Preoperative Echocardiogram Does Not Contribute to Surgical Risk Assessment in Patients With Large Curve Scoliosis and No Cardiac Risk Factors.

OBJECTIVE: Severe scoliosis can affect thoracic organs, potentially leading to cardiovascular abnormalities. Thus, echocardiograms have been suggested for use in preoperative screening in patients with significant scoliosis. However, the utility of preoperative heart screenings in patients without known or suspected heart problems is not well understood. This study aims to find the incidence of cardiac findings in patients with severe scoliosis ≥90° without cardiac history. METHODS: A single-institution retrospective chart review was performed. Inclusion criteria were scoliosis patients with curves ≥90 degrees and a screening echocardiogram performed within 6 months of spine surgery. Patients with a previous cardiac history, diagnosis associated with cardiac comorbidities (eg, connective tissue disease), or major coronal curves <90 degrees were excluded. Echocardiogram reports and perioperative clinical notes from involved services (including orthopaedics, cardiology, and anesthesia) were reviewed. Any postoperative use of vasopressors and reasons for their use were recorded. RESULTS: Overall, 50 patients met the inclusion criteria. The mean age at surgery was 14.0 ± 4.9 years old (range: 2 to 33). The mean major curve was 108 ± 19 degrees (range: 90 to 160 degrees). A normal echocardiogram was seen in 38 (76%), whereas 6 patients (12%) had mild dilation of the aortic sinus or root, 4 (8%) had mild valvular regurgitation, 1 patient had a small atrial septal defect, and 1 had a trace pericardial effusion. No patient had any changes made to their perioperative plan and one patient was advised to see a cardiologist postoperatively. Postoperatively, 8 patients (16%) received vasopressors to raise blood pressure to meet preset goal MAP, but only one of these 8 had a positive echocardiogram (mild valvular insufficiency), which was not seen as a contributing factor to the use of pressors. CONCLUSIONS: This study suggests that screening echocardiograms for patients without a cardiac history or related symptoms does not contribute to the evaluation of perioperative risk or anesthetic management. Creating clear, evidence-based guidelines for the utilization of perioperative testing, like echocardiograms, can reduce the social, time, and financial burdens on families. Such guidelines are vital for appropriate risk assessment and proper utilization of health care resources. LEVEL OF EVIDENCE: Level III.

A New Look at Vertebral Body Tethering (VBT): Through the Modified Clavien-Dindo-Sink (mCDS) Classification.

INTRODUCTION: Anterior vertebral body tethering (AVBT) is increasingly popular as an option for surgical treatment of idiopathic scoliosis (IS). While the technology remains new, it is important for families and patients to be able to compare it to the current standard of care, posterior spinal fusion (PSF). The purpose of this study is to describe the complication rate of AVBT in IS using the mCDS and to compare it to the recently reported complication rate of PSF in IS. METHODS: A multicenter pediatric spine deformity database was queried for all idiopathic scoliosis patients who underwent vertebral body tethering. There were 171 patients with a minimum 9-month follow-up included in this study. Complications were retrospectively graded by 2 attending pediatric spine surgeons using the mCDS classification system. RESULTS: Data from 171 patients with idiopathic scoliosis was available for analysis, with 156/171 (91%) of patients being female and an average age of 12.2 years old at surgery. There were 156 thoracic tethers (1 with an LIV below L2), 5 lumbar tethers, 9 staged double tethers, and only 1 patient with same-day double tether. Fifty-five (55) (32%) patients experienced a total of 69 complications. The most common complication type for VBT by mCDS was Grade IIIb, encompassing 29/69 (42%) of complications. The second most frequent complication grade was Grade I at 23/69 (33%). Thirty-four (34) out of 69 (49%) of the VBT complications reported required either procedural/surgical intervention or admission to the ICU. CONCLUSIONS: This is the first study to directly compare the complication profile of VBT to PSF using the mCDS. Forty-nine percent (49%) of the VBT complications reported were at least Grade III, while only 7% of complications in the control PSF cohort from the literature were Grade III or higher. The mCDS complication classification brings light to the early learning experience of a new technique compared to the widely accepted standard of PSF for IS. LEVEL OF EVIDENCE: III - Retrospective comparative study.

Institutional Variability in Anesthesia Time for Mehta Casting in Early-Onset Scoliosis (EOS).

