Radiation exposure in multiple hereditary exostoses: A retrospective review.

Background: Exposure to ionizing radiation in patients with Multiple Hereditary Exostoses (MHE) is inevitable and necessary for the diagnosis and treatment of MHE. Radiation exposure has many potentially dangerous consequences, including the increased risk of developing cancer. This is especially concerning in the pediatric patient population since children are more likely to develop adverse effects from radiation than adults. This study aimed to quantify radiation exposure over a five-year period among patients diagnosed with MHE since such information is not currently available in the literature. Methods: Diagnostic radiographs, computed tomography (CT) scans, nuclear medicine studies, and intraoperative fluoroscopy exposures were analyzed for radiation exposure in 37 patients diagnosed with MHE between 2015 and 2020. Results: Thirty-seven patients with MHE underwent 1200 imaging studies, 976 of which were related to MHE and 224 unrelated to MHE. The mean estimated MHE cumulative radiation dose per patient was 5.23 mSv. Radiographs related to MHE contributed the most radiation. Patients from the ages of 10- to 24-years-old received the most imaging studies and exposure to ionizing radiation, especially compared to those under age 10 (P = 0.016). The 37 patients also received a total of 53 surgical-excision procedures, with a mean of 1.4 procedures per person. Conclusions: MHE patients are exposed to increased levels of ionizing radiation secondary to serial diagnostic imaging, with those ages 10-24 years old being exposed to significantly higher doses of radiation. Because pediatric patients are more sensitive to radiation exposure and are at an overall higher risk, the use of radiographs should always be justified in those patients.

Perioperative multimodal analgesic injection for patients with adolescent idiopathic scoliosis undergoing posterior spinal fusion surgery.

PURPOSE: This retrospective cohort study compared postoperative as-needed (PRN) opioid consumption pre and postimplementation of a perioperative multimodal analgesic injection composed of ropivacaine, epinephrine, ketorolac, and morphine in patients undergoing posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS). Secondary outcomes include pain score measurements, time to ambulation, length of stay, blood loss, 90-day complication rate, operating room time, nonopioid medication usage, and total inpatient medication cost before and after the initiation of this practice. METHODS: Consecutive patients weighing ≥ 20 kg who underwent PSF for a primary diagnosis of AIS between January 2017 and December 2020 were included. Data from 2018 were excluded to account for standardization of the practice. Patients treated in 2017 only received PCA. Patients treated in 2019 and 2020 only received the injection. Excluded were patients who had any diagnoses other than AIS, allergies to any of the experimental medications, or who were nonambulatory. Data were analyzed utilizing the two-sample t-test or Chi-squared test as appropriate. RESULTS: Results of this study show that compared with 47 patients treated postoperatively with patient-controlled analgesia (PCA), 55 patients treated with a multimodal perioperative injection have significantly less consumption of PRN morphine equivalents (0.3 mEq/kg vs. 0.5 mEq/kg; p = 0.02). Furthermore, patients treated with a perioperative injection have significantly higher rates of ambulation on postoperative day 1 compared with those treated with PCA (70.9 vs. 40.4%; p = 0.0023). CONCLUSION: Administration of a perioperative injection is effective and should be considered in the perioperative protocol in patients undergoing PSF for AIS. LEVEL OF EVIDENCE: Therapeutic Level III.

Interobserver and Intraobserver Agreement are Unsatisfactory When Determining Abstract Study Design and Level of Evidence.

