinated multidisciplinary care is required at each stage. Future research should focus on the utility of intraoperative echocardiography, optimal anesthetic agent selection, and targeted fluid management to reduce postoperative cardiac complications.

Impact of Social Determinants of Health on Adolescent Idiopathic Scoliosis Curve Severity.

INTRODUCTION: Social determinants of health have been shown to influence the health and outcomes of pediatric patients. Adolescent idiopathic scoliosis (AIS) may be particularly sensitive to such factors as early diagnosis and treatment can obviate the need for surgical intervention. The purpose of this study was to analyze the effect that social determinants of health have on the severity of AIS at presentation and at the time of surgery. METHODS: A retrospective review was conducted for consecutive patients who underwent posterior spinal fusion for AIS from 2020 to 2022. Demographic data was collected, while insurance status (private vs. public) and childhood opportunity index (COI) categories (LOW vs. HIGH) were used as a proxy for socioeconomic status. Curve magnitude at the initial presentation and at the latest preoperative visit were recorded with a threshold of 25 to 40 degrees considered within the bracing range. Univariate and multivariate analysis was done to compare differences between subgroups as appropriate. RESULTS: A total of 180 patients with mean initial and preoperative major curve angles of 48 and 60 degrees were included. Statistically significant differences in race and insurance types were appreciated, with the LOW COI group having a higher proportion of underrepresented minority and publicly insured patients than the HIGH COI group ( P <0.001). Patients within the LOW COI group presented with an initial curve that was, on average, 6 degrees more severe than those within the HIGH group ( P =0.009) and a preoperative curve that was 4 degrees larger than those within the HIGH group ( P =0.015). Similarly, only 13% of patients within the LOW COI group presented with curves within the bracing threshold, compared with 31% in the HIGH COI group ( P =0.009). CONCLUSION: Socioeconomic status plays a significant role in the severity of AIS. Specifically, patients with lower COI tend to present with curve magnitudes beyond what is responsive to nonsurgical treatment, leading to larger curves at the time of surgery. Future work should focus on addressing social inequalities to optimize the treatment and outcomes of AIS patients. LEVEL OF EVIDENCE: Level III- Retrospective Comparative Study.

Rib-on-Pelvis Deformity and Reported Pain in Neuromuscular Early-Onset Scoliosis.

BACKGROUND: Neuromuscular early-onset scoliosis (N-EOS) often presents with a long sweeping thoracolumbar scoliosis and pelvic obliquity. With severe pelvic obliquity, the ribs come into contact with the high side of the pelvis, termed rib-on-pelvis deformity (ROP). The goal of this study is to evaluate whether ROP is associated with reported pain and other health-related quality of life (HRQOL) measures. We hypothesize that ROP is associated with increased pain and negative HRQOL. METHODS: A multicenter international registry was queried for all nonambulatory patients with N-EOS from 2012 to 2022. Both surgical and nonsurgical patients were included. ROP was classified as a binary radiographic assessment of preoperative (surgical patients) and most recent follow-up (nonsurgical patients) upright radiographs. Reported pain and other HRQOL measures were assessed through the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24). Patients with nonupright radiographs or EOSQ-24 questionnaires and corresponding radiographs >4 months apart were excluded. RESULTS: Totally, 225 patients (8.4±3.1 y, 55% female) were included. The median major curve was 63.3 (IQR: 40.6 to 81.2) degrees and median pelvic obliquity was 15.5 degrees (IQR: 8.8 to 26.4). Eighty-three patients (37%) had ROP. ROP was associated with both frequency (P<0.001) and severity (P<0.001) of pain. ROP was associated with worse general health (P=0.01), increased difficulty with vocalization (P=0.02), increased frequency of shortness of breath (P=0.002), and increased difficulty sitting upright (P=0.04). Regarding overall EOSQ-24 domains, ROP was associated with worse general health, pain/discomfort, pulmonary function, and physical function (P<0.01). In a subanalysis of 76 patients who underwent surgical intervention with at least 2 years of follow-up, patients with preoperative ROP experienced significantly greater improvements in both frequency (P=0.004) and severity (P=0.001) of pain than the patients without preoperative ROP at 2 years postoperatively. CONCLUSIONS: The overall incidence of ROP in N-EOS is about 37%. ROP is associated with greater pain and worse HRQOL through the EOSQ-24 questionnaire. Furthermore, these patients experienced a greater reduction in pain after surgery. Clinicians and parents must be aware that ROP is possibly a pain generator, but responds positively to surgical intervention. LEVEL OF EVIDENCE: Level III.

Why Don't Adolescents Wear Their Brace? A Prospective Study Investigating Psychosocial Characteristics That Predict Scoliosis Brace Wear.

