Search

Introduction: Synovial sarcoma is one of the most common soft tissue sarcomas in children. Guidelines regarding the adequate extent of resection margins and the role of re-resection are lacking. We sought to evaluate the adequate resection margin and the role of re-resection in predicting outcomes in children with synovial sarcomas., Methods: A cohort of 36 patients less than 18 years of age at diagnosis who were treated for localized synovial sarcoma at three tertiary pediatric hospitals between January 2004 and December 2020 were included in this study. Patient and tumor demographics, treatment information, and margin status after surgical resection were collected from the medical record. Clinical, treatment, and surgical characteristics, as well as outcomes including hazard ratios (HRs), event-free survival (EFS), and overall survival (OS) were compared by resection margins group and re-resection status., Results: Patients in the R1 resection group were significantly more likely to relapse or die compared to patients in the R0 resection group. However, there was no significant difference in EFS (HR 0.52, p = 0.54) or OS (HR 1.56, p = 0.719) in R0 patients with less than 5 mm margins compared to R0 patients with more than 5 mm margins. Patients with R1 on initial or re-resection had significantly worse OS than patients who had R0 resection on initial or re-resection (HR = 10.12, p = 0.005)., Conclusion: This study re-affirms that R0 resection is an independent prognostic predictor of better OS/EFS in pediatric synovial sarcoma. Second, our study extends this finding to report negative margins on initial resection or re-resection is associated with better OS/EFS than positive margins on initial resection or re-resection. Lastly, we found that there is no difference in outcomes associated with re-resection or <5 mm margins for R0 patients, indicating that re-resection and <5 mm margins are acceptable if microscopic disease is removed., (© 2024 The Author(s). Cancer Medicine published by John Wiley & Sons Ltd.)

