Does radiographic calibration affect linear radiographic measurements in a large pediatric spine registry?

PURPOSE: Large registries are increasingly at the forefront of modern pediatric spine research, with manual, centralized, trained radiographic measurement serving as the gold standard for spine research. However, there is limited data regarding the reliability of registry measurements which may be subject to differences in radiographic calibration. We undertook this study to evaluate reliability of T1-T12 height, L1-S1 height, and coronal balance measurements for a large registry of early onset scoliosis patients. METHODS: Three trained technicians from the Pediatric Spine Study Group measured 43 radiographs for T1-T12, L1-S1, and coronal balance using 3 different calibration techniques. All radiographs were AP views of patients with magnetically controlled growing rods with known diameters. The calibration techniques used a pre-export manually drawn line, a digital automatically generated computerized marker, and the diameter of a magnetically controlled growing rod. The intraclass correlation coefficient (ICC) was calculated to determine reliability. RESULTS: 1161 measurements were performed. For each of the three raters, coronal balance, T1-T12 height and L1-S1 height had excellent agreement regardless of the calibration technique (alpha 0.93-1.0). Among the parameters, coronal balance had the worst inter-rater reliability, whereas there was excellent interrater reliability regarding T1-T12 height and L1-S1 height (alpha 0.91-0.99). CONCLUSION: There was excellent agreement among reviewers and between the 3 different calibration techniques. While calibration using rod diameter served as the gold standard, this data shows that other standard calibration methods were adequate and achieved excellent reliability for registry radiographs.

Evaluating the Efficacy of Rib-to-pelvis Growth-friendly Surgery for the Treatment of Non-ambulatory Early-Onset Scoliosis Myelomeningocele Patients.

INTRODUCTION: Early-onset scoliosis (EOS) is a well-known orthopaedic manifestation in patients with myelomeningocele. The rib-based growing system (RBGS) has been proposed as an alternative for these individuals because of the poor outcomes with traditional surgical techniques. We aimed to describe the effect of RBGS in patients with nonambulatory EOS myelomeningocele. METHODS: We retrospectively reviewed the Pediatric Spine Study Group Multicenter Database for all patients with nonambulatory EOS myelomeningocele treated with RBGS from 2004 to 2019. Demographics, surgical data, radiographic findings, and postoperative complications were obtained. The quality-of-life parameters were assessed postoperatively using the Early-onset Scoliosis Questionnaire-24. RESULTS: Thirty patients (18 women; 60%) were patients with nonambulatory EOS myelomeningocele treated with RBGS. The mean age at the initial surgery was 5.3 years. The thoracic (T1-T12) spine height showed a significant increase from initial surgery to the most recent follow-up (P < 0.001). Spine (T1-S1) height was also significantly increased (P < 0.001). The postoperative complication rate was 87%. The Early-onset Scoliosis Questionnaire-24 demonstrates significant improvements in the quality-of-life scores (P = 0.037). CONCLUSION: This study demonstrated that RBGS could improve the reported quality-of-life scores in patients with nonambulatory EOS myelomeningocele when assessed with an EOS-oriented tool. Moreover, we confirmed the ability of RBGS to hold or even correct spinal deformity.

Can distraction-based surgeries achieve minimum 18 cm thoracic height for patients with early onset scoliosis?