BACKGROUND: Although bracing for adolescent idiopathic scoliosis can prevent curve progression and reduce the risk for future surgery, children frequently do not wear their braces as prescribed. The purpose of this study is to investigate how a broad array of psychosocial characteristics predict future compliance with scoliosis brace wear. METHODS: This was a single institution, prospective cohort study. All adolescents prescribed a first-time brace for adolescent idiopathic scoliosis were eligible. Patients and their parents completed a separate series of questionnaires that assessed baseline psychosocial characteristics across 6 domains: (1) brace-specific attitudes; (2) body image and self-esteem; (3) school performance and social relationships; (4) psychological health; (5) family functioning; and (6) demographics and scoliosis-specific details (242 total questions across 12 validated questionnaires). Objective brace compliance was collected using temperature-sensitive monitors. Defining compliance as percentage of brace prescription completed, comparative analyses were performed to identify baseline psychosocial characteristics that were associated with future wear. A composite measure (Bracing Fidelity Follow-Up Scale [BFFS]) of the 12 most predictive individual questions across all domains (both parent and adolescent) was constructed to help assess which adolescents were at highest risk of failure to wear their brace. Total BFFS score for each parent-adolescent dyad who completed all the included surveys was then determined by awarding one point for each factor that positively influenced future brace wear (maximum 12 points), and a correlation was calculated between total score and percent adherence to prescribed brace wear. RESULTS: A total of 41 patients were included. On average, patients with high self-esteem, above average peer relationships and poor brace-specific attitudes had lower brace compliance, although patients with increased loneliness and parental religiousness had higher compliance. Body image, socioeconomic status, family dynamics, and school performance had no significant relationship with brace use. Total score on the Bracing Fidelity Follow-Up Scale (BFFS) was significantly associated with improved brace wear (r=0.687, P <0.001). Those with a score of 6 or above (n=15/33 [45%], median compliance 96%) were more reliable users (15/15 with compliance >75%), and those with a score of 5 or less (n=18/33 [55%], median compliance 50%) had less consistent brace wear (9/18 with compliance <50%). CONCLUSION: This prospective study identifies numerous baseline psychosocial factors that are associated with future compliance with scoliosis brace wear. Although in need of further validation before widespread clinical application, the novel BFF scale offers a potential opportunity to partially discriminate between compliant and noncompliant scoliosis brace users such that supportive resources (eg, supportive counseling, peer-support groups, additional provider-based education, etc.) can be targeted to those patients most likely to benefit. LEVEL OF EVIDENCE: II.

Pregnancy and Childbirth After Spinal Fusion for Adolescent Idiopathic Scoliosis.

BACKGROUND: Little data exist on pregnancy and childbirth for adolescent idiopathic scoliosis (AIS) patients treated with a spinal fusion. The current literature relies on data from patients treated with spinal fusion techniques and instrumentation, such as Harrington rods, that are no longer in use. The objective of our study is to understand the effects of spinal fusion in adolescence on pregnancy and childbirth. METHODS: Prospectively collected data of AIS patients undergoing posterior spinal fusion that were enrolled in a multicenter study who have had a pregnancy and childbirth were reviewed. Results were summarized using descriptive statistics and compared with national averages using χ 2 test of independence. RESULTS: A total of 78 babies were born to 53 AIS patients. As part of their pre-natal care, 24% of patients surveyed reported meeting with an anesthesiologist before delivery. The most common types of delivery were spontaneous vaginal delivery (46%, n=36/78) and planned cesarean section (20%, n=16/78). Compared with the national average, study patients had a higher rate of cesarean delivery ( P =0.021). Of the women who had a spontaneous vaginal birth, 53% had no anesthesia (n=19/36), 19% received intravenous intermittent opioids (n=7/36), and 31% had regional spinal or epidural anesthesia (n=11/36). spontaneous vaginal delivery patients in our study cohort received epidural or spinal anesthesia less frequently than the national average ( P <0.001). Of those (n=26 pregnancies) who did not have regional anesthesia (patients who had no anesthesia or utilized IV intermittent opioids), 19% (n=5 pregnancies) were told by their perinatal providers that it was precluded by previous spine surgery. CONCLUSION: The majority of AIS patients reported not meeting with an anesthesiologist before giving birth and those who had a planned C-section did so under obstetrician recommendation. The presence of instrumentation after spinal fusion should be avoided with attempted access to the spinal canal but should not dictate a delivery plan. A multidisciplinary team consisting of obstetrician, anesthesiologist, and orthopaedic surgeon can provide the most comprehensive information to empower a patient to make her decisions regarding birth experience anesthesia based on maternal rather than provider preference. LEVEL OF EVIDENCE: IV.

A novel risk calculator predicting surgical site infection after spinal surgery in patients with cerebral palsy.

