Early-Onset Scoliosis Questionnaire Research Articles

Reliability and validity of the adapted Finnish version of the early onset scoliosis questionnaire (EOSQ-24).

EOSQ-24 is a disease specific patient-reported outcome score used to assess the quality of life in patients with early-onset scoliosis. The aim of this study was to translate and cross-culturally adapt the English version of the EOSQ-24 to Finnish language and to assess the reliability and validity of the translation. Cross-cultural adaptation and cross-cultural validation were performed to the Finnish translation of the EOSQ-24. Patients and/or their caretakers were then recruited to assess the psychometric properties of the translation. We assessed the internal consistency, test-retest reliability, floor and ceiling effects, and discriminative abilities. One-hundred-and-three patients filled the questionnaire. EOSQ-24 was successfully translated into Finnish. The translation showed excellent internal consistency (Cronbach alpha 0.94), satisfactory item-total correlations ranging from 0.6 to 0.9, and moderate to strong inter item correlations. Test-retest reliability ranged from 0.7 to 0.96 indicating good to excellent agreement. Patients with neuromuscular and syndromic scoliosis reported lower EOSQ-24 scores when compared to patients' idiopathic and congenital scoliosis. There was a significant negative correlation between major curve and EOSQ-24 scores in patients with idiopathic early onset scoliosis. The internal consistency and test-retest reliability of the measure were found to be satisfactory. A marked ceiling effect was observed, indicating a potential source of error.

Do Caregivers and Patients With Early-Onset Scoliosis Share the Same Perspective on Health-Related Quality of Life? A Comparison of 24-item Early-Onset Scoliosis Questionnaire and 22-item Scoliosis Research Society Questionnaire Scores.

The 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24) is validated in patients with early onset scoliosis (EOS) aged 0 to 18 years and the 22-item Scoliosis Research Society (SRS-22) questionnaire is validated in idiopathic scoliosis patients 10 years and older. EOSQ-24 is completed by the caregiver and SRS-22 is completed by the patient. A prior study comparing patient-reported outcome measures completed by older pediatric patients and their parents showed a low level of agreement. Our purpose was to compare EOSQ-24 and SRS-22 scores completed at the same time point and at subsequent time points, in patients aged 5 to 18 years with idiopathic or congenital EOS without developmental delay. This was a multicenter retrospective study. We identified pairs of EOSQ-24 and SRS-22 completed on the same day or within 6 months. Some patients had multiple pairs of surveys over time. EOSQ-24 and SRS-22 questions were matched and domain scores for Pain, Function, Mental Health, and Satisfaction were compared. Patients with a change in treatment between surveys were excluded. Pearson correlation coefficients (r) were used to compare domain scores, with r≥0.7 indicating a strong relationship. There were 228 patient-caregiver dyads who completed 411 pairs of EOSQ-24 and SRS-22 on the same day. A strong correlation was found only for the Pain domain (r=0.77). Function, Mental Health, and Satisfaction domains had positive but not strong correlations (r= 0.58, r= 0.50, r= 0.41, respectively). Subanalysis based on age also showed a strong correlation only for Pain. There were 76 patient-caregiver dyads who completed 134 pairs of surveys with SRS-22 completed within 6 months after an EOSQ-24. All domains demonstrated a positive but not strong correlation, with Pain showing the highest correlation (r= 0.64). EOSQ-24 and SRS-22 had a strong correlation only for Pain when completed at the same time point. EOSQ-24 and SRS-22 completed within 6 months lacked a strong correlation for all domains. Our findings suggest that parents and children with EOS may not share the same perspective on their health. Self-reported questionnaires should be used when possible to assess health-related quality of life in older children and adolescents with EOS who are developmentally neurotypical. Level III-diagnostic.

