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3q29 deletion syndrome (3q29del) is a rare genomic disorder caused by a 1.6 Mb deletion (hg19, chr3:195725000-197350000). 3q29del is associated with neurodevelopmental and psychiatric phenotypes, including an astonishing >40-fold increased risk for schizophrenia, but medical phenotypes are less well-described. We used the online 3q29 registry of 206 individuals (3q29deletion.org) to recruit 57 individuals with 3q29del (56.14% male) and requested information about musculoskeletal phenotypes with a custom questionnaire. 85.96% of participants with 3q29del reported at least one musculoskeletal phenotype. Congenital anomalies were most common (70.18%), with pes planus (40.35%), pectus excavatum (22.81%), and pectus carinatum (5.26%) significantly elevated relative to the pediatric general population. 49.12% of participants reported fatigue after 30 min or less of activity. Bone fractures (8.77%) were significantly elevated relative to the pediatric general population. Participants commonly report receiving medical care for musculoskeletal complaints (71.93%), indicating that these phenotypes impact quality of life for individuals with 3q29del. This is the most comprehensive description of musculoskeletal phenotypes in 3q29del to date, suggests ideas for clinical evaluation, and expands our understanding of the phenotypic spectrum of this syndrome., (© 2023 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals LLC.)

Objective: Aneurysmal bone cysts (ABC) are benign but locally aggressive lesions. Treating children with spinal ABC poses risks due to the proximity of the lesion to the spinal cord and the need to preserve spinal stability after surgery. This study reports the updated outcomes of a uniform aggressive initial surgical technique for the treatment of spinal ABC in children., Methods: Twenty-nine cases of spinal ABC were collected from a tertiary pediatric tumor center over a 24-year period (January 1990-September 2014). The study patients were divided into 2 groups based on the performed procedure: a traditional approach consisting of curettage and bone grafting (group 1) and a 4-step approach consisting of intralesional curettage, high-speed bur, electrocautery, and bone grafting (group 2)., Results: The study population was composed of 12 males and 17 females with a mean age of 12.2 years at the time of diagnosis and a mean postoperative follow-up of 4.03 years. Twenty-one patients underwent the 4-step approach, while 8 patients were treated with the traditional technique. The recurrence rate was 50% (4/8 patients) with the traditional technique (group 1) and 19% (4/21) in the 4-step technique (group 2) ( P = .164). Regardless of the technique used, the presence of sensory symptoms at the time of presentation was a statistically significant factor for recurrence ( P = .016)., Conclusions: We suggest that addressing spinal ABC in pediatric patients with a 4-step approach is a safe technique that may decrease long-term recurrence and reoperation rates compared to the traditional technique of intralesional curettage and bone grafting., Level of Evidence: 4., (This manuscript is generously published free of charge by ISASS, the International Society for the Advancement of Spine Surgery. Copyright © 2020 ISASS.)

The article Is the new ASNM intraoperative neuromonitoring supervision "guideline" a trustworthy guideline? A commentary, written by Stanley A. Skinner, Elif Ilgaz Aydinlar, Lawrence F. Borges, Bob S. Carter, Bradford L. Currier, Vedran Deletis, Charles Dong, John Paul Dormans, Gea Drost, Isabel Fernandez‑Conejero, E. Matthew Hoffman, Robert N. Holdefer, Paulo Andre Teixeira Kimaid, Antoun Koht, Karl F. Kothbauer, David B. MacDonald, John J. McAuliffe III, David E. Morledge, Susan H. Morris, Jonathan Norton, Klaus Novak, Kyung Seok Park, Joseph H. Perra, Julian Prell, David M. Rippe, Francesco Sala, Daniel M. Schwartz, Martín J. Segura, Kathleen Seidel, Christoph Seubert, Mirela V. Simon, Francisco Soto, Jeffrey A. Strommen, Andrea Szelenyi, Armando Tello, Sedat Ulkatan, Javier Urriza and Marshall Wilkinson, was originally published electronically on the publisher's internet portal (currently SpringerLink) on 05 January 2019 without open access. With the author(s)' decision to opt for Open Choice the copyright of the article changed on 30 January 2019 to © The Author(s) 2019 and the article is forthwith distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits use, duplication, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license and indicate if changes were made. The original article has been corrected.

Study Design: Prospective case series of nine children with early-onset scoliosis (EOS) treated by a single surgeon with a novel implant, the magnet-driven growing rod (MdGR) in a publicly funded health care service accounting for "payer costs" (PC) incurred., Objective: The aim of this study was to compare the cost-effectiveness of MdGR versus conventional growing rods (CGRs) with respect to the PC incurred for treating EOS at 5 years., Summary of Background Data: Cost estimate and mathematical modeling study projections of MdGR have shown despite high insertional costs, it breaks even with CGR by 3 to 4 years. However, no clinical study to date exists either supporting or refuting this hypothesis., Methods: Nine patients with EOS secondary to idiopathic (two), congenital (one), syndromic (three), and neuromuscular (three) etiologies treated by submuscular insertion of MdGR against stringent inclusion criteria formed the study cohort. We collected costs incurred with all aspects of care over the lifetime of device (or at least 5 years) from payers' perspective to compute and report average PC incurred per patient. We performed this cost analysis by comparing the MdGR PC against literature reported PC for CGR at 5 years., Results: There were five single rod (SR) and two dual rod (DR) de novo MdGR insertions, while two patients had conversion of CGR to MdGR. MdGR alone accounted for at least 50% of overall budget. The MdGR was at least 40% more cost-effective in comparison to the CGR (£34,741 vs. £52,293) and there were seven MdGR graduates., Conclusion: The first study reporting direct PC incurred in EOS treated by MdGR that is devoid of any mathematical modeling and deterministic sensitivity analysis is presented. The true societal/human cost savings taking into consideration indirect costs are likely to be significantly higher. MdGR is a promising novel implant that may eventually become the "standard of care" for certain EOS etiologies., Level of Evidence: 4.

Introduction: Pelvic sarcomas may present with vague symptoms. The aim of this study was to examine the characteristics and clinical presentations of different types of pelvic sarcoma in children., Methods: This is a retrospective cohort study of patients up to 21 years of age with the diagnosis of pelvic sarcoma between January 2000 and June 2013. Data on demographics, tumor type and location, and clinical presentation were collected from the medical records., Results: A total of 59 patients [37 males (62.7%) and 22 females (37.3%)] were examined in this study. Mean age at presentation was 11.3 ± 5 years (range 0.8-21 years). Thirty-six patients had Ewing sarcoma (61%), 9 osteosarcoma (6.8%), 4 undifferentiated sarcoma (6.8%), 2 (3.4%) rhabdomyosarcoma, 2 synovial cell sarcoma, and one (1.7%) of each fibrosarcoma, dermatofibrosarcoma, fibromyxoid sarcoma, chondrosarcoma, chordoma, and epithelioid sarcoma. Pain at presentation was reported in 41 patients, 13 mass, 8 limping, and 5 neurologic symptoms. Most of the bony tumors were painful (77%), while most of the soft tissue tumors were painless (70%). Nine patients presented with constitutional symptoms. Most patients presented within 4-12 months from symptoms beginning. Twenty-one patients (35.6%) presented with metastases (14 Ewing sarcoma, 6 osteosarcoma, and 1 synovial cell sarcoma). Pelvic radiographs showed lytic lesion in 11 patients, 4 sclerotic lesions, 6 mixed lesion, 6 had only soft tissue mass, 1 radiograph showed osteopenia, and 2 radiographs were reported normal., Conclusion: Ewing sarcoma was the most common pelvic sarcoma tumor in children. In most cases, pelvic sarcoma in children presented with pain mimicking other benign conditions. Some patients presented with metastatic disease with no prognostic clinical or radiographical signs or symptoms. Pelvic sarcoma should be considered a differential diagnosis as part of children work up., (© The Authors, published by EDP Sciences, 2018.)

