Search

Abstract Background A number of infant pain measures have been developed over the past 15 years incorporating behavioural and physiologic indicators; however, no reliable or valid measure exists for infants who are at risk for neurological impairments (NI). The objective of this study was to establish consensus about which behavioural, physiologic and contextual indicators best characterize pain in infants at high, moderate and low levels of risk for NI. Methods A 39- item, self-administered electronic survey that included infant physiologic, behavioral and contextual pain indicators was used in a two round Delphi consensus exercise. Fourteen pediatric pain experts were polled individually and anonymously on the importance and usefulness of the pain indicators for the 3 differing levels of risk for NI. Results The strength of agreement between expert raters was moderate in Round 1 and fair in Round 2. In general, pain indicators with the highest concordance for all three groups were brow bulge, facial grimace, eye squeeze, and inconsolability. Increased heart rate from baseline in the moderate and severe groups demonstrated high concordance. In the severe risk group, fluctuations in heart rate and reduced oxygen saturation were also highly rated. Conclusion These data constitute the first step in contributing to the development and validation of a pain measure for infants at risk for NI. In future research, we will integrate these findings with the opinions of (a) health care providers about the importance and usefulness of infant pain indicators and (b) the pain responses of infants at mild, moderate and high risk for NI.

Background: Congenital dislocation of the knee (CDK) may be idiopathic or associated with another condition, such as Larsen syndrome or arthrogryposis. Surgical reduction of type-3 dislocation may require quadricepsplasty (QP) or femoral diaphyseal shortening (FS). Because it is unknown which treatment is more effective, we evaluated long-term outcomes using patient-reported questionnaires and gait analysis, comparing results by surgery type and underlying diagnosis., Methods: Twelve patients (mean age, 19 mo) were treated surgically for CDK from 1985 to 2015 and studied 9 to 30 years postoperatively. Three participants had idiopathic CDK, 5 had Larsen syndrome, and 4 had arthrogryposis. Eleven knees underwent QP and 7 underwent FS. Participants were evaluated in our movement science laboratory and completed patient-reported outcome questionnaires. Data were compared with healthy, age-matched control values at the same visit., Results: Surgically treated knees had less flexion during swing ( P <0.01), less overall motion ( P <0.01), greater coronal instability ( P <0.04), and slower gait ( P <0.01) compared with controls. QP knees had more instability in midstance ( P =0.03) and less flexion during gait compared with FS knees, less sagittal power generation than controls ( P <0.01), and trended toward lower scores on Knee Injury and Osteoarthritis Outcome and Lysholm Knee Questionnaires than FS patients did. The idiopathic group had the gait most similar to that of controls, followed by the Larsen syndrome group and then the arthrogryposis group. The idiopathic group also had a better UCLA Activity Score ( P =0.03) than the arthrogryposis group did., Conclusions: Surgical treatment of type-3 CDK will not likely restore normal knee function, suggesting teratologic joint abnormality. In this small series, FS produced better gait mechanics and patient-reported outcomes compared with QP. Not surprisingly, patients with idiopathic CDK had better outcomes than those with a syndromic diagnosis, likely related to having only a single joint affected., Level of Evidence: Level III., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is a rare autosomal recessive disorder affecting the formation of the spine, characterized by a complete bilateral fusion of the ribs at the costovertebral junction, producing a "crablike" appearance of the thorax. Despite being declared a core indication for a V-osteotomy vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty of the posterior thorax, the natural history of STD in untreated subjects remains poorly documented. In this study, we report radiographic and pulmonary function findings and Patient-Reported Outcomes Measurement Information System (PROMIS) and 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) scores for untreated adult subjects with STD to gain insights into the natural history., Methods: We identified 11 skeletally mature, untreated subjects with STD. Findings on medical evaluation, demographics, radiographic parameters, pulmonary function, genetic testing results, PROMIS measures, and EOSQ-24 scores were assessed., Results: Five male and 6 female subjects (mean age, 32.3 years [range, 15 to 70 years]) with a confirmed STD diagnosis based on radiographs and genetic testing were evaluated. Mean body mass index (BMI) was 24.4 kg/m 2 (range, 18 to 38.9 kg/m 2 ), and mean thoracic height was 16 cm (range, 12 to 17 cm). Pulmonary function tests (PFTs) showed a mean forced vital capacity (FVC) of 22% of predicted, mean forced expiratory volume in 1 second (FEV1) of 24% of predicted, and FEV1/FVC ratio of 107% of predicted. The mean PROMIS dyspnea score was 40 ± 8 points (range, 27.7 to 52.1 points). The mean total EOSQ-24 score was 77.3 ± 18 points (range, 43.9 to 93.2 points)., Conclusions: Our study characterizes the natural history of STD in untreated subjects. We confirmed the expected restrictive pattern in pulmonary function, but interestingly, our subjects exhibited better EOSQ scores compared with those reported in neuromuscular populations. PFT results and thoracic height did not correspond to PROMIS and EOSQ scores, questioning the use of those parameters as a surgical indication. We therefore suggest that the STD diagnosis as an absolute indication for VEPTR expansion thoracoplasty surgery be reconsidered., Level of Evidence: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence., Competing Interests: Disclosure: No external funding was received for this work. The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article ( http://links.lww.com/JBJS/H817 )., (Copyright © 2023 by The Journal of Bone and Joint Surgery, Incorporated.)

Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest form is provided with the online version of the article (http://links.lww.com/JBJS/H782).

Purpose: To compare objective outcomes for EOS patients age 6-10 years treated by growth-sparing (GS) surgery or definitive one-stage correction and fusion (DF)., Methods: We reviewed surgical, radiographic, PFT's, and EOSQ-24 outcomes for EOS patients > age 6 at index surgery treated at a single institution, minimum 2-year follow-up. Neuromuscular diagnoses were excluded., Results: 47 patients underwent index surgery between age 6 and 10.9 years. Twenty-one had DF, 26 had GS surgery (13 MCGR, 13 TGR). Diagnoses included 15 congenital, 15 idiopathic, 17 syndromic. Age at index was 9.1 years DF, 7.8 GS (p < .001). Follow-up was 63-78 months. 18/26 GS cases converted to DF, 13 due to complications, which occurred in 8/21 DF cases vs 19/26 GS (p = .016). DF patients had fewer post-index surgeries (0.6 vs 3.7, p < .001). At follow-up there were no differences in curve magnitudes, %correction, T1-12/T1-S1 segment lengths, EOSQ-24 scores or PFTs. 18 patients converting to DF after initial GS had equal outcomes as DF initially. 31 patients > age 8 at index ("tweeners") were studied separately. 13 had GS surgery (7 MCGR), 18 had DF. At > 60 months follow-up, curve magnitudes, spine lengths, PFT's, or EOSQ scores were equivalent. DF patients had fewer procedures and complications., Conclusion: For patients age 6-10.9 years, outcomes were no different at > 5 year follow-up between DF and GS groups. DF patients had fewer total surgeries and complications. Equal outcomes also occurred for tweeners. As a result, GS treatment does not appear to benefit patients > age 8., (© 2023. The Author(s).)

