Maturation of the vertebral ring apophysis is delayed in girls with adolescent idiopathic scoliosis compared to the normal population.

PURPOSE: The ring apophysis is a secondary ossification center on both sides of each vertebral body, to which the annulus of the intervertebral disc inserts. Recently, its pattern of ossification and fusion to the vertebral body was described for the normal growing spine. The aim of the present study was to investigate the ossification and fusion of the ring apophysis in patients with adolescent idiopathic scoliosis (AIS) and compare it to the normal growing population. METHODS: Ring apophysis maturation along the entire thoracic and lumbar spine was analyzed on CT scans of 99 female, pre-operative AIS patients and compared to 134 CT scans of non-scoliotic girls, aged 12 to 20. RESULTS: The ring apophysis maturation in AIS patients was delayed at all spinal levels in AIS patients compared to non-scoliotic controls. Ossification starts at T4-T11 at age 12, followed by T1-T5 and L3-S1 at age 15. The fusion process in AIS patients continues longer in the midthoracic region as compared to the other regions and as compared to non-scoliotic controls, with many incomplete fusions still at age 20. CONCLUSION: The ring apophysis maturation in AIS is delayed compared to that in the normal population and lasts longer in the mid/low thoracic spine. Delayed maturation of the spine's most important stabilizer, while the body's dimensions continue to increase, could be part of the patho-mechanism of AIS.

Early-term outcome of apical fusion with vertebral body tethering for thoracolumbar curves in adolescent idiopathic scoliosis: a preliminary study.

INTRODUCTION: Vertebral body tethering (VBT) has become an alternative option for select patients with idiopathic scoliosis. However, studies have shown a high number of tether breakages, specifically after thoracolumbar (TL) VBT, that can have a negative impact on the outcome, when the breakage occurs within the first year after surgery. In order to overcome this problem, we have started to apply an apical fusion (AF) in combination with TL VBT for select patients. This study aims to analyze the outcome after AF plus VBT. METHODS: This is a retrospective single surgeon's data analysis. All patients were included who have had TL VBT after January 2022 and a follow-up of 12 months. Patients were grouped based on whether they only had VBT or VBT + AF. RESULTS: Twenty-five patients were analyzed (15 VBT, 10 VBT + AF). Both groups showed a significant curve correction for thoracic and TL curves. Minor loss of correction was observed in both groups. A significant difference was seen regarding early tether breakages, which were found in 60% of VBT patients and 10% of VBT + AF patients. CONCLUSION: The preliminary data shows a significant reduction of early tether breakages when TL VBT is applied in combination with AF.

The Mid-term Outcome of Intervertebral Disc Degeneration after Direct Vertebral Rotation in Adolescent Idiopathic Scoliosis: Magnetic Resonance Imaging-based Analysis for a Mean 11.6-year Follow-up.

STUDY DESIGN: A retrospective cohort study. OBJECTIVE: To evaluate the mid-term effect of intervertebral disc degeneration (DD) in adolescent idiopathic scoliosis (AIS) patients who underwent pedicle screw instrumentation (PSI) and rod derotation (RD) with direct vertebral rotation (DVR). SUMMARY OF BACKGROUND DATA: Posterior spinal fusion is a mainstay of surgical treatment in AIS, and DVR is considered a main corrective maneuver for vertebral rotation. However, the mid-term effect of intervertebral DD after DVR is still unknown in AIS. METHODS: A total of 336 vertebrae for 48 AIS patients who underwent PSI and RD with DVR were retrospectively assessed for intervertebral DD. They were divided into two groups based upon intervertebral DD, defined as Pfirmann grade more than IV. The Pfirrmann grade and modic change were evaluated at the disc above the uppermost instrumented vertebra (UIV), the disc below the lowest instrumented vertebra (LIV), and the lumbar disc levels. RESULTS: With the 11.6 years of mean follow-up, 41.7% (20/48) of patients exhibited DD, while modic changes were observed in 4.2% (2/48) of included patients. The disc below the LIV, L4-5, and L5-S1 were significantly shown to have an increasing trend of Pfirmann grade. The preoperative thoracic kyphosis was significantly lower in the DD group (22.0°) than in the non-DD group (31.4°) (P = 0.025) and negatively correlated with DD (r = -0.482, P = 0.018). The Pfirrmann grade of L5-S1 showed a high level of correlation with DD (r = 0.604, P < 0.001). CONCLUSIONS: The degenerative change at the disc below the LIV, L4-5, and L5-S1 levels was observed following PSI and RD with DVR. Thoracic hypokyphosis may negatively influence intervertebral discs in AIS patients required for deformity correction. Therefore, restoration of thoracic kyphosis is important to prevent long-term DD in AIS.

