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Chordoma is a rare primary bone malignancy that arises in the skull base, spine and sacrum and originates from remnants of the notochord. These tumors are typically resistant to conventional chemotherapy, and to date there are no FDA-approved agents to treat chordoma. The lack of in vivo models of chordoma has impeded the development of new therapies for this tumor. Primary tumor from a sacral chordoma was xenografted into NOD/SCID/IL-2R γ-null mice. The xenograft is serially transplantable and was characterized by both gene expression analysis and whole genome SNP genotyping. The NIH Chemical Genomics Center performed high-throughput screening of 2,816 compounds using two established chordoma cell lines, U-CH1 and U-CH2B. The screen yielded several compounds that showed activity and two, sunitinib and bortezomib, were tested in the xenograft. Both agents slowed the growth of the xenograft tumor. Sensitivity to an inhibitor of IκB, as well as inhibition of an NF-κB gene expression signature demonstrated the importance of NF-κB signaling for chordoma growth. This serially transplantable chordoma xenograft is thus a practical model to study chordomas and perform in vivo preclinical drug testing. [ABSTRACT FROM AUTHOR]

Necrotizing enterocolitis (NEC) is a devastating disease affecting premature infants. Broadband optical spectroscopy (BOS) is a method of noninvasive optical data collection from intra-abdominal organs in premature infants, offering potential for disease detection. Herein, a novel machine learning approach, iterative principal component analysis (iPCA), is developed to select optimal wavelengths from BOS data collected in vivo from neonatal intensive care unit (NICU) patients for NEC classification. Neural network models were trained for classification, with a reduced-feature model distinguishing NEC with an accuracy of 88%, a sensitivity of 89%, and a specificity of 88%. While whole-spectrum models performed the best for accuracy and specificity, a reduced feature model excelled in sensitivity, with minimal cost to other metrics. This research supports the hypothesis that the analysis of human tissue via BOS may permit noninvasive disease detection. Furthermore, a medical device optimized with these models may potentially screen for NEC with as few as seven wavelengths., (© 2024 The Author(s). Journal of Biophotonics published by Wiley‐VCH GmbH.)

Background: Utilization of the laparoscopic approach for inguinal hernia repair has increased significantly over the past decade. The purpose of this study is to compare rates of second hernia operation and same side recurrence following open and laparoscopic inguinal hernia repair in a large national cohort., Methods: This retrospective analysis utilized the Pediatric Health Information System database to identify children <18 years-old who underwent laparoscopic or open primary inguinal hernia repair from 2017 to 2021. Data were collected through 2022 to allow minimum one year follow-up. Second hernia operation rates, inclusive of same side recurrence and metachronous contralateral hernia, and same side recurrence rates were compared by multivariable mixed effects model controlling for confounders and institutional clustering. Misclassification rates were determined through data validation at four constituent institutions. Sensitivity analyses determined true outcome rates., Results: We identified 53,287 operations (15.6% laparoscopic). Rate of second hernia operation was greater following laparoscopic repair (2.9% vs 2.6%, p = 0.04) with no difference on multivariable analysis (OR 1.14, 95% CI 0.98-1.32). Same side recurrence rate was greater following laparoscopic repair (1.5% vs 0.4%, p < 0.001) which persisted on multivariable analysis (OR 3.72, 95% CI 2.90-4.78). Sensitivity analysis demonstrated true laparoscopic and open repair rates of 14.2% and 85.8%, respectively. True rates of second hernia operation and same side recurrence were identical to those determined by PHIS., Conclusion: Laparoscopic inguinal hernia repair in children has more than three times the odds of same side hernia recurrence than open repair which is balanced by a reduced rate of second operation for metachronous hernia., Level of Evidence: Treatment Study - Level III., Competing Interests: Conflict of interest All authors have no conflicts of interest to disclose. There is no funding to declare. Each author has made final approval of the manuscript and has certified this material has not and will not be published or submitted to any other publication before its appearance in Journal of Pediatric Surgery., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Study Objective: Isolated tubal torsion (ITT) is a surgical emergency that is less well characterized than ovarian torsion and presents with its own diagnostic challenges. We retrospectively examined patients with ITT and compared them to patients with ovarian torsion to better understand the unique features of this pathophysiologic process., Methods: The medical records of patients who were identified as having ITT or ovarian torsion intraoperatively between January 2019 and November 2022 were reviewed. Patient demographics, clinical documentation, diagnostic imaging reports, operative reports, and pathology results were collected., Results: A total of 82 patients met inclusion criteria of which 62 (75.6%) had ovarian torsion and 20 (24.4%) had ITT. Most patients (63.4%) underwent 2 or more imaging studies prior to the operating room. A diagnosis of torsion occurred more often on preoperative imaging for the ovarian torsion