PURPOSE: Karol et al. introduced the concept that 18 cm thoracic height is the critical point where a patient with early onset scoliosis (EOS) can maintain adequate pulmonary function. Our purpose was to determine if distraction-based surgeries will increase thoracic spine height to at least 18 cm in patients with EOS. METHODS: Patients with EOS treated with distraction-based systems (minimum 5 years follow up, minimum five lengthenings). Radiographic analysis of thoracic spine height (T1-T12) at the last lengthening procedure. RESULTS: One hundred and fifty-three patients (67 congenital, 21 neuromuscular, 38 syndromic, 27 idiopathic) with pre-operative mean age 4.6 years, scoliosis 75°, kyphosis 47° were evaluated. Their mean age at final lengthening procedure was 11 years (6-16), average number of lengthening procedures was 10.5 (4-21), mean final scoliosis was 53°, and mean final kyphosis was 58°. Final thoracic height was > 18 cm in 65% and was > 22 cm in 31% of patients. Based on etiology, only 48% of the congenital patients reached 18 cm compared to 81% neuromuscular, 84% syndromic and 67% idiopathic. This height gain was closely related to the percentage of scoliosis correction achieved for each etiology. Comparing congenital etiology to other etiologies, there was a lower percentage of patients in the congenital group that passed the 18 cm threshold (48% vs. 78%) (p < 0.05). CONCLUSION: At minimum 5 years follow up, distraction-based surgeries increased thoracic height for patients with EOS to greater than 18 cm in 65% of patients; however, only 48% of congenital patients reached this thoracic height threshold. DESIGN: Retrospective review of prospectively collected registry data. LOI III.

Comparing health-related quality of life and burden of care between early-onset scoliosis patients treated with magnetically controlled growing rods and traditional growing rods: a multicenter study.

STUDY DESIGN: Multicenter retrospective cohort study. OBJECTIVES: To compare pre-operative and post-operative EOSQ-24 scores in magnetically controlled growing rods (MCGR) and traditional growing rod (TGR) patients. Since the introduction of MCGR, early-onset scoliosis patients have been afforded a reduction in the number of surgeries compared to the TGR technique. However, little is known about (health-related quality of life) and burden of care outcomes between these surgical techniques. METHODS: This is a retrospective cohort study using a multicenter registry on patients with EOS undergoing MCGR or TGR between 2008 and 2017. The EOSQ-24 was administered at preoperative and postoperative 2-year assessments. The EOSQ-24 scores were compared between MCGR and TGR as well as preoperatively and postoperatively within each procedure. RESULTS: 110 patients were analyzed in this study (TGR, N = 32; MCGR, N = 78). There were no significant differences in preoperative age, gender, etiology, main coronal curve or maximum kyphosis between TGR and MCGR groups. Patients with TGR had averaged 3.9 surgical lengthenings and MCGR had averaged 7.7 non-invasive lengthenings by the 2-year follow-up. When changes in preoperative to postoperative scores were compared, MCGR had more improvements in pain, emotion, child satisfaction and parent satisfaction than TGR although there were no statistical significance. When analyzed separately, MCGR cohort had improvement in scores for all four domains and four sub-domains; while, TGR cohort only had improvement in financial burden domain and pulmonary function sub-domain. CONCLUSION: Although there was no statistical significance, the improvement in pain, emotion and satisfaction scores was larger in MCGR than TGR. Since these areas can be influenced more by mental well-being than other sub-domains, the results may prove our hypothesis that compared to TGR, MCGR with reduced number of surgeries have better psychosocial effects. LEVEL OF EVIDENCE: III.

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.

Distraction-based surgeries increase thoracic sagittal spine length after ten lengthening surgeries for patients with idiopathic early-onset scoliosis.

