Health-related quality of life in early-onset-scoliosis patients treated with growth-friendly implants is influenced by etiology, complication rate and ambulatory ability.

BACKGROUND: Progressive Early-Onset Scoliosis (EOS) in children may lead to surgical interventions with growth-friendly implants, which require repeated lengthening procedures in order to allow adequate growth. Quality of life was studied using the validated German version of the EOS-Questionnaire (EOSQ-24-G) in surgically treated EOS children with different lengthening modalities. METHODS: EOSQ-24-G and the KINDLR questionnaire were given to families with EOS children who had been treated by either vertical expandable prosthetic titanium rib implants and repetitive lengthening surgeries every 6 months or children who had received a magnetically expansion controlled implant, which was externally lengthened every 3 months. Results were compared according to differences between the two tests, and with possible influencing factors such as surgical method, severity of scoliosis, relative improvement of curvature, etiology, weight, age, travelling distance, complications, ambulatory ability and others. RESULTS: 56 children with an average curve angle of 69° corrected to 33° (52%; average age 5.6 yrs) answered the EOSQ-24-G and the KINDLR after an average follow-up of 3.9 years. Health-related quality of life (HRQoL) was not affected by the initial scoliosis correction, the number of surgeries or the implant type. However, there was a negative correlation with non-ambulatory status, complications during treatment and for children with a neuromuscular scoliosis. CONCLUSION: Using the validated EOSQ-24-G, no statistically significant differences were found between the group of children receiving repetitive surgeries and children with external lengthening procedures without surgery. However, results were influenced by the etiology, complication rate or ambulatory ability. LEVEL OF EVIDENCE/CLINICAL RELEVANCE: Therapeutic Level IV.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

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.

PURPOSE: The 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. METHODS: All 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. RESULTS: Forty-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. CONCLUSION: HRQoL 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 EVIDENCE: III.

Increased lengthening frequency does not adversely affect the EOSQ scores in magnetically controlled growing rod surgeries in 133 subjects followed to final fusion.

PURPOSE: Magnetically Controlled Growing Rod (MCGR) allows frequent outpatient rod lengthening when treating Early Onset Scoliosis (EOS) patients. But there is lack of expert consensus on the optimal MCGR lengthening interval. EOS 24-Item Questionnaire (EOSQ) is validated for assessing health-related quality of life (HrQOL), family burden, and satisfaction. This is the first study assessing how MCGR lengthening intervals affects patient-perceived outcomes. METHODS: This is a multicentred cohort study with subjects recruited from 2012 to 2018 and followed till fusion. EOS subjects who underwent MCGR surgeries were grouped into high, medium or low lengthening interval subgroups based on 16 and 20 week cut-offs. Repeated measure analysis was performed on EOSQ's specified 12 domains. EOSQ results were taken: before index surgery, after index surgery, and prior to definitive treatment. Demographic, clinical and radiographic data were included in model adjustment. RESULTS: 133 subjects with mean follow-up of 3.5 (± 1.3) years were included, with 60 males and 73 females; 45 idiopathic, 23 congenital, 38 neuromuscular, and 27 syndromic patients. Mean Cobb angle at surgery was 67° (± 22°) with mean age of 8.3 (± 2.5) years. Between groups, clinical and radiographic parameters were comparable. Higher EOSQ scores in medium lengthening interval subgroup was present in fatigue (p = 0.019), emotion (p = 0.001), and parental impact (p = 0.049) domains, and overall score (p = 0.046). Trendline contrast between subgroups were present in general health (p = 0.006) and physical function (p = 0.025) domains. CONCLUSION: Patient-perceived outcome improvements appear similar between lengthening interval subgroups. All MCGR lengthening intervals were tolerated by patients and family, with no negative impact observed. LEVEL OF EVIDENCE: Prognostic Level III.

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

PURPOSE: 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 2 years following surgical intervention remains unclear. METHODS: 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. RESULTS: 150 children with EOS met inclusion criteria (mean 7.09 ± 2.6 years, 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. CONCLUSION: EOS etiology remains the most significant influence on EOSQ scores 2 years 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 2 years.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Validation of the Brazilian Portuguese Version of the 24-Item Early-Onset Scoliosis Questionnaire.

STUDY DESIGN: Multicenter prospective study. OBJECTIVES: To validate the Early-Onset Scoliosis 24 Questionnaire (EOSQ-24) questionnaire for the Brazilian Portuguese language, a widely used tool for assessing the impact of different treatments and interventions in EOS patients. METHODS: The EOSQ-24 questionnaire was cross-culturally adapted following guidelines already published. After language adjustments by a group of experts, the final version of the Brazilian Portuguese EOSQ-24 was applied to a group of 76 patients (35 male and 41 female). Internal consistency was evaluated using the Cronbach α coefficient and item-total correlations. Continuous variables were recorded as median values and interquartile ranges and categorical variables as percentages. RESULTS: In the study group, 76 patients were evaluated. The total EOSQ-24 Cronbach α coefficient was 0.883, indicating excellent reliability. The internal consistency of EOSQ-24 was assessed in 3 domains: patient quality of Life, parental burden, and satisfaction (Cronbach α: 0.816-0.934). The range across all subdomains was 0.473 to 0.934. Floor effects for the 24 items were between 1.3% and 43.4% and ceiling effects, between 3.9% and 42.1%. CONCLUSIONS: The Brazilian Portuguese adaptation of the EOSQ-24 shows excellent reliability and can be a valid tool for psychometric assessment of children with EOS.

Association between health-related quality of life outcomes and pulmonary function testing.