BACKGROUND: Understanding differences between types of study design (SD) and level of evidence (LOE) are important when selecting research for presentation or publication and determining its potential clinical impact. The purpose of this study was to evaluate interobserver and intraobserver reliability when assigning LOE and SD as well as quantify the impact of a commonly used reference aid on these assessments. METHODS: Thirty-six accepted abstracts from the Pediatric Orthopaedic Society of North America (POSNA) 2021 annual meeting were selected for this study. Thirteen reviewers from the POSNA Evidence-Based Practice Committee were asked to determine LOE and SD for each abstract, first without any assistance or resources. Four weeks later, abstracts were reviewed again with the guidance of the Journal of Bone and Joint Surgery (JBJS) LOE chart, which is adapted from the Oxford Centre for Evidence-Based Medicine. Interobserver and intraobserver reliability were calculated using Fleiss' kappa statistic (k). χ2 analysis was used to compare the rate of SD-LOE mismatch between the first and second round of reviews. RESULTS: Interobserver reliability for LOE improved slightly from fair (k=0.28) to moderate (k=0.43) with use of the JBJS chart. There was better agreement with increasing LOE, with the most frequent disagreement between levels 3 and 4. Interobserver reliability for SD was fair for both rounds 1 (k=0.29) and 2 (k=0.37). Similar to LOE, there was better agreement with stronger SD. Intraobserver reliability was widely variable for both LOE and SD (k=0.10 to 0.92 for both). When matching a selected SD to its associated LOE, the overall rate of correct concordance was 82% in round 1 and 92% in round 2 (P<0.001). CONCLUSION: Interobserver reliability for LOE and SD was fair to moderate at best, even among experienced reviewers. Use of the JBJS/Oxford chart mildly improved agreement on LOE and resulted in less SD-LOE mismatch, but did not affect agreement on SD. LEVEL OF EVIDENCE: Level II.

Patient similarity network of newly diagnosed multiple myeloma identifies patient subgroups with distinct genetic features and clinical implications.

The remarkable genetic heterogeneity of multiple myeloma poses a substantial challenge for proper prognostication and clinical management of patients. Here, we introduce MM-PSN, the first multiomics patient similarity network of myeloma. MM-PSN enabled accurate dissection of the genetic and molecular landscape of the disease and determined 12 distinct subgroups defined by five data types generated from genomic and transcriptomic profiling of 655 patients. MM-PSN identified patient subgroups not previously described defined by specific patterns of alterations, enriched for specific gene vulnerabilities, and associated with potential therapeutic options. Our analysis revealed that co-occurrence of t(4;14) and 1q gain identified patients at significantly higher risk of relapse and shorter survival as compared to t(4;14) as a single lesion. Furthermore, our results show that 1q gain is the most important single lesion conferring high risk of relapse and that it can improve on the current International Staging Systems (ISS and R-ISS).

Radiation Reduction Strategies in Pediatric Orthopaedics.

INTRODUCTION: Pediatric orthopaedic patients have the potential for significant radiation exposure from the use of imaging studies, such as computed tomography and bone scintigraphy. With the potential for long-term treatment, such as is required for scoliosis or osteogenesis imperfecta, patients are at even greater risk of radiation-induced carcinogenesis. DISCUSSION: Although an association between radiation and cancer risk is evident, causation is difficult to prove because comorbidities or genetic predispositions may play a role in the higher baseline rates of malignancy later in life. Efforts have been made over the years to reduce exposure using more modern imaging techniques and simple radiation reduction strategies. Educational efforts and clinical practice guidelines are decreasing the rate of computed tomography scan use in pediatrics. Although considerable work is being done on the development of radiation-free imaging modalities, imaging that uses ionizing radiation will, in the near term, be necessary in specific circumstances to provide optimal care to pediatric orthopaedic patients. CONCLUSION: Knowledge of the ionizing radiation exposure associated with commonly used tests as well as radiation-reduction strategies is essential for the optimal and safe care of pediatric orthopaedic patients.

Can Pediatric Orthopaedic Surgery be Done Safely in a Freestanding Ambulatory Surgery Center? Review of 3780 Cases.

BACKGROUND: The purpose of this study was to determine the intraoperative and 30-day postoperative complication rates in a large consecutive cohort of pediatric patients who had orthopaedic surgery at a freestanding ambulatory surgery center (ASC). The authors also wanted to identify the rates of same-day, urgent hospital transfers, and 30-day hospital admissions. The authors hypothesized that pediatric orthopaedic procedures at a freestanding ASC can be done safely with a low rate of complications. METHODS: A retrospective review identified patients aged 17 years or younger who had surgery at a freestanding ASC over a 9-year period. Adverse outcomes were divided into intraoperative complications, postoperative complications, need for the secondary procedure, unexpected hospital admission on the same day of the procedure, and unexpected hospital admission within 30 days of the index procedure. Complications were graded as grade 1, the complication could be treated without additional surgery or hospitalization; grade 2, the complication resulted in an unplanned return to the operating room (OR) or hospital admission; or grade 3, the complication resulted in an unplanned return to the OR or hospitalization with a change in the overall treatment plan. RESULTS: Adequate follow-up was available for 3780 (86.1%) surgical procedures. Overall, there were 9 (0.24%) intraoperative complications, 2 (0.08%) urgent hospital transfers, 114 (3%) complications, and 16 (0.42%) readmissions. Seven of the 9 intraoperative complications resolved before leaving the OR, and 2 required return to the OR.Neither complications nor hospitalizations correlated with age, race, gender, or length or type of surgery. There was no correlation between the presence of medical comorbidities, body mass index, or American Society of Anesthesiologists score and complication or hospitalization. CONCLUSIONS: Pediatric orthopaedic surgical procedures can be performed safely in an ASC because of multiple factors that include dedicated surgical teams, single-purpose ORs, and strict preoperative screening criteria. The rates of an emergency hospital transfer, surgical complications, and 30-day readmission, even by stringent criteria, are lower than those reported for outpatient procedures performed in the hospital setting. LEVEL OF EVIDENCE: Level IV-case series.