Optimal orientation for magnetically controlled growing rods (MCGRs) is unclear. The objective of this study was to investigate associations of rod orientation with implant-related complications (IRCs) and spinal height gains. Using an international early-onset scoliosis (EOS) database, we retrospectively reviewed 57 patients treated with dual MCGRs from May 2013 to July 2015 with minimum 2-year follow-up. Outcomes of interest were IRCs and left/right rod length gains and thoracic (T1-T12) and spinal (T1-S1) heights. We compared patients with two rods lengthened in the cephalad ( standard; n  = 18) versus opposite ( offset; n  = 39) directions. Groups did not differ in age, sex, BMI, duration of follow-up, EOS cause, ambulatory status, primary curve magnitude, baseline thoracic height, or number of distractions/year. We compared patients whose constructs used ≥1 cross-link (CL group; n  = 22) versus no CLs (NCL group; n  = 35), analyzing thoracic height gains per distraction ( α  = 0.05). Offset and standard groups did not differ in left or right rod length gains overall or per year or in thoracic or spinal height gain. Per distraction, the CL and NCL groups did not differ significantly in left or right rod length or thoracic or spinal height gain. Complications did not differ significantly between rod orientation groups or between CL groups. MCGR orientation and presence of cross-links were not associated with differences in rod length gain, thoracic height, spinal height, or IRCs at 2-year follow-up. Surgeons should feel comfortable using either MCGR orientation. Level of evidence: 3, retrospective., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

In recent years, new species descriptions for the North American darters have proliferated. Most species concepts accepted by contemporary ichthyologists require that a valid species be both monophyletic and diagnoseable, yet many lineages exhibit modal or range differences in morphological characteristics without individuals being diagnosable. Such scenarios present difficulties with regards to proper taxonomic recognition of divergent lineages and often prohibit appropriate conservation action. Following the example of recent authors, we provide meristic, geometric morphometric, and pigmentation data to support the recognition of three subspecies of Etheostoma rupestre, a species endemic to the Mobile Basin. These morphological data cohere with previous genetic work for E. rupestre. The nominate subspecies Etheostoma rupetsre rupestre (Tsais Rock Darter) is endemic to the Tombigbee River and Black Warrior River watersheds in Alabama and Mississippi and is characterized by having lower numbers of lateral blotches, lower range and mean of lateral line scales, lower modal number of scales above the lateral line, and lower degrees of nape squamation than other subspecies. Etheostoma rupestre piersoni (Shamrock Darter), ssp. nov., is endemic to the Cahaba and Alabama River Watersheds in Alabama and is characterized by intermediate counts of lateral blotches and higher scale counts and nape squamation than E. r. rupestre. Etheostoma rupestre uphapeense (Jade Darter), ssp. nov., is restricted to several small, disjunct populations in the Coosa and Tallapoosa watersheds in Alabama, Georgia, and Tennessee. Etheostoma r. uphapeense is characterized by having a higher mean number of lateral blotches than both other subspecies and higher scale counts than E. r. rupestre. While E. r. rupestre and E. r. piersoni are widespread and abundant within their respective ranges, E. r. uphapeense has a disjunct range and is often uncommon where it occurs. Etheostoma r. uphapeense should be monitored where it occurs to discern population trends.

Background: The term "Tweener" is colloquially used to refer to early-onset scoliosis (EOS) patients whose age and development make them candidates for multiple surgical options. The purpose of this study was to establish expert consensus on a definition to formally characterize the Tweener population., Methods: A 3-round survey of surgeons in an international EOS study group was conducted. Surgeons were provided with various patient characteristics and asked if each was part of their definition for Tweener patients. Responses were analyzed for consensus (≥70%), near-consensus (60% to 69%), and no consensus (<60%)., Results: Consensus was reached (89% of respondents) for including chronological age in the Tweener definition; 8 to 10 years for females and 9 to 11 years for males. Surgeons agreed for inclusion of Sanders score, particularly Sanders 2 (86.0%). Patients who have reached Sanders 4, postmenarche, or have closed triradiate cartilage should not be considered Tweeners. Bone age range of 8 years and 10 months to 10 years and 10 months for females (12 y for males) could be part of the Tweener definition., Conclusions: This study suggests that the Tweener definition could be the following: patients with open triradiate cartilage who are not postmenarche and have not reached Sanders 4, and if they have one of the following: Sanders 2 or chronological age 8 to 10 years for females (9 to 11 y for males) or bone age 8 years and 10 months to 10 years and 10 months for females (12 y for males). This definition will allow for more focused and comparative research on this population., Level of Evidence: Level V-expert opinion., Competing Interests: B.A.A. reports personal fees from DePuy Spine, NuVasive, and Stryker Spine outside the submitted work. J.B.A. reports personal fees from Johnson and Johnson outside the submitted work. J.T.B. reports grants from POSNA and personal fees from Orthopediatrics and Depuy Synthes outside the submitted work. J.B.E. reports personal fees from Zimmer/Biomet outside the submitted work. J.R.S. reports personal fees from Elsevier, Orthopediatrics, Medtronic Spine, and Depuy Synthes outside the submitted work. J.F. reports personal fees from The Research Grants Council of Hong Kong, American Society for Bone and Mineral Research, Biomet, Wolters Kluwer Health-Lippincott Williams and Wilkins, and leadership in American Board of Orthopaedic Surgery outside the submitted work. C.E.J. reports personal fees from Medtronic, Elsevier, and Shriners Hospital Montreal PQ outside the submitted work. R.F.M. reports personal fees from Globus Medical and Stryker outside the submitted work. R.M. reports personal fees from Medtronic and Orthopediatrics outside the submitted work. B.R. reports personal fees from Elsevier Publishing, grants from Pediatric Orthopaedic Society of North America and Orthopaedic Research and Education Foundation outside the submitted work. M.C.W. reports grants or contracts from Zimmer Biomet, POSNA, and Shriners Hospital for Children, personal fees from Zimmer Biomet Spine, Depuy Synthes Spine, Nuvasive Spine, Stryker/K2M Spine, CHOP FDA course, Samaritan Health Services Grand Rounds, Saint Alphonsus Grand Rounds, and Peace Health Grand Rounds, leadership in PSSG, SRS, POSNA, and Shriners Spine Surgeon Study Group outside the submitted work. H.M. reports personal fees from Pediatric Spine Foundation, grants from Scoliosis Research Society, grants from Pediatric Orthopaedic Society of North America outside the submitted work. M.G.V. reports nonfinancial support from Pediatric Spine Foundation, during the conduct of the study; grants from Setting Scoliosis Straight Foundation, grants and other from Children’s Spine Foundation, grants from Orthopaedic Scientific Research Foundation, grants and other from POSNA, other from OMeGA, personal fees from Stryker, personal fees from Biomet, personal fees from Nuvasive outside of the submitted work. B.D.R. reports grants from Pediatric Orthopaedic Society of North America, Orthopaedic Scientific Research Foundation, and Scoliosis Research Society outside the submitted work. Pediatric Spine Study Group reports support from Zimmer Biomet, DePuy Synthes Spine, Pediatric Spine Foundation, OrthoPediatrics, Nuvasive, Medtronic, Globus Medical Inc., and Stryker during the conduct of the study. The remaining authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: Magnetically controlled growing rods (MCGR) have become the dominant distraction-based implant for the treatment of early onset scoliosis (EOS). Recent studies, however, have demonstrated rising rates of implant failure beyond short-term follow-up. We sought to evaluate a single-center experience with MCGR for the treatment of EOS to define the rate of MCGR failure to lengthen, termed implant stall, over time., Methods: A single-center, retrospective review was conducted identifying children with EOS undergoing primary MCGR implantation. The primary endpoint was the occurrence of implant stalling, defined as a failure of the MCGR to lengthen on three consecutive attempted lengthening sessions with minimum of 2 years follow-up. Clinical and radiographic variables were collected and compared between lengthening and stalled MCGRs. A Kaplan-Meier survival analysis was conducted to assess implant stalling over time., Results: A total of 48 children met inclusion criteria (mean age 6.3 ± 1.8 years, 64.6% female). After a mean 56.9 months (range of 27 to 90 months) follow-up, 25 (48%) of children experienced implant stalling at a mean of 26.0 ± 14.1 months post-implantation. Kaplan-Meier survival analysis demonstrated that only 50% of MCGR continue to successfully lengthen at 2 years post-implantation, decreasing to < 20% at 4 years post-implantation., Conclusion: Only 50% of MCGR continue to successfully lengthen 2 years post-implantation, dropping dramatically to < 20% at 4 years, adding to the available knowledge regarding the long-term viability and cost-effectiveness of MCGR in the management of EOS. Further research is needed to validate these findings., (© 2022. The Author(s), under exclusive licence to Scoliosis Research Society.)