PURPOSE: Mehta casting is a potentially curative intervention for early-onset scoliosis (EOS) that typically requires multiple anesthetics. The Food and Drug Administration (FDA) reported that >3 hours of anesthesia under the age of 3 years old may alter brain development; however, no standard exists for the duration of anesthesia during casting. The purpose of this study is to quantify the variability in anesthesia during Mehta casting. We hypothesize that significant institutional variability exists and may be attributed to modifiable factors. METHODS: An EOS registry was used to identify patients who underwent at least one Mehta casting procedure. Anesthesia exposure was quantified, and site variability was assessed by patient characteristics, cast placement, procedure type, and equipment used. RESULTS: Our cohort consisted of 208 patients from 5 institutions (age 2.6±1.4 y). There were 1097 Mehta casting procedures, with 5.4±3.6 castings per patient. Of these patients, 106 (51%) were female, with an average age of 2.11±1.29 years old at the time casting was initiated. Patient etiologies included 154 idiopathic (74.0%), 22 syndromic (10.6%), 18 congenital (8.7%), 11 neuromuscular (5.3%), and 3 unknown (1.4%). Anesthesia time was 69±31 minutes and varied significantly between sites (59±14 to 117±46 min; P <0.001). Cumulative anesthesia time for patients under 3 years was 320±197 minutes with 120/161 (74.5%) patients exceeding 3 hours. Anesthesia time was lower after the FDA warning in 2016 compared with pre-2016 (71±30 vs. 66±32, P =0.008). CONCLUSIONS: Patients undergoing Mehta casting are at significant risk of exceeding 3 hours of anesthesia, which the FDA has stated may be harmful for children <3 years. Significant site variability indicates that standardization protocols should be developed to encourage best practices and minimize anesthetic times. LEVEL OF EVIDENCE: Prognostic Level II.

Reliability of Preoperative Supine Versus Bending Radiographs in Estimating the Structural Nature of Curves in EOS.

BACKGROUND: The study sought to evaluate the utility of a single supine radiograph in determining curve flexibility in early-onset scoliosis (EOS) patients. METHODS: EOS patients with upright (standing/seated), supine, and side-bending radiographs who underwent spinal deformity surgery were identified. Coronal parameters included: proximal thoracic (PT) curve, main thoracic (MT) curve, and thoracolumbar/lumbar (TL/L) curve. Each radiograph was measured twice by 2 different raters. Correlation coefficients were utilized to investigate associations between the different radiographs. Interrater Correlation Coefficient (ICC) assessed intrarater and interrater reliability. RESULTS: Thirty-seven EOS patients were identified (age at diagnosis: 7.0±2.9 y, preoperative age: 13.0±2.9 y; 73% female; etiologies: 54% idiopathic, 30% syndromic, and 16% neuromuscular). Supine PT and MT curve measurements were highly associated with corresponding side-bending measurements (PT: r=0.75, P<0.001; MT: r=0.80, P<0.001), and TL/L curves were very highly associated (TL/L: r=0.92, P<0.001). The mean absolute differences between supine and side-bending measurements were PT: 11.3±7.8 degrees, MT: 14.8±8.3 degrees, and TL/L: 16.2±7.6 degrees, where the side-bending was on average smaller than the supine measurement. The intrarater reliabilities were excellent, with an ICC ranging from 0.93 to 0.96 for side-bending films and 0.94 to 0.97 for supine films. The interrater reliability was excellent with ICC value of 0.88 for side-bending films and 0.93 for supine films. CONCLUSIONS: A single, preoperative supine radiograph was highly predictive of side-bending radiographs in patients with EOS. Supine curves measured an average of 15 degrees larger than bending curves in the MT and TL/L region. A single supine film may eliminate the need for effort-related, dual side-bending radiographs. LEVEL OF EVIDENCE: Level II-retrospective study.

Definition of Tweener: Consensus Among Experts in Treating Early-onset Scoliosis.

BACKGROUND: The term "Tweener" is colloquially used to refer to early-onset scoliosis (EOS) patients whose age and development make them candidates for multiple surgical options. The purpose of this study was to establish expert consensus on a definition to formally characterize the Tweener population. METHODS: A 3-round survey of surgeons in an international EOS study group was conducted. Surgeons were provided with various patient characteristics and asked if each was part of their definition for Tweener patients. Responses were analyzed for consensus (≥70%), near-consensus (60% to 69%), and no consensus (<60%). RESULTS: Consensus was reached (89% of respondents) for including chronological age in the Tweener definition; 8 to 10 years for females and 9 to 11 years for males. Surgeons agreed for inclusion of Sanders score, particularly Sanders 2 (86.0%). Patients who have reached Sanders 4, postmenarche, or have closed triradiate cartilage should not be considered Tweeners. Bone age range of 8 years and 10 months to 10 years and 10 months for females (12 y for males) could be part of the Tweener definition. CONCLUSIONS: This study suggests that the Tweener definition could be the following: patients with open triradiate cartilage who are not postmenarche and have not reached Sanders 4, and if they have one of the following: Sanders 2 or chronological age 8 to 10 years for females (9 to 11 y for males) or bone age 8 years and 10 months to 10 years and 10 months for females (12 y for males). This definition will allow for more focused and comparative research on this population. LEVEL OF EVIDENCE: Level V-expert opinion.

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.

Degree of Postoperative Curve Correction Decreases Risks of Postoperative Pneumonia in Patients Undergoing Both Fusion and Growth-friendly Surgical Treatment of Neuromuscular Scoliosis.