AIM: To develop and validate a risk calculator based on preoperative factors to predict the probability of surgical site infection (SSI) in patients with cerebral palsy (CP) undergoing spinal surgery. METHOD: This was a multicenter retrospective cohort study of pediatric patients with CP who underwent spinal fusion. In the development stage, preoperative known factors were collected, and a risk calculator was developed by comparing multiple models and choosing the model with the highest discrimination and calibration abilities. This model was then tested with a separate population in the validation stage. RESULTS: Among the 255 patients in the development stage, risk of SSI was 11%. A final prediction model included non-ambulatory status (odds ratio [OR] 4.0), diaper dependence (OR 2.5), age younger than 12 years (OR 2.5), major coronal curve magnitude greater than 90° (OR 1.3), behavioral disorder/delay (OR 1.3), and revision surgery (OR 1.3) as risk factors. This model had a predictive ability of 73.4% for SSI, along with excellent calibration ability (p = 0.878). Among the 390 patients in the validation stage, risk of SSI was 8.2%. The discrimination of the model in the validation phase was 0.743 and calibration was p = 0.435, indicating 74.3% predictive ability and no difference between predicted and observed values. INTERPRETATION: This study provides a risk calculator to identify the risk of SSI after spine surgery for patients with CP. This will allow us to enhance decision-making and patient care while providing valid hospital comparisons, public reporting mechanisms, and reimbursement determinations.

Pediatric Hand Fractures and Congenital Differences: An Analysis of Data From the American Board of Orthopaedic Surgery and the American Board of Plastic Surgery.

PURPOSE: Pediatric patients with hand trauma and congenital differences are treated across multiple surgical subspecialties. The purpose of this study was to assess operative trends over an 11-year period using the American Board of Orthopaedic Surgery and the American Board of Plastic Surgery databases to better understand which surgeons were caring for pediatric hand fractures and birth differences in the first 2 years of their practice. METHODS: We queried the American Board of Orthopaedic Surgery and the American Board of Plastic Surgery databases for surgical procedures performed by applicants for the oral examinations between 2004 and 2014. Candidates self-identified as general orthopedic surgeon, pediatric orthopedic surgeon, hand surgeon (orthopedic and plastic), and general plastic surgeon. This included a total of 2,453 Board applicants. A total of 6,835 surgeries for birth differences or hand trauma were identified and reviewed for patients <18 years of age. RESULTS: There were 5,759 trauma and 1,076 congenital difference surgeries. A total of 4,786 (70%) surgeries were performed by orthopedic surgeons. Fellowship-trained hand surgeons (orthopedic and plastic) performed 3,809 (56%) surgeries. Pediatric orthopedic surgeons performed 608 (9%) surgeries. Over the 11 years, general orthopedic surgeons performed 4.2 fewer surgeries per year, whereas surgeons in hand orthopedics and pediatric orthopedics performed 10.8 and 4.7 additional surgeries per year. There were 3.1 fewer general orthopedic surgeons per year, whereas there were 3.6 and 1.4 additional surgeons in hand orthopedics and pediatric orthopedics each year, respectively. The number of surgeries and the number of surgeons submitting surgeries did not significantly change for those in general plastics or hand plastics. CONCLUSIONS: This analysis of early practice patterns over 11 years demonstrates that the increasing numbers of surgeons in pediatric orthopedics and hand orthopedics are performing more surgeries compared with other fields. CLINICAL RELEVANCE: The care of children with hand injuries and congenital differences is evolving, with direct implications for residency and fellowship education.

Early Knee Range of Motion Following Operative Treatment for Tibial Tubercle Avulsion Fractures Is Safe.

BACKGROUND: Tibial tubercle avulsion fractures (TTAF) often require surgical reduction and stabilization. Traditional teachings recommend postoperative knee immobilization for 4 to 6 weeks; however, the necessity of these restrictions is unclear and the actual practice varies. This study's purpose was to: (1) retrospectively review operatively managed TTAFs at a single center to examine the spectrum of postoperative rehabilitation guidelines, and (2) compare the outcomes of patients based on the timing of initiation of postoperative knee range of motion (ROM). METHODS: Operatively managed TTAFs treated at a single center from 2011-2020 were identified. Patients with polytrauma, associated lower extremity compartment syndrome, or treatment other than screw fixation were excluded. Patient demographics, mechanism of injury, Ogden Classification, associated injuries, operative technique, postoperative ROM progression, and time to release to unrestricted activities were collected. Patients were grouped based on the initiation of postoperative ROM as Early (<4 wk, EROM) or Late (≥4 wk, LROM). Bivariate analysis was used to compare characteristics between these 2 groups. RESULTS: Study criteria identified 134 patients, 93.3% (n=125) of whom were male. The mean age of the cohort was 14.77 years [95% confidence interval (CI: 14.5 to 15.0]. Forty-nine patients were designated EROM; 85 patients were categorized as LROM. The groups did not differ significantly with regards to age, race, injury characteristics, or surgical technique. Both groups progressed similarly with regards to postoperative range of motion. Ultimate activity clearance was achieved at ~20 weeks postoperatively without differences between groups ( P >0.05). Four instances of postoperative complication were identified, all of which occurred in the LROM group. CONCLUSION: A broad range of postoperative immobilization protocols exists following the screw fixation of TTAFs. Across otherwise similar cohorts of patients undergoing operative treatment, initiation of knee range of motion before 4 weeks was without complication and provided equivalent outcomes to traditional immobilization practices. Given the clear benefits to patients and caregivers provided by the permission of early knee motion and the avoidance of casting, providers should consider more progressive postoperative rehab protocols following operatively treated TTAFs. LEVEL OF EVIDENCE: Level III, Retrospective Cohort.