Purpose: The purpose of this study was to describe contraindications to the magnetically controlled growing rod (MCGR) in patients with early onset scoliosis (EOS) by establishing consensus amongst expert surgeons who treat these patients frequently., Methods: Nine pediatric spine surgeons from an international EOS study group participated in semi-structured interviews via email to identify factors that influence decision making in the use of MCGR. A 39-question survey was then developed to specify these factors as contraindications for MCGR-these included patient age and size, etiology, medical comorbidities, coronal and sagittal curve profiles, and skin and soft tissue characteristics. Pediatric spine surgeons from the EOS international study group were invited to complete the survey. A second 29-item survey was created to determine details and clarify results from the first survey. Responses were analyzed for consensus (> 70%), near consensus (60-69%), and no consensus/variability (< 60%) for MCGR contraindication., Results: 56 surgeons of 173 invited (32%) completed the first survey, and 64 (37%) completed the second survey. Responders had a mean of over 15 years in practice (range 1-45) with over 6 years of experience with using MCGR (range 2-12). 71.4% of respondents agreed that patient size characteristics should be considered as contraindications, including BMI (81.3%) and spinal height (84.4%), although a specific BMI range or a specific minimum spinal height were not agreed upon. Among surgeons who agreed that skin and soft tissue problems were contraindications (78.6%), insufficient soft tissue (98%) and skin (89%) to cover MCGR were specified. Among surgeons who reported curve stiffness as a contraindication (85.9%), there was agreement that this curve stiffness should be defined by clinical evaluation (78.2%) and by traction films (72.3%). Among surgeons who reported sagittal curve characteristics as contraindications, hyperkyphosis (95.3%) and sagittal curve apex above T3 (70%) were specified. Surgeons who indicated the need for repetitive MRI as a contraindication (79.7%) agreed that image quality (72.9%) and not patient safety (13.6%) was the concern. In the entire cohort, consensus was not achieved on the following factors: patient age (57.4%), medical comorbidities (46.4%), etiology (53.6%), and coronal curve characteristics (58.9%)., Conclusion: Surgeon consensus suggests that MCGR should be avoided in patients who have insufficient spinal height to accommodate the MCGR, have potential skin and soft tissue inadequacy, have too stiff a spinal curve, have too much kyphosis, and require repetitive MRI, particularly of the spine. Future data-driven studies using this framework are warranted to generate more specific criteria (e.g. specific degrees of kyphosis) to facilitate clinical decision making for EOS patients., Level of Evidence: Level V-expert opinion., (© 2022. The Author(s), under exclusive licence to Scoliosis Research Society.)

Background: Thoracic spine height is cited as a crucial outcome measure in the treatment of early-onset scoliosis (EOS) because of its reported relationship to pulmonary function tests (PFTs). An 18-cm threshold has been proposed, although this single parameter might be overly simplistic for cases of different etiologies and deformity magnitude. We aimed to reevaluate pulmonary function in patients undergoing corrective surgery, assessing the role of residual scoliosis as well as spine elongation., Methods: Patients undergoing EOS correction with a minimum of 5 years of follow-up since initial treatment were evaluated. Standard spirometry (forced vital capacity [FVC], forced expiratory volume in 1 second [FEV1]) was correlated to deformity magnitude and T1-T12 height. Patients were compared by age at first surgery (<5 or ≥5 years), final thoracic height (≤18 or >18 cm), and percentage of predicted pulmonary function (<60% or ≥60%)., Results: Twenty-nine patients (15 congenital, 11 syndromic, and 3 idiopathic cases) were tested at a mean of 8.5 years following initial surgery. Twenty-two patients (mean initial age, 4.8 years) had growth-sparing instrumentation, and 7 patients (age, 5.1 years) had definitive fusion performed. Age at initial surgery was not associated with a difference in PFT results at the time of follow-up, and both age groups had ominously low percentages of predicted pulmonary-function volumes (50% to 55%). Only 18 of the 29 patients achieved a T1-T12 height of >18 cm. Those with a thoracic height of ≤18 cm had similar percentage-of-predicted spirometry results at the time of follow-up as those with greater thoracic height, possibly because of increased deformity correction. Only 14 of 29 patients had spirometry of ≥60% of predicted volume at the time of follow-up. These 14 had slightly smaller curves and slightly greater T1-T12 heights but significantly better spirometry results than the 15 subjects with <60% of predicted volume. For those with a T1-T12 height of ≤18 cm, the residual Cobb angle negatively correlated with spirometry results. In those with a final T1-T12 height of >18 cm, spirometry did correlate with thoracic height, especially when residual deformity was ≥60°., Conclusions: Regardless of thoracic height of ≤18 or >18 cm, with residual curves of >50 o , pulmonary function was ominously low in fully half of the patients, raising doubt about the value of this threshold as an EOS outcome parameter., Level of Evidence: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSOA/A348)., (Copyright © 2021 The Authors. Published by The Journal of Bone and Joint Surgery, Incorporated. All rights reserved.)