INTRODUCTION: Due to a combination of poor respiratory muscle control and mechanical lung compression secondary to spine and chest wall deformities, patients with neuromuscular (NM) early-onset scoliosis (EOS) are at a high risk for pulmonary complications including pneumonia. The purpose of this study is to examine the effect of surgical intervention on the prevalence and risk of postoperative pneumonia in patients with NM EOS. METHODS: In this retrospective cohort study, pediatric (18 y old and below) patients with NM EOS undergoing index fusion or growth-friendly instrumentation from 2000 to 2018 were identified. Patients were then categorized into 2 groups: those with ≥50% curve correction and those with <50% curve correction of the coronal deformity at the first postoperative visit. The primary outcome of interest was postoperative pneumonia occurring between 3 weeks and 2 years postoperatively. Manual chart review was supplemented with phone call surveys to ensure all occurrences of preoperative/postoperative pneumonia (ie, in-institution and out-of-institution visits) were accounted for. RESULTS: A total of 35 patients (31% female, age at surgery: 10.3±4.3 y) with NM EOS met inclusion criteria. Twenty-three (66%) patients experienced at least 1 case of preoperative pneumonia. Twenty-six (74%) patients had ≥50% and 9 (26%) patients had <50% immediate postoperative curve correction. In total, 12 (34%) patients experienced at least 1 case of postoperative pneumonia (7 in-institution, 5 out-of-institution). Seven (27%) patients with ≥50% curve correction versus 5 (56%) with <50% curve correction experienced postoperative pneumonia. Relative risk regression demonstrated that patients with <50% curve correction had increased risk of postoperative pneumonia by 2.1 times compared with patients with ≥50% curve correction (95% confidence interval: 0.9; 4.9, P =0.099). CONCLUSION: The prevalence of preoperative and postoperative pneumonia is high in patients with NM EOS. This study presents preliminary evidence suggesting that percent curve correction is associated with the occurrence of postoperative pneumonia in patients with NM EOS undergoing surgical correction.

Mortality in Neuromuscular Early Onset Scoliosis Following Spinal Deformity Surgery.

BACKGROUND: The purpose of this study was to report mortality and associated risk factors in neuromuscular early onset scoliosis following spinal deformity surgery. METHODS: This is a multicenter retrospective cohort study of patients with cerebral palsy (CP), spinal muscular atrophy, myelodysplasia, muscular dystrophy, or myopathy undergoing index spine surgery from 1994 to 2020. Mortality risk was calculated up to 10 years postoperatively. Proportional hazard modeling was utilized to investigate associations between risk factors and mortality rate. RESULTS: A total of 808 patients [mean age 7.7 y; 439 (54.3%) female] were identified. Postoperative 30-day, 90-day, and 120-day mortality was 0%, 0.001%, and 0.01%, respectively. 1-year, 2-year, 5-year, and 10-year mortality was 0.5%, 1.1%, 5.4%, and 17.4%, respectively. Factors associated with increased mortality rate: CP diagnosis [hazard ratio (HR): 3.14, 95% confidence interval (CI): 1.71; 5.79, P<0.001]; nonambulatory status (HR: 3.01, 95% CI: 1.06; 8.5, P=0.04)]; need for respiratory assistance (HR: 2.17, 95% CI: 1.00; 4.69, P=0.05). CONCLUSIONS: In neuromuscular patients with early onset scoliosis, mortality risk at 10 years following spine surgery was 17.4%. As mortality was 1.1% at 2 years, premature death was unlikely a direct result of spine surgery. Diagnosis (CP) and markers of disease severity (nonambulatory status, respiratory assistance) were associated with increased mortality rate. LEVEL OF EVIDENCE: Prognostic level II.

The Impact of Unplanned Return to the Operating Room on Health-related Quality of Life at the End of Growth-friendly Surgical Treatment for Early-onset Scoliosis.