INTRODUCTION: Investigations in associations between subjective health-related quality of life (HRQoL) measures and objective clinical assessments in patients with early-onset scoliosis (EOS) are limited. The purpose of this study is to investigate the association between pulmonary function rated by parents and pulmonary function testing (PFT) in patients with EOS. MATERIALS/METHODS: In this cross-sectional study, patients with EOS at any stage of treatment from 2011 to 2018 were identified in 2 registries including 33 centers. Parents' perception of pulmonary function was evaluated using pulmonary function (PF) domain in the Early-Onset Scoliosis 24 item Questionnaire (EOSQ-24). PFT measures included FVC% predicted, FEV1/FVC, and TLC% predicted. All PFT predicted values utilized arm span. PFT and EOSQ-24 questionnaire were completed within 180 days of each other with an average day difference of 26 days. RESULTS: 176 patients (mean age: 10.4 years old, female: 56%) were identified. 33% of patients were of congenital/structural etiology, 27% neuromuscular, 26% syndromic, and 14% idiopathic. Wide variance and lower scores of PF domain were reported by parents at lower FVC% predicted values (< 50%). As FVC% predicted values increased, PFD scores increased with simultaneous decreases in variance with few exceptions. CONCLUSION: More variability and frequent lower pulmonary function values are reported by parents when percent forced vital capacity (FVC%) is < 50%. This likely reflects the degree to which children adapt to restrictive lung disease and the limits on adaptation that occur increasingly as lung function falls below 50% predicted. As a direct linear association with high correlation was expected, more research into the character of what the PF domain is measuring is necessary. LEVEL OF EVIDENCE: IV.

Serial casting for early onset scoliosis and its effects on health-related quality of life during and after discontinuation of treatment.

INTRODUCTION: Serial body casting plays an important role in the treatment of early onset scoliosis (EOS), serving as a safer method compared to surgical intervention. There is no published evidence documenting the impact of casting on health-related quality of life (HRQoL) of patients and their caregivers. The purpose of this study was to utilize the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) to compare the HRQoL of patients with EOS and the burden of care for their caregivers before, during, and after treatment with Mehta casting. METHODS: In this multicenter retrospective cohort study, two EOS databases were queried for patients with EOS who underwent serial casting from 2005 to 2016. Patients who had treatment prior to their initial cast application, including bracing or surgical intervention, were excluded from the study. Patients were stratified into two subgroups and analyzed separately: those with idiopathic etiology, and those with non-idiopathic etiology. HRQoL and burden of care were assessed using the EOSQ-24 completed before, during, and after cast treatment. RESULTS: 91 patients were identified in this study (mean age at the index casting: 2.1 ± 1.2 years, mean age at final cast removal: 4.1 ± 1.3 years). 59 (64.8%) had EOS of idiopathic etiology, while 32 (35.2%) had EOS of non-idiopathic etiology, including 10 congenital, 6 neuromuscular, and 16 syndromic. Idiopathic patients and non-idiopathic patients experienced an improvement in the coronal deformity from 45° pre-cast to 26° post-cast, and from 59° pre-cast to 34° post-cast, respectively. Patients with idiopathic scoliosis experienced a decrease from baseline in nearly all EOSQ-24 sub-domain scores except for general health. Furthermore, even after the removal of the cast, patients with idiopathic etiology suffered residual negative effects of casting on emotion and transfer sub-domains. Among patients with non-idiopathic etiology, decreased scores from baseline were observed only in transfer and emotion sub-domains during the casting intervention. After brace removal, patients with non-idiopathic etiology had increased scores compared to their baseline scores in most sub-domains. DISCUSSION: Serial body casting can prevent curve correction in patients with EOS. However, the psychosocial stresses secondary to this non-operative intervention can have significant negative impacts on HRQoL for both idiopathic and non-idiopathic patients during the course of treatment. Although non-idiopathic patients experience improved HRQoL following treatment, idiopathic patients do not seem to exhibit much improvement in HRQoL from baseline even years after the final cast removal.

Validation of the German version of the 24-item Early-Onset Scoliosis Questionnaire.

OBJECTIVE: During childhood, early-onset scoliosis (EOS) may show severe progressive deformity, which consequently leads to aggressive treatment strategies, such as serial casting, long-term bracing, or surgical interventions. The latter usually includes repeated surgeries for implant lengthening every 6 months in order to allow sufficient growth of the thorax and spine. In 2011, the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24) was introduced to measure health-related quality of life for this patient group and their families. Since then, cross-culturally adapted versions of the EOSQ-24 have been published in Spanish, Turkish, traditional Chinese, and Norwegian. The purpose of the study was to transculturally adapt the original English version of the EOSQ-24 into the German language and evaluate the reliability of the German version. METHODS: After adaptation and forward/backward translation, the German version of the EOSQ-24 was given to the parents or caregivers of 67 EOS patients (33 male, 34 female) Data quality was evaluated by mean, standard deviation, percentage of data missing, and extent of ceiling and floor effects. Reliability was estimated by internal consistency using Cronbach α and item-total correlations. RESULTS: In the study group (n = 67), 12 children were either observed (n = 7) or treated with a brace (n = 5). The other 55 patients were treated surgically with growth-friendly implants. The item response to the German EOSQ-24 was high with a minimum of missing data (1.7%). All items showed very good to excellent internal consistencies (0.879-0.903). Floor effects for the 24 items were between 0% and 31% and ceiling effects between 9% and 78%. The calculated Cronbach α for the 24-item scale was 0.9003, indicating excellent reliability. CONCLUSIONS: The German adaptation of the EOSQ-24 shows excellent reliability and therefore is a valid tool to measure objective health-related quality of life in children with EOS.