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.

Heath-related quality of life and functional outcomes in patients with congenital or juvenile idiopathic scoliosis after an average follow-up of 25 years: a cohort study.

BACKGROUND CONTEXT: Congenital and juvenile scoliosis are both early-onset deformities that develop before the age of 10. Children are treated to prevent curve progression and problems in adulthood such as back pain and a decreased quality of life but literature on long-term outcomes remains scarce. PURPOSE: To evaluate the health-related quality of life (HRQoL) and potential disability of children with congenital scoliosis (CS) or juvenile idiopathic scoliosis (JIS) after a minimum of 20 years follow-up. STUDY DESIGN: Comparative cohort study. PATIENT SAMPLE: A consecutive cohort of CS and JIS patients were retrospectively identified from a single-center scoliosis database. Patients born between 1968 and 1981 and treated during skeletal growth were eligible for participation. OUTCOME MEASURES: HRQoL (SF-36, SRS-22r, ODI). METHODS: The primary aim was to evaluate the HRQoL of CS and JIS patients using the general SF-36 questionnaire. Both patient cohorts were compared with age-matched national norms. The secondary aim was to analyze the differences between conservatively and surgically treated patients using the scoliosis-specific Scoliosis Research Society-22r questionnaire (SRS-22r) and the Oswestry Disability Index (ODI). T-tests were used for statistical comparison. RESULTS: In total, 114 patients (67% of the eligible patients) completed the questionnaire, with a mean follow-up of 25.5±5.5 years after their final clinical follow-up. Twenty-nine patients with CS were included with a mean age of 44.4±3.8 years (79.3% female), and 85 patients with JIS with a mean age of 43.7±4.2 years (89.4% female). Of the SF-36 domains, only the vitality score (60.6±18.0 for CS and 58.1±17.6 for JIS cohort) and mental health score (70.0±18.4 for CS and 72.1±18.1 for JIS cohort) were significantly lower compared with the general population (68.6±19.3 for vitality, and 76.8±17.4 for mental health). These decreased scores were larger than the determined minimum clinically important difference threshold of 4.37. Surgically treated JIS patients had a significantly lower score on the SRS-22r pain domain than their nonsurgically treated peers (3.6±0.9 vs 4.1±0.7l p=.019). Surgically treated CS patients had a significantly higher score on the SRS-22r mental health domain than their nonsurgically treated peers (4.3±0.5 vs 3.5±1.0; p=.023). No significant differences were found in the other domains. CONCLUSIONS: Except for vitality and mental health domains, congenital and juvenile idiopathic scoliosis patients treated during skeletal growth had similar HRQoL on most SF-36 domains in adulthood compared with national norms. Surgical treated JIS patients experienced more pain compared with brace treated patients, while braced CS patients had a significantly lower mental scores compared with surgical treated patients. These long-term outcomes are essential to inform patients and can guide shared decision-making between clinicians and patients.

Monitoring of Curve Progression in Patients with Adolescent Idiopathic Scoliosis Using 3-D Ultrasound.