Background: The present study compares prosthetic treatment options for proximal femoral focal deficiency in terms of gait analysis, oxygen consumption, and patient-reported outcomes., Methods: Twenty-three patients who had been managed with a prosthesis for unilateral proximal femoral focal deficiency underwent gait analysis; this group included 7 patients who had received an equinus prosthesis, 6 who had received a rotationplasty prosthesis, and 10 who had undergone Syme amputation and had received an above-the-knee prosthesis. Cadence parameters, kinematic and kinetic data, and oxygen consumption were measured, and the Gait Deviation Index (GDI) was calculated. Medical records and radiographs were reviewed. The Pediatric Outcomes Data Collection Instrument (PODCI) was completed by the child's parent., Results: Patients underwent gait analysis at a mean age of 11.6 years (range, 4 to 19 years). Proximal femoral focal deficiency classification was not predictive of the chosen treatment. Patients in the rotationplasty group had undergone more procedures than those in the Syme amputation and equinus groups (mean, 3.3, 1.8, and 0.7 procedures, respectively) (p = 0.001). Oxygen cost did not differ between groups; however, all required greater energy expenditure than normal (170%, 144%, and 159%, in the equinus, rotationplasty, and Syme amputation groups, respectively) (p = 0.427). Likewise, hip power, abductor impulse, and GDI did not differ, but all groups had GDI scores >3 standard deviations below normative values. Patients in the equinus group walked faster (97% of normal for age) than those in the rotationplasty (84%) and Syme amputation groups (83%) (p = 0.018), whereas those in the Syme amputation group had superior knee range of motion (55° from the prosthetic knee) than those in the equinus (20°) and rotationplasty groups (15° generated from the ankle) (p = 0.003). There were no differences in terms of the PODCI subscales for pain, sport/physical function, happiness, or global function. Transfer/basic mobility improved with age (r = 0.516, p = 0.017), but no other associations were found between gait variables and PODCI scores., Conclusions: Rotationplasty provided no patient-reported benefit and no functional benefit in terms of gait parameters or oxygen consumption, despite requiring more surgical procedures compared with other prosthetic options. Patients with an equinus prosthesis walked the fastest, whereas treatment with a Syme amputation and prosthetic knee yielded equivalent gait parameters and oxygen consumption as compared with those for patients using an equinus prosthesis. These findings contradict those of previous reports that rotationplasty provides superior function over other proximal femoral focal deficiency prosthetic treatment options., Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJS/G613)., (Copyright © 2021 by The Journal of Bone and Joint Surgery, Incorporated.)

Purpose: Hyperkyphosis, including Scheuermann and postural kyphosis, is associated with decreased perceived cosmesis and well being. No patient reported outcome questionnaire specific to kyphosis exists. We sought to assess the internal consistency, test-retest reliability and concurrent validity of a new Kyphosis-specific Spinal Appearance Questionnaire (K-SAQ)., Methods: A K-SAQ was developed from a modified SAQ to measure kyphosis-specific aspects of appearance. Patients with hyperkyphosis (ages 10-20 years) curves ≥ 50° completed the K-SAQ and SRS-22R at baseline and the K-SAQ 2 weeks later., Results: 55 patients completed the K-SAQ and SRS-22R. 28 patients completed the K-SAQ 2 weeks later. The K-SAQ total averages showed excellent internal consistency (Cronbach's α = 0.91) and test-retest reliability (ICC = 0.84). Moderate associations were seen between the SRS-22R subtotal average score and K-SAQ total average score and (r = - 0.62, p < 0.001) and the SRS-22R self-image domain with the K-SAQ total average score (r = - 0.57, p < 0.001). Higher BMI and increased age scored worse on the K-SAQ total average, whereas only higher BMI scored worse on the SRS-22R subtotal average., Conclusion: The K-SAQ is a reliable patient reported outcome measure of kyphosis-specific aspects of appearance., (© 2021. Scoliosis Research Society.)

Purpose: Increasingly, patients with early onset scoliosis (EOS) are completing a growth friendly surgical program followed by observation, removal of implants or a definitive spinal fusion. These patients are colloquially referred to as "graduates". A standardized definition of a graduate is needed for research and comparing the outcomes, family counseling, and a better understanding of the population., Methods: A 15-question electronic survey was completed by 39 experienced pediatric spine surgeons to identify factors salient to the definition of a graduate of EOS surgical programs. A Delphi/Nominal group technique session with nine questions was then performed face-to-face with 21 members of the Pediatric Spine Study Group to discuss and refine the definition. A follow-up electronic survey was then distributed to these same 21 members to gain consensus on the final definition., Results: From the initial survey, it was identified that a graduate did not require definitive spinal fusion after a growing program. From the Delphi session, it was determined that skeletal maturity was the most important factor in defining a graduate. A strictly defined minimum length of follow-up was not felt to be a prerequisite for qualification of graduation. After the final electronic version was distributed, > 80% of respondents agreed upon the final definition, thereby achieving consensus., Conclusion: The Pediatric Spine Study Group recommends adoption of the following definition: a "graduate" is a patient who has undergone any surgical program to treat early onset scoliosis, and has reached skeletal maturity and does not have a planned surgical intervention for EOS in the future., Level of Evidence: V.

Purpose: The study was undertaken to determine the feasibility of growth-friendly distraction-based surgery in children with OI., Methods: Two multi-center databases were queried for children with OI who had undergone GR or VEPTR surgery. Inclusion criteria were a minimum 2-year follow-up and three lengthening procedures following the initial implantation. Details of the surgical techniques, surgical complications, and radiographic measurements of deformity correction, T1-T12 and T1-S1 elongation and growth were recorded., Results: Five patients were identified. There was one patient with type I OI and two patients each with type III and type IV. Four patients had GR constructs and one a VEPTR construct. The initial scoliosis deformity averaged 80° (70°-103°), and the subsequent corrections averaged 32% for initial correction, 48% at last follow-up, and 54% for the two patients that had a final fusion. The T1-T12 and T1-S1 growth averaged 31 mm and 44 mm respectively, and yearly growth averaged 4 mm and 6 mm, respectively. Growth was notably much less in those with more severe disease. There were 13 complications in 4 patients. Nine of the 10 surgical complications were anchor failures which were corrected in 7 planned and 2 un-planned procedures. Significant migration occurred in one patient with severe OI type III., Conclusion: The results varied in this heterogeneous population. In general, satisfactory deformity corrections were obtained and maintained, modest growth was obtained, and complications were similar to those reported in other series of growth-friendly surgery. Limited growth and significant anchor migration are to be anticipated in this population., Level of Evidence: IV.