When Is an Isolated Olecranon Fracture Pathognomonic for Osteogenesis Imperfecta?

BACKGROUND: Isolated fractures of the olecranon process of the ulna in pediatric patients with open physes are classically considered pathognomonic for osteogenesis imperfecta (OI). The purpose of this study was to distinguish the clinical manifestations of isolated olecranon fractures in patients with and without OI to help practitioners assess when further evaluation for OI may be necessary. METHODS: All patients younger than 18 years old who were treated for an isolated olecranon fracture at a pediatric tertiary care center between 2009 and 2021 were identified. Patients without radiographs available for review, those with known skeletal dysplasia other than OI, and patients with multiple fractures (eg, polytraumas) or with concomitant dislocations were excluded. Of the 701 patients identified, 403 were included for analysis. Demographic variables, mechanism of injury, treatment type, and determination of OI diagnosis were collected. Patients with a previously confirmed diagnosis of OI or with genetic confirmation of OI following their fracture were designated as OI (+), and the remainder were designated OI (-). The Mann-Whitney U and χ2 tests were used to compare groups. RESULTS: Of the 403 patients, the median age was 7.8 years (interquartile range 5.2 to 12.5), and 270 (67%) were male. There were 14 confirmed cases of OI (3.5%). The OI (+) and OI (-) groups did not differ significantly by age or sex (P>0.05). OI (+) patients were more likely to sustain an injury from low-energy mechanisms (86% vs. 32%, P<0.001), sustain displaced fractures (86% vs. 21%, P<0.001) and undergo operative treatment (86% vs. 20%, P<0.001), and to report a history of previous fracture (79% vs. 16%, P<0.001) than OI (-) patients. 36% of OI (+) patients sustained a second olecranon fracture during the study period; there were no subsequent olecranon fractures in the OI (-) group. CONCLUSIONS: Isolated olecranon fractures may not be pathognomonic for OI. However, orthopaedists must be vigilant about the possibility of OI in patients who sustain displaced, isolated olecranon fractures under low-energy mechanisms with a history of previous fracture(s). LEVEL OF EVIDENCE: Level III.

A Modern Day Timeline for In-Hospital Monitoring in Perfused, Pulseless Pediatric Supracondylar Humerus Fractures.

BACKGROUND: The perfused, pulseless supracondylar humerus fracture (ppSCHF) remains a consistent topic of discussion in the literature. Inpatient observation of these patients postoperatively for worsening vascular exam or compartment syndrome is frequently recommended but not well studied. The purpose of this study was to evaluate the postoperative complications in a cohort of ppSCHF patients and their timeline to discharge. METHODS: This study is a retrospective review of a prospectively maintained database of all operatively treated supracondylar humerus fractures from a tertiary pediatric hospital from 2013 to 2019. All patients without a palpable pulse were included. We excluded patients with <4 weeks follow-up. Demographic, operative, and postoperative data were collected, including time from presentation to operating room (OR), time from OR to discharge, and incidence of postoperative complications, including return to OR, compartment syndrome, new neurovascular deficits, and Volkmann contractures. The descriptive statistics were used to summarize the data. RESULTS: Among 1371 operatively treated supracondylar humerus fractures, 39 (2.8%) presented with a ppSCHF. Five (15%) had a signal on doppler ultrasound, whereas 34 (85%) had no signal. Thirty-seven (95%) patients had a Gartland type III fracture and 2 (5%) had type IV fractures. Twenty-two (56%) patients had a neurological deficit, of which 14 had an anterior interosseous nerve deficit. The average time to OR was 6.9 (range 2.2 to 15) hours; 6 (15%) required open reduction. At the time of discharge, 85% of patients had a palpable pulse and 13% had a dopplerable signal. Postoperatively, no patients were returned to the OR for any secondary procedures. The average length of stay after the operation was 25 (range 8.5 to 40) hours, with 92% of patients being discharged by 36 hours postoperatively. No patients developed compartment syndrome, new neurological deficits, or Volkmann contractures at a mean follow-up of 112 (range 34 to 310) days. CONCLUSIONS: In our study of 39 patients presenting with ppSCHF, no patient required an unexpected return to the OR, or developed post-treatment compartment syndrome, neurological deficits, or Volkmann contractures. The average time from OR to discharge for ppSCHF was 25 hours. LEVEL OF EVIDENCE: Level II.