OBJECTIVE: The aim of the work described here was to determine whether 3-D ultrasound can provide results comparable to those of conventional X-ray examination in assessing curve progression in patients with adolescent idiopathic scoliosis (AIS). METHODS: One hundred thirty-six participants with AIS (42 males and 94 females; age range: 10-18 y, mean age: 14.1 ± 1.9 y) with scoliosis of different severity (Cobb angle range: 10º- 85º, mean: of 24.3 ± 14.4º) were included. Each participant underwent biplanar low-dose X-ray EOS and 3-D ultrasound system scanning with the same posture on the same date. Participants underwent the second assessment at routine clinical follow-up. Manual measurements of scoliotic curvature on ultrasound coronal projection images and posterior-anterior radiographs were expressed as the ultrasound curve angle (UCA) and radiographic Cobb angle (RCA), respectively. RCA and UCA increments ≥5º represented a scoliosis progression detected by X-ray assessment and 3-D ultrasound assessment, respectively. RESULTS: The sensitivity and specificity of UCA measurement in detecting scoliosis progression were 0.93 and 0.90, respectively. The negative likelihood ratio of the diagnostic test for scoliosis progression by the 3-D ultrasound imaging system was 0.08. CONCLUSION: The 3-D ultrasound imaging method is a valid technique for detecting coronal curve progression as compared with conventional radiography in follow-up of AIS. Substituting conventional radiography with 3-D ultrasound is effective in reducing the radiation dose to which AIS patients are exposed during their follow-up examinations.

Adolescent Idiopathic Scoliosis: Is the Feasible Option of Minimally Invasive Surgery using Posterior Approach?

The purpose of this systematic review and meta-analysis is to perform a systematic review and meta-analysis of previous studies on minimally invasive scoliosis surgery (MISS) in adolescents with idiopathic scoliosis (AIS). Some data on MISS in AIS compared with conventional open scoliosis surgery (COSS) are conflicting. A systematic literature search was conducted in Medline, Embase, and Cochrane Library, including studies reporting outcomes for MISS in AIS. The meta-analysis compared the operative, radiological, and clinical outcomes and complications between MISS and COSS in patients with AIS. Of the 208 records identified, 15 nonrandomized studies with 1,369 patients (reviews and case reports are excluded) were included in this systematic review and meta-analysis. The mean scale was 6.1, and eight of the 15 included studies showed satisfactory quality using the Newcastle-Ottawa scale. For operative outcomes, MISS had significant benefits in terms of estimated blood loss (standard mean difference [SMD], -1.87; 95% confidence interval [CI], -2.94 to -0.91) and hospitalization days (SMD, -2.99; 95% CI, -4.45 to -1.53) compared with COSS. However, COSS showed significantly favorable outcomes for operative times (SMD, 1.71; 95% CI, 0.92-2.51). No significant differences were observed in radiological outcomes, including Cobb's angle of the main curve and thoracic kyphosis. For clinical outcomes, MISS showed significant benefits on the visual analog scale score (SMD, -0.91; 95% CI, -1.36 to -0.47). The overall complication rates of MISS were similar to those of COSS (SMD, 0.96; 95% CI, 0.61-1.52). MISS using the posterior approach provides equivalent radiological and clinical outcomes and complication rates compared with COSS. Considering the lower estimated blood loss, shorter hospitalization days, and longer operative times in MISS, COSS is still the mainstay of surgical treatment in AIS; however, MISS using the posterior approach is also one of the surgical options of choice in the case of moderate AIS.

Rotational Changes Following Use of Direct Vertebral Rotation in Adolescent Idiopathic Scoliosis: A Long-term Radiographic and Computed Tomography Evaluation.