Health-related quality of life in early onset scoliosis patients treated with the spring distraction system: what to expect in the first 2 years after surgery

PurposeThe Spring Distraction System (SDS) is a novel “growth-friendly” implant for the treatment of Early-Onset Scoliosis (EOS). This prospective study aims to determine the evolution of the “24-Item Early-Onset Scoliosis Questionnaire” (EOSQ-24) scores during 2-year follow-up after SDS surgery. Secondary aims include investigating the relation between EOSQ-24 scores and EOS etiology, and evaluating the impact of an unplanned return to the operating room (UPROR) on HRQoL.MethodsAll SDS patients with at least 2-year follow-up were included. Caregivers completed the EOSQ-24 pre-operatively, post-operatively, and at 6, 12, and 24 month follow-up. Mean total and -domain scores were graphed over time. Repeated-measures ANOVA analyzed the influence of etiology on EOSQ-24 scores. Multiple regression analyzed associations between UPRORs and EOSQ-24 scores.ResultsForty-nine patients were included. Mean total EOSQ-24 scores decreased from 70 pre-operatively to 66 post-operatively, then gradually increased to 75 (24 months). Most domains exhibited changes over time, with initial declines, but eventually surpassing pre-operative levels after 2-year follow-up. Neuromuscular/Syndromic patients had lower scores, but showed similar improvements over time compared with other etiologies. Multiple regression showed lower Parental Burden domain score (− 14 points) in patients with UPRORs, although no significant reductions were found in total score, or in other domains.ConclusionHRQoL decreases immediately following SDS surgery but quickly recovers and exceeds pre-operative levels at 2-year follow-up in all domains. Neuromuscular/Syndromic patients have lower initial scores, but progress similarly over time. UPRORs do not influence EOSQ-24 scores, except for a negative impact on the Parental Burden domain in the short term.Level of EvidenceIII.

Health-Related Predictive Factors of Brazilian Children With Early Onset Scoliosis Using the EOSQ-24 and CHQ-PF50 Questionnaires.

Assessing the quality of life (QOL) of children with early onset scoliosis (EOS) has been discussed recently. Therefore, the study aimed to apply and correlate the 24-item Early Onset Scoliosis Questionnaire (EOSQ-24) with the 50-item Childhood Health Questionnaire (CHQ-PF50) to find predictive factors impacting QOL. Cross-sectional study involving a population of caregivers of patients with EOS. The sample consisted of 72 patients. Two research assistants applied the Portuguese version of the EOSQ-24 and CHQ-PF50 in 3 treatment centers. The EOSQ-24 assesses the subjective response of children with EOS from the parent's point of view. The CHQ is a self-administered questionnaire or parental proxy assessment of the psychological and social status of children aged 5 to 18 years. Of 72 patients, 41 (56.9%) were females, mean age of 11.9 ± 4.2 years. The most common scoliosis was of neuromuscular origin (32%). The CHQ-PF50 showed that family-related items had significant scores. The most affected subcategory was physical function (45.5), and the least affected was mental health (90.8). Thus, the CHQ-PF50 PhS summary index was 27, and the CHQ-PF50 PsS was 71.7. Moreover, the critical categories for the EOSQ-24 questionnaire were daily life and physical function (45.1 and 47.8, respectively), and the least affected categories were transfer and pulmonary function (70.8 and 68.9, respectively). Four subcategories showed a strong correlation between both questionnaires: general health (r = 0.749, P < 0.001), physical function (r = 0.645, P < 0.001), bodily pain (r = 0.714, P < 0.001), and mental health (r = 0.424, P < 0.001). Using CHQ-PF50 as a dependent variable in multiple regression analysis (P = 0.028), the only variable affecting the scores was syndromic scoliosis (P = 0.019; 95% CI -27.4 to -2.5). A strong correlation between both questionnaires was seen for general health, physical function, bodily pain, and mental health. Syndromic scoliosis was a predictor of worse QOL according to the CHQ-PF50.

Reliability and validity of the Chinese version of the Early-Onset Scoliosis Self-Report Questionnaire in children aged 8 to 18 years with early-onset scoliosis.