To obtain a wide resection and safe margins in recurrent spine osteosarcoma, the surgical approach can include - posterior only, combined posterior and anterior, and combined posterior and anterior with a return to posterior in multiple stages. In our case, we used a novel approach of multiple extensile exposures circumferentially in a single stage with a single surgical prep. We present the case of a 9-year-old female with a history of metastatic osteosarcoma, who previously underwent an attempted en bloc resection with an L3 corpectomy and left below knee amputation. At 1 year follow-up, she developed a recurrent solitary spine lesion at the previous surgical resection site. An additional attempt at complete surgical resection was performed with a complex en bloc L2, L3, L4 corpectomy with removal of deep spinal implants and anterior and posterior spinal fusion with instrumentation and revision decompressive laminectomy. The patient had a good functional outcome without neurological deficits, except those resulting from resection of involved lumbar nerve roots. At last follow-up of 5 months, there was no local recurrence or distant metastasis. This approach for revision resection of recurrent spinal osteosarcoma can be performed successfully with clean margins in a safe manner., (© The Authors, published by EDP Sciences, 2018.)

Purpose: Pelvic sarcoma is uncommon in children and challenging to treat. This study examined different surgical approaches to treat pelvic sarcoma with the aim of assessing the oncologic, and functional outcomes., Methods: We retrospectively examined the medical records of patients younger than 21 years of age who underwent surgery for pelvic sarcoma at our institution from 1992 to 2010. The functional status of the patients was examined after a minimum follow-up of two years., Results: Twenty-six patients were included in the analysis. Nineteen (73%) patients were male and seven (27%) were female. Mean age at presentation was 12.0 ± 3.9 years. Nineteen patients had Ewing sarcoma (73%), five had osteosarcoma (19%), one had chondrosarcoma (4%) and one had rhabdomyosarcoma (4%). Iliac wing resection with no reconstruction was done in three patients. Reconstruction with free fibular graft A-frame was performed in four patients, saddle endoproshtesis in five patients, iliac autoclave in one patient, and internal hemipelvectomy in nine patients. Hindquarter amputation was performed in five patients. Median follow-up was 4.6 years (range, 2.6-16 years). Nineteen patients were alive (73%); of those, 13 were known to be without disease, three were with disease and three did not have known tumor status. Six patients were reported deceased, three had osteosarcoma and three had Ewing sarcoma. Function was assessed in 17 patients; 64% were asymptomatic and ambulatory and 36% were symptomatic and ambulatory., Conclusions: Salvage reconstruction for pelvic sarcoma can be performed through various procedures on the extent of necessary bony resection. Survival rate and functional outcomes were promising in the performed study.

Intraoperative radiographic guidance has traditionally been utilized in orthopedic surgery through 2-D navigation with the C-arm and recently with 3-D navigation with the O-arm. The aim of this study was to describe the outcome of surgical treatment of spinal osteoblastoma and osteoid osteoma with the utilization of the O-arm and conventional C-arm guidance. This is a retrospective cohort study of patients with spinal osteoid osteoma and or osteoblastoma who were treated at our institution between 2002 and 2011. Seventeen patients were examined in this study including seven with spinal osteoblastoma and 10 with spinal osteoid osteoma. The mean age of the patients at surgery was 11.5±3.9 years. The O-arm was used in seven patients and the C-arm in 10 patients. The C-arm failed to identify the tumor in one case and needed transport to perform a computed tomographic-scan. The length of surgery was shorter when the O-arm was used, especially in the osteoblastoma group. Thirteen patients were pain free at the last follow-up visit and two patients developed recurrence. Radiographs at the last follow-up did not show signs of vertebral instability following tumor resection. Safe and effective localization of spine tumors and confirmation of tumor removal during surgery was achieved by intraoperative radiographic guidance specifically with the O-arm 3-D navigation system., Level of Evidence: III.

Purpose: Is there any relationship between lengthening intervals and rod fracture in traditional growing rod (TGR) surgery?, Methods: A multicenter EOS database was queried for patients who had: (1) dual growing rods for EOS; (2) minimum 2-year follow-up; (3) a minimum of 2 lengthenings; and (4) revision surgery due to rod fracture. Of 138 patients who met the criteria: 56 patients experienced at least one-rod fracture (RF group) and 82 patients had no rod fractures (NRF group). In addition to each patient's lengthening intervals, demographics, construct details, and radiographic parameters were compared., Results: RF and NRF patients had a mean pre-op age of 5.7 years (range 1.3-10.7) and 7.3 years (range 1.6-12.8), respectively (p < 0.001). There was no significant association between etiologies and rod fracture or between BMI and rod fracture (p = 0.979). There was no significant difference between lengthening intervals between the RF and NRF groups (p > 0.05). RF and NRF patients had statistically similar mean pre-op major curve size and max kyphosis (p = 0.279; p = 0.619, respectively). Stainless steel rods fractured more frequently compared with Titanium rods (SS 49.2 % vs. Ti 38 %; p = 0.004). Rod fracture occurred more in rods smaller than 4 mm (p = 0.011)., Conclusions: Lengthening intervals were not statistically different in RF and NRF groups and there was no association between lengthening interval and rod fracture in TGR cases. It was shown that patients who had rod fracture were younger and were more likely to have SS rods with smaller than 4 mm diameter.

In children, hematogenous osteomyelitis is an infection that primarily affects the most vascularized regions of the growing skeleton. The disease has increased in frequency, virulence, and degree of soft-tissue involvement. The change in clinical manifestations and management over the past 2 decades should be reflected in the current imaging approach to the disease. Imaging of infection must depict the location of a single focus or of multiple foci of involvement and the presence of drainable collections. This review provides an overview of the imaging implications directed by the changing epidemiology, the newer insights of anatomy and pathophysiology, the imaging characteristics with emphasis on specific locations and disease complications, and the differential diagnosis considerations. In addition, basic imaging guidelines for appropriate extent of area to image based on patient age are provided. © RSNA, 2017.

Background: Cervical kyphosis may occur with neurofibromatosis type I (NF1) and is often associated with vertebral dysplasia. Outcomes of cervical spinal fusion in patients with NF1 are not well described because of the rarity of the condition. We aimed to (1) characterize the clinical presentation of cervical kyphosis and (2) report the outcomes of posterior and anteroposterior cervical fusion for the condition in these children., Methods: The medical records and imaging studies of 22 children with NF1 who had undergone spinal fusion for cervical kyphosis (mean, 67°) at a mean age of 11 years and who had been followed for a minimum of 2 years were reviewed., Results: Thirteen children presented with neck pain; 10, with head tilt; 9, with a previous cervical laminectomy or fusion; and 5, with a neurologic deficit. Two patients had spontaneous dislocation of the mid-cervical spine without a neurologic deficit. Eleven had scoliosis, with the major curve measuring a mean of 61°. Nine patients underwent posterior and 13 underwent anteroposterior surgery. Twenty-one received spinal instrumentation, and 1 was not treated with instrumentation. Preoperative halo traction was used for 9 patients, and it reduced the mean preoperative kyphosis by 34% (p = 0.0059). At the time of final follow-up, all spinal fusion sites had healed and the cervical kyphosis averaged 21° (mean correction, 69%; p < 0.001). The cervical kyphosis correction was significantly better after the anteroposterior procedures (83%) than after the posterior-only procedures (58%) (p = 0.031). Vertebral dysplasia and erosion continued in all 17 patients who had presented with dysplasia preoperatively. Thirteen patients had complications, including 5 new neurologic deficits and 8 cases of junctional kyphosis. Nine patients required revision surgery. Junctional kyphosis was more common in children in whom ≤5 levels had been fused (p = 0.054)., Conclusions: Anteroposterior surgery provided better correction of cervical kyphosis than posterior spinal fusion in children with NF1. Erosion of vertebral bodies continued during the postoperative follow-up period in all patients who had presented with dysplastic changes preoperatively. The cervical spine should be screened in all children with NF1. Fusion should include at least 6 levels to prevent junctional kyphosis., Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence., (Copyright © 2016 by The Journal of Bone and Joint Surgery, Incorporated.)