Hunger Games: Impact of Fasting Guidelines for Orthopedic Procedural Sedation in the Pediatric Emergency Department.

BACKGROUND: Fasting guidelines for pediatric procedural sedation have historically been controversial. Recent literature suggests that there is no difference in adverse events regardless of fasting status. OBJECTIVES: The goal of this study was to examine adverse outcomes and departmental efficiency when fasting guidelines are not considered during pediatric emergency department (PED) sedation for orthopedic interventions. METHODS: Retrospective chart review identified 2674 patients who presented to a level I PED and required procedural sedation for orthopedic injuries between February 2011 and July 2018. This was a level III, retrospective cohort study. Patients were categorized into the following groups: already within American Society of Anesthesiologists (ASA) fasting guidelines on presentation to the PED (n = 671 [25%]), had procedural sedation not within the ASA guidelines (n = 555 [21%]), and had procedural sedation after fasting in the PED to meet ASA guidelines (n = 1448 [54%]). Primary outcomes were length of stay, time from admission to start of sedation, length of sedation, time from end of sedation to discharge, and adverse events. DISCUSSION: There was a significant difference in the length of stay and time from admission to sedation-both approximately 80 min longer in those with procedural sedation after fasting in the PED to meet ASA guidelines (p < 0.001). There was no significant difference among groups in length of sedation or time to discharge after sedation. Adverse events were uncommon, with only 55 total adverse events (0.02%). Vomiting during the recovery phase was the most common (n = 17 [0.006%]). Other notable adverse events included nine hypoxic events (0.003%) and five seizures (0.002%). There was no significant difference in adverse events among the groups. CONCLUSIONS: Length of stay in the PED was significantly longer if ASA fasting guidelines were followed for children requiring sedation for orthopedic procedures. This is a substantial delay in a busy PED where beds and resources are at a premium. Although providing similar care with equivalent outcomes, the value of spending less time in the PED is evident. Overall, adverse events related to sedation are rare and not related to fasting guidelines.

Minimum 5-Year Follow-up on Graduates of Growing Spine Surgery for Early Onset Scoliosis.