The Early-Onset Scoliosis Self-Report Questionnaire (EOSQ-SELF) is a novel self-report instrument to evaluate the health-related quality of life (HRQoL) of early onset scoliosis (EOS) patients, as a complementary HRQoL assessment tool for the proxy-repot 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24). This study aimed to translate and adapt the EOSQ-SELF into a Chinese version and evaluate its reliability and validity in EOS patients. A cross-sectional study was performed from July 2022 to September 2022. Patients aged 8 to 18 years with EOS were recruited. Forwards-backwards translation and cross-cultural adaptation of the original EOSQ-SELF were performed. The Chinese EOSQ-SELF was administered and collected twice through social media, with a 2 weeks interval. Reliability was evaluated by using internal consistency, and test-retest reliability was assessed by the intraclass correlation coefficient (ICC) of data between the two time points. A Delphi survey in the expert committee was used to investigate the content validity. SRS-22r, PedsQL4.0, and EQ-5D-Y scales were used to assess the criterion-related validity. Nonparametric tests and multiple linear regression were performed to evaluate the discriminative ability. A total of 101 valid responses from EOS patients were received. The mean age was 12.08±2.19 years, and 53.47% were female. The mean Cobb angle was 60.56°±19.48°. Floor effects of 0.00% to 7.92% and ceiling effects of 0.00% to 52.48% were observed. Excellent internal consistency and test-retest reliability of the Chinese EOSQ-SELF was observed, with a Cronbach's α coefficient of 0.942, McDonald's ω coefficient of 0.940, and ICC of 0.930. All domains of the Chinese EOSQ-SELF were significantly correlated with SRS-22r (0.709 to 0.878), PedsQL4.0 (0.568 to 0.718), and EQ-5D-Y (-0.598 to -0.625), depicting excellent criterion-related validity. Discriminative ability was validated in aetiology (P<0.001), severity of spinal deformity (P<0.001), treatment status (P<0.001), and ambulatory ability (P<0.001). The Chinese EOSQ-SELF is a reliable and valid tool for the assessment of self-report HRQoL in patients aged 8 to 18 years with EOS. It can be easily applied in clinical settings and for research purposes, as a complementary tool for the proxy-report EOSQ-24.

Surgical treatment of neuromuscular Early Onset Scoliosis with a bilateral posterior one-way rod compared to the Spring Distraction System: study protocol for a limited-efficacy Randomized Controlled Trial (BiPOWR)

BackgroundEarly Onset Scoliosis (EOS) is a progressive spinal deformity in children, and a potentially life-threatening disease. “Growth-friendly” surgical techniques aim to control the deformity, while allowing the spine and trunk to maintain growth. Current “growth-friendly” systems such as the traditional growing rod (TGR) and magnetically controlled growing rod (MCGR) have limitations that reduce their efficacy and cost-effectiveness. Recently, two “growth-friendly” systems have been developed that mitigate many of these limitations, the Spring Distraction System (SDS) and the One Way Self-Expanding Rod (OWSER). The purpose of the multicenter BiPOWR trial is to investigate, describe and compare the 1-year limited-efficacy and -safety of both strategies in the treatment of neuromuscular EOS.MethodsAfter informed consent, 28 neuromuscular EOS patients will be randomized to receive either the SDS or the OWSER. Patients and caregivers will be blinded to allocation until after surgery. Primary outcomes will be maintenance of coronal curve correction and the occurrence of serious adverse events. In addition, spinal growth, implant lengthening, and perioperative findings are recorded systematically. At each follow-up moment, the Early Onset Scoliosis Questionnaire (EOSQ-24) will be used to assess health-related quality of life. All outcomes will be compared between groups.DiscussionThe BiPOWR trial is the first randomized controlled trial that compares two specific “growth-friendly” implants in a specified EOS population. It will determine the 1-year limited-efficacy and safety of the SDS and OWSER implants.Trial registrationClinicaltrials.gov: NCT04021784 (13–06-2019). CCMO registry: NL64018.041.17 (06–05-2019).

Does Transitioning to a Brace Improve HRQoL After Casting for Early Onset Scoliosis?