While the expansion of anthropogenic noise studies in aquatic habitats has produced conservation-based results for a range of taxa, relatively little attention has been paid to the potential impacts on stream fishes. Recent work has shown responses to road noise in single species of stream fish; however, assemblage-wide effects of anthropogenic noise pollution have not yet been investigated. By examining five metrics of disturbance across four ecologically and evolutionarily disparate species of stream fishes, a series of laboratory experiments aimed to describe the effects of and species susceptibility to anthropogenic noise playback. Each species studied represented a unique combination of hearing sensitivity and water column position. Physiological and behavioral metrics were compared across the presence and absence of rail-noise noise playback in four target species. Through repeated subsampling, the temporal dynamics of cortisol secretion in response to noise in two target species were additionally described. Rail-noise playback had no statistically significant effect on blood glucose or water-borne cortisol levels, with the exception of decreased cortisol in noise-exposed largescale stoneroller ( Campostoma oligolepis ). Time-course cortisol experiments revealed rapid secretion and showed minimal effects of noise at most observation points. The presence of noise produced significant changes in ventilation rate and swimming parameters in a portion of the four species observed representing the most conserved responses. Overall, effects of noise were observed in species contrary to what would be hypothesized based on theoretical hearing sensitivity and water column position demonstrating that predicting susceptibility to this type of stressor cannot be accomplished based off these course considerations alone. More importantly, we show that anthropogenic noise can disrupt a variety of behavioral and physiological processes in certain taxa and should be further investigated via measures of fitness in the wild., (© The Author(s) 2020. Published by Oxford University Press and the Society for Experimental Biology.)

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.

Aims: The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management., Patients and Methods: A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation., Results: Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10)., Conclusion: Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563-1569.

Background: With observed success and increased popularity of growth modulation techniques, there has been a trend toward use in progressively younger patients. Younger age at growth modulation increases the likelihood of complete deformity correction and need for implant removal before skeletal maturity introducing the risk of rebound deformity. The purpose of this study was to quantify magnitude and identify risk factors for rebound deformity after growth modulation., Methods: We performed a retrospective review of all patients undergoing growth modulation with a tension band plate for coronal plane deformity about the knee with subsequent implant removal. Exclusion criteria included completion epiphysiodesis or osteotomy at implant removal, ongoing growth modulation, and <1 year radiographic follow-up without rebound deformity. Mechanical lateral distal femoral angle, mechanical medial proximal tibial angle, hip-knee-ankle angle (HKA), and mechanical axis station were measured before growth modulation, before implant removal, and at final follow-up., Results: In total, 67 limbs in 45 patients met the inclusion criteria. Mean age at growth modulation was 9.8 years (range, 3.4 to 15.4 y) and mean age at implant removal was 11.4 years (range, 5.3 to 16.4 y). Mean change in HKA after implant removal was 6.9 degrees (range, 0 to 23 degrees). In total, 52% of patients had >5 degrees rebound and 30% had >10 degrees rebound in HKA after implant removal. Females below 10 years and males below 12 years at time of growth modulation had greater mean change in HKA after implant removal compared with older patients (8.4 vs. 4.7 degrees, P=0.012). Patients with initial deformity >20 degrees had an increased frequency of rebound >10 degrees compared with patients with less severe initial deformity (78% vs. 22%, P=0.002)., Conclusions: Rebound deformity after growth modulation is common. Growth modulation at a young age and large initial deformity increases risk of rebound. However, rebound does not occur in all at risk patients, therefore, we recommend against routine overcorrection., Level of Evidence: Level IV-retrospective study.

Background: This study assessed the outcomes, at skeletal maturity, for 34 patients in whom congenital pseudarthrosis of the tibia (CPT) had been treated with intramedullary (IM) rod fixation., Methods: The results in skeletally mature patients in whom type-4 CPT had been treated with an IM rod at an average of 11.9 years earlier were reviewed. The rod procedures varied according to whether both the tibia and the fibula were resected and both bones (type A) or just the tibia (type B) were fixed with an IM rod or whether only the tibia was resected and the fibula received no surgery (type C). Outcome grading ranged from unequivocal union with brace-free function (grade 1) to a functional limb with residual angulation or cortical defects (grade 2) to a severely impaired extremity with insufficient union or refracture (grade 3)., Results: Thirty-four patients were evaluated at a mean age of 16.9 years, a mean of 11.9 years after their initial surgical procedure. Seventeen patients had a grade-1 result; 11 patients, grade 2; and 6, grade 3. Thus, 82% (28) of the 34 patients had a functional extremity at maturity. All patients with a final grade-3 outcome eventually requested amputation. The final outcomes were not affected by the age at the initial fracture or surgery, the presence of neurofibromatosis-1, or cross-ankle fixation. A total of 58 IM rod procedures were performed in the 34 patients. Twenty-four (73%) of the 33 type-A procedures produced grade-1 or 2 outcomes, as did 14 (88%) of the 16 type-B procedures. Of the 9 type-C procedures, none produced a grade-1 result and 4 produced a grade-2 outcome. The results of types A and B combined were superior to those of type-C procedures (p = 0.03). Refracture occurred in 13 of 33 patients with initial stability/union after rod fixation, with 3 of those fractures remaining ununited at the latest follow-up. A dystrophic fibula had no effect on the eventual achievement of a grade-1 or 2 outcome., Conclusions: This review, in which all patients had reached skeletal maturity, documents functional (grade-1 or 2) outcomes in 82% of cases of IM rod fixation for CPT. This finding was almost identical to the result in our earlier report and confirms the long-term value of permanent IM rod fixation in maintaining union and function in patients with CPT. Procedures not addressing the fibula produced inferior results. Although 13 patients had a refracture following initial union, only 3 of these fractures failed to heal with additional treatment., Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Study Design: A retrospective review of a prospective, multicenter database., Objective: The aim of this study was to compare surgical and quality-of-life outcomes at the end of growing rod treatment in patients with severe versus moderate early-onset scoliosis (EOS)., Summary of Background Data: Knowledge of the outcomes of severe EOS after growth-friendly treatment is limited because this condition is uncommon., Methods: We identified 40 children with severe EOS (major curve ≥90°) treated with growing rods before age 10 with minimum 2-year follow-up after last lengthening or final fusion. From the same registry, we matched 40 patients with moderate EOS (major curve < 90°). Twenty-seven patients in the severe group and 12 in the moderate group underwent final fusion (P < 0.001)., Results: Mean preoperative curves were 102° (range, 90°-139°) in the severe group and 63° (range, 33°-88°) in the moderate group (P < 0.001). At final follow-up, mean curves were 56° (range, 10°-91°) and 36° (range, 12°-89°), respectively (P < 0.001). Fourteen (35%) children in the severe group and 32 (80%) in the moderate group had scoliosis of < 45° at final follow-up [risk ratio (RR), 0.44; 95% confidence interval (95% CI), 0.20-0.57]. At final follow-up, 30 (75%) children in the severe group and 35 (88%) in the moderate group had achieved T1-T12 length of ≥18 cm (RR, 0.86; 95% CI, 0.70-1.09). Thirty-five children in the severe group and 26 in the moderate group had at least one complication (RR, 1.35; 95% CI, 1.05-1.73). Mean 24-Item Early-Onset Scoliosis Questionnaire scores were similar between groups at final follow-up., Conclusion: Delaying surgery until the major curve has progressed beyond 90° is associated with larger residual deformity and more complications than treating at a lesser curve magnitude. Quality-of-life outcomes were similar between those with severe and moderate EOS., Level of Evidence: 3.