BACKGROUND: Limiting complications, especially unplanned return to the operating room (UPROR), is a major focus in the surgical management of early-onset scoliosis (EOS). Although UPROR remains common in this population, its effect on long-term health-related quality of life (HRQoL) remains unclear. The purpose of this study was to investigate the association between UPROR and end-of-treatment HRQoL in EOS patients treated with growth-friendly instrumentation. METHODS: Patients with EOS who underwent growth-friendly instrumentation at age less than 10 years from 1993 to 2018, and completed treatment, were identified in a multicenter EOS registry. UPROR events were recorded, and end-of-treatment (defined as skeletal maturity and/or definitive spinal fusion) HRQoL was assessed via the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24). RESULTS: A total of 825 patients were identified, and 325 patients (age at surgery: 6.4 y, follow-up: 8.1 y) had end-of-treatment HRQoL data necessary for our investigation. Overall, 129/325 (39.7%) patients experienced 264 UPROR events; the majority (54.2%) were implant-related. Aside from age and etiology, no other variables were determined to be confounders or effect modifiers. Congenital patients with UPROR had worse pain/discomfort by 10.4 points (P=0.057) and worse pulmonary function by 7.8 points (P=0.102) compared with non-UPROR patients adjusting for age. Neuromuscular patients with UPROR had worse pulmonary function by 10.1 points compared with non-UPROR patients adjusting for age (P=0.037). Idiopathic and syndromic patients with UPROR reported consistently worse domain scores than their non-UPROR counterparts, but smaller (<5-point) differences were seen. CONCLUSIONS: UPROR during growth-friendly surgical treatment for EOS is associated with worse HRQoL in all patients, but particularly in those with neuromuscular or congenital etiologies. Ongoing efforts to avoid UPROR are critical. LEVEL OF EVIDENCE: Level II. This is a multicenter retrospective cohort study investigating the effect of UPROR on HRQoL (prognostic study).

The Effect of Surgeon Experience on Outcomes Following Growth Friendly Instrumentation for Early Onset Scoliosis.

BACKGROUND: The purpose of this study was to utilize a multicenter, multisurgeon cohort to assess the effect of surgeon experience on outcomes of growth friendly instrumentation (GFI) in early onset scoliosis (EOS). We hypothesized that unplanned return to the operating room (UPROR), estimated blood loss (EBL), and surgical time would be greater amongst early career surgeons (ECSs) when compared with advanced career surgeons (ACSs). METHODS: An international pediatric spine database was queried for patients ages 2 to 10 years treated by posterior distraction-based GFI with at least of 2-year follow up. Two groups were created for analysis based on surgeon experience: ECSs (with ≤10 y of experience) and ACSs (with >10 y of experience). The primary outcome was UPROR. Additional outcomes included: operating room time, EBL, neurological deficits, infection rate, hardware failure, and the Early Onset Scoliosis Questionnaire (EOSQ-24). Subgroup analysis was performed for further assessment based on procedure type, superior anchor type, etiology, and curve severity. RESULTS: A total of 960 patients met inclusion criteria including 243 (25.3%) treated by ECS. Etiology, sex, superior anchor, and EOSQ-24 scores were similar between groups (P>0.05). There were no clinically significant differences in patient age or preoperative major coronal curve. UPROR (35.8% vs. 32.7%, P=0.532), infection (17.0% vs. 15.6%, P=0.698), operating room time (235 vs. 231 min, P=0.755), and EBL (151 vs. 155 mL, P=0.833) were comparable between ECS and ACS groups. The frequency of having at least 1 complication was relatively high but comparable among groups (60.7% vs. 62.6%, P=0.709). EOSQ-24 subdomain scores were similar between groups at 2-year follow-up (P>0.05). Subgroup analysis revealed that ECS had increased surgical time compared with ACS in severe curves >90 degrees (270 vs. 229 min, P=0.05). CONCLUSIONS: This study represents the first multicenter assessment of surgeon experience on outcomes in EOS. Overall, surgeon experience did not significantly influence UPROR, complication rates, EBL, or surgical time associated with GFI in this cohort of EOS patients. LEVEL OF EVIDENCE: Level III.

Uncorrected Pelvic Obliquity Is Associated With Worse Health-related Quality of Life (HRQoL) in Children and Their Caregivers at the End of Surgical Treatment for Early Onset Scoliosis (EOS).

BACKGROUND: While largely of concern in nonambulatory patients, pelvic obliquity (PO) can be found in many patients with early onset scoliosis (EOS) and may remain following surgery. However, its association with health-related quality of life (HRQoL) in children and their caregivers at the end of treatment is not well understood. The purpose of this study was to investigate the association between residual PO and HRQoL in children and their caregivers at the end of surgical treatment in ambulatory patients with EOS. METHODS: In this retrospective cohort study, a multicenter EOS registry was queried to identify ambulatory patients who underwent definitive posterior spinal fusion (PSF) from 2012 to 2019. Patients with fusions extending to the pelvis were excluded. PO was measured at least 1 year following PSF. HRQoL, Parental Burden, Financial Burden, and Satisfaction were assessed through the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) also at a minimum of 1 year following PSF. RESULTS: A total of 155 patients (12.5±2.1 y, 73.5% female) were included. Etiology distribution was 30.3% congenital, 12.9% neuromuscular, 21.3% syndromic, and 35.5% idiopathic. In congenital patients, those with residual PO >8 degrees had worse Satisfaction by 23.2 points compared with those with PO ≤8 degrees. In neuromuscular patients, those with residual PO >7 degrees had worse HRQoL by 16.1 points and Parental Burden by 22.3 points compared with their counterparts. In syndromic patients, those with residual PO >8 degrees had worse HRQoL by 14.8 points, Parental Burden by 16.4 points, and Satisfaction by 21.2 points compared with their counterparts. In idiopathic patients, those with >9 degrees of residual PO had worse HRQoL by 15.0 points and Financial Burden by 26.8 points compared with their counterparts. CONCLUSIONS: Remaining PO at the end of surgical treatment is associated with worse HRQoL in ambulatory children and their caregivers. These results suggest that correction of PO should remain a primary goal of treatment in patients with EOS undergoing surgery. LEVEL OF EVIDENCE: Level II-multicenter retrospective cohort study investigating prognosis.