group (67.7% ovarian torsion vs 35.0% ITT, p=0.01). Doppler flow to the ovary was expectedly absent in 65.9% of ovarian torsion patients but interestingly also absent in 25% of ITT patients (p=0.01). Patients with ovarian torsion were more likely to have ovarian cysts (56.5% vs 10.0%, p<0.001), while patients with ITT were significantly more likely to have a paratubal cyst (90.0% vs 38.7%, p<0.001)., Conclusion: ITT is a surgical emergency that poses a risk to future fertility. Ultrasound studies, including doppler, may not be as definitive in patients with ITT and early cross-sectional imaging should be considered. When there is a high clinical suspicion for ovarian torsion or ITT, there should be a low threshold for diagnostic laparoscopy., Competing Interests: Declaration of competing interest The authors have nothing to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Purpose: Limited operating room availability constrains hospital scheduling capacity for elective surgical cases. Leveraging weekends for elective surgical cases could increase operative capacity but must be balanced with practical considerations. Our study aimed to characterize trends and outcomes for elective pediatric surgeries performed during weekends., Methods: This retrospective cohort study used the Pediatric Health Information System database from 2016 to 2019 to identify surgeries in children <18 years of age from 38 hospitals. Six elective surgeries, commonly performed on the weekend, were selected for analysis. Trends in elective surgeries during weekends (Saturday or Sunday) were evaluated using the Mann-Kendall trend test. Multivariable regression models were used to compare complications and costs between weekend and weekday surgeries., Results: Of the 233,266 elective surgeries evaluated, 357 (0.15%) were performed during weekend hours. The proportion of surgeries performed on weekends was stable over time (p = 0.65). Following adjustment for clinicodemographic and hospital-level factors, no differences were observed when comparing weekend to weekday surgeries in terms of surgical complications [adjusted Odds Ratio: 1.59; 95% Confidence Interval (CI): 0.65-3.90; p = 0.32] or mortality (n = 1 in cohort). Weekend surgeries were associated a small additional cost compared to weekday surgeries (β-coefficient $312; 95% CI: $152 to $473; p < 0.01)., Conclusion: Elective pediatric surgeries performed during weekends were uncommon, stable in occurrence, and not associated with substantial increases in complications or costs compared to weekday surgeries. Increasing surgical capacity by extending into weekend scheduling merits further assessment of patient and provider satisfaction, unexpected human resource costs, and thoughtful case selection to ensure patient safety., Level of Evidence: III., Type of Study: Retrospective Cohort Study., Competing Interests: Conflicts of interest There are no conflicts of interest regarding these data., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background and Objective: Thymectomy as a management strategy for juvenile myasthenia gravis (JMG) has been increasingly adopted with the advent of minimally invasive surgical techniques. This review evaluates existing evidence regarding the surgical management of JMG, including the benefits of surgical compared to medical therapy, important considerations when evaluating surgical candidacy and determining optimal timing of intervention. In addition, we provide an overview of the open, thoracoscopic and robotic surgical approaches available for thymectomy and compare the existing data to characterize optimal surgical management., Methods: A thorough literature review was conducted for full length research articles, including systematic reviews, retrospective cohort studies and case series, published between January 2000 and July 2023 regarding open, thoracoscopic or robotic thymectomy for management of JMG. Reference lists of the identified articles were manually searched for additional studies. Evidence was summarized in a narrative fashion with the incorporation of the authors' knowledge gained through clinical experience., Key Content and Findings: Although data specific to JMG are limited to small retrospective cohort studies, available evidence supports equal to greater disease control following thymectomy versus pharmacologic management. Furthermore, outcomes may be optimized when surgery is performed earlier in the disease course, particularly for patients who are post-pubertal with generalized or severe disease and those necessitating high-dose steroid administration thereby limiting its metabolic and growth inhibitory effects. Open transsternal resection is the historic gold-standard; however, as surgeons become more comfortable with thoracoscopic and robotic-assisted thymectomy, an increasing proportion of patients are expected to undergo thymectomy. At present, the data available is unable to support conclusions regarding which surgical approach is superior; however, minimally invasive approaches may be non-inferior while offering superior cosmesis and decreased morbidity., Conclusions: Higher-level investigation through the use of multi-institutional databases and randomized prospective trials is warranted in order to understand which child warrants thymectomy, at what point in their disease course and their development, and which surgical approach will optimize postoperative outcomes., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://med.amegroups.com/article/view/10.21037/med-23-41/coif). The authors have no conflicts of interest to declare., (2024 Mediastinum. All rights reserved.)