ChatGPT - currently the most popular generative artificial intelligence system - has been revolutionizing the world and healthcare since its release in November 2022. ChatGPT is a conversational chatbot that uses machine learning algorithms to enhance its replies based on user interactions and is a part of a broader effort to develop natural language processing that can assist people in their daily lives by understanding and responding to human language in a useful and engaging way. Thus far, many potential applications within healthcare have been described, despite its relatively recent release. This manuscript offers the pediatric surgical community a primer on this new technology and discusses some initial observations about its potential uses and pitfalls. Moreover, it introduces the perspectives of medical journals and surgical societies regarding the use of this artificial intelligence chatbot. As ChatGPT and other large language models continue to evolve, it is the responsibility of the pediatric surgery community to stay abreast of these changes and play an active role in safely incorporating them into our field for the benefit of our patients. LEVEL OF EVIDENCE: V., Competing Interests: Declarations of competing interest None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Trauma is the leading cause of death in children. Resuscitative endovascular balloon occlusion of the aorta (REBOA) provides temporary hemorrhage control, but its potential benefit has not been assessed in children. We hypothesized that there are pediatric patients who may benefit from REBOA., Methods: Trauma patients <18 years old at a level 1 pediatric trauma center between 2009 and 2019 were queried for deaths, pre-hospital cardiac arrest, massive transfusion protocol activation, transfusion requirement, or hemorrhage control surgery. These patients defined the cohort of severely injured patients. From this cohort, patients with intraabdominal injuries for which REBOA may provide temporary hemorrhage control were identified, including solid organ injury necessitating intervention, vascular injury, or pelvic hemorrhage., Results: There were 239 severely injured patients out of 6538 pediatric traumas. Of these, 38 had REBOA-amenable injuries (15.9%) with 34.2% mortality, accounting for 10.2% of all pediatric trauma deaths at one center. Eleven patients with REBOA-amenable injuries had TBI (28.9%). Patients with REBOA-amenable injuries represented 0.6% of all pediatric traumas., Conclusion: Nearly 20% of severely injured pediatric patients could potentially benefit from REBOA. The overall proportion of pediatric patients with REBOA-amenable injuries is similar to adult studies., Type of Study: Retrospective comparative study., Level of Evidence: Level III., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Müllerian duct anomalies are rare in the general population, occurring in less than 3% of women, but much more prevalent in female patients with anorectal malformation, occurring in up to 30% of these patients. Unicornuate uterus with a rudimentary non-communicating horn is a congenital anomaly of Mullerian development which can be seen in isolation or in conjunction with other anomalies, with several case reports described in patients with VACTERL association. These anomalies may be asymptomatic until the patient develops dysmenorrhea or devastating obstetrical complications. We describe the successful surgical management of an obstructive Müllerian anomaly in a post-pubertal female patient with anorectal malformation., Competing Interests: Conflict of interest: The following authors have no financial disclosures: CMT, AFT, SAC, KM, PS Declaration of interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Background: Trauma is the leading cause of death in children, and most deaths occur within 24 hours of injury. A better understanding of the causes of death in the immediate period of hospital care is needed., Methods: Trauma admissions younger than 18 years from 2009 to 2019 at a Level I pediatric trauma center were reviewed for deaths (n = 7,145). Patients were stratified into ages 0-6, 7-12, and 13-17 years old. The primary outcome was cause of death, with early death defined as less than 24 hours after trauma center arrival., Results: There were 134 (2%) deaths with a median age of 7 years. The median time from arrival to death was 14.4 hours (interquartile range, 0.5-87.8 hours). Half (54%) occurred within 24 hours. However, most patients who survived initial resuscitation in the emergency department died longer than 24 hours after arrival (69%). Traumatic brain injury was the most common cause of death (66%), followed by anoxia (9.7%) and hemorrhage (8%). Deaths from hemorrhage were most often in patients sustaining gunshot wounds (73% vs. 11% of all other deaths, p < 0.0001), more likely to occur early (100% vs. 50% of all other deaths, p = 0.0009), and all died within 6 hours of arrival. Death from hemorrhage was more common in adolescents (21.4% of children aged 13-17 vs. 6.3% of children aged 0-6, and 0% of children aged 7-12 p = 0.03). The highest case fatality rates were seen in hangings (38.5%) and gunshot wounds (9.6%)., Conclusion: Half of pediatric trauma deaths occurred within 24 hours. Death from hemorrhage was rare, but all occurred within 6 hours of arrival. This is a critical time for interventions for bleeding control to prevent death from hemorrhage in children. Analysis of these deaths can focus efforts on the urgent need for development of new hemorrhage control adjuncts in children., Level of Evidence: Epidemiological study, level IV., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: The paradigm that children maintain normal blood pressure during hemorrhagic shock until 30%-45% hemorrhage is widely accepted. There are minimal data supporting when decompensation occurs and how a child's vasculature compensates up to that point. We aimed to observe the arterial response to hemorrhage and when mean arterial pressure (MAP) decreased from baseline in pediatric swine., Methods: Piglets were hemorrhaged in 20% increments of their total blood volume to 60%. MAP and angiograms of the thoracic aorta (TA) and abdominal arteries were obtained. Percent change in area of the vessels from baseline was calculated., Results: Piglets (n = 8) had a differential vasoconstriction starting at 20% hemorrhage (celiac artery 36.3% [31.4-44.6] vs TA 16.7% [10.7-19.1] p = 0.0012). At 40% hemorrhage, the differential vasoconstriction favored shunting blood away from the abdominal visceral branches to the TA (celiac artery 54.7% [36.9-60.6] vs TA 29.5% [23.9-36.2] p = 0.0056 superior mesenteric artery 46.7% [43.9-68.6] vs TA 29.5% [23.9-36.2] p = 0.0100). This was exacerbated at 60% hemorrhage. MAP decreased from baseline at 20% hemorrhage (66.4 ± 6.0 mmHg vs 41.4 ± 10.4 mmHg, p < 0.0001), and worsened at 40% and 60% hemorrhage., Conclusion: In piglets, a differential vasocontriction shunting blood proximally occurred in response to hemorrhage. This did not maintain normal MAP at 20%, 40% or 60% hemorrhage., Level of Evidence: Level II., Competing Interests: Disclosures Author MAJ is a founder and shareholder of Certus Critical Care Inc., (Published by Elsevier Inc.)