Mortality in Early-Onset Scoliosis During the Growth-friendly Surgery Era.

INTRODUCTION: Early-onset scoliosis (EOS) is a spinal deformity that occurs in patients 9 years of age or younger. Severe deformity may result in thoracic insufficiency, respiratory failure, and premature death. The purpose of this study is to describe the modern-day natural history of mortality in patients with EOS. METHODS: The multicenter Pediatric Spine Study Group database was queried for all patients with EOS who are deceased, without exclusion. Demographics, underlying diagnoses, EOS etiology, operative and nonoperative treatments or observation, complications, and date of death were retrieved. Descriptive statistics and survival analysis with Kaplan-Meier curves were performed. RESULTS: There were 130/8009 patients identified as deceased for a registry mortality rate of 16 per 1000 patients. The mean age at death was 10.6 years (range: 1.0 to 30.2 y) and the most common EOS etiology was neuromuscular (73/130, 56.2%; P<0.001). Deceased patients were more likely be treated operatively than nonoperatively or observed (P<0.001). The mean age of death for patients treated operatively (12.3 y) was older than those treated nonoperatively (7.0 y) or observed (6.3 y) (P<0.001) despite a larger deformity and similar index visit body mass index and ventilation requirements. Kaplan-Meier analysis confirmed an increased survival time in patients with a history of any spine operation compared with patients without a history of spine operation (P<0.0001). Operatively treated patients experienced a median of 3.0 complications from diagnosis to death. Overall, cardiopulmonary related complications were the most common (129/271, 47.6%; P<0.001), followed by implant-related (57/271, 21.0%) and wound-related (26/271, 9.6%). The primary cause of death was identified for 78/130 (60.0%) patients, of which 57/78 (73.1%) were cardiopulmonary related. CONCLUSIONS: This study represents the largest collection of EOS mortality to date, providing surgeons with a modern-day examination of the effects of surgical intervention to better council patients and families. Both fatal and nonfatal complications in children with EOS are most likely to involve the cardiopulmonary system. LEVEL OF EVIDENCE: Level IV-therapeutic.

Best Practice Guidelines for Surgical Site Infection in High-risk Pediatric Spine Surgery: Definition, Prevention, Diagnosis, and Treatment.

BACKGROUND: Prior "best practice guidelines" (BPG) have identified strategies to reduce the risk of acute deep surgical site infection (SSI), but there still exists large variability in practice. Further, there is still no consensus on which patients are "high risk" for SSI and how SSI should be diagnosed or treated in pediatric spine surgery. We sought to develop an updated, consensus-based BPG informed by available literature and expert opinion on defining high-SSI risk in pediatric spine surgery and on prevention, diagnosis, and treatment of SSI in this high-risk population. MATERIALS AND METHODS: After a systematic review of the literature, an expert panel of 21 pediatric spine surgeons was selected from the Harms Study Group based on extensive experience in the field of pediatric spine surgery. Using the Delphi process and iterative survey rounds, the expert panel was surveyed for current practices, presented with the systematic review, given the opportunity to voice opinions through a live discussion session and asked to vote regarding preferences privately. Two survey rounds were conducted electronically, after which a live conference was held to present and discuss results. A final electronic survey was then conducted for final voting. Agreement ≥70% was considered consensus. Items near consensus were revised if feasible to achieve consensus in subsequent surveys. RESULTS: Consensus was reached for 17 items for defining high-SSI risk, 17 items for preventing, 6 for diagnosing, and 9 for treating SSI in this high-risk population. After final voting, all 21 experts agreed to the publication and implementation of these items in their practice. CONCLUSIONS: We present a set of updated consensus-based BPGs for defining high-risk and preventing, diagnosing, and treating SSI in high-risk pediatric spine surgery. We believe that this BPG can limit variability in practice and decrease the incidence of SSI in pediatric spine surgery. LEVEL OF EVIDENCE: Not applicable.

Spinal fusion in pediatric patients with marfan syndrome: a nationwide assessment on short-term outcomes and readmission risk.