STUDY DESIGN: A retrospective cohort study. OBJECTIVE: To evaluate long-term rotational changes in the vertebrae of patients with adolescent idiopathic scoliosis (AIS) who underwent direct vertebral rotation (DVR). SUMMARY OF BACKGROUND DATA: DVR using thoracic pedicle screws, a rotational corrective maneuver used in the surgical treatment of AIS, was introduced in 2004. Although DVR is considered to be the main axial corrective maneuver, the long-term rotational changes of vertebrae following this treatment are not well understood. METHODS: A total of 135 vertebrae that underwent DVR using thoracic pedicle screws with a minimum 5-year follow-up were retrospectively assessed for the vertebral rotation angle. Vertebral rotation of the apical vertebra (AV), and distal end vertebra (EV) was evaluated using the Nash-Moe scale, the rotational angle to the sacrum (RAsac), and the Aaro and Dahlborn method. Student's t-test (paired means) was used for continuous variables, and the chi-square test was used for categorical variables, as appropriate. A comparison of two and three groups used a one-way repeated measures analysis of variance, and the post hoc analysis used the Bonferroni test. RESULTS: The mean Nash-Moe scale of distal EV showed statistically significant differences between preoperative and postoperative values (P=0.034) and no statistically significant difference between postoperative and last follow-up values (P=1.000). The last follow-up RAsac of AV did not differ significantly from the preoperative RAsac of AV (P=0.515). The last follow-up RAsac of distal EV was significantly lower than the preoperative RAsac of distal EV (P=0.001). Pearson's correlation analysis showed that the last follow-up RAsac of distal EV was correlated with Cobb angle of the main curve (r=0.459, P=0.004), loss of correction (r=0.541, P=0.001), and LIV tilt angle (r=0.504, P=0.001). CONCLUSIONS: The rotation regression phenomenon in AV and rotation maintenance in distal EV were observed after DVR over an average of 10-year follow-up. These findings suggest that the DVR in the surgical treatment of AIS has a positive long-term effect on the stabilization of distal EV from the point of view of axial rotation. LEVEL OF EVIDENCE: IV.

Three-dimensional ultrasonography could be a potential non-ionizing tool to evaluate vertebral rotation of subjects with adolescent idiopathic scoliosis.

Background: Three-dimensional (3D) ultrasonography is nonionizing and has been demonstrated to be a reliable tool for scoliosis assessment, including coronal and sagittal curvatures. It shows a great potential for axial vertebral rotation (AVR) evaluation, yet its validity and reliability need to be further demonstrated. Materials and Methods: Twenty patients with adolescent idiopathic scoliosis (AIS) (coronal Cobb: 26.6 ± 9.1°) received 3D ultrasound scan for twice, 10 were scanned by the same operator, and the other 10 by different operators. EOS Bi-planar x-rays and 3D scan were conducted on another 29 patients on the same day. Two experienced 3D ultrasonographic researchers, with different experiences on AVR measurement, evaluated the 3D ultrasonographic AVR of the 29 patients (55 curves; coronal Cobb angle: 26.9 ± 11.3°). The gold standard AVR was determined from the 3D reconstruction of coronal and sagittal EOS radiographs. Intra-class correlation coefficients (ICCs), mean absolute difference (MAD), standard error measurements (SEM), and Bland-Altman's bias were reported to evaluate the intra-operator and inter-operator/rater reliabilities of 3D ultrasonography. The reliability of 3D ultrasonographic AVR measurements was further validated using inter-method with that of EOS. Results: ICCs for intra-operator and inter-operator/rater reliability assessment were all greater than 0.95. MAD, SEM, and bias for the 3D ultrasonographic AVRs were no more than 2.2°, 2.0°, and 0.5°, respectively. AVRs between both modalities were strongly correlated (R 2 = 0.901) and not significantly different (p = 0.205). Bland-Altman plot also shows that the bias was less than 1°, with no proportional bias between the difference and mean of expected and radiographic Cobb angles. Conclusion: This study demonstrates that 3D ultrasonography is valid and reliable to evaluate AVR in AIS patients. 3D ultrasonography can be a potential tool for screening and following up subjects with AIS and evaluating the effectiveness of nonsurgical treatments.

Automatic generation of subject-specific finite element models of the spine from magnetic resonance images.