Better Patient Care Through Physician Extenders and Advanced Practice Providers.

Physician extenders and advanced practice providers (APPs) are now common in most adult and pediatric orthopaedic clinics and practices. Their utilization, with physician leadership, can improve patient care, patient satisfaction, and physician satisfaction and work/life balance in addition to having financial benefits. Physician extenders can include scribes, certified athletic trainers, and registered nurses, while APPs include nurse practitioners and physician assistants/associates. Different pediatric orthopaedic practices or divisions within a department might benefit from different physician extenders or APPs based on particular skill sets and licensed abilities. This article will review each of the physician extender and APP health care professionals regarding their training, salaries, background, specific skill sets, and scope of practice. While other physician extenders such as medical assistants, cast technicians, and orthotists/prosthetists have important roles in day-to-day clinical care, they will not be reviewed in this article. In addition, medical trainees, including medical students, residents, fellows, and APP students, have a unique position within some academic clinics but will also not be reviewed in this article. With the many different local, state, and national regulations, a careful understanding of the physician extender and APP roles will help clinicians optimize their ability to improve patient care.

Impact of growth friendly interventions on spine and pulmonary outcomes of patients with spinal muscular atrophy.

PURPOSE: Patients with spinal muscular atrophy (SMA) are often treated with growth friendly devices such as vertical expandable prosthetic titanium rib(VEPTR) and magnetically controlled growing rods(MCGR) to correct spinal deformity and improve pulmonary function. There is limited data on this topic, and the purpose of this study was to assess the effect of these constructs and the addition of chest wall support (CWS) on spinal deformity, thorax morphology and pulmonary outcomes. METHODS: This is a retrospective analysis of prospectively collected data. We included patients with chest wall deformity and scoliosis secondary to SMA who were treated with growth friendly interventions and had two-year follow-up. Descriptive statistics and univariate analyses were performed. RESULTS: This study included 66 patients (25% MCGR, 73% VEPTR, 2% unknown). Approximately 23% of constructs included CWS. The average Cobb angle improved from 67° (SD: 27°) to 50° (SD: 26°) at 2 years in patients with CWS (p = 0.02), and from 59° (SD: 20°) to 46° (SD: 15°) at 2 years in patients without CWS (p < 0.01). Hemithorax height improved in patients treated with and without CWS (p = 0.01), but hemithorax width only improved in patients with CWS (p = 0.01). One patient with CWS and two patients without CWS required additional respiratory support at 2 years. The rates of postoperative complications were not significantly different in patients treated with and without CWS (p = 0.31). CONCLUSIONS: Growth friendly constructs improve spinal deformity and may be effective in altering the progression toward respiratory failure in patients with SMA. Patients treated with CWS have significant improvements in thorax morphology compared to patients without CWS.

Can Rapid Progression in Nonambulatory Cerebral Palsy Scoliosis Be Predicted Using Humeral Head Ossification?

BACKGROUND: Patients with cerebral palsy scoliosis (CPS) experience higher complication rates compared with idiopathic scoliosis and often present for surgery with larger curves. Prediction of an inflection point for rapid deformity progression has proven difficult. A proximal humerus-based skeletal maturity staging system (HS) has been recently validated and is commonly visible on the posteroanterior radiograph. The authors hypothesize that this system can be used to identify a period at which CPS may progress rapidly, perhaps facilitating discussion of timely surgical intervention. METHODS: A retrospective review was conducted for nonambulatory pediatric patients with CPS who presented between 2009 and 2018 at our institution. All patients were considered for inclusion regardless of operative or nonoperative management. Patients who were skeletally mature at initial evaluation or had prior spine surgery were excluded. The authors analyzed radiographs in each HS available. Survival was calculated for cutoffs of 60 and 70 degrees (numbers found to increase intraoperative and postoperative complications for CPS). RESULTS: Eighty-six patients with CPS were identified (54 male individuals). Major curves increased significantly between HS 1 and 2 (27.7 to 46.6 degrees, P=0.009) and HS 3 and 4 (53.1 to 67.9 degrees, P=0.023). The proportion of curves ≥70 degrees were significantly different between HS (P<0.001), with the greatest increase between HS 3 and 4 (24% to 51%; ≥70 degrees). The largest drop in the 60/70-degree survival curves was between HS 3 and 4. In a subanalysis, 69% of patients with curves ≥40 degrees but <70 degrees in stage 3 would progress ≥70 degrees by stage 4. CONCLUSIONS: Identifying a period of rapid curve progression may guide surgical planning before CPS curves become large, stiff, and more difficult to fix. Our findings suggest that humeral skeletal maturity staging is a valuable decision-making tool in neuromuscular scoliosis, with the HS 3 to 4 transition representing the time of the greatest risk of progression. Consider a surgical discussion or shortened follow-up interval for patients with CPS with curves ≥40 degrees who are HS 3. LEVEL OF EVIDENCE: Level II.