Serial casting is favored for the initial treatment of early onset scoliosis (EOS), but there is concern about significant morbidity and caregiver burden. Studies have examined the utility of bracing as an alternative to casting, but little is known about differences in health-related quality of life (HRQoL) between treatments. We hypothesized that patients with a diagnosis of idiopathic EOS experience an improvement in HRQoL when transitioning from serial casting to bracing as measured by the 24-Item Early Onset Scoliosis Questionnaire (EOSQ). Subjects with idiopathic EOS were retrospectively identified from a multicenter database. EOSQ scores were compared before treatment, after index casting, after transition out of cast to brace, and at the most recent follow-up. Available major curve magnitudes were also compared during these time points. Data were compared using repeated-measures ANOVA with post hoc Bonferroni correction. Sixty-six subjects met the inclusion criteria. Thirty-seven (56%) subjects were male and the average age at the time of index treatment was 1.9 (0.37-6.4) years. The average follow-up was 3.2 (0.90-6.8) years. In 57 subjects, the major curve magnitude improved from a mean of 33 (CI 28-37) degrees before treatment initiation to 27 (CI 23-30) degrees after casting and to 24 (CI 20-29) degrees at the most recent follow-up. The HRQoL subdomain showed a significant decrease in HRQoL during casting treatment 75.7 (CI 72.9-78.5) from pre-index treatment 84.9 (CI 81.4-88.5), during brace treatment 84.8 (CI 81.8-88.0) and at most recent follow-up 87.0 (CI 83.6-90.3) ( P <0.001). The parental impact subdomain improved from the beginning to the end of treatment (77.7 to 87.7, P =0.001) (n=64). Satisfaction improved from casting to bracing (73.4 to 86.7, P <0.001) (n=63) and to the most recent follow-up (73.4 to 87.9, P <0.001). Patients treated with casting for EOS experience reversible declines in HRQoL. After patients transition from casting to bracing, EOSQ scores recover to pretreatment baseline levels and are maintained at follow-up. This information must be balanced with the effectiveness of treatment for EOS with either method and customized for each patient. Level III-retrospective comparative study.

Impact of surgical treatment on parent-reported health related quality of life measures in early-onset scoliosis: stable but no improvement at 2years.

The etiology of early-onset scoliosis (EOS) has been shown to significantly influence baseline parent-reported health-related quality of life (HRQoL) measures as assessed by the Early Onset Scoliosis Questionnaire (EOSQ). We sought to assess the influence of distraction-based surgery and scoliosis etiology on EOSQ 2years following surgical intervention remains unclear. A retrospective review of a multi-center prospective EOS database was performed. Children untreated with distraction-based, growth friendly instrumentation for EOS with completed baseline and 2-year post-surgical EOSQ were included. Children were subdivided by curve etiology individually and in combined cohorts (congenital/idiopathic [C/I], neuromuscular/syndromic [NMS]). EOSQ domains and compositive HRQoL score at presentation and 2-year follow-up were compared across C-EOS etiologies. Minimal clinically important difference (MCID) was defined as ≥ 20% change in domain score and compared across etiologies. 150 children with EOS met inclusion criteria (mean 7.09 ± 2.6years, 58.9% female). There were no differences in EOSQ domains between Congenital vs. Idiopathic nor NM vs. Syndromic etiologies at any timepoint. Combined C/I children demonstrated significantly higher EOSQ scores than combined NMS at initial and 2-years post-treatment. Etiology remained the only independent predictor of 2-year EOSQ composite HRQoL score. Overall, the vast majority of children demonstrated stable HRQoL composite scores, with a trend toward more positive MCID in NMS etiologies. EOS etiology remains the most significant influence on EOSQ scores 2years following surgical intervention. However, the majority of patients, regardless of etiology, maintain stable HRQoL scores suggesting that surgery may only serve to stabilize HRQoL at 2years.

Development and Validation of a Health-Related Quality-of-Life Measure in Older Children and Adolescents with Early-Onset Scoliosis