INTRODUCTION: After discontinuation of growth friendly (GF) surgery for early onset scoliosis, patients undergo spinal fusion or continued observation. This last planned treatment is colloquially called "definitive" treatment, conferring these patients as "graduates" of a growing program. The 5-year radiographic and clinical outcomes of this cohort are unknown. METHODS: An international pediatric spine database was queried for patients from a GF program (spine or rib-based) with minimum 5-year follow-up from last planned surgery (GF or spinal fusion). Radiographs and charts were reviewed for main coronal curve angle and maximum kyphosis as well as occurrence of secondary surgery. RESULTS: Of 580 graduates, 170 (29%) had minimum 5-year follow-up (37% male). Scoliosis etiology was congenital in 41 (24%), idiopathic 36 (21%), neuromuscular 51 (30%), and syndromic 42 (25%). Index surgery consisted of spine-based growing rods in 122 (71%) and rib-based distraction in 48 (29%). Mean age at index surgery was 6.8 years, and patients underwent an average of 5.4 lengthenings over an average of 4.9 years (range, 6 mo to 11 y). Last planned treatment was at an average age of 11.8 years (range, 7 to 17 years). Last planned treatment consisted of spinal fusion in 114 patients, 47 had growing implants maintained, 9 had implants removed. Average follow-up was 7.3 years (range, 5 to 13 y).When compared from postdefinitive treatment to 2-year follow-up, there was noted progression of the coronal curve angle (46±19 to 51±21 degrees, P=0.046) and kyphosis (48±20 to 57±25 degrees, P=0.03). However, between 2 and 5 years, no further progression occurred in the coronal (51±21 to 53±21 degrees, P=0.26) or sagittal (57±25 to 54±28 degrees, P=0.93) planes. When stratified based on etiology, there was no significant coronal curve progression between 2- and 5-year follow-up. When comparing spinal fusion patients to those who had maintenance of their growing construct, there was also no significant curve progression.Thirty-seven (21%) underwent at least 1 (average, 1.7; range, 1 to 7) revision surgery following graduation, and 15 of 37 (41%) underwent 2 or more revision surgeries. Reason for revision was implant revision (either GF or spinal fusion) in 34 patients, and implant removal in 3. On an average, the first revision was 2.5 years after the definitive treatment plan (range, 0.02 to 7.4 y). In total, 15 of 37 (41%) revisions occurred over 2 years following the final decision for treatment plan, and 7 of 37 (19%) occurred 5 or more years after the definitive treatment.Patients who underwent spinal fusion as a definitive treatment strategy were more likely to undergo revision surgery (27%) than patients who had their GF implants maintained (11%) (P=0.04). CONCLUSIONS: Five years following "graduation" from growing surgery for early onset scoliosis, there is progression of curve magnitude in both the coronal and sagittal planes up to 2 years, with no further progression at 5 years. A total of 21% of patients undergo at least 1 revision surgery, and average time to revision surgery is over 2 years from last planned surgery. Risk of revision surgery was higher in patients who underwent a spinal fusion as their definitive treatment strategy. LEVEL EVIDENCE: Level III-retrospective comparative. TYPE OF EVIDENCE: Therapeutic.

The Frequency of Mediastinal Injury in Acute Posterior Sternoclavicular Dislocations: A Multicenter Study.

BACKGROUND: Acute posterior sternoclavicular dislocations (APSCD) are rare injuries that historically have prompted concern for injury to the great vessels and other mediastinal structures from initial trauma or subsequent treatment, resulting in the recommendation that a thoracic or vascular surgeon be present or available during operative treatment. The objectives of the study were to characterize the demographic, clinical, and radiographic characteristics of a large series of APSCDs in skeletally immature patients and to describe the rate and nature of any vascular or mediastinal complications that occurred during treatment. METHODS: Following Institutional Review Board approval, records of consecutive patients under 25 years of age treated for APSCD were collected from each of 6 participating centers. Only acute injuries (sustained fewer than 10 days before presentation) were included. Patient demographics, injury mechanism, associated mediastinal injuries, and need for thoracic/vascular surgery were recorded. Mediastinal structures injured or compressed by mass effect were specifically characterized by review of preoperative computed tomography imaging. RESULTS: Review identified 125 patients with a mean age of 14.7 years; 88% were male. APSCD most commonly resulted from a sporting injury (74%) followed by falls from standing height (10%) and high-energy motor vehicle trauma (10%). The most common finding on cross-sectional imaging was compression without laceration of the ipsilateral brachiocephalic vein (50%). Eleven patients had successful closed reduction, and 114 (90%) had open reduction and internal fixation, with 25 failed or unstable closed reductions preceding open treatment. There were no vascular or mediastinal injuries during reduction or fixation that required intervention. CONCLUSIONS: In this multicenter series of 125 APSCDs no injuries to the great vessels/mediastinal structures requiring intervention were identified. Although more than half of patients had evidence of extrinsic vascular compression at the time of injury, careful open reduction of acute injuries can be safely performed. Although vascular injuries following APSCD seem to be quite rare, vascular complications can be catastrophic. Treating providers should consider these data and their own institutional resources to maximize patient safety during the treatment of APSCD. LEVEL OF EVIDENCE: Level III-therapeutic case control study.

Prospectively collected surgeon indications for discontinuation of a lengthening program for early-onset scoliosis.