The generation of subject-specific finite element models of the spine is generally a time-consuming process based on computed tomography (CT) images, where scanning exposes subjects to harmful radiation. In this study, a method is presented for the automatic generation of spine finite element models using images from a single magnetic resonance (MR) sequence. The thoracic and lumbar spine of eight adult volunteers was imaged using a 3D multi-echo-gradient-echo sagittal MR sequence. A deep-learning method was used to generate synthetic CT images from the MR images. A pre-trained deep-learning network was used for the automatic segmentation of vertebrae from the synthetic CT images. Another deep-learning network was trained for the automatic segmentation of intervertebral discs from the MR images. The automatic segmentations were validated against manual segmentations for two subjects, one with scoliosis, and another with a spine implant. A template mesh of the spine was registered to the segmentations in three steps using a Bayesian coherent point drift algorithm. First, rigid registration was applied on the complete spine. Second, non-rigid registration was used for the individual discs and vertebrae. Third, the complete spine was non-rigidly registered to the individually registered discs and vertebrae. Comparison of the automatic and manual segmentations led to dice-scores of 0.93-0.96 for all vertebrae and discs. The lowest dice-score was in the disc at the height of the implant where artifacts led to under-segmentation. The mean distance between the morphed meshes and the segmentations was below 1 mm. In conclusion, the presented method can be used to automatically generate accurate subject-specific spine models.

Morphological Changes of the Intervertebral Disc During Growth.

STUDY DESIGN: Cross-sectional. OBJECTIVE: To describe morphological changes of the annulus fibrosus (AF) and nucleus pulposus (NP) in children during growth using magnetic resonance imaging (MRI). SUMMARY OF BACKGROUND DATA: Little is known of intervertebral disc (IVD) maturation as opposed to degeneration, such as changes in relative AF/NP proportions and orientation during growth. Studies suggest the IVD plays a role in the etiology of pediatric spinal deformities. Therefore, understanding the morphological development of the AF and NP during growth is key. METHODS: An existing database of children aged 0-18 that had magnetic resonance imaging (MRI) for indications unrelated to the spine were analyzed. The AF/NP were segmented semi-automatically from T1-L5. The parameters: mean IVD height, cross-sectional area, slenderness (height/width ratio), volume (ratio) and relative position of the centroid of the NP within the IVD in three directions (x,y,z) were extracted, and compared between age, sex and spinal level. RESULTS: IVD height increased modestly and predominantly in the low-thoracic and lumbar spine during the first 5-10 years of life. Cross-sectional area and thus volume increased steadily at all levels throughout growth. IVD slenderness decreased sharply the first years of life and remains relatively stable throughout the remainder of growth. IVDs were smaller and more slender in females, especially in the mid-thoracic spine at early adolescence. In the upper- and mid-thoracic spine the NP comprises 10-12% of total IVD volume during growth, this percentage increases in the low-thoracic and lumbar spine towards 20-25%. In the anterior-posterior direction, the position of the nucleus increasingly shifts with age, possibly in line with the developing sagittal profile of the spine. CONCLUSION: This study describes the development of thoracic and lumbar IVDs during growth and may be used as a reference for future studies on the IVD its role in the etiology of disc related disorders.

The three-dimensional coupling mechanism in scoliosis and its consequences for correction.