The 24-question Early-Onset Scoliosis Questionnaire (EOSQ-24) is a proxy measure assessing health-related quality of life (HRQoL) among patients with early-onset scoliosis (EOS). There exists an increasing need to assess HRQoL through a child's own perspective, particularly for older children and adolescents with EOS. The purpose of this study was to develop and validate a self-reported questionnaire, the Early-Onset Scoliosis Self-Report Questionnaire (EOSQ-SELF), to assess HRQoL in older children and adolescents with EOS. A literature review, an expert focus group, and patient interviews were used to generate a preliminary survey of appropriate domains and question items. This survey was provided to English-speaking patients with EOS who were 8 to 18 years of age and capable of answering survey questions. Content validity was assessed for clarity and relevance of questions. Confirmatory factors analysis was performed to reduce the number of items and determine domains that fit items. Reliability was evaluated by measuring the internal consistency of items and test-retest reliability. Construct validity was evaluated by convergent, discriminant, and known-group validity. The literature review, expert focus group, and patient interviews identified 59 questions in 14 domains. Psychometric analysis reduced these to 30 questions across 12 domains: General Health, Pain/Discomfort, Pulmonary Function, Transfer, Physical Function/Daily Living, Participation, Fatigue/Energy Level, Sleep, Appearance, Relationships, Emotion, and Satisfaction. The final questionnaire was found to have good content and construct validity and adequate reliability. The EOSQ-SELF is a valid and reliable instrument for measuring self-reported HRQoL among older children and adolescents with EOS (ages 8 to 18 years). This will serve as an important research outcome measure and enhance clinical care by providing a better understanding of HRQoL for these patients. Diagnostic Level II . See Instructions for Authors for a complete description of levels of evidence.

Health-Related Quality of Life Assessment in Idiopathic Early-Onset Scoliosis Treated by Serial Mehta Casting.

This was a retrospective clinical study of patients with idiopathic early-onset scoliosis (IEOS) treated by serial Mehta derotational cast in a single facility. A standardized casting protocol was used for patients aged ≤5 years with IEOS (curve ≥20°). Patients' demographics, clinical and radiological outcomes, and major curve Cobb angle were recorded, and health-related quality of life (HRQoL) using the 24-item early-onset scoliosis questionnaire (EOSQ-24) was assessed. A total of 15 patients with IEOS were managed by serial Mehta cast between October 2014 and September 2018 with a mean follow-up period of 42.33 ± 6.37 (range 32-55) months. The mean precast curve was 56.8° ± 12.01°, and the mean final postcast curve was 26.4° ± 19.12° (P = 0.0008). The EOSQ-24 was inversely proportional to the magnitude of the residual curve. There were 2 cases with superficial skin irritation and a single case with repeated vomiting after casting. IEOS can be managed effectively by serial Mehta cast, which is proved not only by the clinical and radiological outcome but also by the improvement of the quality of life of the patients and their families as measured by the EOSQ-24.

Outcomes Evaluating Quality of Life and Their Measurement Properties in Early-onset Scoliosis: A Systematic Review.

Early-onset scoliosis (EOS) is a spinal deformity affecting children under the age of 10. Understanding the impact of EOS on quality of life can be achieved through appropriate patient/carer-reported outcome measures (PROMs). The aim of this systematic review was to identify, evaluate and summarize the evidence for the measurement properties of health-related quality of life (HR-QoL) outcome measures in the EOS population. A 2-stage search methodology was conducted across the PubMed, MEDLINE, EMBASE, EMCARE, PsychINFO, and CINAHL databases. Search 1 identified PROMs used to evaluate the quality of life in EOS, and search 2 identified studies of the measurement properties of these PROMs. Two reviewers performed searching, study screening and selection and assessed studies for risk of bias using the Consensus-based Standards for the selection of health Measurement INstruments (COSMIN) Risk of Bias checklist. One reviewer performed data extraction. Evidence for each measurement property per PROM was summarized and evaluated using a modified GRADE approach. Search 1 identified 19 (10 disease-specific, 9 generic) PROMs that have been used to assess HR-QoL or a relevant domain in the EOS population. Search 2 identified only one PROM (the 24-item Early-Onset Scoliosis Questionnaire, EOSQ) with eligible studies (n=4) evaluating measurement properties. There is low-quality evidence for sufficient content validity, very low-quality evidence for sufficient reliability and low-quality evidence for sufficient criterion validity, specifically regarding the pulmonary function domain) of the EOSQ. Construct validity and responsiveness were rated as indeterminate. There were no studies on measurement error. Our search strategy found that measurement properties have only been evaluated for one PROM, the EOSQ. There is low-quality evidence for sufficient measurement properties for the EOSQ in patients under the age of 10 with EOS. Further research is needed to improve the quality of evidence for EOSQ measurement properties and investigate other PROMs in this population. Level II.