INTRODUCTION: At some point after children with early-onset scoliosis (EOS) undergo implantation of a distraction construct to control deformity and promote growth, a decision is made to discontinue lengthening. The purpose of this study was to evaluate surgeon indications for discontinuation of a lengthening program and to evaluate patient outcomes. METHODS: As a part of a multicenter database, surgeons prospectively completed a questionnaire at the completion of growth-friendly treatment. Surgeon indications for discontinuation included patient age, pain/functional status, implant status, and spinal deformity parameters. Patient demographics, scoliosis type, deformity parameters, and length of time in a growing program were queried. Patients were treated with a final fusion or observation, and rate of secondary surgeries was analyzed. RESULTS: Questionnaires were completed on 121 patients (61% female). EOS etiology was 31% neuromuscular, 43% congenital, 16% idiopathic, and 10% syndromic. Average age at initiation of growing program was 6.8 ± 3.1 years, and average age at discontinuation was 12.7 ± 2.5 years. The most commonly cited indications for discontinuation of a lengthening program included bone age/skeletal maturity (n = 46), patient age (n = 33), and diminishing returns with expansions (n = 33). A larger coronal Cobb angle was found in patients who underwent definitive fusion (65°) when compared with continued observation (55°, p = 0.001). Twenty-nine (24%) patients were initially treated with observation after completion of a growing construct. In this subgroup, at a minimum of 2 years' (average 3.8 years') follow-up, 26/29 (90%) patients remained stable with observation alone; whereas, three (10%) underwent delayed final fusion surgery. CONCLUSIONS: The most common surgeon-cited indications for discontinuation of a lengthening program in EOS patients are skeletal maturity and patient age. The majority of patients (76%) underwent definitive spinal fusion after discontinuation of a lengthening program; whereas, those treated with observation alone had a survivorship of 90% at a minimum follow-up of two years.

Flexible intramedullary nailing of femoral shaft fractures: closed versus open reduction.

Children's femoral shaft fractures are commonly treated with flexible intramedullary nailing after closed or open reduction, but there is little information concerning indications for open reduction. The purpose of this study was to determine radiographic and clinical features likely to lead to open reduction before flexible intramedullary nailing. Record review identified 158 femoral shaft fractures treated with flexible intramedullary nailing. In addition to patient demographics and mechanism of injury, data obtained included surgeon name, estimated blood loss, type of reduction, type and diameter of nail, type of operating table, the use of percutaneous reduction techniques or supplemental casting, time to and duration of surgery, total time in operating room, and time to union. Fracture ratios were calculated based on established radiographic protocol. Of 158 fractures, 141 were treated with closed reduction and 17 with open reduction. The anteroposterior fracture index (1.3 ± 0.4, P = 0.0007), surgeon (P = 0.002), and flattop operating table (0.05) were associated with open reduction. Smaller lateral diameter of bone at the fracture site, transverse fracture, and surgeon were all found to be independent risk factors for open reduction; patient characteristics, including age, sex, and BMI, did not seem to influence the choice of open reduction. Fractures with a lower fracture index or pattern resembling a transverse fracture rather than oblique or spiral had an increased risk of converting to an open reduction. Surgeon preference and use of flattop tables also had a significant influence on how the fracture was treated.

Daratumumab in high-risk relapsed/refractory multiple myeloma patients: adverse effect of chromosome 1q21 gain/amplification and GEP70 status on outcome.

Gain of chromosome 1q21 and the gene expression-based GEP70 risk score are established prognostic markers for newly diagnosed Multiple Myeloma (MM) patients. Here we addressed the prognostic impact of these two markers in 81 relapsed/refractory (RR) MM patients treated with the CD38-antibody daratumumab. Fluorescence in situ hybridization for 1q21 was performed at initial presentation, while the GEP70 score was determined at initial presentation and prior to daratumumab treatment. While the GEP70 at initial presentation showed a trend for inferior survival, the GEP70 collected prior to daratumumab treatment was significantly associated with poor outcome (P < 0·05). The worst outcome was seen for patients who were positive for gain(1q) and classified as GEP70 high risk prior to daratumumab [progression-free (PFS) and overall survival (OS) of 0·3 years (95% CI: 0·15-1·4 years) and 0·8 years (95% CI: 0·5-1·9 years) respectively], while the median PFS and OS were not reached by patients without gain(1q) and GEP70 low-risk status. In conclusion, gain(1q) and the GEP70 are powerful prognostic markers for RR MM patients treated with daratumumab, and patients classified as high risk according to these markers experience shorter treatment response.

Operating Room Personnel Determine Efficiency of Pediatric Spinal Fusions for Scoliosis.

