Idiopathic Early-onset Scoliosis Research Articles

Minimally Invasive Controlled Growing Rods for the Surgical Treatment of Early-Onset Scoliosis—A Surgical Technique Video

Background: Spinal deformities in children and adolescents can be easily divided into those occurring and diagnosed before the age of 10—early-onset scoliosis—and those occurring and diagnosed after the age of 10—late-onset scoliosis. When the curvature continues to progress and exceeds a Cobb angle of more than 60–65 degrees, surgical treatment should be considered. The most common treatment procedure for EOS is the surgical correction of the deformity using standard growing rods (SGRs), and in the case of congenital defects with additional hemivertebrae, it is the resection of the hemivertebra and short fusion. Minimally invasive controlled growing rods (MICGRs) need to be distracted every 6–9 months through a minimally invasive approach that involves sedation and neuromonitoring to obtain the best possible correction while minimizing complications. The aim of our study is to present a less-invasive surgical technique for MICGR implantation based on a two-case presentation—early-onset idiopathic scoliosis and congenital kyphosis. The surgical technique is the less-invasive percutaneous and subfascial implantation of MICGRs without long incisions in the back. Conclusions: The use of MICGRs is an alternative and safe surgical technique for patients undergoing surgical treatment for EOS. Without the risk of metallosis, like in other implant systems, and the need for replacement after 2 years of use, like in using magnetically controlled growing rods (MCGRs), the MICGR system can be used as a less-invasive procedure, allowing for the avoidance of many periodic invasive procedures in children with a wider opening of the spine (like in using standard growing rods), minimizing the number of planned hospitalizations, reducing the length of hospital stays, and reducing the physical and mental burdens on young patients, parents, and families.

Spinal height for growth guidance treatment in early onset idiopathic scoliosis: analysis through final surgical treatment.

The growth guidance (GG) method for treatment of early onset scoliosis has as its primary goal the restoration of apical spinal alignment, facilitating normal spinal growth to achieve a suitable adult thoracic height. To evaluate whether GG surgical treatment achieves comparable thoracic and spinal height to distraction-based treatment (DBT) in idiopathic early onset scoliosis (I-EOS) patients. We hypothesized that GG would prove superior to DBT at the time of definitive fusion surgery. All I-EOS patients who underwent GG at a single center were reviewed. T1-L1 and T1-S1 heights were measured using the traditional coronal method as well as the Halifax sagittal spinal length (SSL) technique. The same measurements were obtained from a comparable control group obtained from a multi-center pediatric early onset scoliosis database who underwent treatment with traditional growing rods (TGR) or magnetically controlled growing rods (MCGR). Of the I-EOS patients who underwent GG 2004-2019, 15 patients underwent final fusion after GG completion, with a mean 5.5years of GG treatment prior to fusion (range 2.0-11.4years). Mean age at GG implantation was 8.4years (range 2.0-11.7years); 7 were male and 8 female. GG patients experienced a mean coronal thoracic height increase of 6.2cm during treatment through final fusion, and a mean coronal spinal height increase of 8.8cm. At the time of final fusion, GG patients achieved greater significant mean increases than DBT patients by 2.9cm in coronal thoracic height (p = 0.0023), 4.5cm in coronal spinal height (p = 0.001), and 4.0cm in SSL spinal height (p = 0.01). No GG patient concluded treatment with a thoracic height less than 18cm in either coronal or sagittal plane. Not only did 100% of GG patients reach minimum thoracic height of 18cm at time of final fusion, but GG also proved to be superior to distraction-based constructs in a comparison cohort on 3 of 4 spinal elongation measures. 3.