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).

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. 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. 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. 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 II-multicenter retrospective cohort study investigating prognosis.

Does ventilator use status correlate with quality of life in patients with early-onset scoliosis treated with rib-based growing system implantation?

Pulmonary function and quality of life (QOL) are important outcome measures for patients with early-onset scoliosis (EOS) undergoing rib-based growing system (RBGS) implantation. The Assisted Ventilation Rating (AVR) measures ventilator requirements in this population. A higher, more severe, score implies negative changes in QOL. The EOS Questionnaire (EOSQ) is a validated outcome measure. Paired measurements for both ratings were compared to clarify correlation between ventilator status and QOL. Secondary analysis aimed at defining QOL between more broad subgroups defined by ventilator use. AVR and EOSQ scores were extracted from the Pediatric Spine Study Group database for patients 10 years of age and under. Instances were excluded if the time between AVR and EOSQ assessment was greater than 6 months. Scores were compared using Spearman correlation coefficient. Subgroup analysis included control for age, gender, and etiology. Secondary analysis was performed for broad functional grouping using ranked analysis of variance for repeated measures using median scores. Two thousand five hundred and forty-two instances of paired EOSQ and AVR in 329 patients were analyzed. A statistically significant weak correlation between AVR and EOSQ was identified in Child's Health Related QOL and Family Impact sections, in nine subsets. Subgroup analysis showed little variation, except increased correlation in female patients to near moderate level. Analysis of variance for demonstrated decreased medians for all subdomains when comparing those mechanically ventilated to patients who did not require ventilation. Ventilator status tracks with QOL were measured by EOSQ. A more severe AVR is negatively correlated with most domains of the EOSQ for patients with EOS who have undergone RBGS implantation. The strength of this correlation is weak, and so AVR alone may be insufficient to precisely determine QOL in this population. Level-III, Retrospective.

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

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). 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. 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). 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 III.

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.

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. 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). 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. 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 II. This is a multicenter retrospective cohort study investigating the effect of UPROR on HRQoL (prognostic study).

Pulmonary function and health-related quality of life in patients with early onset scoliosis after repeated traditional growing rod procedures.

PurposeThe purpose of this study was to investigate pulmonary function and health-related quality of life after traditional growing rod (TGR) procedures.MethodsBetween January 2006 and December 2017, a retrospective observational study of 17 early onset scoliosis (EOS) patients with a mean follow-up of 6.2 years (2.3 to 10.4) was conducted. The forced expiratory volume in one second (FEV1), expiratory forced vital capacity (FVC) and 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) score before the index surgery and at last follow-up were investigated.ResultsThe mean percentage of predicted FEV1 improved from 50% (20% to 86%) to 53% (15% to 80%; p = 0.08); and the mean percentage of predicted FVC improved from 51% (24% to 81%) to 55% (25% to 89%; p = 0.06). The mean EOSQ-24 score was 78.2 (58 to 90) preoperatively and 77.2 (55 to 88) at last follow-up, there was no statistical difference (p = 0.70). The subdomain scores of pulmonary function (p < 0.01) and daily living (p < 0.01) significantly improved, whereas the subdomain scores of pain (p < 0.01), emotion (p < 0.01) and satisfaction (p = 0.02) significantly declined at last follow-up.ConclusionThe TGR procedure was associated with stable pulmonary function and decline in EOSQ-24 pain, emotion and satisfaction scores.Level of EvidenceLevel III, retrospective cohort study.

Management and results of early-onset scoliosis with dual magnetically controlled growing rods: Additional preliminary results of spinal fusion surgery.