STUDY DESIGN: Retrospective, comparative, multicenter. OBJECTIVES: To determine if the choice of proximal anchor affects thoracic sagittal spine length (SSL) for children with idiopathic early-onset scoliosis (EOS). Debate exists as to whether spine growth is maintained during treatment for EOS. As rib- (RB) and spine-based (SB) distraction procedures may be kyphogenic, the traditional measurement of spine growth on coronal radiographs may not identify out-of-plane increase in spine length. A measure of SSL, along the spine's sagittal arc of curvature, has been validated to reliably assess the length of the thoracic spine. METHODS: Patients with idiopathic EOS treated with distraction-based systems (minimum 5-year follow-up, five lengthening surgeries) with radiographic analysis preoperatively, postimplant (L1), and during lengthening periods (L2-L5, L6-L10) were evaluated with primary outcome of T1-T12 SSL. RESULTS: We identified 34 patients (14 RB, 20 SB) with preoperative age 4.9 years (4.2 RB vs. 5.4 SB), scoliosis 72° (60° RB vs. 77° SB; p < 0.05), kyphosis 39° (50° RB vs. 34° SB; p < 0.05), and SSL 17.8 cm (15.5 RB vs. 18.5 SB; p < 0.05). After initial scoliosis correction from implantation, scoliosis remained constant over time. RB patients had greater kyphosis than SB patients: L1, 46° RB vs. 19° SB (p < 0.05); L2-L5, 50° RB vs. 27° SB (p < 0.05); L6-L10, 56° RB vs. 26° SB (p < 0.05). SSL increased for both groups from preoperative to the tenth lengthening (p < 0.05). As compared with RB patients, SB patients had higher SSL preoperatively and maintained this difference to the tenth lengthening (p < 0.05). After ten lengthening surgeries, when normalized to preoperative SSL, relative thoracic growth was greater for RB (27%) than for SB patients (19%) (p < 0.05). CONCLUSION: Regardless of proximal anchor choice, thoracic length continued to increase during the distraction phase of treatment for idiopathic EOS. LEVEL OF EVIDENCE: Level III.

Growth Friendly Surgery and Serial Cast Correction in the Treatment of Early-onset Scoliosis for Patients With Prader-Willi Syndrome.

BACKGROUND: Prader-Willi syndrome (PWS) patients can present with scoliosis which can be treated with serial cast correction (SCC) or with growth friendly surgery (GFS). This study's purpose was to describe the results of SCC as well as GFS for PWS patients with early-onset scoliosis (EOS). METHODS: PWS patients were identified from 2 international multicenter EOS databases. Scoliosis, kyphosis, spine height (T1-S1), right/left hemithoracic heights/widths (RHTH, LHTH, RHTW, LHTW) were measured pretreatment, postoperation, and at 2-year follow-up. Complications were recorded. RESULTS: Overall, 23 patients with 2-year follow-up were identified. Pretreatment; patients treated with SCC (n=10) had mean age of 1.8±0.6 years; body mass index (BMI), 16±1.5 kg/m; scoliosis, 45±18 degrees; kyphosis, 56±9 degrees; T1-S1, 22.4±2.4 cm; RHTH, 8.0±2.0 cm; LHTH, 8.5±1.7 cm; RHTW, 6.6±1.3 cm; and LHTW, 8.0±1.0 cm. Patients treated with GFS (n=13) had mean age of 5.8±2.6 years; BMI, 21±5.4 kg/m; scoliosis, 76±14 degrees; kyphosis, 59±25 degrees; T1-S1, 24.1±3.6 cm; RHTH, 10.0±1.6 cm; LHTH, 10.6±1.6 cm; RHTW, 9.4±2.5 cm; and LHTW, 8.1±2.8 cm. At 2-year follow-up, patients treated with SCC had mean scoliosis 37±11 degrees (18% correction, P=0.06); kyphosis, 42±6 degrees (NS); T1-S1, 26.4±2.1 cm (P<0.01); RHTH, 9.0±1.1 cm (13%; P=0.30); LHTH, 10.0±1.5 cm (18%, P<0.01); RHTW, 7.4±1.1 cm (12%, P<0.01); and LHTW, 8.0±1.0 cm (0%, P=0.34). At 2-year follow-up, patients treated with GFS had mean scoliosis 42±13 degrees (45% correction, P<0.000001); kyphosis, 53±13 degrees (10%, P=0.19); T1-S1, 31.5±5.4 cm (P<0.00001); RHTH, 12.0±2.4 cm (20%; P<0.01); LHTH, 12.0±1.7 cm (13%; P<0.01); RHTW, 9.8±1.3 cm (4%; P=0.27); and LHTW, 7.9±2.3 cm (3%;P=0.11). As an entire group, patients with a BMI>17 kg/m² had more device-related than disease-related complications (P=0.09). Patients treated with SCC had 0.9 complications per patient. Patients treated with GFS had 2.2 complications per patient [≤5 y more often had ≥2 complications (P=0.05)]. CONCLUSIONS: At 2-year follow-up, SCC and GFS were both effective in treating EOS in PWS patients. Patients treated with SCC had significant improvements in spine height and LHTH. Patients treated with GFS had significant improvements in scoliosis magnitude, spine height, RHTH, and LHTH. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Thoracogenic spinal deformity: a rare cause of early-onset scoliosis.