The efficacy of bracing in the treatment of progressive early-onset scoliosis

Serial casting as one of the applications to treat early-onset scoliosis has been reported efficiently to improve deformity, but no report has focused on the efficacy of braces in the treatment of congenital early-onset scoliosis and comparison with progressive idiopathic early-onset scoliosis. Patients with progressive EOS treated with braces in our institution with a minimum of 4 years follow-up were reviewed. Two groups according to the etiological diagnosis were analyzed and compared: the congenital scoliosis (CS) group and idiopathic scoliosis (IS) group. The success cases and the failure cases were also compared. 27 patients with an average main Cobb angle of 38.19° (20–55) underwent initial bracing at an average age of 55.7 months (24–108), the average follow-up time was 76.19 months (49–117). In IS group the main Cobb angle was corrected to 18.69 ± 12.06° (48.61%) following the first bracing; the final Cobb angle was 23.08 ± 22.15°(38.76%) after brace removal. In CS group the main Cobb angle was corrected to 33.93 ± 10.31°(17.1%) following the first bracing and 37.93 ± 14.74°(3.53%) after brace removal. Both coronal chest width and T1-T12 height increased dramatically from pre-bracing to the last follow-up. Patients diagnosed as IS tended to have a better result in main Cobb angle correction than that of CS (P = 0.049). By the time of last follow-up, 8 patients had undergone surgery, and the operation time was postponed by 68.88 ± 26.43 months. For patients with progressive early-onset scoliosis, bracing is an efficient nonsurgical alternative to casting, and some of them can be cured; if not, eventual surgical intervention can be delayed for a period of time without restrictions on the thoracic cavity.

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.

Clinical Outcomes of 111 Patients with Early Onset Idiopathic Scoliosis (EOIS) Receiving Brace Treatment: A Longitudinal Retrospective Cohort Study

Introduction: Bracing is one of the first-line treatment for early-onset idiopathic scoliosis (EOIS) to control curves from progression. This study aimed to explore the determinants that govern bracing effectiveness in EOIS. Methods: One hundred and eleven patients with EOIS (mean age of 8.6 ± 1.25 at diagnosis) received bracing treatment and had a final follow-up beyond skeletal maturity were identified from records between 1988 and 2021. Demographic data and clinical features of spinal curvature were obtained for correlation analyses to determine the associations between curve outcomes and clinical features. Results: Most patients were female (85.6%) and had a major curve on the left side (67%). The mean baseline Cobb angle of major curves was 21.73 ± 7.92°, with a mean Cobb angle progression of 18.05 ± 19.11°. The average bracing duration was 5.3 ± 1.9 years. Only 26 (23.4%) of them underwent surgery. The final Cobb angle and curve progression at the final follow-up with a Cobb angle of ≥50° were positively correlated with the initial Cobb angle (r = 0.206 and r = 0.313, respectively) and negatively correlated with maturity parameters. The lumbar curve type was found to correlate with a smaller final Cobb angle. Conclusions: The majority of patients had a final Cobb angle < 50°, which was considered a successful bracing outcome. The final Cobb angle correlated with the initial Cobb angle and curve types observed in EOIS.

PATOLOGIA NEUROAXIAL OCULTA NA ESCOLIOSE IDIOPÁTICA: PESQUISA ORIGINAL

ABSTRACT Objective: To evaluate the prevalence of hidden neuraxial pathology (NAP) revealed in idiopathic scoliosis (IS) in neurologically normal patients. Methods: We selected 401 patients with IS who visited our clinic. We identified patterns of NAP and its frequency. In addition to the main part of the study, we assessed the reliability of Magnetic Resonance Imaging (MRI) measuring of the pedicles to plan screw width and trajectory. Results: Among the 401 patients, 53 (13%) presented NAP. The proportion of males in this group was higher (42% vs. 21%, p=0.004), the age of onset of the deformity was lower (8.9±3.77 vs. 9.9±3.93 years old, p=0.045), the left-sided thoracic curve was more frequent (21% vs. 8%, p=0.016), thoracic kyphosis was more pronounced (p=0.070), and the percentage of revision surgeries for deformity progression or non-fusion was higher (13% vs. 5%, p=0.147). Conclusions: The spine MRI should be performed in the early stages of IS, as in some cases of NAP (Chiari, tethered spinal cord), there is the possibility of an early neurosurgical operation that will prevent the development of scoliosis. The main signs of hidden NAP in IS are early-onset IS, IS with left-sided thoracic curve, male gender, and thoracic kyphosis > 40°Cobb. Level of Evidence II; Retrospective Study.