STUDY DESIGN: Retrospective chart review. OBJECTIVES: To investigate the effect of different surgeons, anesthesiologists, and cRNAs individually and in teams on various perioperative and operative time intervals in a large, high-volume children's hospital. SUMMARY OF BACKGROUND DATA: Along with individual factors, studies have indicated that team factors play a role in efficiency, with larger teams leading to increased procedure times. An operating room (OR) staff dedicated to orthopedics has been reported to decrease turnover time; however, the characteristics and behaviors of surgical team members, to our knowledge, have not been analyzed as possible factors contributing to pediatric OR efficiency, and limited research has been conducted in the field of orthopedic personnel. METHODS: Chart review identified consecutive pediatric and adolescent patients who had primary posterior spinal fusion (PSF) of ≥7 levels for correction of spinal deformity. Time intervals and delays were recorded based on previous studies looking at OR efficiency and adjusted to the specific time points available in our perioperative nursing records. RESULTS: Adjusted for etiology, osteotomy, fusion levels, distance from hospital, staff switch, and body mass index, there was a significant difference in patient wait time among anesthesiologists, surgeon-anesthesiologist, and anesthesiologist-certified registered nurse anesthetist (cRNA) teams; in surgery prep time and total prep among surgeons and SA teams; and in surgery time and total room time among surgeons. There were no significant differences among cRNAs, individually, in any time interval. CONCLUSIONS: Anesthesiologists have a significant effect before and surgeons have a significant effect after entry into the OR. Identification of this variability provides an opportunity to study the differences in habits and processes of high- and low-efficiency teams, which can then be applied to all teams with the goal of improving performance of all surgical teams. LEVEL OF EVIDENCE: Level IV, case series.

Outcomes of Primary and Conversion Magnetically Controlled Growth Rods Are Different at Two-Year Follow-up: Results of North American Release.

STUDY DESIGN: Multicenter retrospective review. OBJECTIVES: To compare the radiographic outcomes and complication rates in patients with primary and conversion magnetically controlled growing rod (MCGR) implants at one and two years after surgery. SUMMARY OF BACKGROUND DATA: Many initial early-onset scoliosis (MCGR) implantations in the United States were conversions from other types of growth-friendly systems, and the outcome similarities and differences between primary and conversion MCGR implantation procedures are still relatively unknown. METHODS: Multicenter retrospective review of EOS patients from two multicenter EOS registries identified consecutive EOS patients treated from 2014 to 2017 with a minimum of one-year follow-up. In addition, a subset of these patients who had two-year follow-up were further analyzed. RESULTS: In total, 383 MCGR patients were identified, of which 272 (71%) were primary (P) and 111 (29%) were conversion (C). Group P patients had significantly greater coronal curves at the time of MCGR implantation and greater initial coronal correction. There was no statistically significant difference in Cobb correction at one year or between follow-up at one and two years. The preimplantation thoracic spine height was identical in both groups, with statistically greater improvement at initial implantation in P than in C patients. Significantly greater height gains were seen in P than in C patients in the one-year follow-up cohort. There was a higher rate of complications in the C group than in the P group; however, the difference was not statistically significant. Overall, most complications were implant-related. No loss of curve correction occurred in either group. CONCLUSIONS: Patients with primary MCGR insertion can be expected to have greater radiographic correction and spine length gain than those with conversion from growth-friendly instrumentation to MCGR, most likely because of increased spine stiffness in conversion patients. The rate of complications, primarily implant-related, remains higher in conversion than in primary insertion patients. LEVEL OF EVIDENCE: Level III.

An acquired high-risk chromosome instability phenotype in multiple myeloma: Jumping 1q Syndrome.