PURPOSE: The purpose of this study was to utilize the National Readmission Database (NRD) to determine estimates for complication rates, 90-day readmission rates, and hospital costs associated with spinal fusion in pediatric patients with Marfan syndrome. METHODS: The 2012-2015 NRD databases were queried for all pediatric (< 19 years old) patients diagnosed with Marfan syndrome undergoing spinal fusion surgery. The primary outcome variables in this study were index admission complications and 90-day readmissions. RESULTS: A total of 249 patients with Marfan syndrome underwent spinal fusion surgery between 2012 and 2015 (mean age ± standard deviation at the time of surgery: 14 ± 2.0, 132 (53%) female). 25 (10.1%) were readmitted within 90 days of the index hospital discharge date. Overall, 59.7% of patients experienced at least one complication during the index admission. Unplanned 90-day readmission could be predicted by older age (odds ratio 2.3, 95% confidence interval 1.3-4.2, p = 0.006), Medicaid insurance status (56.0, 3.8-820.0, p = 0.003), and experiencing an inpatient medical complication (42.9, 4.6-398.7, p = 0.001). Patients were readmitted for wound dehiscence (8 patients, 3.2%), nervous system related complications (3 patients, 1.2%), and postoperative infectious related complications (4 patients, 1.6%). CONCLUSION: This study is the first to demonstrate on a national level the complications and potential risk factors for 90-day hospital readmission for patients with Marfan syndrome undergoing spinal fusion. Patients with Marfan syndrome undergoing spinal fusion often present with multiple medical comorbidities that must be managed carefully perioperatively to reduce inpatient complications and early hospital readmissions.

High Risk of Mismatch Between Sanders and Risser Staging in Adolescent Idiopathic Scoliosis: Are We Guiding Treatment Using the Wrong Classification?

BACKGROUND: Despite known limitations, Risser staging has traditionally been the primary marker of skeletal maturity utilized in decision-making for treatment of adolescent idiopathic scoliosis (AIS). The purpose of this study is to assess the incidence and factors associated with mismatch between Risser Staging and Sanders classification, and determine interobserver reliability. METHODS: We reviewed the medical records of consecutive patients aged 10 to 18 referred to our institution for evaluation of AIS from January to June 2016 with a closed triradiate cartilage. Data collected included sex, age, race, height, weight, body mass index percentile, menarchal status, Risser stage, Sanders classification, and major curve. Risser and Sanders stage was determined by 2 fellowship-trained pediatric spine surgeons and 1 pediatric orthopaedic nurse practitioner. Mismatch was defined as Risser stage 2 to 4 corresponding to Sanders 3 to 5, and Risser 0 to 1 corresponding to Sanders 6 to 7. RESULTS: A total of 165 consecutive patients were identified (mean age: 13.9±1.7 y, major curve 28.2±15.4 degrees, 76% female). The risk of skeletal maturity mismatch, based on the criteria of Risser 2 to 5 (limited growth remaining) corresponding to Sanders 3 to 5 (significant growth remaining) was 21.8%, indicating that 1 of 5 patients would be undertreated if managed by Risser criteria. Conversely, the mismatch risk for Risser 0 to 1 corresponding to Sanders 6 to 7 was 3.6%, leading such patients to be treated conservatively longer than necessary. Males and those of Hispanic ethnicity were at a higher risk of mismatch (23.1% vs. 11.9%, P=0.08; 33.3% vs. 8.8%, P=0.04, respectively). Body mass index percentile, race, and major curve were not associated with mismatch. The unweighted and weighted interobserver κ for Risser staging was 0.74 and 0.82, respectively, and 0.86 and 0.91 for Sanders classification, respectively. CONCLUSION: Given the limited sensitivity of Risser staging during peak growth velocity, high mismatch risk, and lower interobserver reliability, the Sanders classification should be utilized to guide treatment options in patients with AIS. Compared with Sanders, utilizing Risser staging results in mistreatment in a total of 1 of 4 patients, with the vast majority being undertreated. LEVEL OF EVIDENCE: Level II.

The Association Between the Utilization of Traction and Postoperative Complications Following Growing Rod Instrumentation for Early-onset Scoliosis.

BACKGROUND: Preoperative and/or intraoperative traction have been proposed as adjunctive methods to limit complications associated with growth-friendly instrumentation for early-onset scoliosis (EOS). By gradually correcting the deformity before instrumentation, traction can, theoretically, allow for better overall correction without the complications associated with the immediate intraoperative correction. The purpose of this multicenter study was to investigate the association between preoperative/intraoperative traction and complications following growth-friendly instrumentation for EOS. METHODS: Patients with EOS who underwent growth rod instrumentation before 2017 were identified from 2 registries. Patients were divided into 2 groups: preoperative traction group versus no preoperative traction group. A subgroup analysis was done to compare intraoperative traction only versus no traction. Data was collected on any postoperative complication from implantation to up to 2 years postimplantation. RESULTS: Of 381 patients identified, 57 (15%) and 69 (18%) patients received preoperative and intraoperative traction, respectively. After adjusting for etiology and degree of kyphosis, there was no evidence to suggest that preoperative halo traction reduced the risk of any complication following surgical intervention. Although not statistically significant, a subgroup analysis of patients with severe curves demonstrated a trend toward a markedly reduced hardware failure rate in patients undergoing preoperative halo traction [preoperative traction: 1 (3.1%) vs. no preoperative traction: 11 (14.7%), P=0.083]. Nonidiopathic, hyperkyphotic patients treated with intraoperative traction were 61% less likely to experience any postoperative complication (P=0.067) and were 74% (P=0.091) less likely to experience an unplanned return to the operating room when compared with patients treated without traction. CONCLUSIONS: This multicenter study with a large sample size provides the best evidence to date of the association between the use of traction and postoperative complications. Our results justify the need for future Level I studies aimed at characterizing the complete benefit and risk profile for the use of traction in surgical intervention for EOS. LEVEL OF EVIDENCE: Level III.