Do Pedicle Screws That Terminate in the Costovertebral Joint Compromise Proximal Anchor Fixation in a Dual Growing Rod Construct?

Growing rods (GRs) are used to treat early-onset scoliosis (EOS) recalcitrant to bracing and casting. Proximal anchor pullout, a known complication of GR constructs, can result in spinal cord injury if pedicle screw anchors are placed with a lateral-to-medial trajectory. To mitigate this risk, a more straightforward and potentially safer trajectory may result in screws that terminate within the costovertebral joint (CVJ). We asked, how often does CVJ placement occur and does this technique increase the rate of failure in GR constructs? We retrospectively reviewed 35 patients with EOS treated with dual GR with >2-year follow-up excluding patients with skeletal dysplasia or history of previous posterior instrumentation. Patient demographics, radiographic parameters, and implant constructs were assessed. Of patients meeting the inclusion criteria, 18/35 (51%) were females with an average age of 7.96 (range: 4.0 to 15.2) years at surgery with a follow-up of 3.7 (range: 2.0 to 7.7) years. Five (14%) patients had idiopathic EOS, 5 (14%) had congenital EOS, 12 (34%) had neuromuscular EOS, 10 (29%) had syndromic scoliosis, and 3 (9%) had another etiology for EOS. Among 195 proximal pedicle screws placed, 19 (10%) terminated within the CVJ, and 13 patients (37%) had at least 1 CVJ screw. Two patients (6%) experienced unilateral proximal pullout. In both patients, the anchors on the affected side included 1 of 2 screws within the CVJ. Both patients had constructs that included 2 screws on the side that pulled out and 3 screws on the side that did not. None of the remaining 17 CVJ screws led to implant failure. Pedicle screw placement within the CVJ is common and does not appear to significantly contribute to proximal screw pullout; however, it may contribute to unilateral implant failure in constructs employing only 2 proximal screws, where 1 of those 2 screws terminates within the CVJ. Construct modifications should be considered in this scenario. Level III.

A Comprehensive Assessment of Psychosocial Well-being Among Growing Rod Graduates: A Preliminary Investigation.

Traditional growing rods (TGR) encompass a long process, in which patients experience physical and psychosocial difficulties. However, the effect of repeating surgeries on the overall psychological functioning of graduated patients has not been thoroughly investigated in the literature. The aim of this study is to evaluate the psychological well-being of graduated idiopathic early-onset scoliosis patients in terms of psychopathology, neurocognition, and psychosocial functioning, and determine the accuracy of scoliosis outcome questionnaires in these regards. TGR graduates with idiopathic early-onset scoliosis without known intellectual disabilities or neuromuscular impairments were included. Patients were thoroughly evaluated using psychological instruments [Wechsler Adult Intelligence Scale, Auditory Consonant Trigram Test, Verbal Fluency Test, Beck Depression Inventory, Beck Anxiety Inventory, Rosenberg Self-Esteem Scale, Symptom Checklist-90, Post-Traumatic Growth Inventory, Strengths and Difficulties Questionnaire, Spinal Appearance Questionnaire, Scoliosis Research Society 22-item questionnaire (SRS-22)]. Results were compared with normative data when available. Spearman correlations were performed between the results of these tests, the total treatment duration, and the number of spinal surgeries. Of the 15 patients included in the study, 9 were females, and the mean age was 18.73 (16 to 23). The mean age at index surgery was 6.38 (3 to 10) whereas that of graduation was 14.00 (12 to 16). The average number of spinal surgeries was 14.28 (7 to 20). Two patients performed below the range of adult intellectual functioning. Auditory Consonant Trigram Test showed normal verbal working memory and attention control. Six patients had abnormal Verbal Fluency Test performance. Eight patients had abnormal ratings on at least one of the assessment scales of psychopathology (Symptom Checklist-90, Beck Depression Inventory, and Beck Anxiety Inventory). Eight patients had low-to-moderate self-esteem (Rosenberg self-esteem scale). The median spinal appearance questionnaire and SRS-22 scores were 34 and 4.18, respectively. Pain and function subdomains of SRS-22 scored higher than self-image and mental health. No correlation was found between the treatment duration and number of surgeries and test scores. SRS-22 showed correlations with multiple psychological tests. Completed TGR treatment yields acceptable correction of deformities and surgical outcomes, however, may fail to improve psychological well-being. This is the first study to find various psychosocial abnormalities in two-thirds of cases. Level IV; cross-sectional study.