For the past 35 years, we have used halo gravity traction (HGT) to treat patients with a wide variety of underlying spinal deformities. This clinical concepts review will cover the indications, contraindications, HGT technique details, and our preferred method of dynamic HGT. Emphasis will be placed on our protocol, and recommendations that help to avoid complications. HGT is best applied by a standardized team approach intending to medically and nutritionally optimize the patient in preparation for an operative or medical/interventional treatment that will maintain and improve on the deformity correction achieved while avoiding peri- and postoperative complications., (Copyright © 2018 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

Background: To propose a modified classification of infantile tibia vara based on the morphology of the metaphyseal/epiphyseal tibial slope that better correlates with treatment outcomes than the traditional Langenskiold classification., Methods: We performed a retrospective review of 82 patients and 115 limbs that underwent surgery for infantile tibia vara over a 22-year period (1990 to 2012) at a single institution. A modified Langenskiold classification was applied to all patients preoperatively and the outcomes were assessed. The modified system created a 3-stage classification (types A, B, and C): type A has a partially lucent medial metaphyseal defect, with or without "beaking"; type B deformity has downward-sloping curvature of the lateral and inferior rim of a completely lucent metaphyseal defect, which then has an upslope at the medial rim, resembling a ski-jump, with no epiphyseal downward slope; type C has vertical, downsloping deformity of both the epiphysis and metaphysis, with no upward curvature projecting medially at the inferior extent, while the epiphysis slopes downward into the metaphyseal defect., Results: Sixty-seven limbs did not develop recurrence following corrective osteotomy, whereas 48 limbs required at least 1 repeat surgery for recurrent deformity. Preoperative mechanical axis deviation, medial proximal tibial angle, lateral distal tibial angle, and body mass index did not differ significantly between those with recurrence and those with without. Mean age at surgery was significantly different for those who developed recurrence compared with those who did not. Patients without recurrence were 4.3 years of age (range, 2.4 to 10.3 y) compared with 6.2 years of age (range, 2.9 to 10.1 y) for those who recurred (P<0.01). Of patients who developed recurrent deformity, there were significantly more patients with type C changes (71.7%, P<0.01) then either type A (22.5%) or type B (20.7%). High rates of recurrence were seen for both Langenskiold stage III (50%) and stage IV (69.6%)., Conclusions: Consistent with prior studies, age 5 seems to be a critical transition in the risk for recurrent deformity after tibial osteotomy. Extreme vertical sloping of the medial metaphyseal defect, as in some classic Langenskiold III lesions and more precisely described by type C in a newer, modified classification, carries a poor prognosis for successful correction by high tibial osteotomy alone or in combination with epiphysiolysis., Level of Evidence: Level II.

Study Design: Single-center retrospective chart review., Objectives and Summary: Halo gravity traction (HGT) is a safe and effective intervention to improve spinal deformity prior to corrective instrumentation. Our study aimed to report on a large series of patients undergoing HGT, demonstrate the correlation between thoracic height achieved and pulmonary function, and evaluate the efficacy of nutritional assessment and intervention while in HGT for these often malnourished or nutritionally compromised patients., Methods: 107 patients underwent HGT for severe spinal deformity. Major coronal and sagittal Cobb angles, T1-T12 ht, and T1-S1 ht were collected pre-HGT, during HGT, postoperation, and 2 years postoperation. Pulmonary function tests (PFTs) recorded forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1). Nutritional interventions such as formal nutrition consult, resulting nutritional supplementation, or interventions were recorded., Results: Patients were in HGT for mean of 82.1 days and mean maximum percentage body weight in traction was 49.5%. Mean major coronal Cobb angle prior to HGT was 92.6°, improving to 65.8° in maximal traction and to 47° after surgical intervention. Traction accounted for 78% of T1-T12 height and 79% of T1-S1 length gains from pre- to postoperation. We showed a positive correlation between gain in T1-T12 height and percentage predicted changes in FVC and FEV1. Weight Z score for the entire cohort of patients improved from -2.8 pretraction to -2.4 in traction and then to -2.3 postoperative., Conclusions: Our study again demonstrated that HGT achieves radiographic improvement safely and effectively in severe spinal deformity. We demonstrated a positive correlation between improvement in PFTs and increase in thoracic height seen with HGT. Additionally, improvement in weight Z score was seen in the entire population and the most at risk patients for malnutrition, results that have not been shown before., Level of Evidence: Level IV., (Copyright © 2018 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

Background: Software applications (apps) could potentially promote exercise adherence. However, it is unclear whether adolescents with painful hyperkyphosis will use an app designed for a home exercise program. The purpose of this study is to assess factors regarding adherence to an app-based home exercise program in adolescents with hyperkyphosis and back pain who were provided a one-time exercise treatment., Methods: Twenty-one participants were instructed in a one-time exercise treatment and asked to complete a home exercise program 3 times a week for 6 months using an app called PT PAL. At a 6-month follow-up, 14 participants completed a survey assessing factors related to their experiences using the app and their treatment engagement., Results: Although most participants did not use the app, they reported performing their exercises a few times per week. The adolescent participants considered the app to be more of a barrier than a supportive measure for promoting exercise adherence. Most participants still reported bothersome back pain., Conclusions: Although adherence to the 6-month app-based home exercise program was not successful, adolescents still viewed technology support such as text reminders as a potential solution., Trial Registration: ClinicalTrials.gov Identifier: NCT03212664. Registered 11 July 2017. Retrospectively registered., Competing Interests: This study was approved by the Institutional Review Board of the primary investigator’s affiliated institute and registered with ClinicalTrials.gov. Prior to data collection, each adolescent participant’s assent and caregiver’s consent to participate in the study were obtained.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Study Design: Surgeon survey., Objectives: To determine if magnetic resonance imaging (MRI) following implantation of magnetically controlled growing rods (MCGRs) is associated with any adverse events., Summary of Background Data: Magnetically controlled growing rods have been shown to reduce the need for repeated surgical procedures and improve costs when compared to traditional growing rods, but concerns about MRI compatibility exist. MRIs are often clinically indicated in the EOS population., Methods: Pediatric spine surgeons who are members of the Growing Spine Study Group, Children's Spine Study Group, and early international users of this technology were surveyed regarding MRI use after performing MCGR surgery., Results: A total of 118 surgeons were surveyed. Four surgeons reported that 10 patients had an MRI with an implanted MCGR. Loss of fixation (0%, 0/10), movement of implants (0%, 0/10), unintended lengthening/shortening (0%, 0/10), or noticeable heating of MCGR (0%, 0/10) were not observed. No problems were observed with function of the MCGR following MRI, and a mean of 2.1 mm was obtained at the next lengthening (range, 0.5-3.0 mm). Two patients had brain MRIs, both of which could be interpreted. All cervical spine MRIs could be interpreted without excessive artifact (100%, 7/7). Six patients had MRIs of the thoracic or lumbar spine, but these were considered uninterpretable as a result of artifact from the MCGR device (0%, 0/6)., Conclusion: These are the first reported cases of MRI use in humans with MCGR. There were no adverse events observed. MCGR rods lengthened as expected following MRI. MRIs of the brain and cervical spine were able to be interpreted, but MRIs of the thoracolumbar spine could not be interpreted because of MCGR artifact. MRIs can be safely performed in patients with MCGRs; however, MRIs of thoracic and thoracolumbar spine may be of limited clinical benefit because of artifact., Level of Evidence: Level IV, case series., (Copyright © 2017 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