Purpose: Although adolescent idiopathic scoliosis (AIS) is known to impact the 3D orientation of the spine and pelvis, the impact of the vertebral position relative to the X-ray scanner on the agreement between 2D and 3D measurements of a curve has not been evaluated. The purpose of this study was to investigate the agreement between 2D and 3D measurements of the scoliotic curve as a function of the 3D spinal parameters in AIS., Methods: Three independent observers measured the thoracic and lumbar Cobb angles, Kyphosis, and lordosis on the posterior-anterior and lateral X-rays of AIS patients. The 3D reconstructions were created from bi-planar X-rays and the 3D spinal parameters were calculated in both radio and patient planes using SterEOS software. The degree of agreement between the 2D and 3D measurements was tested and its relationship with the curve axial rotation was determined., Results: 2D and 3D measurements of the sagittal plane spinal parameters were significantly different (p < 0.05). The differences between the 2D and 3D measurements were related to the apical vertebrae rotation, the orientation of the plane of maximum curvature, pelvic axial rotation, and the curve magnitude. Differences between the radio plane and patient plane measurements were related to the pelvic axial rotation, Cobb angles, and apical vertebrae rotation, p < 0.05., Conclusion: Clinically and statistically significant differences were observed between the 2D and 3D measurements of the scoliotic spine. The differences between the 2D and 3D techniques were significant in sagittal plane and were related to the spinal curve and pelvic rotation in transverse plane.

Purpose: A prospective, cross-sectional study to determine the impact of arm position on the 3D spine and pelvic parameters and postural balance in adolescent idiopathic scoliosis., Methods: A total number of 37 adolescent idiopathic scoliosis patients were enrolled prospectively. Three arm positions, (1) 45° shoulder flexion with knuckles on clavicles, (2) 90° shoulder and elbow flexion with forearms and palms on the front wall, and (3) arms hanging on either side, were instructed to the cohort. Bi-planar low dose X-ray images of the spine and pelvis were registered in a stereoradiography system in the first and second arm positions. A pressure mat recorded the position of the center of pressure in each arm position. Spinal and pelvic parameters were measured for the cohort. Statistical analysis was performed to determine the agreement between the spinal and pelvic parameters and standing balance in different arm positions., Results: Thoracic kyphosis, sacral slope, sagittal vertical axis, T1 tilt, and spinal height were significantly different between the knuckles on clavicle and forearms on the wall positions p < 0.05. Significant differences were observed in the pressure distribution under the feet between the wall and freestanding positions. Bland-Altman plots determined disagreements between the first and second arm positions in clinical measurements and postural assessment of adolescent idiopathic scoliosis., Conclusion: The knuckles on clavicles position replicates the functional standing balance in AIS. Significant differences between the spinal and pelvic parameters suggest that the wall and clavicle arm positions should not be used interchangeably in AIS postural assessment., Level of Evidence: Diagnostic level II.

Background: Surgical site infections (SSIs) can have devastating consequences for children who undergo spinal instrumentation. Prospective evaluations of prophylactic cefazolin in this population are limited. The purpose of this study was to describe the pharmacokinetics and skeletal muscle disposition of prophylactic cefazolin in a paediatric population undergoing complex spinal surgery., Methods: This prospective pharmacokinetic study included 17 children with adolescent idiopathic scoliosis undergoing posterior spinal fusion, with a median age of 13.8 [interquartile range (IQR) 13.4-15.4] yr and a median weight of 60.6 (IQR 50.8-66.0) kg. A dosing strategy consistent with published guidelines was used. Serial plasma and skeletal muscle microdialysis samples were obtained during the operative procedure and unbound cefazolin concentrations measured. Non-compartmental pharmacokinetic analyses were performed. The amount of time that the concentration of unbound cefazolin exceeded the minimal inhibitory concentration for bacterial growth for selected SSI pathogens was calculated., Results: Skeletal muscle concentrations peaked at a median of 37.6 (IQR 26.8-40.0) µg ml(-1) within 30-60 min after the first cefazolin 30 mg kg(-1) dose. For patients who received a second 30 mg kg(-1) dose, the peak concentrations reached a median of 40.5 (IQR 30.8-45.7) µg ml(-1) within 30-60 min. The target cefazolin concentrations for SSI prophylaxis for meticillin-sensitive Staphylococcus aureus (MSSA) and Gram-negative pathogens were exceeded in skeletal muscle 98.9 and 58.3% of the intraoperative time, respectively., Conclusions: For children with adolescent idiopathic scoliosis undergoing posterior spinal fusion, the cefazolin dosing strategy used in this study resulted in skeletal muscle concentrations that were likely not to be effective for intraoperative SSI prophylaxis against Gram-negative pathogens., (© The Author 2016. Published by Oxford University Press on behalf of the British Journal of Anaesthesia. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Study Design: Observational quantitative evaluation of all Scoliosis Research Society annual meeting (SRS-AM) podium presentations over a 6-year period in the new millennium for changing trends in Level of Evidence (LoE)., Objectives: To report the LoE of SRS-AM podium presentations over a 13-year period in the new millennium and to compare the trends in LoE over a 3-year period between the two decades, that is, 2001-2003 versus 2011-2013., Summary of Background Data: LoE is a rating system introduced by the American Academy of Orthopaedic Surgeons (AAOS) in 2003 to reflect the quality of clinical studies. No study has looked into any of AAOS-affiliated spinal specialist societies' LoE, hence this study., Methods: The SRS-AM proceedings books from two 3-year periods, 2001-2003 and 2011-2013, were retrieved. Two orthopedic surgeons independently graded each abstract on two occasions, 1 month apart, assigning them LoE from I to V, in accordance with the stringent guidelines published by AAOS. They also categorized all studies into types (ie, therapeutic, prognostic, diagnostic, economic or decision-modeling analysis, or LoE not applicable). Any disagreement between the two assessors over the LoE or the nature of study was discussed, and agreed upon by consensus. Thus, analyzed abstracts of the two groups were subsequently compared and subjected to statistical tests to determine significance., Results: 637 free-papers from six annual meeting proceedings (264 in Group I and 373 in Group II) were analyzed in total. LoE I and II studies have more than doubled (p<.0001), and LoE IV studies have fallen by half in 2011-2013 as compared to 2001-2003., Conclusion: The SRS-AM podium presentation has shown an increase in LoE I and II studies in the new millennium. SRS is the first AAOS-affiliated subspecialty society to have undertaken an in-depth analysis of its AM podium presentations, setting a standard for other societies to emulate., Level of Evidence: Not applicable., (Copyright © 2016 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

Study Design: A cross-sectional survey of surgeon members of the Scoliosis Research Society (SRS)., Objective: This study sought to characterize the incidence, clinical presentation, diagnostic workup, treatment, and neurologic prognosis following delayed postoperative neurologic deficit (DPND) in patients undergoing spinal deformity surgery., Summary of Background Data: DPND is a potentially devastating condition following spinal surgery, characterized by the development of a neurological deficit within hours or days of the surgical procedure. To date, only case reports and small case series have been published on the topic., Methods: We developed a survey to characterize DPND following spinal deformity surgery. This survey was distributed to surgeon members of the SRS through email and standard mail. The overall response rate was 38% (352/929)., Results: Our results suggest an estimated DPND incidence of 1 of 9910 cases (0.01%). Eighty-one surgeons (23%) experienced at least 1 DPND in the past 10 years (92 total cases). Most common diagnoses were scoliosis (69%), kyphosis (23%), and spondylolisthesis (14%); 20% were revision surgeries. The number of hours to deficit onset was as follows: 1 to 12 (36%), 13 to 24 (27%), 25 to 48 (27%), more than 48 (10%). The most commonly cited sources of injury included ischemic injury (38%) and cord compression (15%). Forty-one percent experienced complete neurologic recovery, 26% partial, and 33% no recovery. Twenty-one percent of patients achieved final neurologic status within 1 week, 38% by 1 month, and 73% by 6 months. Patients with compression-related DPND had a significantly greater likelihood of experiencing some neurologic recovery (≥1 ASIA Grade) than ischemia-related DPND (86% versus 51%, P = 0.049)., Conclusions: DPND occurs at an estimated incidence of 0.01%. Sixty-three percent of DPND cases occurred within the first 24 hours and 90% within 48 hours. Complete (41%) or partial (26%) neurologic recovery may be expected, especially in compression-related DPND, emphasizing the need for perioperative vigilance, prompt recognition, and early intervention., Level of Evidence: 4.