INTRODUCTION: In idiopathic scoliosis, the anterior spinal column has rotated away from the midline and has become longer through unloading and expansion of the intervertebral discs. Theoretically, extension of the spine in the sagittal plane should provide room for this longer anterior spinal column, allowing it to swing back towards the midline in the coronal and axial plane, thus reducing both the Cobb angle and the apical vertebral rotation. METHODS: In this prospective experimental study, ten patients with primary thoracic adolescent idiopathic scoliosis (AIS) underwent MRI (BoneMRI and cVISTA sequences) in supine as well as in an extended position by placing a broad bolster, supporting both hemi-thoraces, under the scoliotic apex. Differences in T4-T12 kyphosis angle, coronal Cobb angle, vertebral rotation, as well as shape of the intervertebral disc and shape and position of the nucleus pulposus, were analysed and compared between the two positions. RESULTS: Extension reduced T4-T12 thoracic kyphosis by 10° (p < 0.001), the coronal Cobb angle decreased by 9° (p < 0.001) and vertebral rotation by 4° (p = 0.036). The coronal wedge shape of the disc significantly normalized and the wedged and lateralized nucleus pulposus partially reduced to a more symmetrical position. CONCLUSION: Simple extension of the scoliotic spine leads to a reduction of the deformity in the coronal and axial plane. The shape of the disc normalizes and the eccentric nucleus pulposus partially moves back to the midline.

Comment on Burgos et al. Fusionless All-Pedicle Screws for Posterior Deformity Correction in AIS Immature Patients Permit the Restoration of Normal Vertebral Morphology and Removal of the Instrumentation Once Bone Maturity Is Reached. J. Clin. Med. 2023, 12, 2408.

With great interest, we read the recently published paper "Fusionless All-Pedicle Screws for Posterior Deformity Correction in AIS Immature Patients Permit the Restoration of Normal Vertebral Morphology and Removal of the Instrumentation Once Bone Maturity is Reached" by Burgos et al. [...].

Radiographic outcome after vertebral body tethering of the lumbar spine.

INTRODUCTION: Multiple studies have analyzed the outcome after thoracic Vertebral Body Tethering (VBT). The results seem reproducible with most studies reporting coronal correction rates around 50% and a tether breakage rate near 20% at two years follow-up. There is a paucity of data on lumbar VBT, and no study has yet analyzed the radiographic outcome after lumbar VBT in a double tether technique at two years follow-up, which was the aim of this study. METHODS: This is a retrospective, single surgeons' data analysis of all consecutive immature patients who have had VBT of the lumbar spine (to L3 or L4) between January 2019 and September 2020. Primary interest focused on coronal curve correction at two years post-operatively. Suspected tether breakages were analyzed separately and defined as an angular change of more than 5° between two adjacent screws. RESULTS: Forty-one patients were eligible for this study and 35 (85%) had complete two-year follow-up data. Average age at surgery was 14.3 years. All patients had a Sanders stage of 7 or below. Average curve correction for thoracolumbar/lumbar curves at two years follow-up was 50%. 90% of patients had at least one level with a suspected tether breakage. No patient required a revision surgery within two years from surgery but two patients were surgically revised after two years. CONCLUSION: VBT in the lumbar spine resulted in 50% coronal curve correction two years post-operatively despite a tether breakage in 90% of patients.

Updated clinical practice recommendations for managing adults with 22q11.2 deletion syndrome.

This review aimed to update the clinical practice guidelines for managing adults with 22q11.2 deletion syndrome (22q11.2DS). The 22q11.2 Society recruited expert clinicians worldwide to revise the original clinical practice guidelines for adults in a stepwise process according to best practices: (1) a systematic literature search (1992-2021), (2) study selection and synthesis by clinical experts from 8 countries, covering 24 subspecialties, and (3) formulation of consensus recommendations based on the literature and further shaped by patient advocate survey results. Of 2441 22q11.2DS-relevant publications initially identified, 2344 received full-text review, with 2318 meeting inclusion criteria (clinical care relevance to 22q11.2DS) including 894 with potential relevance to adults. The evidence base remains limited. Thus multidisciplinary recommendations represent statements of current best practice for this evolving field, informed by the available literature. These recommendations provide guidance for the recognition, evaluation, surveillance, and management of the many emerging and chronic 22q11.2DS-associated multisystem morbidities relevant to adults. The recommendations also address key genetic counseling and psychosocial considerations for the increasing numbers of adults with this complex condition.

Updated clinical practice recommendations for managing children with 22q11.2 deletion syndrome.