Study Design: Multicenter retrospective cohort., Objective: To compare improvement in nutritional status seen in early-onset scoliosis (EOS) patients following treatment with various growth-friendly techniques, especially in underweight patients (<20th weight percentile)., Background: Thoracic insufficiency resulting from EOS can lead to severe cardiopulmonary disease. In this age group, pulmonary function tests are often difficult or impossible to perform. Weight gain has been used in prior studies as a proxy for improvement and has been demonstrated following VEPTR and growing rod implantation. In this study, we aim to analyze weight gain of EOS patients treated with four different spinal implants to evaluate if significant differences in weight percentile change exist between them., Methods: Retrospective review of patients treated surgically for EOS was performed from a multicenter database. Exclusion criteria were index instrumentation at >10 years old and <2 years' follow-up., Results: 287 patients met the inclusion criteria and etiologies were as follows: congenital = 85; syndromic = 79; neuromuscular = 69; and idiopathic = 52. Average patient age at surgery was 5.41 years, with an average follow-up of 5.8 years. Preoperatively, 55.4% (162/287) fell below the 20th weight percentile. There was no significant difference in preoperative weight between implants (p = .77), or diagnoses (p = .25). Among this group, the mean change in weight percentile was 10.5% (range: -16.7% to 88.7%) and all implant groups increased in mean weight percentile at final follow-up. There were no significant differences in weight percentile change between the groups when divided by implant type (p = .17)., Conclusions: Treatment of EOS with growth-friendly constructs resulted in an increase in weight percentile for underweight patients (<20th percentile), with no significant difference between constructs., Level of Evidence: Level III., (Copyright © 2017 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

Background: Quengel casting was introduced in 1922 for nonsurgical treatment of knee flexion contractures (KFC) associated with hemophilic arthropathy. It consists of an extension-desubluxation hinge fixed to a cast allowing for gradual correction of a flexion deformity while preventing posterior tibial subluxation. The purpose of this study is to report 1 center's experience with this technique for the treatment of pediatric KFC., Methods: A retrospective review was conducted over a 26-year period. All patients with KFC treated with Quengel casting were included. Demographic data, associated medical conditions, adjunctive soft tissue releases, complications, and the need for late surgical intervention were recorded. Tibiofemoral angle measurements in maximal extension were recorded at initiation and termination of casting, 1-year follow-up, and final follow-up. Success was defined as no symptomatic recurrence of KFC or need for subsequent surgery., Results: Eighteen patients (26 knees) were treated for KFC with Quengel casting. Average age at initiation of casting was 8.1 years with average follow-up of 59.9 months. Fifteen knees (58%) underwent soft tissue releases before casting. An average of 1.5 casts per knee were applied over an average of 23.9 days. Average KFC before casting was 50.6 degrees (range, 15 to 100 degrees) which improved to 5.96 degrees (0 to 40 degrees) at cast removal (P<0.00001). Sixteen patients (22 knees) had 1-year follow-up or failed casting before 1 year. Of these, 11 knees (50%) had a successful outcome. Residual KFC of those treated successfully was 6.8 degrees (range, 0 to 30 degrees) at 1 year and 8.2 degrees (range, 0 to 30 degrees) at final follow-up, averaging 71.4 months (P=0.81). Of the 11 knees deemed failures, all had recurrence of deformity within an average of 1 year from cast removal. Surgical release before Quengel casting did not improve the chances for success (P=0.09)., Conclusions: Quengel casting can improve pediatric KFC an average of 44.2 degrees with minimal complications. Although 50% of treated patients will demonstrate significant recurrence or need later surgery, the majority of those treated successfully have durable results at intermediate term follow-up., Level of Evidence: Level IV-therapeutic study.

Background: In this study, we sought to evaluate radiographic, functional, and quality-of-life outcomes of patients who have completed growth-sparing management of early-onset scoliosis., Methods: This prospective study involved patients with early-onset scoliosis who underwent growth-sparing treatment and either "final" fusion or observation for ≥2 years since the last lengthening procedure. Demographics, radiographic parameters, pulmonary function test (PFT) values, and scores of patient-reported assessments (Early-Onset Scoliosis Questionnaire [EOSQ] and Scoliosis Research Society [SRS]-30) were obtained. At the most recent follow-up, patients performed 2 additional functional outcome tests: step-activity monitoring and a treadmill exercise-tolerance test., Results: Twelve patients were evaluated as "graduates" of growth-sparing management of early-onset scoliosis (mean of 37 months since the most recent surgery). The major scoliosis curve measurement averaged 88° before treatment and 47° at the most recent follow-up. T1-S1 height increased from a mean of 22.3 cm to 34.7 cm and T1-T12 height, from 13.3 to 22.3 cm. At the most recent follow-up, the mean forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) as a percentage of the predicted volume were 52.1% and 55.3%, respectively, and were essentially unchanged from the earliest PFT that patients could perform (FEV1 = 53.8% of predicted and FVC = 53.5% of predicted). There was no difference between graduates and controls with respect to activity time or total steps in step-activity monitoring, and in the exercise-tolerance test, graduates walked at the same speed but at a higher heart rate and at a significantly higher (p <0.001) VO2 cost (rate of oxygen consumed per distance traveled). The EOSQ mean score was 102.2 of a possible 120 points, and the SRS mean score was 4.1 of a possible 5 points., Conclusions: A realistic long-term goal for the management of early-onset scoliosis appears to be spine elongation and maintenance of pulmonary function at a level that is no less than the percentage of normal at initial presentation. Functional testing and patient-reported outcomes at a mean of 3 years from the last surgery suggest that activity levels were generally equal to those of controls but required greater physiologic demand. General health and physical function outcomes revealed continued impairment in these domains., Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Scoliosis deformity has been linked with deleterious changes in the thoracic cavity that affect pulmonary function. The causal relationship between spinal deformity and pulmonary function has yet to be fully defined. It has been hypothesized that deformity correction improves pulmonary function by restoring both respiratory muscle efficiency and increasing the space available to the lungs. This research aims to correlate pulmonary function and thoracic volume before and after scoliosis correction. Retrospective correlational analysis between thoracic volume modeling from plain x-rays and pulmonary function tests was conducted. Adolescent idiopathic scoliosis patients enrolled in a multicenter database were sorted by pre-operative Total Lung Capacities (TLC) % predicted values from their Pulmonary Function Tests (PFT). Ten patients with the best and ten patients with the worst TLC values were included. Modeled thoracic volume and TLC values were compared before and 2 years after surgery. Scoliosis correction resulted in an increase in the thoracic volume for patients with the worst initial TLCs (11.7%) and those with the best initial TLCs (12.5%). The adolescents with the most severe pulmonary restriction prior to surgery strongly correlated with post-operative change in total lung capacity and thoracic volume (r 2  = 0.839; p < 0.001). The mean increase in thoracic volume in this group was 373.1 cm 3 (11.7%) which correlated with a 21.2% improvement in TLC. Scoliosis correction in adolescents was found to increase thoracic volume and is strongly correlated with improved TLC in cases with severe restrictive pulmonary function, but no correlation was found in cases with normal pulmonary function. © 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 35:175-182, 2017., (© 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc.)