Study Design: Retrospective cohort study using the Pediatric Health Information Systems database., Objective: To determine the association between antifibrinolytic use and red cell transfusions in spinal fusion operations performed at 37 US Children's Hospitals., Summary of Background Data: Evidence from randomized clinical trials and systematic reviews suggests that antifibrinolytic therapy can significantly reduce blood loss in children undergoing scoliosis surgery; however, the effectiveness of these agents as used in surgeries performed at US children's has not been studied., Materials and Methods: We included children aged 0-18 years with diagnoses indicating adolescent idiopathic scoliosis (AIS) or neuromuscular scoliosis (NMS) for whom a spinal fusion procedure was performed between January 1, 2006 and September 30, 2009. Patients with malignancy, trauma, coagulation disorders, or for whom a cell salvage device was employed were excluded. Multilevel logistic regression was used to determine associations between ε-aminocaproic acid (EACA), tranexamic acid (TXA), and aprotinin (APR) use and blood transfusions, controlling for patient and surgery characteristics., Results: Cohorts consisted of 2722 AIS and 1547 NMS procedures. Antifibrinolytic use varied across hospitals (AIS 3.3%, interquartile range, 0%-42%; NMS 12 interquartile range, 0%-46%), and was significantly associated with NMS, posterior fusion, number of vertebrae fused. Overall, 15% of children received EACA, 7% TXA, and 2% APR. The median hospital-specific rate of red cell transfusions was 24% for AIS and 43% for NMS. In AIS operations, EACA use, but not TXA use, was associated with significantly lower odds of transfusion (odds ratio, 0.42; P<0.001 vs. odds ratio, 1.0; P=0.8). In NMS operations, neither EACA nor TXA use was associated with a decrease in odds of red cell transfusions., Conclusions: The effectiveness of antifibrinolytics as used outside of clinical trials is unclear and should continue to be explored. Future prospective research is needed to evaluate which administration protocols will most benefit patients, as well as to determine the comparative effectiveness of these drugs in the context of other blood conservation strategies.

Background: Aicardi syndrome (ACS) is a rare neurodevelopmental disorder that was classically characterized by the triad of agenesis of corpus callosum, infantile spasms, and chorioretinal lacunae. As new cases emerge and new common phenotypic features are being described in subsequent reports, new modified diagnostic criteria have been proposed that now classify the observed costovertebral abnormalities as supporting diagnostic features. To our knowledge there are no previous studies focusing and describing the scoliosis observed in children with ACS., Methods: We screened billing lists to identify patients seen in the Division of Orthopaedic Surgery at our institution with a diagnosis of ACS that were treated for scoliosis after 2001. A total of 5 patients were identified. Medical records and radiographs were retrospectively reviewed in all cases. In all of the patients the diagnosis of ACS had been confirmed through complete genetic evaluation and advanced imaging., Results: The mean age when scoliosis was first noticed was 3.9±4.2 years (range, 0.5 to 10.5 y) with a mean Cobb angle of 22.5±6.7 degrees (range, 10 to 27 degrees). The mean age at the first orthopedic visit was 5.8±5.0 years (range, 1.5 to 13 y) with a progressed mean Cobb angle of 39.5±17.3 degrees (range, 15 to 57 degrees). Congenital vertebral anomalies were observed in 1 patient. Three patients were treated surgically; 1 of the 3 patients had a surgical complication with loss of intraoperative neuromonitoring signals. Two patients had not undergone surgery at the last visit with a mean Cobb angle of 75.5 degrees. The mean postoperative follow-up for the surgical group (cases 1 to 3) was 3±3.6 years (range, 0.6 to 7.2 y) and the mean total follow-up for both groups was 6.6±2.5 years (range, 2.6 to 8.6 y)., Conclusions: Scoliosis in ACS can represent a clinically significant problem that is underdiagnosed and overshadowed by the other severe medical complications associated with the syndrome. Our data suggest that scoliosis in patients with ACS is rapidly progressive and bracing is ineffective; early screening, close observation, and low threshold for referral to an orthopedic surgeon are crucial., Level of Evidence: Level IV-case series.

Background: Superior mesenteric artery (SMA) syndrome has been reported as an uncommon condition of external vascular compression of the SMA particularly after rapid weight loss, body casts, or after corrective surgery for spinal deformities, usually within the first few weeks after surgery., Methods: This is a retrospective report of a case of a non-verbal autistic female patient who started to develop SMA syndrome at the age of 16, 4 years after posterior spinal fusion surgery for scoliosis. She was treated conservatively by increasing oral caloric intake, which resulted in increased body weight and relief of symptoms., Results: Seen at 10 years' follow up, the patient is doing well, and is functional within the limits of her suboptimal cognitive and verbal conditions. She maintains good trunk balance with solid spinal fusion and intact instrumentation at latest follow up., Conclusion: Spinal surgeons should maintain a high index of suspicion for diagnosis of SMA syndrome even years after scoliosis surgery, especially for patients with communication problems, like the case we present here. Appropriate conservative measures can succeed in relieving the symptoms, increasing body weight, and preventing complications including the risk of death.

Background: The use of the conventional halo is accompanied by the possibility of serious complications, especially in the pediatric population. Complications could include penetration of pin into the skull, pin loosening, pin tract infection, cranial nerve palsies, and vest-related pressure sores. The noninvasive "pinless" halo was introduced in an attempt to mitigate these problems while retaining the effectiveness of the conventional halo. The purpose of this study is to determine the indications and complications related to pinless halo application., Methods: We retrospectively reviewed 61 patients, whose treatment included the use of a pinless halo device, presenting to our institution between 2004 and 2012., Results: There were 35 male and 26 female patients with an average age of 6.04 years. Indications of pinless halo application included postoperative immobilization for congenital muscular torticollis in 38 cases, conservative management of atlantoaxial rotatory subluxation in 11 cases, postoperative immobilization following cervical fusion in 10 cases, and immobilization for occipital condyle fracture in 2 cases. The average duration of the pinless halo application was 32.68 days. Thirteen patients had complications, among which major complications were seen in 2 patients, each of whom developed a pressure sore; one on the scalp and the other on the chest. Both the pressure sores responded to local treatment; however, 1 resulted in permanent alopecia., Conclusions: The use of the noninvasive pinless halo was found to be safe with few complications in our study. The complications were infrequent and patients were compliant to treatment, indicating that this modality is patient-friendly. Effectiveness of this treatment in comparison with invasive halos and other cervical orthoses was not determined and is a limitation of this study., Level of Evidence: Level IV-Case series.

Synovial chondromatosis is a benign disorder characterized by the formation of multiple cartilaginous nodules in the synovium of large joints. Synovial chondromatosis of the spine is very rare; this is the first reported case in the skeletally immature pediatric spine. A 12-year-old female presented with a posteriolateral neck mass and discomfort. A two-stage surgical resection of the mass was performed. This case shows that synovial chondromatosis can occur in the immature pediatric spine. Synovial chondromatosis should be included in the differential diagnosis of a calcified paravertebral or lateral neck mass in children.