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.

Spinal mobility and muscle function in middle-aged patients treated for early onset idiopathic scoliosis: compared with untreated and treated adolescent onset patients

PurposeTo determine long-term outcome in terms of spinal range of motion (ROM) and trunk muscle endurance (TME) patients treated for idiopathic scoliosis, diagnosed before the age of ten, were evaluated and compared with untreated or treated patients with idiopathic scoliosis with adolescent onset (AIS).MethodsSixty-three braced and 53 operated patients underwent examination of spinal ROM and TME. Validated questionnaires were used for evaluation of back function.ResultsA total of 116 patients were examined 26.5 years after treatment. Braced EOS patients had longer bracing time and operated EOS patients had longer fusions compared to the respective AIS groups. Braced EOS patients had similar total ROM (thoracic ROM 40°, lumbar ROM 78°) and TME (trunk flexors 140 s, trunk extensors 255 s) as untreated AIS patients (thoracic ROM 34°, lumbar ROM 88°, trunk flexor endurance 158 s, trunk extensor endurance 234 s). Braced patients also had significantly better results than braced AIS patients. Operated EOS patients were slightly but significantly stronger and more mobile compared to AIS peers. The lumbar ROM was found to affect the back function in the operated EOS group (Oswestry Questionnaire, rs = 0.49, p < 0.001).ConclusionsThe braced EOS patients had mostly similar muscle strength and mobility as the untreated but younger AIS group, while the braced AIS group showed reductions of both strength and mobility. Similar significant, but small, differences were also found between operated EOS and AIS patients. Especially for muscle strength were findings at a level that would be of significant clinical importance.Levels of evidenceIII.

Outcomes of Definitive Spine Fusion Using All-pedicle-Screw Constructs in Skeletally Immature Patients Aged 8 to 10 Years With Severe Idiopathic Early-Onset Scoliosis.

The choice between growth-sparing techniques or definitive spine fusion for severe idiopathic early-onset scoliosis (IEOS) in skeletally immature patients aged 8 to 10 years represents a challenging dilemma. Although growth-sparing techniques show high complication rates in severe IEOS, the outcomes of definitive fusion in borderline skeletally immature patients with severe IEOS have not been investigated. We aimed to investigate the outcomes of early definitive fusion using all-pedicle-screw constructs in skeletally immature patients aged 8 to 10 years with severe IEOS. The inclusion criteria were as follows: IEOS, age 8 years or above, major coronal curve ≥90 degrees, thoracic height >18 cm, no history of previous spine surgery, no intraspinal anomalies and at least 3 years of postoperative follow-up. Patients underwent instrumented spinal fusion with all-pedicle-screw constructs and multiple Ponte osteotomies. All patients completed the Scoliosis Research Society 22 revision (Arabic version) questionnaire and Body Image Disturbance Questionnaire-Scoliosis version (BIDQ-S) preoperatively and at the last follow-up. Fifty-five patients (24 males, 31 females; mean age: 8.96 y; range: 8 to 10 y) with severe IEOS met the inclusion criteria (mean follow-up period: 4.1±0.6 y; range: 3 to 5 y). The mean major coronal Cobb angle improved significantly (P<0.001) from 107±12.5 degrees to 26.8±6.8 degrees. Mean thoracic kyphosis improved significantly (P<0.001) from 57.2±15.8 degrees to 31.2±4.4 degrees. The loss of correction at the latest follow-up was nonsignificant. The total Scoliosis Research Society 22 revision (SRS-22r) score improved significantly from 2.5±1 to 4.3±0.7. The mean BIDQ-S score improved significantly from 4.1±0.3 to 1.6±0.3. The immediate postoperative gains in the mean thoracic height (T1-T12) and spinal height (T1-S1) were 14.9% and 19.6%, respectively, and the overall height increase at the latest follow-up was 17.8% and 23.8%, respectively. One patient underwent revision for implant failure (rod breakage). Early definitive fusion for skeletally immature patients with severe IEOS yielded excellent correction with major improvements in patient quality of life. Severe IEOS poses a significant risk, but definitive fusion can potentially mitigate that risk in patients aged 8 to 10 years.