Risk of Scoliosis Progression in Nonoperatively Treated Adolescent Idiopathic Scoliosis Based on Skeletal Maturity.

BACKGROUND: Hand radiographs for skeletal maturity staging are now frequently used to evaluate remaining growth potential for patients with adolescent idiopathic scoliosis (AIS). Our objective was to create a model predicting a patient's risk of curve progression based on modern treatment standards. METHODS: We retrospectively reviewed all AIS patients presenting with a major curve <50 degrees, available hand radiographs, and complete follow up through skeletal maturity at our institution over a 3-year period. Patients with growth remaining underwent rigid bracing of curves >25 degrees, whereas patients between 10 and 25 degrees were observed. Treatment success was defined as reaching skeletal maturity with a major curve <50 degrees. Four risk categories were identified based on likelihood of curve progression. RESULTS: Of 609 AIS patients (75.4% female) presenting with curves over 10 degrees and reaching skeletal maturity at most recent follow up, 503 (82.6%) had major thoracic curves. 16.3% (82/503) of thoracic curves progressed into surgical treatment range. The highest risk group (Sanders 1 to 6 and curve 40 to 49 degrees, Sanders 1 to 2 and curve 30 to 39) demonstrate a 30% success rate with nonoperative treatment. This constitutes an 111.1 times (95% confidence interval: 47.6 to 250.0, P<0.001) higher risk of progression to surgical range than patients in the lowest risk categories (Sanders 1 to 8 and curve 10 to 19 degrees, Sanders 3 to 8 and curve 20 to 29 degrees, Sanders 5 to 8 and curve 30 to 39 degrees). CONCLUSIONS: Skeletal maturity and curve magnitude have strong predictive value for future curve progression. The results presented here represent a valuable resource for orthopaedic providers regarding a patient's risk of progression and ultimate surgical risk. LEVEL OF EVIDENCE: Level III-retrospective cohort study.

Rib-based Distraction Device Implantation Before Age 3 Associated With Higher Unplanned Rate of Return to the Operating Room.

BACKGROUND: Surgical treatment of early-onset scoliosis (EOS) with rib-based implants such as the vertical expandable prosthetic titanium rib (VEPTR) is associated with a high rate of complications including surgical site infection, skin breakdown, and implant migration. Many of these complications warrant the need for unplanned reoperations, increasing the burden on an already vulnerable patient population, and introducing the further risk of infection. To provide insight into the risks of early intervention, we investigate the relationship between initial device implantation before the age of 3 and the rate of unplanned reoperation. METHODS: A retrospective review was performed of all patients at a single institution who had undergone VEPTR insertion for EOS with at least a 2-year follow-up from 2007 to 2016. Patients were stratified into the case-cohort (0 to 2 y of age) or the comparison cohort (3 to 10 y of age) based on age at the time of device implantation. Multivariate regression accounting for age and scoliosis etiology was performed to identify factors predictive of unplanned reoperation. RESULTS: A total of 137 of 185 patients treated with VEPTR were identified with 76 (56%) undergoing at least 1 unplanned reoperation during the study time period. There were 68 and 69 patients in the age 0- to 2-year and 3- to 10-year cohorts, respectively. Patients aged 0 to 2 years underwent a higher number of total procedures compared with those aged 3 to 10 (13.1±6.5 vs. 10.6±4.8, P=0.032). A significant difference was found in the rate of unplanned reoperation between the 2 cohorts with 44 (65%) patients aged 0 to 2 and 32 (46%) patients aged 3 to 10 undergoing at least 1 unplanned reoperation (P=0.031). Binary logistic multivariate regression accounting for age and scoliosis etiology demonstrated that patients aged 0 to 2 had a significantly greater odds of undergoing an unplanned reoperation (odds ratio=3.050; 95% confidence interval: 1.285-7.241; P=0.011) compared with patients aged 3 to 10 years. CONCLUSION: Overall, EOS patients aged 0 to 2 at initial VEPTR implantation are up to 3 times higher risk of undergoing an unplanned reoperation compared with those aged 3 to 10. LEVEL OF EVIDENCE: Level III.

Hand Bone Age Radiography: Comparison Between Slot-scanning and Conventional Techniques.