Hereditary Multiple Exostoses (HME) is an autosomal-dominant disorder characterized by benign cartilage tumors (exostoses) forming near the growth plates, leading to severe health problems. EXT1 and EXT2 are the two genes known to harbor heterozygous loss-of-function mutations that account for the vast majority of the primary genetic component of HME. However, patients present with wide clinical heterogeneity, suggesting that modifier genes play a role in determining severity. Our previous work has pointed to an imbalance of β-catenin signaling being involved in the pathogenesis of osteochondroma formation. TCF7L2 is one of the key 'gate-keeper' TCF family members for Wnt/β-catenin signaling pathway, and TCF7L2 and EXT2 are among the earliest associated loci reported in genome wide appraisals of type 2 diabetes (T2D). Thus we investigated if the key T allele of single nucleotide polymorphism (SNP) rs7903146 within the TCF7L2 locus, which is strongly over-represented among T2D cases, was also associated with HME. We leveraged genotype data available from ongoing GWAS efforts from genomics and orthopedic centers in the US, Canada and Italy. Collectively 213 cases and 1890 controls were analyzed and, surprisingly, the T allele was in fact significantly under-represented in the HME patient group [P = 0.009; odds ratio = 0.737 (95% C.I. 0.587-0.926)]; in addition, the direction of effect was consistent within each individual cohort. Immunohistochemical analyses revealed that TCF7L2 is differentially expressed and distributed in normal human growth plate zones, and exhibits substantial variability in human exostoses in terms of staining intensity and distribution. In summary, the data indicate that there is a putative genetic connection between TCF7L2 and EXT in the context of HME. Given this observation, we suggest that these loci could possibly modulate shared pathways, in particular with respect to β-catenin, and their respective variants interplay to influence HME pathogenesis as well as T2D., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: Melorheostosis is a progressive hyperostotic bone disease that commonly affects the appendicular skeleton. Melorheostosis has a significant degree of overlap with other hyperostosis conditions including osteopoikilosis and likely represent varying degrees of a clinical spectrum., Methods: This is a report of 2 patients with melorheostosis who presented with different clinical presentations and involvement of different anatomic locations., Results: One of the patients presented with foot size asymmetry along with intermittent foot pain and limping. This patient also had irregular nonblanching yellow plaque-like lesion on the forehead. The second patient presented with abnormal gait but no pain. Radiographs demonstrated endosteal hyperostosis, which were consistent with a mixed picture of melorheostosis and osteopoikilois. Genetic testing of the LEMD3 gene from a blood sample was negative in both cases., Conclusions: The diagnosis of melorheostosis can be made based on the clinical and radiographic features and can be challenging to differentiate from other hyperostosis conditions., Level of Evidence: Level V.

Background: Postural orthostatic tachycardia syndrome (POTS) is the most common of several types of dysautonomia, characterized by dysfunction of the autonomic nervous system manifesting with symptoms of orthostatic intolerance with or without associated orthostatic hypotension and excessive autonomic excitation. Given the numerous presenting musculoskeletal symptoms of POTS and its known associations with other clinical entities like Ehlers-Danlos syndrome, POTS constitutes an unusual treatment challenge of which the orthopaedic surgeon and other related healthcare providers should be aware., Case Description: We describe two patients with a diagnosis of POTS and musculoskeletal manifestations. The first is a 13-year-old boy with a concurrent diagnosis of Ehlers-Danlos syndrome and worsening back pain. The patient had resolution of his musculoskeletal symptoms after treatment with a Boston Overlap LSO brace and physical therapy and resolution of his cardiovascular symptoms after pharmacologic treatment. The second patient is a 17-year-old girl with an L1 vertebral hemangioma and pain of sudden onset radiating to her lower extremities. Her symptoms were controlled with therapy and gabapentin., Literature Review: POTS is a poorly understood and controversial clinical entity with lack of awareness and knowledge regarding the syndrome by the majority of medical specialists. Its complex and wide range of clinical presentation has led to being commonly mistaken with malingering, depression and anxiety disorders. Review of the literature did not yield any previous studies addressing POTS and the special considerations for the orthopaedic surgeon caring for these patients; previous studies of large patient series focus on investigating the pathologic substrate of POTS and the efficacy of various treatment modalities on the cardiovascular dysregulation., Clinical Relevance: These two cases show that understanding and knowledge of this syndrome and its comorbidities should guide any diagnostic approach or therapeutic intervention by the orthopaedic surgeon caring for patients with this syndrome. It is important that the surgeon is aware of the benefits of detailed patient education and physical conditioning, the increased perioperative complications, and the need for specialized anesthesia.

Purpose: The management of proximal femoral deformity in fibrous dysplasia (FD) is a challenge to the orthopaedic surgeon. The purpose of this study was to analyze the various presentations of FD of proximal femur and the results of the various treatment modalities for the same., Methods: This is a retrospective cohort study of 23 patients (24 femora) with FD who underwent surgery for the proximal femur. The study sample included 14 males, nine females. Ten patients had a monostotic disease, eight patients had polyostotic disease, and five patients had McCune-Albright syndrome., Results: Group 1: shepherd crook deformity-included five patients who underwent femoral neck osteotomy. Four patients had intramedullary (IM) nailing with neck cross-pinning and all patients showed union. One patient was stabilized with external fixation, which failed. Group 2: nine patients (ten femora) presented with frank pathological fracture. Nine underwent fixation with IM nailing, one with locking plate and screws. Three patients had to undergo more than one procedure and all fractures showed good union. Group 3: nine patients who presented with bone cyst and pain. All patients underwent biopsy; four of them had curettage with bone graft., Conclusion: Shepherd crook deformity can be treated by a well-planned osteotomy and fixation with intramedullary implants with neck cross-pinning. Frank pathological fractures fixation with an intramedullary nail has excellent results even if not accompanied by resolution of the fibrodysplastic lesion. More than one procedure may be required. External fixation is not an optimal choice for fixation of femoral osteotomies in FD.

Study Design: Retrospective study., Objectives: To compare radiographic outcomes between primary thoracic and primary thoracolumbar/lumbar curves in patients with early-onset scoliosis (EOS) after growing rod (GR) surgery., Summary of Background Data: Previous studies have shown the efficacy of GR surgery for progressive EOS. However, there is no information on the behavior of different curve patterns in EOS after GR surgery., Methods: A multicenter international EOS database query identified 175 patients who met the following inclusion criteria: non-congenital etiology, GR surgery, ≤ 10 years of age at index surgery, minimum 2-year follow-up, and at least 3 lengthenings. Patients were categorized into 2 groups based on the Scoliosis Research Society definition of the anatomical location of primary curves: group 1 included thoracic apices (T2 to T11/12 disc) and group 2 included thoracolumbar (T12 to L1) and lumbar (L1/2 disc to L4) apices. Radiographic measurements were performed before and after index surgery and at latest follow-up., Results: A total of 139 patients (79%) had primary thoracic (group 1) and 36 (21%) had primary thoracolumbar or lumbar curves (group 2). Mean number of levels instrumented was statistically greater in group 2 (15.0) versus group 1 (13.6) (p < .05). Group 2 had statistically better mean curve correction than group 1 after the index GR surgery (51% and 44%, respectively; p < .05). However, there was no significant difference in mean percent curve correction at latest follow-up (46% and 39%, respectively; p > .05). Implant complication rate was 45% and 47% for groups 1 and 2, respectively. Preoperative curve flexibility was greater in group 2 (45%) compared with group 1 (40%) (p > .05)., Conclusions: Overall, thoracolumbar/lumbar and thoracic curves achieve similar major curve correction and have a similar complication profile., (Copyright © 2014 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

Background: Cervical spondylolysis with spondylolisthesis is a rare condition in the pediatric population. The nature of this condition and clinical presentation are important to provide appropriate management., Methods: This is a case report of 3 adolescent siblings who had C6 cervical spondylolysis with spondylolisthesis., Results: Two cases were diagnosed insidiously with absence of neurological deficits and no instability on imaging and were treated conservatively. One case had a traumatic presentation associated with instability on imaging and was managed with cervical fusion and instrumentation. All 3 patients were doing well on a follow-up of >2 years., Conclusions: These cases suggest that cervical spondylolysis could be familial. The treatment was offered based on clinical presentation and presence of instability on radiographic studies., Level of Evidence: Level IV.