This review aimed to update the clinical practice guidelines for managing children and adolescents with 22q11.2 deletion syndrome (22q11.2DS). The 22q11.2 Society, the international scientific organization studying chromosome 22q11.2 differences and related conditions, recruited expert clinicians worldwide to revise the original 2011 pediatric clinical practice guidelines in a stepwise process: (1) a systematic literature search (1992-2021), (2) study selection and data extraction by clinical experts from 9 different countries, covering 24 subspecialties, and (3) creation of a draft consensus document based on the literature and expert opinion, which was further shaped by survey results from family support organizations regarding perceived needs. Of 2441 22q11.2DS-relevant publications initially identified, 2344 received full-text reviews, including 1545 meeting criteria for potential relevance to clinical care of children and adolescents. Informed by the available literature, recommendations were formulated. Given evidence base limitations, multidisciplinary recommendations represent consensus statements of good practice for this evolving field. These recommendations provide contemporary guidance for evaluation, surveillance, and management of the many 22q11.2DS-associated physical, cognitive, behavioral, and psychiatric morbidities while addressing important genetic counseling and psychosocial issues.

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).

BACKGROUND: Early 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. METHODS: After 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. DISCUSSION: The 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 REGISTRATION: Clinicaltrials.gov: NCT04021784 (13-06-2019). CCMO registry: NL64018.041.17 (06-05-2019).

Three-dimensional correction of scoliosis by a double spring reduction system as a dynamic internal brace: a pre-clinical study in Göttingen minipigs.

BACKGROUND CONTEXT: Adolescent idiopathic scoliosis (AIS) is a major skeletal deformity that is characterized by a combination of apical rotation, lateral bending and apical lordosis. To provide full 3D correction, all these deformations should be addressed. We developed the Double Spring Reduction (DSR) system, a (growth-friendly) concept that continuously corrects the deformity through two different elements: A posterior convex Torsional Spring Implant (TSI) that provides a derotational torque at the apex, and a concave Spring Distraction System (SDS), which provides posterior, concave distraction to restore thoracic kyphosis. PURPOSE: To determine whether the DSR components are able to correct an induced idiopathic-like scoliosis and to compare correction realized by the TSI alone to correction enforced by the complete DSR implant. STUDY DESIGN/SETTING: Preclinical randomized animal cohort study. PATIENT SAMPLE: Twelve growing Göttingen minipigs. OUTCOME MEASURES: Coronal Cobb angle, T10-L3 lordosis/kyphosis, apical axial rotation, relative anterior lengthening. METHODS: All mini-pigs received the TSI with a contralateral tether to induce an idiopathic-like scoliosis with apical rotation (mean Cobb: 20.4°; mean axial apical rotation: 13.1°, mean lordosis: 4.9°). After induction, the animals were divided into two groups: One group (N=6) was corrected by TSI only (TSI only-group), another group (N=6) was corrected by a combination of TSI and SDS (DSR-group). 3D spinal morphology on CT was compared between groups over time. After 2 months of correction, animals were euthanized. RESULTS: Both intervention groups showed excellent apical derotation (TSI only-group: 15.0° to 5.4°; DSR-group: 11.2° to 3.5°). The TSI only-group showed coronal Cobb improvement from 22.5° to 6.0°, while the DSR-group overcorrected the 18.3° Cobb to -9.2°. Lordosis was converted to kyphosis in both groups (TSI only-group: -4.6° to 4.3°; DSR-group: -5.2° to 25.0°) which was significantly larger in the DSR-group (p<.001). CONCLUSIONS: The TSI alone realized strong apical derotation and moderate correction in the coronal and sagittal plane. The addition of distraction on the posterior concavity resulted in more coronal correction and reversal of induced lordosis into physiological kyphosis. CLINICAL SIGNIFICANCE: This study shows that dynamic spring forces could be a viable method to guide the spine towards healthy alignment, without fusing it or inhibiting its growth.