Analysis of friction and wear processes in an innovative spine stabilization system. Part 2. A study and model of the wear of a metal rod-polymer cord friction joint

Purpose: The purpose of this study was to model and describe the processes and phenomena occurring during the sliding interaction between biometal rods (titanium alloys Ti6Al4V and Ti6Al7Nb, austenitic steel AISI 316L, alloy CoCrMo) and PE-UHMW cords, used in spine stabilization systems to treat early-onset idiopathic scoliosis. Methods: The wear of friction joints in two lubricating solutions (acidic sodium lactate and distilled water) at stabilized temperature T = 38 C was studied. The wear of the polymeric cords was investigated through analyses of the chemical composition of the surface of the cords and microscopic examinations of the changes occurring on this surface. In addition, microscopic examinations and EDS analyses of the wear products filtered out from the lubricating medium were carried out. Results: Metallic particles were found to be present in both lubricating solutions at each stage of the friction process. The largest amount of metallic particles was recorded after 5000 motion cycles. Conclusions: The presence of metallic wear products is an evidence of the wear of the harder metal rod due to its friction against the PE-UHMW cord. This means that the use of guided-growth implants poses a risk of inflammations in the peri-implant tissues.

Analysis of friction and wear processes in an innovative spine stabilization system. Part 1. A study of static and kinetic friction of a metal rod-polymer cord friction joint

Purpose: This analysis is the first part of research that aims to develop a model of the tribological wear of PE-UHMW cord – biometal rod combination. This type of sliding joint is applied in spine stabilization systems that enable the treatment of early-onset idiopathic scoliosis. Methods: The friction tests included force measurements, followed by the determination of static and kinetic friction coefficients as a function of the number of the performed movement cycles, and static friction coefficient with regards to the string tension force FN in the range of 50–300 N. Additionally, the surface roughness and microscopic observations of the metal rods were made. The friction measurements were carried out at a stabilized temperature T = 38 °C in the presence of distilled water and acidic sodium lactate. Results: The measurements confirmed the impact of both the number of completed movement cycles and the value of the force loaded on the cord on the static friction coefficient. Similar values of kinetic friction force occur for the pairs with the titanium alloys rods, as well as for the pairs with the steel and CoCr rod. The type of lubricant affected the obtained measurement results unevenly: (Ti6Al4V and Ti6Al7Nb – slight impact, steel 316L and CoCrMo – large impact). During microscopic observations, numerous wear products, were visible, including harder than the base material large conglomerates. Conclusions: Susceptibility of polymer fibres results in its increased resistance to wear, but it can be also combined with an increase in wear of the surface of the metal rod.

Magnetically Controlled Growing Rods (MCGR) Versus Single Posterior Spinal Fusion (PSF) Versus Vertebral Body Tether (VBT) in Older Early Onset Scoliosis (EOS) Patients: How Do Early Outcomes Compare?