Early amphibious tetrapods may have detected aquatic sound pressure using sound-induced lung vibrations, but their lack of tympanic middle ears would have restricted aerial sensitivity. Sharing these characteristics, salamanders could be models for the carryover of auditory function across an aquatic-terrestrial boundary without tympanic middle ears. We measured amphibious auditory evoked potential audiograms in five phylogenetically and ecologically distinct salamanders (Amphiuma means, Notophthalmus viridescens, Ambystoma talpoideum, Eurycea spp., and Plethodon glutinosus) and tested whether metamorphosis and terrestrial niche were linked to aerial sensitivity. Threshold differences between media varied between species. A. means' relative aerial sensitivity was greatest at 100 Hz and decreased with increasing frequency. In contrast, all other salamanders retained greater sensitivity up to 500 Hz, and in A. talpoideum and Eurycea, relative sensitivity at 500 Hz was higher than at 100 Hz. Aerial thresholds of terrestrial P. glutinosus above 200 Hz were similar to A. talpoideum and Eurycea, but lower than N. viridescens and A. means. Metamorphosis did not affect aerial sensitivity in N. viridescens or A. talpoideum. These results fail to support a hypothesis of terrestrial hearing specialization across ontogeny or phylogeny. We discuss methodological limitations to our amphibious comparisons and factors affecting variation in amphibious performance.

Study Design: Prospectively enrolled early-onset scoliosis (EOS) patients undergoing growing rod treatment, who have had no surgery for >1 year and/or have received definitive fusion (growing rod "graduates")., Objectives: To assess oxygen consumption during exercise and determine if a diminished conventional pulmonary function test (PFT) correlates with metabolic, pulmonary, and cardiovascular measures during exercise., Summary of Background Data: Based on clinical impression and sequential PFT values, EOS patients who have undergone extensive treatment are thought to have limited capacity during exercise. The use of PFTs in this population has been a primary outcome measure of respiratory capacity; however, PFTs are dependent on effort, and thus subjective. This led us to find a new assessment of outcome, to better understand their pulmonary capacity., Methods: Patients underwent oxygen consumption (VO 2 ) testing while walking at self-selected speed over-ground and during a graded exercise test. Maximal VO 2 was predicted in those who completed the test to 85% of maximal heart rate (HR). Statistical analysis included Mann-Whitney U test and Spearman correlation coefficient (α = 0.05)., Results: 12 patients participated. Over-ground walking showed that EOS graduates chose to walk at the same speed, but at a higher VO 2 Cost (0.28 mL/kg/m) than controls (0.22 mL/kg/m; p < .001). Treadmill exercise testing showed 9 of 12 subjects able to complete the 85% of predicted maximum protocol. The EOS group had lower VO 2 during the final stage (27.9 mL/kg/min) compared to controls (34.2 mL/kg/min; p = .021); however, their heart rate reached the same values. Subjects completing the protocol had lower predicted VO 2 max (38.5 mL/kg/min) compared with controls (45.0 mL/kg/min), but this was not significant., Conclusions: Although PFT data suggest clinically relevant pulmonary compromise in EOS patients, the current study shows that these children are able to keep up with their peers in daily activities and also have the capacity to exercise., Level of Evidence: Level II, therapeutic., (Copyright © 2016 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

Background: The purpose of this study is to assess radiographic shoulder measures from the preoperative to the postoperative time period, specifically to determine whether T1 tilt could be used as an intraoperative proxy for shoulder balance determination. This study focused on radiographic shoulder measures of 619 adolescent idiopathic scoliosis patients who underwent spinal deformity surgery., Methods: A prospective, multicenter database of adolescent idiopathic scoliosis was queried to identify all patients who had undergone spinal deformity surgery with >2 years of follow-up postoperatively. Radiographic analysis focused on measures of shoulder balance: T1 tilt, clavicle angle, and radiographic shoulder height., Results: A total of 619 patients were included in this analysis. Mean age at surgery was 14.8 years with 83% female. Mean preoperative curve size was 58.0 degrees. Mean T1 tilt preoperatively was -0.10 degrees and postoperatively 2.42 degrees. Mean clavicle angle preoperatively was -1.39 degrees and postoperatively 0.79 degrees. Mean radiographic shoulder height preoperatively was -7.04 mm and postoperatively 1.63 mm. All 3 radiographic parameters demonstrated reasonable correlation preoperatively and postoperatively to each other. To assess the viability of T1 tilt as an intraoperative proxy for shoulder balance, standardized ratios between the variables were created. Analysis of these ratios demonstrated little or no relationship preoperatively to postoperatively, hence the relationship of T1 tilt to radiographic shoulder height does not remain constant., Conclusions: Analysis of the relationship of T1 tilt to radiographic shoulder height from preoperative to postoperative did not demonstrate consistency. Lenke 3 and 6 curve patterns demonstrated preoperative to postoperative correlation, both with nonstructural proximal thoracic curves; however, for the remaining curve patterns T1 tilt cannot be used as an intraoperative proxy for shoulder balance., Level of Evidence: Level IV.

Although the adult brain contains neural stem cells (NSCs) that generate new neurons throughout life, these astrocyte-like populations are restricted to two discrete niches. Despite their terminally differentiated phenotype, adult parenchymal astrocytes can re-acquire NSC-like characteristics following injury, and as such, these 'reactive' astrocytes offer an alternative source of cells for central nervous system (CNS) repair following injury or disease. At present, the mechanisms that regulate the potential of different types of astrocytes are poorly understood. We used in vitro and ex vivo astrocytes to identify candidate pathways important for regulation of astrocyte potential. Using in vitro neural progenitor cell (NPC)-derived astrocytes, we found that exposure of more lineage-restricted astrocytes to either tumor necrosis factor alpha (TNF-α) (via nuclear factor-κB (NFκB)) or the bone morphogenetic protein (BMP) inhibitor, noggin, led to re-acquisition of NPC properties accompanied by transcriptomic and epigenetic changes consistent with a more neurogenic, NPC-like state. Comparative analyses of microarray data from in vitro-derived and ex vivo postnatal parenchymal astrocytes identified several common pathways and upstream regulators associated with inflammation (including transforming growth factor (TGF)-β1 and peroxisome proliferator-activated receptor gamma (PPARγ)) and cell cycle control (including TP53) as candidate regulators of astrocyte phenotype and potential. We propose that inflammatory signalling may control the normal, progressive restriction in potential of differentiating astrocytes as well as under reactive conditions and represent future targets for therapies to harness the latent neurogenic capacity of parenchymal astrocytes.