Patients with multiple myeloma (MM) accumulate adverse copy number aberrations (CNAs), gains of 1q21, and 17p deletions during disease progression. A subset of these patients develops heightened 1q12 pericentromeric instability and jumping translocations of 1q12 (JT1q12), evidenced by increased copy CNAs of 1q21 and losses in receptor chromosomes (RC). To understand the progression of these aberrations we analyzed metaphase cells of 50 patients with ≥4 CNAs of 1q21 by G-banding, locus specific FISH, and spectral karyotyping. In eight patients with ≥5 CNAs of 1q21 we identified a chromosome instability phenotype similar to that found in ICF syndrome (immunodeficiency, centromeric instability, and facial anomalies). Strikingly, the acquired instability phenotype identified in these patients demonstrates the same transient structural aberrations of 1q12 as those found in ICF syndrome, suggesting similar underlying pathological mechanisms. Four types of clonal aberrations characterize this phenotype including JT1q12s, RC deletions, 1q12-21 breakage-fusion-bridge cycle amplifications, and RC insertions. In addition, recurring transient aberrations include 1q12 decondensation and breakage, triradials, and 1q micronuclei. The acquired self-propagating mobile property of 1q12 satellite DNA drives the continuous regeneration of 1q12 duplication/deletion events. For patients demonstrating this instability phenotype, we propose the term "Jumping 1q Syndrome."

Changes in Pediatric Spine Surgery Patterns Over the Last 10 Years Among ABOS Part II Candidates.

STUDY DESIGN: A retrospective review of prospectively collected data. OBJECTIVE: Our purpose was to evaluate the volume of pediatric spine cases being done by surgeons applying for American Board of Orthopaedic Surgeons (ABOS) certification. SUMMARY OF BACKGROUND DATA: Pediatric orthopedic surgery has become increasingly subspecialized over the past decade. METHODS: Data were reviewed from the ABOS for surgeons undergoing part II of ABOS certification between 2004 and 2014. Applicants were divided into pediatric orthopedic surgeons and spine surgeons based on their self-declared subspecialty for the ABOS Part II examination. A total of 102,424 cases were reviewed to identify spine cases performed on patients <18 years old. RESULTS: Between 2004 and 2014, the total number of ABOS part II pediatric candidates increased significantly, from a low of 15 to a high of 44 (r = 0.68, P = 0.001). During this time frame, there has been no significant increase in the total number of pediatric spine cases reported (r = 0.09, P = 0.19). In 2004, 46.5% (33/71) of the pediatric spine cases were done by spine surgeons, which decreased to 17.3% (28/162) in 2014. Conversely in 2004, 53.5% (38/71) of pediatric spine cases were done by pediatric orthopedists, which increased to 82.7% (134/162) in 2014. The number of pediatric candidates performing pediatric spine cases decreased 35% from 2004 to 2014, but the percentage performing >20 spine cases during their candidate year has increased from 0% to 7% (r = 0.31, P = 0.04). CONCLUSION: The share of pediatric spine surgeries performed by pediatric candidates has increased from 54% in 2004 to 83%, with a corresponding fall in the share surgeries performed by spine candidates (47% to >17%). The percentage of pediatric candidates performing more than 20 spine cases/year increased from 0% to 7%, reflecting a trend of spine subspecialization within pediatric surgery. LEVEL OF EVIDENCE: 3.

Radiation exposure in adult and pediatric patients with osteogenesis imperfecta.

Diagnostic radiographs, computed tomography (CT), nuclear medicine studies, and intraoperative fluoroscopy durations were analyzed for radiation exposure. Cumulative and yearly effective ionizing radiation doses, cumulative background radiation, and total radiograph studies were compared between pediatric and adult populations. In 24 patients with 1,246 imaging studies (average 5.5 years longitudinal treatment duration), the mean estimated cumulative effective radiation dose per patient was 30.0 mSv (range 2.3-115.0), with an average yearly dose of 4.9 mSv (range 0.4-24.8). Pediatric patients had significantly more radiograph studies per year than adults and greater average yearly effective radiation doses.

Poor overall survival in hyperhaploid multiple myeloma is defined by double-hit bi-allelic inactivation of TP53.

Hyperhaploid multiple myeloma is a rare numerical aberration group defined by a range of 24-34 chromosomes, which is associated with a poor prognosis with a 5-year survival rate of 23%. Hyperhaploid patient samples (n=8) were sequenced and copy number and mutations identified. Samples had a median of 13 monosomies (range 12-14), which in general were those not associated with trisomies in hyperdiploid samples. The chromosomes traditionally trisomic in hyperdiploid myeloma were disomic in hyperhaploid myeloma with retention of heterodisomy. We examined the hyperhaploid samples for frequently mutated genes and found that 8/8 (100%) hyperhaploid samples had a mutation in TP53, exceeding the overall rate of mutation in newly diagnosed patients (5.5%), indicating an oncogenic dependency in this group. All samples with TP53 mutation also had monosomy of chromosome 17, indicating bi-allelic inactivation of TP53. As such, this high risk group is part of double-hit myeloma.