Fluorescence-guided surgery (FGS) is an increasingly available and popular method of visual field augmentation. The basic premise of FGS entails injection of fluorescent indocyanine green (ICG) and subsequent detection with a near-infrared (NIR) camera. For pediatric surgical oncologists, FGS remains experimental but is a promising modality for identifying tumor margins, locating metastases, performing sentinel lymph node biopsies, protecting peritumoral structures of interest, and facilitating reconstruction. Familiarity with basic ICG pharmacokinetics and NIR detection optics is critical for surgeons wishing to judiciously use FGS, as its success is firmly grounded in a thorough understanding of its capabilities and limitations. In this practical guide, we outline several well-described and innovative FGS applications by disease type, including their methods of administration, modes of detection, and typical ICG dosing paradigms. LEVEL OF EVIDENCE: V., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Introduction: Increasingly, for pediatric patients with short bowel syndrome (SBS), intestinal lengthening procedures such as serial transverse enteroplasty (STEP) are being offered with the hope of improving patients' chances for achieving enteral autonomy. However, it remains unclear to what extent STEP reduces the long-term need for intestinal transplant or improves survival., Methods: Based on existing literature, a decision analytic Markov state transition model was created to simulate the life of 1,000 pediatric SBS patients. Two simulations were modeled: 1) No STEP: patients were listed for transplant once medical management failed and 2) STEP: patients underwent STEP therapy and subsequent transplant listing if enteral autonomy was not achieved. Sensitivity analysis of small bowel length and anatomy was completed. Base case patients were defined as neonates with a small bowel length of 30cm., Results: For base case patients with an ostomy and a NEC SBS etiology, STEP was associated with increased rates of enteral autonomy after 10 years for patients with an ICV (53.9% [STEP] vs. 51.1% [No STEP]) and without an ICV (43.4% [STEP] vs. 36.3% [No STEP]). Transplantation rates were also reduced following STEP therapy for both ICV (17.5% [STEP] vs. 18.2% [No STEP]) and non-ICV patients (20.2% [STEP] vs. 22.1% [No STEP]). 10-year survival was the highest in the (+) STEP and (+) ICV group (85.4%) and lowest in the (-) STEP and (-) ICV group (83.3%)., Conclusions: For SBS patients, according to our model, STEP increases rates of enteral autonomy, reduces need for intestinal transplantation, and improves long-term survival., Type of Study: Economic/Decision Analysis or Modeling Studies LEVEL OF EVIDENCE: Level III., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: The presence of pain may interrupt sleep and impede normal postoperative recovery; however, no prior studies have quantified sleep loss due to pain in children undergoing inpatient surgery. Wearable accelerometers objectively measure sleep patterns in children. We aimed to quantify sleep loss associated with patient reported pain scores after a Modified Nuss operation., Methods: Ten patients undergoing Modified Nuss operations were recruited during their inpatient stay. Children wore an Actigraph GT3X-BT accelerometer postoperatively during their hospital stay. Hourly sleep minutes were recorded using the Actigraph between 10 pm and 6 am. Patient reported pain scores were abstracted from patient charts. Mixed linear regression models, adjusting for within-subject random effects, were estimated to quantify the association between hourly sleep minutes and patient reported pain scores., Results: Patients were 30% female, with an average age of 15.7 years (range 13-22). The majority (70%) of patients were white non-Hispanic. All patients received a patient controlled analgesic pump. Average postoperative length of stay was 4.8 days (range 4.0-6.0; SD = 0.8). A total of 240 sleep hours and associated pain scores were analyzed. Patients slept on average 48 min per hour. Mixed model analysis predicted that a 1-point increase in pain score was associated with 2.5 min per hour less sleep time., Conclusion: Increases in patient-reported pain scores are associated with sleep loss after a Modified Nuss operation. Objectively quantifying sleep loss associated with postoperative pain using accelerometer data may help clinicians better understand their patient's level of pain control. Our findings provide the basis for future studies aimed at more accurately titrating pain medication to optimize sleep and speed up recovery., Level of Evidence: Case Series Without Comparison Group, Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Despite extensive research and improvements in the field of neonatal care, the morbidity and mortality associated with necrotizing enterocolitis (NEC) have remained unchanged over the past three decades. Early detection of ischemia and necrotic bowel is vital in improving morbidity and mortality associated with NEC; however, strategies for predicting and preventing NEC are lacking. Contrast-enhanced ultrasound (CEUS) and near-infrared spectroscopy (NIRS) are novel techniques in pediatrics that have been proven as safe modalities. CEUS has benefits over conventional ultrasound (US) by its improved real-time evaluation of the micro- and macrovascularities of normally and abnormally perfused tissue. US has been implemented as a useful adjunct to X-ray for earlier evaluation of NEC. NIRS is another noninvasive technique that has shown promise in improving early detection of NEC. The purpose of this article is to review the current understanding of changes in bowel perfusion in NEC, discuss the accuracy of abdominal US in detecting NEC, and explain how the use of CEUS and NIRS will enhance the precise and early detection of altered/pathological bowel wall perfusion in the initial development and course of NEC., Competing Interests: None., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)