BACKGROUND: Determination and longitudinal monitoring of progressive skeletal maturity are essential in the management of children with scoliosis. Although different methods for determining skeletal maturity exists, the most widely practiced method relies on the ossification pattern of the bones of the hand and wrist, which is traditionally acquired using conventional techniques and after the acquisition of the spine using the low-dose slot-scanning technique. Whereas the existing published literature has published promising results on the use of the slot-scanning technique to acquire these hand and wrist radiographs, image quality and radiation dose have not been systematically compared between these techniques. Thus, the objective of our study is to compare image quality, interpretation reliability, and radiation dose of hand bone age radiographs between slot-scanning and conventional techniques using age- and sex-matched children. METHODS: This retrospective study included children who underwent hand radiographs using slot-scanning between October 1, 2019 and December 31, 2019; and matched children who underwent conventional radiography. Blinded to technique, 5 readers reviewed all radiographs after randomization to rate image quality and to determine bone age using the Greulich and Pyle classification. Dose area product was recorded. Mann-Whitney and t tests were used to compare variables between techniques and intraclass correlation (ICC) to determine observer agreement. RESULTS: Our study cohort of 194 children (128 girls, 66 boys; mean age: 13.7±2.3 y) included 97 slot-scanning and 97 conventional radiographs. One (1%) slot-scanning and no conventional radiograph was rated poor in image quality. There was almost perfect interpretation reliability with slot-scanning with high interobserver (ICC=0.948) and intraobserver (ICC=0.996) agreements, comparable with conventional radiographs (ICCs=0.919 and 0.996, respectively). Dose area product (n=158) was lower (P<0.002) with slot-scanning than with conventional techniques. CONCLUSION: Almost perfect interobserver reliability and intraobserver reproducibility with slot-scanning radiographs (performed using significantly lower radiation doses) suggest that this technique for hand bone age determination can be a reliable adjunct to scoliosis monitoring. LEVEL OF EVIDENCE: Level III.

Outcomes and Complications in Management of Congenital Myopathy Early-Onset Scoliosis.

BACKGROUND: Congenital myopathies (CMs) are complex conditions often associated with early-onset scoliosis (EOS). The purpose of this study was to investigate radiographic outcomes in CM patients undergoing EOS instrumentation as well as complications. Secondarily, we sought to compare these patients to a population with higher prevalence, cerebral palsy (CP) EOS patients. METHODS: This is a retrospective study of a prospectively collected multicenter registry. The registry was queried for EOS patients with growth-sparing instrumentation (vertical expandable prosthetic titanium ribs, magnetically controlled growing rods, traditional growing rod, or Shilla) and a CM or CP diagnosis with minimum 2 years follow-up. Outcomes included major curve magnitude, T1-S1 height, kyphosis, and complications. RESULTS: Sixteen patients with CM were included. Six (37.5%) children with CM experienced 11 complications by 2 years. Mean major curve magnitude for CM patients was improved postoperatively and maintained at 2 years (P<0.01), with no significant increase in T1-S1 height or maximum kyphosis(P>0.05). Ninety-seven patients with CP EOS were included as a comparative cohort. Fewer CP patients required baseline respiratory support compared with CM patients (20.0% vs. 92.9%, P<0.01). Fifty-four (55.7%) CP patients experienced a total of 105 complications at 2 years. There was no evidence that the risk of complication or radiographic outcomes differs between cohorts at 2 years, though CP EOS patients experienced significant improvement in all measurements at 2 years. CONCLUSIONS: EOS CM children face a high risk of complication after growing instrumentation, with similar curve correction and risk of complication to CP patients. LEVEL OF EVIDENCE: Level III.

Mistakes Made and Lessons Learned: A Mid-Career Pediatric Orthopaedic Surgeon's Journey to Sustain Energy and Avoid Burnout.

INTRODUCTION: Twenty-three years into this journey as a pediatric orthopaedic surgeon, my path is littered with mistakes made. The collision of a "family-first" philosophy with the demands of my busy academic surgical practice-not pretty. As a canary in a toxic coal mine, I have failed to fix the mine, so I have focused on being the very strongest and resilient canary possible. METHODS: This is a retrospective study of mistakes made and lessons learned. The mistakes include submitting to the tyranny of perfectionism; failure to resist the tendency to measure ourselves against others; saying "yes" to every request; thinking my job is Monday to Friday, and career is nights/weekends; being given a big new job, but using same old time/energy management strategy; sloppy time management; failure to manage energy; considering sleep "a waste of time;" failure to manage attention; dragging the challenges from my day through the door each night; thinking that the skills that make me good at work are same that will make me good at home; aiming for work-life "balance;" and sometimes losing sight of our mission. RESULTS: It is too soon to tell whether the lessons I have learned from all my mistakes are essential to preventing burnout. Although I am certain my results to date are significant, I cannot prove statistically significance. I am working to develop Appropriate Use Criteria for time and energy, and Best Practice Guidelines for prioritizing where to focus attention in life. DISCUSSION: We need to manage our energy, time, and priorities with great care, and learn from the experts who are trying to teach corporate executives and professional athletes how to function in a high-pressure environment. CONCLUSIONS: Pediatric orthopaedics is one of the most wonderful professional callings. We can and should be happy and resilient canaries. The owners of the coal mine should hear us singing sweet songs about the children we care for even as they make the air increasingly toxic.