Objectives The aim of this study was to evaluate the clinical and radiographic outcomes and complications of dual magnetically controlled growing rods (MCGRs) in the treatment of early-onset scoliosis (EOS) and to investigate the results of patients with definitive spinal fusion following MCGR.Patients and methods A total of 15 patients (7 males, 8 females; mean age: 8.7±1.7 years; range, 6 to 10 years) with EOS who underwent dual MCGR and were prospectively followed between February 2013 and March 2019 were included in this retrospective study. The Cobb angle, thoracic kyphosis, and the length of the spine between T1-T12 and T1-S1 were measured on preoperative, postoperative, and follow-up radiographs. The 24-Item Early-Onset Scoliosis Questionnaire (EOSQ-24) was used to assess the functional outcomes before and after the operation. All complications during the treatment were recorded.Results The mean follow-up was 27.8±10.4 (range, 12 to 60) months. The mean curve correction immediately after the index surgery and latest follow-up was 47.6% and 42.4%, respectively (p>0.05). At the last follow-up, there were no significant changes in mean Cobb and kyphosis angles. The mean T1-T12 length increase was 26.2±7.1 (range, 16 to 40) mm, while the mean T1-S1 length increase was 43.3±15.0 (range, 24 to 70) mm. Complications developed in four (26.6%) of 15 patients. Definitive spinal fusion surgery was performed in seven patients. Total mean Cobb angle difference between the final follow-up and fusion surgery was 9.3° (p=0.016) and kyphosis angle difference was -2.1° (p=0.349). After fusion surgery, total lengthening in T1-T12 and T1-S1 distance was 10.5 mm (p=0.036) and 15.0 mm (p=0.022), respectively. A significant increase in all subdomain scores of the EOSQ-24 (p<0.05), except for financial impact, was recorded in all patients.Conclusion Dual MCGR technique is an effective, reliable, and robust treatment alternative for primary EOS. However, surgeons should be aware of the relatively high rate of complications. In addition, residual deformity can be corrected successfully with definitive surgery.

Cross-Cultural Adaptation and Validation of the Persian Version of the 24-Item Early-Onset Scoliosis Questionnaire.

Study DesignCross-sectional study.PurposeTo translate and culturally adapt the original English version of the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24) to the Persian language as well as assess its reliability and validity.Overview of LiteratureEarly-onset scoliosis (EOS) patients with progressive curves require active treatments, such as serial elongation–derotation–flexion casting, bracing, and surgery, which are stressful and expensive. In order to evaluate the impact of EOS and its treatment strategies, it is important to consider the patients and their parents’ quality of life as the clinical and radiological parameters. The EOSQ-24 is a parent-based measure that evaluates the health-related quality of life of patients with EOS and their caregivers/parents. Similar to other widely used questionnaires, EOSQ-24 needs to be translated into other languages to make it usable in populations from different cultures and societies.MethodsWe evaluated the translation and back translation of the EOSQ-24 and made the required revisions as per the analysis performed by the expert committee and an international guideline to adapt it for use in this study. Thereafter, we recruited 100 EOS patients in order to evaluate its reliability and validity. The reliability was assessed with internal consistency. Convergent validity was assessed by comparing the scores of the EOSQ-24 and the 22-item Scoliosis Research Society Questionnaire (SRS-22r). Finally, the known groups validity was assessed as per patient’s sex, curve magnitude, and treatment type.ResultsThe Persian EOSQ-24 demonstrated very good internal consistency (Cronbach’s α=0.88). All the items had an acceptable corrected item-total correlation (>0.3). Further, the EOSQ-24 and the SRS-22r scores (p<0.001) were significantly correlated. The EOSQ-24 could discriminate patients with different curve magnitudes.ConclusionsThe Persian EOSQ-24 can serve as a disease-specific instrument with strong validity and reliability in the evaluation of EOS patients. Its applicability in other Persian-speaking countries and regions of the world needs to be investigated further.

Growth-Friendly Spine Surgery in Arthrogryposis Multiplex Congenita.