Orthopaedic Care for Children: Who Provides It? How Has It Changed Over the Past Decade? Analysis of the Database of the American Board of Orthopaedic Surgery.

BACKGROUND: The field of orthopaedic surgery has subspecialized over the past decade with an increasing number of graduates of orthopaedic residency programs entering fellowship training. The number of graduates from pediatric orthopaedic fellowships has also increased over the past decade. We hypothesize as the number of pediatric orthopaedic fellowship graduates has increased, the proportion of orthopaedic cases completed by pediatric surgeons in comparison with adult surgeons has also increased. We have used the database of the American Board of Orthopaedic Surgery (ABOS) to analyze the trends in who is providing the orthopaedic care for children. METHODS: Procedure logs of applicants for ABOS part II certification from 2004 to 2014 were collected and pediatric cases were used for this study. Applicants were divided into pediatric orthopaedic surgeons and adult orthopaedic surgeons based on the self-declared subspecialty for part II examination. CPT codes were used to place the cases into different categories. Descriptive and statistical analysis were performed to evaluate the change in the practice of pediatric orthopaedics over the past decade. RESULTS: ABOS part II applicants performed 102,424 pediatric cases during this period. In total, 66,745 (65%) cases were performed by nonpediatric surgeons and 35,679 cases (35%) by pediatric surgeons. In total, 82% of the pediatric cases were done by adult surgeons in 2004 which decreased to 69% in 2009 and to 53% in 2014 (r=0.8232, P=0.0019). In pediatric sports medicine, pediatric orthopaedic surgeons performed 7% of the cases in 2004 which increased to 14% in 2009 and to 28% in 2014 (300% increase from 2004). Pediatric surgeons also increased their share of pediatric trauma cases. In total, 12% of lower extremity trauma cases were attended by pediatric surgeons in 2004 compared with 47% in 2014 (235% increase from 2004). In upper extremity trauma, pediatric surgeons increased their share of the cases from 12% in 2004 to 43% in 2014 (175% increase from 2004). CONCLUSIONS: Over the past decade, pediatric orthopaedic specialists are caring for an increasing share of pediatric cases. Pediatric trauma, pediatric spine, and pediatric sports medicine have seen the greatest increase in the percentage of cases performed by pediatric orthopaedic surgeons. LEVEL OF EVIDENCE: Level III.

Changes in the Practice of Pediatric Orthopaedic Surgeons Over the Past Decade: Analysis of the Database of the American Board of Orthopaedic Surgery.

BACKGROUND: There has been an increase in the number of the graduates of pediatric orthopaedic fellowship programs over the past decade creating the potential for increased competition in the field. The purpose of this study was to analyze the effect of increased number of pediatric orthopaedic fellowship graduates on case volume as well as the type of procedures performed by recent graduates of pediatric orthopaedic fellowship programs from 2004 to 2014. METHODS: Case logs submitted for the American Board of Orthopaedic Surgery Part II examination by applicants with the self-declared subspecialty of pediatric orthopaedics from 2004 to 2014 were analyzed. Cases were categorized as trauma (upper and lower extremity), spine, sports medicine, hip, deformity correction, foot and ankle, hardware removal, soft tissue procedures, and other. The period was divided into 3 sections: 2004-2007, 2008-2011, 2012-2014. Descriptive analysis was used to report the change in the volume and pattern of practices over the study period. RESULTS: Although the number of pediatric orthopaedic subspecialty applicants increased from 15 to 44 from 2004 to 2014, the average cases per year increased from 2142 in 2004-2007 to 2960 in 2007-2011, and to 4160 in 2012-2014. The number of cases performed per applicant remained stable over the study period. Upper extremity trauma cases were the largest category of cases reported and increased in case volume by 141% from 2004 to 2014. Sports medicine cases increased in volume by 175%. CONCLUSIONS: Despite a large increase in the number of pediatric orthopaedic surgeons over the past decade, there is a concomitant increased in case volume across almost all subspecialties within pediatric orthopaedics. As such, pediatric orthopaedic surgeons who start a new practice can expect to develop a robust practice with a diverse group of pathologies. LEVEL OF EVIDENCE: Level III.