Background: Chest wall osteochondroma is a rare tumor in children. Even though the potential for malignant transformation or serious intrathoracic complications is low, it has led some centers to advocate surgical management of these bony tumors. We present our experience of the surgical management of costal osteochondromata., Methods: Between January 1, 2006 and November 1, 2012 we saw 854 patients with solitary or multiple exostoses in our clinics. By reviewing our billing lists we found 7 children who had surgical management of chest wall osteochondromata. The indications for surgery were pain (3 patients), excision for confirmation of diagnosis (2 patients), recurrent pneumothorax (1 patient), and malignancy (1 patient)., Results: All patients made a good postoperative recovery with a median hospital stay of 1.8 days (range, 0 to 4 d). There was no recurrence of exostosis on follow-up (range, 8 mo to 2.6 y). One patient required surgery for excision of another chest wall osteochondroma at an adjacent location. No patient reported scar-related pain symptoms. No malignant transformation or intrathoracic complications occurred. We found ribs as the first site of presentation of multiple hereditary exostoses in 2 young patients., Conclusions: Surgical management of thoracic osteochondroma, with excision for painful, symptomatic, malignant lesions or lesions adjudged to be at risk of intrathoracic complications, yields good outcomes in terms of symptom control, establishing histologic diagnosis, and prevention of thoracic complications., Level of Evidence: Level IV-case series.

Study Design: Consensus-based creation of a checklist and guideline., Objective: To develop a consensus-based checklist to guide surgeon responses to intraoperative neuromonitoring (IONM) changes in patients with a stable spine and to develop a consensus-based best practice guideline for IONM practice in the United States., Summary of Background Data: Studies show that checklists enhance surgical team responses to crisis situations and improve patient outcomes. Currently, no widely accepted guidelines exist for the response to IONM changes in spine deformity surgery., Methods: After a literature review of risk factors and recommendations for responding to IONM changes, 4 surveys were administered to 21 experienced spine surgeons and 1 neurologist experienced in IONM. Areas of equipoise were identified and the nominal group process was used to determine items to be included in the checklist. The authors reevaluated and modified the checklist at 3 face-to-face meetings over 12 months, including a period of clinical validation using a modified Delphi process. The group was also surveyed on current IONM practices at their institutions. This information and existing IONM position statements were used to create the IONM best practice guideline., Results: Consensus was reached for the creation of 5 checklist headings containing 26 items to consider in the response to IONM changes. Consensus was reached on 5 statements for inclusion in the best practice guideline; the final guideline promotes a team approach and makes recommendations aimed at decreasing variability in neuromonitoring practices., Conclusions: The final products represent the consensus of a group of expert spine surgeons. The checklist includes the most important and high-yield items to consider when responding to IONM changes in patients with a stable spine, whereas the IONM guideline represents the group consensus on items that should be considered best practice among IONM teams with the appropriate resources., (Copyright © 2014 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

We report six new cases of lipofibromatosis, an uncommon pediatric soft tissue neoplasm. This is the only series of patients to be described since the initial case series of 45 patients that characterized this entity in 2000. The purpose of this study was to characterize the presentation of lipofibromatosis to further define the clinical phenotype of this rare entity. Six patients were diagnosed with lipofibromatosis at our institution from 2000 to 2012. Patient age, sex, and ethnicity were recorded, along with tumor site and size, management, and recurrence data. Half of our patients were younger than 2 years old at presentation and the other half were school age. Boys and girls were affected with equal frequency. In five of six patients, lipofibromatosis presented in its "classic" form as a mass on the distal extremities. These tumors typically measured 1 to 2 cm in diameter, in contrast to case reports in the medical literature highlighting the occurrence of lipofibromatosis of greater size and at varied anatomic sites. The tumors in our series were managed using excision, with recurrence noted in 33%. Lipofibromatosis is an uncommon tumor typically found on the distal extremities of infants, although it can appear in various sizes and locations. It should be considered in the differential diagnosis of pediatric soft tissue neoplasms., (© 2014 Wiley Periodicals, Inc.)

Unicameral bone cysts are benign bone lesions that are often asymptomatic and commonly develop in the proximal humerus and femur of skeletally immature patients. The etiology of these lesions remains unknown. Most patients present with a pathologic fracture, but these cysts can be discovered incidentally, as well. Radiographically, a unicameral bone cyst appears as a radiolucent lesion with cortical thinning and is centrally located within the metaphysis. Although diagnosis is frequently straightforward, management remains controversial. Because the results of various management methods are heterogeneous, no single method has emerged as the standard of care. New minimally invasive techniques involve cyst decompression with bone grafting and instrumentation. These techniques have yielded promising results, with low rates of complications and recurrence reported; however, prospective clinical trials are needed to compare these techniques with current evidence-based treatments.

Background: Interest in developing national health care has been increasing in many fields of medicine, including orthopaedics. One manifestation of this interest has been the development of global health opportunities during residency training., Questions/purposes: We assessed global health activities and opportunities in orthopaedic residency in terms of resident involvement, program characteristics, sources of funding and support, partner site relationships and geography, and program director opinions on global health participation and the associated barriers., Methods: An anonymous 24-question survey was circulated to all US orthopaedic surgery residency program directors (n = 153) by email. Five reminder emails were distributed over the next 7 weeks. A total of 59% (n = 90) program directors responded., Results: Sixty-one percent of responding orthopaedic residencies facilitated clinical experiences in developing countries. Program characteristics varied, but most used clinical rotation or elective time for travel (76%), which most frequently occurred during Postgraduate Year 4 (57%) and was used to provide pediatric (66%) or trauma (60%) care. The majority of programs (59%) provided at least some funding to traveling residents and sent accompanying attendings on all ventures (56%). Travel was most commonly within North America (85%), and 51% of participating programs have established international partner sites although only 11% have hosted surgeons from those partnerships. Sixty-nine percent of residency directors believed global health experiences during residency shape future volunteer efforts, 39% believed such opportunities help attract residents to a training program, and the major perceived challenges were funding (73%), faculty time (53%), and logistical planning (43%)., Conclusions: Global health interest and activity are common among orthopaedic residency programs. There is diversity in the characteristics and geographical locations of such activity, although some consensus does exist among program directors around funding and faculty time as the largest challenges.

Heparan sulfate (HS) is an essential component of cell surface and matrix-associated proteoglycans. Due to their sulfation patterns, the HS chains interact with numerous signaling proteins and regulate their distribution and activity on target cells. Many of these proteins, including bone morphogenetic protein family members, are expressed in the growth plate of developing skeletal elements, and several skeletal phenotypes are caused by mutations in those proteins as well as in HS-synthesizing and modifying enzymes. The disease we discuss here is hereditary multiple exostoses (HME), a disorder caused by mutations in HS synthesizing enzymes EXT1 and EXT2, leading to HS deficiency. The exostoses are benign cartilaginous-bony outgrowths, form next to growth plates, can cause growth retardation and deformities, chronic pain and impaired motion, and progress to malignancy in 2-5% of patients. We describe recent advancements on HME pathogenesis and exostosis formation deriving from studies that have determined distribution, activities and roles of signaling proteins in wild-type and HS-deficient cells and tissues. Aberrant distribution of signaling factors combined with aberrant responsiveness of target cells to those same factors appear to be a major culprit in exostosis formation. Insights from these studies suggest plausible and cogent ideas about how HME could be treated in the future., (Copyright © 2013 Wiley Periodicals, Inc.)