Retrospective review of prospective data from multicenter registry. Compare outcomes of posterior spinal fusion (PSF) versus magnetically controlled growing rods (MCGR) versus vertebral body tethers (VBT) in 8- to 11-year-old idiopathic early onset scoliosis (EOS) patients. In EOS, it is unclear at what age the benefit of growth-sparing strategies outweighs increased risks of surgical complications, compared with PSF. One hundred thirty idiopathic EOS patients, 81% female, aged 8-11 at index surgery (mean 10.5 yrs), underwent PSF, MCGR, or VBT. Scoliosis curve, kyphosis, thoracic and spinal height, complications, and Quality of Life (QoL) were assessed preoperatively and at most recent follow-up (prior to final fusion for VBT/MCGR). Of 130 patients, 28.5% received VBT, 39.2% MCGR, and 32.3% PSF. The VBT cohort included more females (P < 0.0005), was older (P < 0.0005), more skeletally mature (P < 0.0005), and had smaller major curves (P < 0.0005). At follow-up, scoliosis curve corrected 41.1 ± 22.4% in VBT, 52.2 ± 19.9% in PSF, and 27.4 ± 23.9% in MCGR (P < 0.0005), however, not all VBT/MCGR patients finished treatment. Fifteen complications occurred in 10 VBTs, 6 requiring unplanned surgeries; 45 complications occurred in 31 MCGRs, 11 requiring unplanned surgeries, and 9 complications occurred in 6 PSFs, 3 requiring unplanned revisions. Cox proportional hazards regression adjusted for age, gender, and preoperative scoliosis curve revealed that MCGR (hazard ratio [HR] = 21.0, 95% C.I. 4.8-92.5; P < 0.001) and VBT (HR = 7.1, 95% C.I. 1.4-36.4; P = 0.019) patients were at increased hazard of requiring revision, but only MCGR patients (HR = 5.6, 95% C.I. 1.1-28.4; P = 0.038) were at an increased hazard for unplanned revisions compared with PSF. Thoracic and spinal height increased in all groups. QoL improved in VBT and PSF patients, but not in MCGR patients. In older idiopathic EOS patients, MCGR, PSF, and VBT controlled curves effectively and increased spinal height. However, VBT and PSF have a lower hazard for an unplanned revision and improved QoL.Level of Evidence: 3.

Development of orthosis following EDF serial casting for infantile scoliosis.

Elongation-de-rotation-flexion (EDF) casting is a popular treatment for early-onset-scoliosis (EOS). However, casting every 2 to 3 months using general anesthesia may affect cognitive function.[1,2] Aims of this study: 1) to develop a new orthosis for EOS treatment based on EDF technique (EDFO) and traction frame; 2) to evaluate emerging radiographic results from treatment. Mehta's EDF serial casting method and AMIL traction frame were used to manually correct the spine for 3D trunk scan. Afterward, a digital spinal model was created and helped design the EDFO with CAD/CAM technology. Radiographic measurements included Cobb angle, RVAD, and thoracic height and width. Six patients (2 girls; 4 boys) diagnosed with idiopathic EOS were enrolled in the study. EDFO was applied at mean 36.5 months of age, after final EDF casting. The average major Cobb angle stabilized after treatment. Average RVAD increased. The average normalized thoracic width at last EDFO out-of-brace was less than prior to EDFO. The new asymmetric EDFO offers an alternative to serial casting and TLSO. EDFO is considered a cost-effective, safer, more breathable, removable, and less invasive modality.

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.

Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure.

There are few reports on the surgical management of early-onset scoliosis (EOS) associated with Marfan syndrome (MFS). Affected patients tend to have more rapid curve progression than those with idiopathic EOS, and their course is further complicated by medical comorbidities. As surgical techniques and implants for growing spines become more widely applied, this study seeks to better delineate the safety and efficacy of growth-friendly spinal instrumentation in treating this population. A prospective registry of children treated for EOS was queried for MFS patients treated between 1996 and 2016. Forty-two patients underwent rib-based or spine-based growing instrumentation and were assessed on preoperative, surgical, and postoperative clinical and radiographic parameters including complications and reoperations. Subgroup analysis was performed based on spine-based versus rib-based fixation. Patients underwent their index surgery at a mean age of 5.5 years, when the major coronal curve and kyphosis measured 77 and 50 degrees, respectively. Over half were treated with traditional growing rods. Patients underwent 7.2 total surgical procedures-4.7 lengthening and 1.9 revision surgeries not including conversion to fusion-over a follow-up of 6.5 (±4.1) years. Radiographic correction was greatest at index surgery but maintained over time, with a final thoracic height measuring 23.8 cm. Patients experienced a mean of 2.6 complications over the course of the study period; however, a small group of 6 patients experienced ≥6 complications while over half of patients experienced 0 or 1. Implant failures represented 42% of all complications with infection and pulmonary complications following. This is the largest report on patients with EOS and MFS. All subtypes of growth-friendly constructs reduced curve progression in this cohort, but complications and reoperations were nearly universal; patients were particularly plagued by implant failure and migration. Further collaborations are needed to enhance understanding of optimal timing and fixation constructs for those with MFS and other connective tissue diseases.