The surgical management of children with rectal prolapse is wide ranging and without consensus within the pediatric surgical community. While the majority of rectal prolapse in infants and children resolves spontaneously or with the medical management of constipation, a small but significant subset of patients may require intervention for persistent symptoms. In this review, we discuss the etiology and pathophysiology of rectal prolapse in both infants and children, options for medical management, described interventions and surgical options and their outcomes, and future avenues for research and investigation., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Resuscitative endovascular balloon occlusion of the aorta (REBOA) is recommended in adults with a noncompressible torso hemorrhage with occlusion times of less than 60 minutes. The tolerable duration in children is unknown. We used a pediatric swine controlled hemorrhage model to evaluate the physiologic effects of 30 minutes and 60 minutes of REBOA., Methods: Pediatric swine weighing 20 kg to 30 kg underwent a splenectomy and a controlled 60% total blood volume hemorrhage over 30 minutes, followed by either zone 1 REBOA for 30 minutes (30R) or 60 minutes (60R). Swine were then resuscitated with shed blood and received critical care for 240 minutes., Results: During critical care, the 30R group's (n = 3) pH, bicarbonate, base excess, and lactate were no different than baseline, while at the end of critical care, these variables continued to differ from baseline in the 60R group (n = 5) and were worsening (7.4 vs. 7.2, p < 0.001, 30.4 mmol/L vs. 18.4 mmol/L, p < 0.0001, 5.6 mmol/L vs. -8.5 mmol/L, p < 0.0001, 2.4 mmol/L vs. 5.7 mmol/L, p < 0.001, respectively). Compared with baseline, end creatinine and creatinine kinase were elevated in 60R swine (1.0 mg/dL vs. 1.7 mg/dL, p < 0.01 and 335.4 U/L vs. 961.0 U/L, p < 0.001, respectively), but not 30R swine (0.9 mg/dL vs. 1.2 mg/dL, p = 0.06 and 423.7 U/L vs. 769.5 U/L, p = 0.15, respectively). There was no difference in survival time between the 30R and 60R pediatric swine, p = 0.99., Conclusion: The physiologic effects of 30 minutes of zone 1 REBOA in pediatric swine mostly resolved during the subsequent 4 hours of critical care, whereas the effects of 60 minutes of REBOA persisted and worsened after 4 hours of critical care. Sixty minutes of zone 1 REBOA may create an irreversible physiologic insult in a pediatric population.

Background: Resuscitative endovascular balloon occlusion of the aorta (REBOA) has not been studied in children. We hypothesized that REBOA was feasible and would improve hemorrhage control and survival time, compared to no aortic occlusion, in a pediatric swine liver injury model., Methods: Pediatric swine were randomized to Zone 1 REBOA or no intervention (control). Piglets underwent a partial liver amputation and free hemorrhage followed by either REBOA or no intervention for 30 min, then a damage control laparotomy and critical care for 4 h., Results: Compared to control piglets (n = 5), REBOA piglets (n = 6) had less blood loss (34.0 ± 1.6 vs 61.3 ± 2.5 mL/kg, p < 0.01), higher end hematocrit (28.1 ± 2.1 vs 17.1 ± 4.1%, p = 0.03), higher end creatinine (1.4 ± 0.1 vs 1.2 ± 0.1 mg/dL, p = 0.05), higher end ALT and AST (56 ± 4 vs 32 ± 6 U/L, p = 0.01 and 155 ± 26 vs 69 ± 25 U/L, p = 0.05) and required more norepinephrine during critical care (1.4 ± 0.3 vs 0.3 ± 0.3 mg/kg, p = 0.04). All REBOA piglets survived, whereas 2 control piglets died, p = 0.10., Conclusion: In pediatric swine, 30 min of REBOA is feasible, decreases blood loss after liver injury and may improve survival., Level of Evidence: Level 1., Competing Interests: Disclosures Author MAJ is a founder and shareholder of Certus Critical Care Inc., (Published by Elsevier Inc.)