Study Design: Therapeutic level II cohort study., Objective: To evaluate the safety of adjunctive local application of vancomycin powder (VP) for infection prophylaxis in posterior instrumented thoracic and lumbar spine wounds in pediatric patients weighing more than 25 kg., Summary of Background Data: Spine surgeons have largely turned to vancomycin prophylaxis in an attempt to decrease the incidence of late surgical site infection and acute surgical site infection from methicillin-resistant Staphylococcus aureus. In adult patients, the adjunctive local application of VP with an intravenous cephalosporin has been shown to decrease postsurgical wound infection rates significantly; however, the safety of VP as an adjunct in pediatric spine surgery has not been reported., Methods: We reviewed data collected under a systematic protocol specifically designed to monitor the safety profile of VP. We measured changes in creatinine and systemic vancomycin levels after intrawound application of 500 mg of unreconstituted VP during spine deformity correction surgery in patients weighing more than 25 kg (patients also received routine intravenous cephalosporin prophylaxis). Laboratory values were measured preoperatively and on postoperative days 1 and 4. Any adverse reactions and infections through available follow-up (2-8 mo) were recorded., Results: Eighty-seven consecutive pediatric patients with spinal deformity weighing more than 25 kg who received intraoperative VP during a 9-month period were identified. Sixty-three percent of the patients in this series had adolescent idiopathic scoliosis, 15% congenital scoliosis, 15% neuromuscular scoliosis, and 5% spondylolisthesis. The average change in creatinine levels between the preoperative and postoperative day 1 draw was -0.03 and between the preoperative and postoperative day 4 draw was -0.075. The postoperative systemic vancomycin levels remained undetectable. None of the patients experienced nephrotoxicity or red man syndrome. Three of the 87 patients developed a surgical site infection., Conclusion: In this cohort there were no clinically significant changes in creatinine level or systemic vancomycin level caused by use of intraoperative VP., Level of Evidence: 2.

Vascular malformations are abnormal proliferations of the endothelial lining of the blood vessels that are most commonly found in skin and can penetrate the local soft tissue and muscle. Only 1% of all vascular malformations are found in the bone. Skeletal vascular malformations of the long bones, including the humerus, are extremely rare entities that are particularly uncommon in children. Only 4 reports involving the humerus have been described in children previously. We present the case of an intraosseous capillary vascular malformation of the right distal humerus in a 3-year-old boy. Definitive treatment was successful with a single operation using curettage and intramedullary decompression with grafting of an osteoconductive calcium sulfate pellet filler and autogenous bone graft. This is, to our knowledge, the youngest reported case of a solitary intraosseous capillary vascular malformation involving the distal humerus.

Chondroblastoma and chondromyxoid fibroma are benign but locally aggressive bone tumors. Chondroblastoma, a destructive lesion with a thin radiodense border, is usually seen in the epiphysis of long bones. Chondromyxoid fibroma presents as a bigger, lucent, loculated lesion with a sharp sclerotic margin in the metaphysis of long bones. Although uncommon, these tumors can be challenging to manage. They share similarities in pathology that could be related to their histogenic similarity. Very rarely, chondroblastoma may lead to lung metastases; however, the mechanism is not well understood.

Study Design: Retrospective cohort study using the Pediatric Health Information System database., Objective: To describe longitudinal patterns of prophylactic antibiotic use and determinants of antibiotic choice for spinal fusion surgical procedures performed at US children's hospitals., Summary of Background Data: Surgical site infections (SSIs) account for a significant proportion of post-spinal surgery complications, particularly among children with complex conditions such as neuromuscular disease. Antimicrobial prophylaxis with intravenous cefazolin or cefuroxime has been a standard practice, but postoperative infections caused by organisms resistant to these antibiotics are increasing in prevalence. Studies describing the choice of antibiotic prophylaxis for pediatric spinal surgery are lacking., Methods: We included children 6 months to 18 years of age discharged from 37 US children's hospitals between January 1, 2006, and June 30, 2009, with (1) an International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) procedure code indicating a spinal fusion and (2) combinations of diagnosis codes indicating adolescent idiopathic scoliosis (AIS) (n = 5617) or neuromuscular scoliosis (NMS) (n = 3633). After identifying antibiotics ordered on the operative day, we described variation in broad-spectrum antibiotic use over time and measured associations between patient/surgery characteristics and antibiotic choice., Results: Prophylactic antibiotic choice varied across hospitals and over time. Broad-spectrum antibiotics were used in 37% of AIS and 52% of NMS operations. Seven (19%) hospitals used broad-spectrum coverage for more 80% of all cases. For NMS procedures, broad-spectrum antibiotic use was associated with patient characteristics known to be associated with high SSI risk. Use of vancomycin and broad gram-negative agents increased over time., Conclusion: Broad-spectrum antimicrobial prophylaxis varied across hospitals and was often associated with known risk factors for SSI. These results highlight the need for future studies comparing the effectiveness of various prophylaxis strategies, particularly in high-risk subgroups. This research can inform the development of best practice for SSI prevention in spinal fusion procedures.

Osteochondromas and enchondromas are the most common tumors affecting the skeleton. Osteochondromas can occur as multiple lesions, such as those in patients with hereditary multiple exostoses. Unexpectedly, while studying the role of β-catenin in cartilage development, we found that its conditional deletion induces ectopic chondroma-like cartilage formation in mice. Postnatal ablation of β-catenin in cartilage induced lateral outgrowth of the growth plate within 2 weeks after ablation. The chondroma-like masses were present in the flanking periosteum by 5 weeks and persisted for more than 6 months after β-catenin ablation. These long-lasting ectopic masses rarely contained apoptotic cells. In good correlation, transplants of β-catenin-deficient chondrocytes into athymic mice persisted for a longer period of time and resisted replacement by bone compared to control wild-type chondrocytes. In contrast, a β-catenin signaling stimulator increased cell death in control chondrocytes. Immunohistochemical analysis revealed that the amount of detectable β-catenin in cartilage cells of osteochondromas obtained from hereditary multiple exostoses patients was much lower than that in hypertrophic chondrocytes in normal human growth plates. The findings in our study indicate that loss of β-catenin expression in chondrocytes induces periosteal chondroma-like masses and may be linked to, and cause, the persistence of cartilage caps in osteochondromas., (Copyright © 2013 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.)

Community-acquired methicillin-resistant Staphylococcus aureus (MRSA) has been recognized as a public health concern since the mid-1990s. Because of the increase in reports of this pathogen, it has become increasingly tempting for clinicians to provide prophylaxis against this entity using antibiotics known to be effective against MRSA. The goal of this study was to assess the use of MRSA prophylaxis to determine whether it is safe and effective. A systematic search of the literature was performed to identify articles that examined the use of vancomycin in clean orthopedic surgery. Infection rates and adverse events were extracted, and the data were aggregated and analyzed using a DerSimonian and Laird random effects model. Publication bias and study quality were also assessed. No benefit of parenteral administration of vancomycin was identified. Local, vancomycin-impregnated cement and powder are associated with lower infection rates. Few adverse events occurred, and most of those that occurred involved infusion rate.Cost, resistance, and side effects are concerns in using vancomycin therapy in addition to standard antibiotic prophylaxis. Given the lack of efficacy of intravenous vancomycin, the authors do not recommend its routine use in clean orthopedic surgery. However, local administration appears to be safe and effective. The data are most compelling in orthopedic spine surgery in which a patient without prophylaxis is more than 4 times as likely to have a deep postoperative wound infection compared with a patient who received local vancomycin. The authors recommend the use of local antibiotics when possible in clean orthopedic surgery., (Copyright 2013, SLACK Incorporated.)

Purpose: Chemotherapy with alternating vincristine-doxorubicin-cyclophosphamide and ifosfamide-etoposide cycles and primary tumor treatment with surgery and/or radiation therapy constitute the usual approach to localized Ewing sarcoma in North America. We tested whether chemotherapy intensification through interval compression could improve outcome., Patients and Methods: This was a prospective, randomized controlled trial for patients younger than 50 years old with newly diagnosed localized extradural Ewing sarcoma. Patients assigned to standard and intensified treatment were to begin chemotherapy cycles every 21 and 14 days, respectively, provided an absolute neutrophil count greater than 750×10(6)/L and a platelet count greater than 75×10(9)/L. Patients received vincristine (2 mg/m2), doxorubicin (75 mg/m2), and cyclophosphamide (1.2 g/m2) alternating with ifosfamide (9 g/m2) and etoposide (500 mg/m2) for 14 cycles, with filgrastim (5 mg/kg per day; maximum, 300 mg) between cycles. Primary tumor treatment (surgery, radiation, or both) was to begin at week 13 (after four cycles in the standard arm and six cycles in the intensified arm). The primary end point was event-free survival (EFS). The study is registered at ClinicalTrials.gov (identifier: NCT00006734)., Results: Five hundred eighty-seven patients were enrolled and randomly assigned, and 568 patients were eligible, with 284 patients in each regimen. For all cycles, the median cycle interval for standard treatment was 21 days (mean, 22.45 days); for intensified treatment, the median interval was 15 days (mean, 17.29 days). EFS at a median of 5 years was 65% in the standard arm and 73% in the intensified arm (P=.048). The toxicity of the regimens was similar., Conclusion: For localized Ewing sarcoma, chemotherapy administered every 2 weeks is more effective than chemotherapy administered every 3 weeks, with no increase in toxicity.