Arthrogryposis multiplex congenita (AMC) is a condition that describes neonates born with ≥2 distinct congenital contractures. Despite spinal deformity in 3% to 69% of patients, inadequate data exist on growth-friendly instrumentation (GFI) in AMC. Our study objectives were to describe current GFI trends in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched idiopathic EOS (IEOS) cohort to determine whether spinal rigidity or extremity contractures influenced outcomes. Children with AMC and spinal deformity of ≥30° who were treated with GFI for ≥24 months were identified from a multicenter EOS database (1993 to 2017). Propensity scoring matched 35 patients with AMC to 112 patients with IEOS with regard to age, sex, construct, and curve. Multivariable linear mixed modeling compared changes in spinal deformity and the 24-item Early Onset Scoliosis Questionnaire (EOSQ-24) across cohorts. Cohort complications and reoperations were analyzed using multivariable Poisson regression. Preoperatively, groups did not differ with regard to age (p = 0.87), sex (p = 0.96), construct (p = 0.62), rate of nonoperative treatment (p = 0.54), and major coronal curve magnitude (p = 0.96). After the index GFI, patients with AMC had reduced percentage of coronal correction (35% compared with 44%; p = 0.01), larger residual coronal curves (49° compared with 42°; p = 0.03), and comparable percentage of kyphosis correction (17% compared with 21%; p = 0.52). In GFI graduates (n = 81), final coronal curve magnitude (55° compared with 43°; p = 0.22) and final sagittal curve magnitude (47° compared with 47°; p = 0.45) were not significantly different at the latest follow-up after definitive surgery. The patients with AMC had reduced T1-S1 length (p < 0.001), comparable T1-S1 growth velocity (0.66 compared with 0.85 mm/month; p = 0.05), and poorer EOSQ-24 scores at the time of the latest follow-up (64 compared with 83 points; p < 0.001). After adjusting for ambulatory status and GFI duration, patients with AMC developed 51% more complications (incidence rate ratio, 1.51 [95% confidence interval (CI), 1.11 to 2.04]; p = 0.009) and 0.2 more complications/year (95% CI, 0.02 to 0.33 more; p = 0.03) compared with patients with IEOS. Patients with AMC and EOS experienced less initial deformity correction after the index surgical procedure, but final GFI curve magnitudes and total T1-S1 growth during active treatment were statistically and clinically comparable with IEOS. Nonambulatory patients with AMC with longer GFI treatment durations developed the most complications. Multidisciplinary perioperative management is necessary to optimize GFI and to improve quality of life in this complex population. Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Effect of Etiology, Radiographic Severity, and Comorbidities on Baseline Parent-Reported Health Measures for Children with Early-Onset Scoliosis.

The Classification of Early-Onset Scoliosis (C-EOS) allows providers to differentiate patients, for clinical and research purposes, on the basis of the etiology of their disease as well as radiographic parameters. The Early Onset Scoliosis Questionnaire (EOSQ) is the first disease-specific, parent-reported HRQOL (health-related quality-of-life) outcome measure for this condition. We sought to determine the influence of the C-EOS etiology designation, radiographic parameters, and medical comorbidities on EOSQ scores to differentiate quality of life in this heterogeneous patient population. We hypothesized that baseline EOSQ scores for patients with EOS would be strongly affected by the C-EOS etiology designation. The analysis included prospectively enrolled patients with EOSQ scores recorded in a multicenter EOS database prior to intervention for the EOS. EOSQ scores were compared across C-EOS etiologies, severity of disease based on radiographic measurements, and patient comorbidities prior to scoliosis intervention. Six hundred and ten patients with EOS were available for analysis; 119 had congenital, 201 had idiopathic, 156 had neuromuscular, and 134 had syndromic EOS. In multivariate analysis, neuromuscular and syndromic etiologies were associated with lower scores than congenital and idiopathic etiologies in many EOSQ domains including general health, transfer, daily living, fatigue/energy level, and emotion. Patients with neuromuscular EOS had the lowest EOSQ scores in general. Congenital and idiopathic EOS did not differ from each other in any EOSQ domain. Coronal Cobb and kyphosis angles had significant inverse but generally weak correlations with EOSQ domains. Individual medical comorbidities had a minor effect on certain domains while American Society of Anesthesiologists (ASA) class and total number of comorbidities had inverse correlations with most domains. The underlying etiology of EOS appears to have a significant influence on the parent-reported HRQOL outcomes of the disease. Specifically, syndromic and neuromuscular C-EOS diagnoses are associated with lower EOSQ scores before treatment compared with congenital and idiopathic diagnoses. Radiographic measurements of severity have a relatively small influence on EOSQ scores. These baseline differences in C-EOS-designated etiology should be accounted for in studies comparing outcomes of treatment for this heterogeneous patient population. Prognostic Level II. See Instructions for Authors for a complete description of levels of evidence.