Introduction: Many management options exist for the treatment of refractory rectal prolapse (RP) in children. Our goal was to characterize current practice patterns among active members of APSA., Methods: A 23-item questionnaire assessed the management of full-thickness RP for healthy children who have failed medical management. The survey was approved by our IRB and by the APSA Outcomes committee., Results: 236 surgeons participated. The respondents were geographically dispersed (44 states, 5 provinces). 32% of respondents had twenty or more years of clinical experience. 71% evaluated 1-5 RP patients in the last 2 years, while 5% evaluated >10. 71% performed 0-1 procedure (operation or local therapy [LT]) for RP over 2 years. 59% would treat a 2-year-old patient differently than a 6-year-old with the same presentation, and were more likely to offer up-front surgery to a 6-year-old (26% vs 15%, p = 0.04), less likely to continue medical management indefinitely (2% vs 7%, p=0.01), and more likely to perform resection with rectopexy (30% vs. 15%, p=0.01). 71% perform LT as an initial intervention: injection sclerotherapy (59%), anal encirclement (8%), and sclerotherapy + anal encirclement (5%). 70% consider LT a failure after 1-3 attempts. If LT fails, surgical management consists of transabdominal rectopexy (46%), perineal proctectomy or proctosigmoidectomy (22%), transabdominal sigmoidectomy + rectopexy (22%), and posterior sagittal rectopexy (9%)., Conclusions: There is wide variability in the surgical management of pediatric rectal prolapse. This suggests a need for development of processes to identify best practices and optimize outcomes for this condition., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: There is wide variability of transfusion practices for children with hemorrhagic injuries across trauma centers. We are planning a multicenter, randomized clinical trial evaluating tranexamic acid in children with hemorrhage. Standardization of transfusion practices across sites is important to minimize confounding. Therefore, we sought to generate consensus-based transfusion guidelines for the trial., Methods: We used a modified Delphi process utilizing a multi-site, multi-disciplinary panel of experts to develop our transfusion guidelines. A survey of 23 clinical categories on various aspects of transfusion practices was developed and distributed via SurveyMonkey®. Statements were graded on a 5-point Likert scale ("Strongly agree" to "This intervention may be harmful"). Statements were accepted if ≥ 80% of the panelists rated the statement as "Strongly agree" or "Agree". After each round, the responses were calculated and the results included on subsequent rounds., Results: 35 panelists from four pediatric trauma centers participated in the study, including 11 (31%) pediatric EM physicians, 8 (23%) pediatric trauma surgeons, 5 (14%) transfusionists, 5 (14%) pediatric anesthesiologists, and 6 (17%) pediatric critical care physicians (range of 8 to 10 from each clinical site). Four survey iterations were performed. In total 176 statements were rated and 39 were accepted by criteria across all 23 categories. An rational algorithm for transfusion in trauma was then developed., Conclusions: We successfully developed transfusion guidelines for various aspects of the management of children with hemorrhagic injuries using a modified Delphi process with broad interdisciplinary participation. We anticipate implementation of these guidelines will help minimize heterogeneity of transfusion practices across clinical sites for the upcoming clinical trial evaluating tranexamic acid in children with hemorrhage.

Although most children with Hirschsprung disease ultimately achieve functional and comfortable stooling, some will experience a variety of problems after pull-through surgery. The most common problems include soiling, obstructive symptoms, enterocolitis, and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative soiling in children with Hirschsprung disease. The American Pediatric Surgical Association Hirschsprung Disease Interest Group engaged in a literature review and group discussions. Expert consensus was then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with soiling symptoms following pull-through for Hirschsprung disease. Causes of soiling after pull-through are broadly categorized as abnormalities in sensation, abnormalities in sphincter control, and "pseudo-incontinence." A stepwise algorithm for the diagnosis and management of soiling after a pull-through for Hirschsprung disease is presented; it is our hope that this rational approach will facilitate treatment and optimize outcomes.