Mid-term results of a modified self-growing rod technique for the treatment of early-onset scoliosis.

To report the mid-term results of a modified self-growing rod (SGR) technique for the treatment of idiopathic and neuromuscular early-onset scoliosis (EOS). We carried out a retrospective analysis of 16 consecutive patients with EOS treated with an SGR construct at a single hospital between September 2008 and December 2014. General demographics and deformity variables (i.e. major Cobb angle, T1 to T12 length, T1 to S1 length, pelvic obliquity, shoulder obliquity, and C7 plumb line) were recorded preoperatively, and postoperatively at yearly follow-up. Complications and revision procedures were also recorded. Only patients with a minimum follow-up of five years after surgery were included. A total of 16 patients were included. Six patients had an idiopathic EOS while ten patients had a neuromuscular or syndromic EOS (seven spinal muscular atrophy (SMA) and three with cerebral palsy or a syndrome). Their mean ages at surgery were 7.1 years (SD 2.2) and 13.3 years (SD 2.6) respectively at final follow-up. The mean preoperative Cobb angle of the major curve was 66.1° (SD 8.5°) and had improved to 25.5° (SD 9.9°) at final follow-up. The T1 to S1 length increased from 289.7 mm (SD 24.9) before surgery to 330.6 mm (SD 30.4) immediately after surgery. The mean T1 to S1 and T1 to T12 growth after surgery were 64.1 mm (SD 19.9) and 47.4 mm (SD 18.8), respectively, thus accounting for a mean T1 to S1 and T1 to T12 spinal growth after surgery of 10.5 mm/year (SD 3.7) and 7.8 mm/year (SD 3.3), respectively. A total of six patients (five idiopathic EOS, one cerebral palsy EOS) had broken rods during their growth spurt but were uneventfully revised with a fusion procedure. No other complications were noted. Our data show that SGR is a safe and effective technique for the treatment of EOS in nonambulatory hypotonic patients with a neuromuscular condition. Significant spinal growth can be expected after surgery and is comparable to other published techniques for EOS. While satisfactory correction of the deformity can be achieved and maintained with this technique, a high rate of rod breakage was seen in patients with an idiopathic or cerebral palsy EOS. Cite this article: Bone Joint J 2020;102-B(11):1560-1566.

A report of two conservative approaches to early onset scoliosis: serial casting and bracing.

Previous reports have demonstrated the effectiveness of casting for EOS. Brace treatment for EOS has not been studied. The purpose of this multicenter retrospective study was to compare radiographic outcomes, complications, and rates of conversion to surgery in children with EOS treated with casting or bracing. Children aged 2-6years with idiopathic or neuromuscular EOS treated with casting or bracing with minimum follow-up of 2years were identified. 68 patients (36 cast, 32 brace) were analyzed. Diagnosis, age at start of treatment, and duration of follow-up were similar. Although the cast patients had a larger pre-treatment major curve magnitude (50° vs 31°, p < 0.001), both groups had a similar major curve magnitude at most recent follow-up (36° vs 32°, p = 0.456). T1-T12 and T1-S1 length increased in both groups. The cast and brace patients had similar complications and conversions to surgery. Sub-analysis showed that while casting resulted in curve improvement regardless of etiology, bracing was able to prevent curve progression in patients with idiopathic EOS but not in patients with non-idiopathic EOS (Δ- 15° vs 27°, p = 0.006). Regression analysis (significance p = 0.10) controlling for baseline age, major curve magnitude, and T1-T12 and T1-S1 length showed that treatment method was associated with difference in major curve magnitude (p = 0.090) and T1-T12 length (p = 0.024). In our study, serial casting led to curve improvement in children with idiopathic and neuromuscular EOS, whereas brace treatment appeared to prevent curve progression in patients with idiopathic EOS but did not appear to control the curve in neuromuscular EOS.