Establishing Consensus on the Best Practice Guidelines for Use of Halo Gravity Traction for Pediatric Spinal Deformity.

BACKGROUND: Although halo gravity traction (HGT) has been used to treat children with severe spinal deformity for decades, there is a distinct lack of high-quality evidence to speak to its merits or to dictate ideal manner of implementation. In addition, no guidelines exist to drive research or assist surgeons in their practice. The aim of this study was to establish best practice guidelines (BPG) using formal techniques of consensus building among a group of experienced pediatric spinal deformity surgeons to determine ideal indications and implementation of HGT for pediatric spinal deformity. METHODS: The Delphi process and nominal group technique were used to formally derive consensus among leaders in pediatric spine surgery. Initial work identified significant areas of variability in practice for which we sought to garner consensus. After review of the literature, 3 iterative surveys were administered from February through April 2018 to nationwide experts in pediatric spinal deformity. Surveys assessed anonymous opinions on ideal practices for indications, preoperative evaluation, protocols, and complications, with agreement of 80% or higher considered consensus. Final determination of consensus items and equipoise were established using the Nominal group technique in a facilitated meeting. RESULTS: Of the 42 surgeons invited, responses were received from 32, 40, and 31 surgeons for each survey, respectively. The final meeting included 14 experts with an average 10.5 years in practice and average 88 annual spinal deformity cases. Experts reached consensus on 67 items [indications (17), goals (1), preoperative evaluations (5), protocols (36), complications (8)]; these were consolidated to create final BPG in all categories, including statements to help dictate practice such as using at least 6 to 8 pins under 4 to 8 lbs of torque, with a small, tolerable starting weight and reaching goal weight of 50% TBW in ∼2 weeks. Nine items remained items of equipoise for the purposes of guiding future research. CONCLUSIONS: We developed consensus-based BPG for the use and implementation of HGT for pediatric spinal deformity. This can serve as a measure to help drive future research as well as give new surgeons a place to begin their practice of HGT. LEVEL OF EVIDENCE: Level V-expert opinion.

Gross Motor Function Classification System Specific Growth Charts-Utility as a Risk Stratification Tool for Surgical Site Infection Following Spine Surgery.

BACKGROUND: There is currently minimal evidence that preoperative malnutrition increases surgical site infection (SSI) risk in children with cerebral palsy (CP) undergoing spinal deformity surgery. Growth charts specifically for patients with CP have been created to aid in the clinical interpretation of body mass index (BMI) as a marker of nutritional status, but to our knowledge these charts have never been used to risk stratify patients before orthopaedic surgery. We hypothesize that patients with CP who have BMI-for-age below the 10th percentile (BMI≤10) on CP-specific growth charts are at increased risk of surgical site infection following spinal deformity surgery compared with patients with BMI-for-age above the 10th percentile (BMI>10). METHODS: Single-center, retrospective review comparing the rate of SSI in patients with CP stratified by BMI-for-age percentiles on CP-specific growth charts who underwent spinal deformity surgery. Odds ratios with 95% confidence intervals and Pearson χ tests were used to analyze the association of the measured nutritional indicators with SSI. RESULTS: In total, 65 patients, who underwent 74 procedures, had complete follow-up data and were included in this analysis. Ten patients (15.4%) were GMFCS I-III and 55 (84.6%) were GMFCS IV-V; 39 (60%) were orally fed and 26 (40%) were tube-fed. The rate of SSI in this patient population was 13.5% with 10 SSIs reported within 90 days of surgery. There was a significant association between patients with a BMI below the 10th percentile on GMFCS-stratified growth charts and the development of SSI (OR, 13.6; 95% CI, 2.4-75.4; P=0.005). All SSIs occurred in patients that were GMFCS IV-V. There was no association between height, weight, feeding method, or pelvic instrumentation and development of SSI. CONCLUSIONS: CP-specific growth charts are useful tools for identifying patients at increased risk for SSI following spinal instrumentation procedures, whereas standard CDC growth charts are much less sensitive. There is a strong association between preoperative BMI percentile on GMFCS-stratified growth charts and SSI following spinal deformity surgery. LEVEL OF EVIDENCE: Level III-Retrospective Study.