Esophageal duplication and congenital esophageal stenosis (CES) may represent diseases with common embryologic etiologies, namely, faulty tracheoesophageal separation and differentiation. Here, we will re-enforce definitions for these diseases as well as review their embryology, diagnosis, and treatment., (Copyright © 2017. Published by Elsevier Inc.)

Background: Folate binding protein(FBP) is an immunogenic protein over-expressed in endometrial(EC) and ovarian cancer(OC). We are conducting a phase I/IIa trial of E39 (GALE 301)+GM-CSF, an HLA-A2-restricted, FBP-derived peptide vaccine to prevent recurrences in disease-free EC and OC patients. This interim analysis summarizes toxicity, immunologic responses, and clinical outcomes to date., Methods: HLA-A2+ patients were vaccinated(VG), and HLA-A2- or -A2+ patients were followed as controls(CG). Six monthly intradermal inoculations of E39+250mcg GM-CSF were administered to VG. Demographic, safety, immunologic, and recurrence rate(RR) data were collected and evaluated., Results: This trial enrolled 51 patients; 29 in the VG and 22 in the CG. Fifteen patients received 1000mcg E39, and 14 received <1000mcg. There were no clinicopathologic differences between groups(all p ≥ 0.1). E39 was well-tolerated regardless of dose. DTH increased pre- to post-vaccination (5.7±1.5 mm vs 10.3±3.0 mm, p = 0.06) in the VG, and increased more in the 1000mcg group (3.8±2.0 mm vs 9.5±3.5 mm, p = 0.03). With 12 months median follow-up, the RR was 41% (VG) vs 55% (CG), p = 0.41. Among the 1000mcg patients, the RR was 13.3% vs 55% CG, p = 0.01. Estimated 2-year DFS was 85.7% in the 1000mcg group vs 33.6% in the CG (p = 0.021)., Conclusions: This phase I/IIa trial reveals that E39+GM-CSF is well-tolerated and elicits a strong, dose-dependent in vivo immune response. Early efficacy results are promising in the 1000 mcg dose cohort. This study proves the safety and establishes the dose of E39 for a larger prospective, randomized, controlled trial in HLA-A2+ EC and OC patients to prevent recurrence.

Background: The optimal treatment of flat epithelial atypia (FEA) found on breast core needle biopsy (CNB) is controversial. We performed a retrospective review of our institutional experience with FEA to determine if excisional biopsy may be deferred., Methods: Surgical records from 2009 to 2012 were reviewed for FEA diagnosis. After exclusion for concomitant lesions, CNBs of pure FEA were classified using a previously agreed upon descriptor of "focal" versus "prominent". Data was analyzed with the Fisher's Exact and Student-t test as appropriate., Results: Of 71 CNBs evaluated, pure FEA was identified on 27 CNBs. Final excisional biopsy was benign in 24 of 27 cases (88%) with associated ductal carcinoma in-situ (DCIS) in 3 of 27 cases (11%). Eighteen of 27 (67%) CNBs were classified as focal while 9 (33%) were described as prominent. Zero of the 18 focal patients had a malignancy compared to 3 of the 9 in the prominent group (0% vs 33%, p=0.02). Of the 27 pure FEA CNBs, 6 patients had a personal history of breast carcinoma, five DCIS and one invasive ductal carcinoma. No malignancies were found in the 21 patients without a personal history of breast carcinoma versus three in the patients with a positive history (0/21 v 3/6, p=0.007)., Conclusions: Our data suggests those women who have adequate sampling and sectioning of CNBs, with focal, pure FEA on pathology, and are without a personal history of breast cancer may undergo a period of imaging surveillance. Conversely, patients with a history of breast cancer or pure, prominent FEA on CNB disease should proceed to excisional biopsy.

Oxidation of [Ru(NH(2)Q)(3)](2+) (NH(2)Q = 8-aminoquinoline) results in intermolecular coupling of 8-aminoquinoline ligands to yield an electroactive polymer. Oxidative polymerization is not observed for [Ru(bpy)(2)(NH(2)Q)](2+) (bpy = 2,2'-bipyridine), where only one 8-aminoquinoline ligand is present.