OBJECTIVESurgery for severe congenital defects, such as congenital diaphragmatic hernia, congenital heart defects, and tracheoesophageal disorders, are life-saving treatments for many infants. However, the incidence of scoliosis following thoracoabdominal surgery has been reported to range from 8% to 50%. Little is known about severe scoliosis that occurs after chest wall procedures in infants. The authors sought to determine the prevalence of thoracogenic scoliosis, disease severity, and need for scoliosis surgery in patients who underwent chest wall procedures in early childhood.METHODSA multicenter database of patients with early-onset scoliosis was queried to identify patients with a history of thoracogenic or acquired iatrogenic scoliosis. Patients with significant congenital spine deformities were excluded. Forty-one patients (1.6%) were noted to have thoracogenic scoliosis. Of these patients, 14 patients were observed; 10 received casts and/or braces; and 17 underwent treatment with rib-based distraction rods, Shilla procedures, or spine-based growing rod devices. Radiographs, complications, and patient characteristics were reviewed.RESULTSThe mean age at scoliosis diagnosis for the 41 patients was 6.0 years. The mean time to follow-up was 2.9 years (4.5 years in the 17 surgical patients). The mean preoperative coronal Cobb angle in the surgical group was 65° and improved to 47° postoperatively (p = 0.01). The mean Cobb angle for the nonoperative group was 31° initially and 32° at follow-up (p = 0.44). Among the 17 patients undergoing surgery for scoliosis, there were 13 complications in 7 patients, including a brachial plexus palsy following rib-based distraction rod placement. This resolved with revision of the rib hooks. There were no known complications in the nonoperative cohort.CONCLUSIONSSevere scoliosis can develop after thoracoabdominal surgeries during infancy. Further work is needed to understand the pathogenesis of scoliosis in this population so as to implement measures for prevention and early diagnosis and to guide appropriate treatment.

Classification of Early Onset Scoliosis has Excellent Interobserver and Intraobserver Reliability.

BACKGROUND: Classification systems can be useful tools for clinical care and research but must be proven as reliable. The purpose of this study was to evaluate the interobserver and intraobserver reliability of the Classification of Early Onset Scoliosis (C-EOS) scheme. METHODS: After IRB approval, 50 cases were drawn from a prospectively collected database of patients with EOS. Cases were selected using a stratified randomization scheme based on etiology. These cases were used to create an internet survey that was sent to pediatric orthopaedic faculty, research coordinators, and fellows involved in EOS care and research. Participants were asked to classify each case and were provided with a written C-EOS scheme that could be referenced while they completed the survey. Surveys were sent to participants twice, 3 weeks apart, to assess both intraobserver and interobserver reliability. Fleiss κ and Cohen κ were used to assess interobserver and intraobserver reliability, respectively. RESULTS: There were 36 total participants, 29 who completed the survey twice (21 faculty, 13 research coordinators, and 2 fellows). Overall Fleiss κ coefficient for interobserver reliability was excellent across the major categories of etiology (0.84), major curve (0.93), and kyphosis (0.96). Overall intraobserver reliability was excellent with Cohen κ values for etiology (0.92), major curve (0.96), and kyphosis (0.98). Faculty members had excellent agreement for etiology (0.90), major curve (0.91), and kyphosis (0.96). Research coordinators had high levels of agreement for etiology (0.78), and excellent for major curve (0.95) and kyphosis (0.96). Intraobserver reliability was excellent across all major categories for all groups: faculty, research coordinators, and fellows. CONCLUSIONS: The study shows high levels of interobserver and intraobserver agreement of the C-EOS scheme. The C-EOS scheme can be used as a reliable tool for classifying EOS patients for clinical communication and research. LEVEL OF EVIDENCE: Level II-diagnostic study.