The Final 24-Item Early Onset Scoliosis Questionnaires (EOSQ-24): Validity, Reliability and Responsiveness.

INTRODUCTION: The goal of early-onset scoliosis (EOS) treatment is to improve health-related quality of life (HRQoL) for patients and to reduce the burden on their parents or caregivers. The purpose of this study is to develop and finalize the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24), and examine the validity, reliability, and responsiveness of the EOSQ-24 in measuring patients' HRQoL, the burden on their caregivers, and the burden on their caregiver's finances. The study also established aged-matched normative values for the EOSQ-24. METHODS: The EOSQ-24 was administered to caregivers of male and female patients aged 0 to 18 years with EOS. Patients with EOS are diagnosed before 10 years of age. Criterion validity was investigated by measuring agreement between its scores and pulmonary function testing. Construct validity was established by comparing values across different etiology groups using the known-group method, and measuring internal consistency reliability. Content validity was confirmed by reviewing caregiver and health provider ratings for the relativity and clarity of the EOSQ-24 questions. Test-retest reliability was examined through intraclass correlation coefficients. Responsiveness of the EOSQ-24 before and after surgical interventions was also investigated. Age-matched, healthy patients, without spinal deformity, were enrolled to establish normative EOSQ-24 values. RESULTS: The pulmonary function subdomain score in the EOSQ-24 was positively correlated with pulmonary function testing values, establishing criterion validity. The EOSQ-24 scores for neuromuscular patients were significantly decreased compared with idiopathic or congenital/structural patients, demonstrating known-group validity. Internal consistency reliability of patients' HRQoL was excellent (0.92), but Family Burden was questionable (0.64) indicating that Parental Burden and Financial Burden should be in separate domains. All 24 EOSQ items were rated as essential and clear, confirming content validity. All EOSQ-24 domains demonstrated good to excellent agreement (0.68 to 0.98) between test and retest scores. Neuromuscular patients improved their HRQoL postoperatively, whereas idiopathic patients worsened their HRQoL postoperatively, indicating the ability of the instrument to respond to different trajectories of HRQoL according to etiology in patients with EOS. DISCUSSION: The developed and finalized EOSQ-24 is a valid, reliable, and responsive instrument that is able to serve as a patient-reported outcome measure evaluating health status for patients with EOS and burden of their caregivers. This instrument will be able to serve as an outcome measure for future research including clinical trials evaluating the effectiveness of various treatments. In addition, the EOSQ-24 allows assessment of patients' HRQoL, and the burden on their caregivers relative to age-matched healthy peers. LEVEL OF EVIDENCE: Level II-diagnostic study with consecutive patients enrolled in national registries.

Psychosocial effects of repetitive surgeries in children with early-onset scoliosis: are we putting them at risk?

BACKGROUND: The use of growing instrumentation in children with early-onset scoliosis (EOS) has created interest in determining if these repetitive procedures are prompting the development of lasting psychosocial problems. Given the increasing role of this treatment modality in the management of EOS, this study aimed to assess the psychological status of this patient population and to determine factors associated with worse scores in various psychosocial domains. METHODS: A cross-sectional assessment of 34 EOS patients was performed utilizing 2 well-established, caregiver-completed psychiatric instruments: the Child Behavior Checklist (CBCL) and the Strength and Difficulties Questionnaire. Scores were calculated for 15 CBCL and 6 Strength and Difficulties Questionnaire domains and subdomains and grouped as "Normal" or "Abnormal" according to published normative values. The prevalence of abnormal scores was within each instrument subdomain and was compared with the national norms. Domain scores were also correlated with age at first scoliosis surgery, total number of operative procedures, and total number of growing instrumentation surgeries. RESULTS: A higher prevalence of Abnormal scores were found in multiple psychosocial domains in our cohort as compared with national normative data. Children with Abnormal CBCL "Total Problems" domain scores were younger at the time of first scoliosis surgery (2.50 vs. 5.52 y). Normal and Abnormal scoring groups showed significant differences in the number of (1) total surgeries; (2) total scoliosis surgeries; and (3) growing instrumentation surgeries in multiple domains on both instruments. Aggression, Rule-breaking, and Conduct were positively correlated with total number of surgeries. CONCLUSIONS: Our findings showed a higher prevalence of Abnormal psychosocial scores in multiple domains in multioperated EOS patients as compared with national norms. Our findings suggest that EOS patients with abnormal psychosocial scores were younger at the time of their initial scoliosis surgery. The number of repetitive surgeries also correlated positively with 3 behavioral problem scores. Although healthier scores were seen in 1 positive behavioral domain in more operated children suggesting the potential for "posttraumatic growth," the other findings of this study are concerning given the increasing use of this treatment modality and indicate a need for ongoing screening and mental health care in this high-risk population. LEVEL OF EVIDENCE: Level III--case-control.