Purpose: Limb salvage in bone sarcomas requires resection and joint and soft-tissue reconstruction. Suboptimal coverage can lead to prosthesis infection, subsequent hardware exposure, or loss with eventual amputation. In the pediatric sarcoma population, it is essential to not only have viable soft-tissue coverage but one that minimizes donor site morbidity and maximizes function and growth over time., Methods: This is a retrospective review of all sarcoma patients with primary gastrocnemius and soleus muscle flap coverage of modular knee endoprosthesis over the 10-year period between 1997 and 2007. All patients had resection of the skin at the tibial biopsy site prior to their primary tumor resection surgery. After resection of the knee joint and proximal tibia with clear margins, the bony defect was reconstructed with an endoprosthesis. The gastrocnemius and soleus muscles were then independently rotated to cover the prosthesis and the patellar tendon., Results: Eleven patients (10 male, 1 female) with an average age of 13 years (range, 10-17) underwent resection of proximal tibia. Pathology included osteosarcoma in 9 patients, Ewing in 1, and spindle cell sarcoma in 1 patient. All patients had endoprosthetic coverage by gastrocnemius and soleus flaps; 4 patients had additional split-thickness skin graft over the gastrocnemius. The average level of tibial resection was 16.5 cm distal to the articular surface of the proximal tibia. Mean soft-tissue mass volume resected with bone was 157 cm. There was one flap failure that was reconstructed with a rectus abdominis free flap. Average follow-up was 32 months (range, 8-92 months). Two patients succumbed to metastatic disease. In this series, there was no limb loss or hardware exposure or infection. All patients achieved independent ambulation. Functionally, patients were evaluated based on the Musculoskeletal Tumor Society scale. The average score was 29 (out of a perfect score of 30). Two patients had transient foot drop that resolved with ankle foot orthosis use. Anticipated leg length discrepancy was detected in 3 patients who underwent opposite limb epiphysiodesis with ultimate limb equalization., Conclusion: Soft-tissue reconstruction with gastrocnemius and soleus muscle flap coverage decreases wound complications and hardware exposure while maintaining function in the pediatric population.

Pathological fractures in children can occur as a result of a variety of conditions, ranging from metabolic diseases and infection to tumours. Fractures through benign and malignant bone tumours should be recognised and managed appropriately by the treating orthopaedic surgeon. The most common benign bone tumours that cause pathological fractures in children are unicameral bone cysts, aneurysmal bone cysts, non-ossifying fibromas and fibrous dysplasia. Although pathological fractures through a primary bone malignancy are rare, these should be recognised quickly in order to achieve better outcomes. A thorough history, physical examination and review of plain radiographs are crucial to determine the cause and guide treatment. In most benign cases the fracture will heal and the lesion can be addressed at the time of the fracture, or after the fracture is healed. A step-wise and multidisciplinary approach is necessary in caring for paediatric patients with malignancies. Pathological fractures do not have to be treated by amputation; these fractures can heal and limb salvage can be performed when indicated.

Quantity of citation is often used as a surrogate measurement of an article's importance or relevance in a given field. To date, there has been no study on citation quantity or density in pediatric orthopedics. We present such an analysis. We reviewed all articles in the ISI web of science under the subheading 'orthopedics' to find articles of relevance to pediatric orthopedic surgeons. We subsequently analyzed the characteristics of the most cited articles in terms of the level of evidence, journal of publication, and subcategory of pediatric orthopedics. The majority of the top 50 articles in pediatric orthopedics were from the 1970s and 1980s. All the articles were in English. Uncontrolled case series comprised the largest single level of evidence (level IV). We describe the top 50 articles in pediatric orthopedics by citation and citation density. The level of evidence for highly cited papers was low. Although many of these articles were written by the great thinkers of our field, a need exists for more rigorous methodology.

Background: Intrathecal morphine (ITM) provides effective analgesia after posterior spinal fusion (PSF). Although most anesthetic drugs have well-characterized effects on evoked potentials, there is little data on the effects of ITM on transcranial electric motor-evoked potentials (tceMEPs). We performed this study to assess the effects of ITM on tceMEPs in the first 30 minutes after administration. We hypothesized that administration of ITM in doses currently used at our institution would not significantly affect mean tceMEP amplitudes and latencies of an ITM study group relative to control patients who did not receive the drug., Methods: tceMEPs were recorded before ITM injection and 5, 10, 20, and 30 minutes after injection in 14 subjects ages 11 through 18 years undergoing PSF. These recordings were compared to an age-matched control group undergoing PSF in which ITM was not injected. The effects of ITM on tceMEP amplitude and latency were compared between the 2 groups., Results: Fourteen subjects were enrolled in the ITM group and 16 served as controls. There were no significant differences in the baseline mean response amplitudes of the 2 groups for any of the 8 muscles studied. Mean response amplitudes over the 30-minute posttreatment period in the ITM group did not differ significantly from those of the control subjects. Average response amplitudes collapsed across all muscles for each subject were not significantly different during the baseline period (95% CI = -38% to 45%; P = 0.783), nor were they significantly different between the 2 groups during the posttreatment period (95% CI = -30% to 78%; P = 0.640). There also were no significant differences in the mean response latencies of the 2 groups in either the baseline or posttreatment periods. Average response latencies collapsed across all muscles for each subject were 4% larger for the ITM group than for controls during the baseline period (95% CI = -5% to 13%; P = 0.377), and 3% larger for the ITM group than for controls during the posttreatment period (95% CI = -4% to 12%; P = 0.359)., Conclusions: Administration of ITM in doses currently used at our institution did not cause more than a 70% attenuation of mean tceMEP amplitudes or latency changes of an ITM study group relative to control subjects during the 30-minute period after injection. Further studies are required to determine if there are delayed effects after this initial time period.

Study Design: A multicenter retrospective analysis., Objective: To evaluate outcomes of closed-suction wound drainage after posterior spinal fusion with instrumentation for adolescent idiopathic scoliosis and to identify surgeon patterns of drain use in this cohort., Summary of Background Data: There is little evidence on the use of drains in spinal surgery, particularly for repair of adolescent idiopathic scoliosis. Studies on hip and knee arthroplasty suggest no advantage to draining. There are few published reports on surgeon technique and rationale for drain use in spinal surgery., Methods: Patients were divided into drain and no drain cohorts and followed for 2 years. Primary outcome was complication rate. A separate survey was conducted from surgeons in the Spinal Deformity Study Group to evaluate drain practice patterns., Results: There were 324 drained and 176 undrained patients. Complication rate did not differ between the drain and no drain cohorts in any of the 4 categories (wound infection, neural injury, other infection, and other complication) at any time (all P > 0.1). More drained patients received postoperative transfusions compared with those without a drain (43% vs. 22%, P < 0.001). Of the 50 surgeons in the group, 36 used drains. Half of these did so out of habit. Surgeons tended to place deep drains with bulb suction, without drain manipulation. Half removed drains on the basis of output, whereas half removed them after 1 to 3 days., Conclusion: More patients tended to receive wound drains than not receive wound drains. Drains did not impact complication rate and drained patients received more blood product. There are no universal criteria for draining and practice patterns vary widely.