The Impact of Posterior Spinal Fusion (PSF) on Coronal Balance in Adolescent Idiopathic Scoliosis (AIS): A New Classification and Trends in the Postoperative Period.

INTRODUCTION: One of the primary goals of scoliosis surgery is to balance the head over the pelvis (or avoid creating imbalance). Historically, a normal coronal balance was defined as the C7 plumb line (C7PL) within 2 cm of the central sacral vertical line (CSVL); however, there is limited published information regarding the speed/magnitude and success/failure of balancing, rebalancing, or unbalancing in the postoperative period. The purpose of this study is to classify and quantify coronal plane balance postoperatively in adolescent idiopathic scoliosis when using pedicle screw instrumentation. METHODS: Evaluated patients with adolescent idiopathic scoliosis who had a posterior spinal fusion withstanding 2-view, posteroanterior and lateral radiographs of the entire spine performed at first erect visit, 6, 12, and 24 months. To measure coronal balance, a C7PL was measured and compared with the CSVL. A negative value denotes the leftward deviation of the C7PL and a positive value a rightward deviation. The authors then created a novel coronal balance classification system. RESULTS: A total of 954 patients met the inclusion criteria. There was a strong trend toward improving coronal balance, especially between first erect and 6 months; the proportion of out of balance patients declined throughout the 2-year period: preoperative 372 of 954 (39%), first erect 297 of 954 (31.1%), 6 months 167 of 954 (17.5%), 1 year 136 of 954 (14.3%), and 2 years 115 of 954 (12.0%). Analyzing the patients most out of balance immediately after posterior spinal fusion, 35 of 50 (70%) in group 3 regained balance by 2 years. Out of the remaining 15 patients, 12 corrected to group 1 (24%), 2 patients to group 2 (4%), and 1 patient remained in group 3 (2%). CONCLUSIONS: This large, longitudinal postoperative study of coronal balance documents a strong trend toward postoperative rebalancing, with the largest gains between first erect image and 6 months. The 31% of patients out of balance at first erect declined to only 12.1% at 2 years.

Expert Consensus for a Principle-based Classification for Treatment of Diaphyseal Pediatric Femur Fractures.

INTRODUCTION: Recent studies demonstrate considerable deviation from the American Academy of Orthopaedic Surgeons (AAOS) evidence-based guidelines for the treatment of pediatric diaphyseal femur fractures (PDFFs). This study aimed to determine if expert-consensus can be reached on a principle-based classification to be applied broadly to a wide variety of PDFF scenarios and if outcomes correspond to adherence to the classification. METHODS: A 2-stage study was performed. First, a survey of experts using a principle-based approach to PDFF. We conducted a survey of 17 thought-leaders (criteria≥20 y' experience+authors of the seminal pediatric femur fracture studies) who were asked to classify 15 cases of PDFF using the principle-based classification for agreement. Next, we conducted a retrospective review of 289 consecutive PDFF treated (2011-2015) at a level 1 pediatric trauma center. For each case, we compared the actual treatment and proposed "ideal" principle-based classification. We then compared clinical results and outcome data points including the length of stay, physician visits, and hospital charge data. RESULTS: A substantial (κ=0.7) expert-agreement was noted for assigning treatment principles with near-perfect (κ=0.93) agreement on conservative versus surgical management. We obtained agreement on employing a flexible implant (κ=0.84) rigid fixation (κ=0.75) and damage control philosophy (κ=0.64). Suboptimal clinical results were noted in 43% of the undertreated patients (24/56), 18.8% of the adequately treated, and 14.3% of overtreated (P<0.01) patients. An increasing trend for the length of hospital stay and a number of clinic visits was noted as the treatment class increased (P<0.01). Charges were 4.2 times higher for an episode of operative versus nonoperative care (P<0.01). Rigid fixation (class 4) had significantly (P=0.01) higher total and material charges than flexible fixation (class 3). DISCUSSION: The proposed classification has a substantial agreement among thought-leaders. Clinical results demonstrated significantly more suboptimal results in undertreated fractures, compared with ideally treated or more invasively treated fractures. More invasive treatments led to increased burden to families and the system in terms of length of stay and hospital charges. LEVEL OF EVIDENCE: Level III.