Survival outcomes in pediatric patients with metastatic Ewing sarcoma who achieve a rapid complete response of pulmonary metastases.

PURPOSE: Our objectives were to compare overall survival (OS) and pulmonary relapse between patients with metastatic Ewing sarcoma (EWS) at diagnosis who achieve rapid complete response (RCR) and those with residual pulmonary nodules after induction chemotherapy (non-RCR). PATIENTS AND METHODS: This retrospective cohort study included children under 20 years with metastatic EWS treated from 2007 to 2020 at 19 institutions in the Pediatric Surgical Oncology Research Collaborative. Chi-square tests were conducted for differences among groups. Kaplan-Meier curves were generated for OS and pulmonary relapse. RESULTS: Among 148 patients with metastatic EWS at diagnosis, 61 (41.2%) achieved RCR. Five-year OS was 71.2% for patients who achieved RCR, and 50.2% for those without RCR (p = .04), and in multivariable regression among patients with isolated pulmonary metastases, RCR (hazards ratio [HR] 0.42; 95% confidence interval [CI]: 0.17-0.99) and whole lung irradiation (WLI) (HR 0.35; 95% CI: 0.16-0.77) were associated with improved survival. Pulmonary relapse occurred in 57 (37%) patients, including 18 (29%) in the RCR and 36 (41%) in the non-RCR groups (p = .14). Five-year pulmonary relapse rates did not significantly differ based on RCR (33.0%) versus non-RCR (47.0%, p = .13), or WLI (38.8%) versus no WLI (46.0%, p = .32). DISCUSSION: Patients with EWS who had isolated pulmonary metastases at diagnosis had improved OS if they achieved RCR and received WLI, despite having no significant differences in rates of pulmonary relapse.

Two-Stage Liver Transplantation and Tricuspid Valve Replacement After Blunt Trauma in a Pediatric Patient.

In cases of uncontrollable hepatic hemorrhage or acute hepatic failure after trauma, liver transplantation can be a lifesaving procedure. Traumatic tricuspid valve injuries are rare, and symptoms can range from indolent to acute right heart failure. When concomitant, traumatic liver transplant and tricuspid injuries have significant physiologic interplay and management implications. We present a 14-year-old male injured in an all-terrain vehicle accident, who sustained a devastating disruption of the common bile duct and celiac artery injury, leading to acute hepatic failure, necessitating a two-stage liver transplantation. He was subsequently found to have a severe traumatic tricuspid injury, which required tricuspid valve replacement. At 4 years post-injury, he is without major complications. This is the first case presentation of the cooccurrence of these complex pathologies. Importantly, we demonstrate the complex decision-making surrounding traumatic liver transplantation and timing of subsequent tricuspid valve repair, weighing the complex interplay of these 2 pathologies.

Framework for pediatric robotic surgery program development.

Pediatric robotic surgery has seen increasing implementation for its many benefits over the past two decades. As more pediatric surgeons gain exposure to robotic surgery, the interest in utilizing this technology is growing. However, there are no guidelines or existing framework for developing pediatric general surgery robotic programs. Programmatic development can be challenging, requiring institutional support, a minimum 12-month multistep process in partnership with the robot manufacturer, and organization of a local dedicated team. A cornerstone to all program building is collaboration and communication with key stakeholders who are committed to establishing a robotic surgery program. In this manuscript, we detail numerous best practices for implementation, followed by three variations of programmatic development, each drawing lessons from one of three practice settings: (i) A children's hospital in a large medical center associated with an adult hospital, (ii) a free-standing children's hospital, and (iii) a community-based practice. We aim for this article to provide a framework that can serve as a guide for those beginning this process, consolidating the key resources and strategies used to develop a robust pediatric robotic surgery program.

The Effect of Propofol on Peri-Induction Hemodynamics and Resuscitation in Operative Penetrating Trauma.

BACKGROUND: Hemorrhaging trauma patients may be disproportionately affected by choice of induction agent during rapid sequence intubation (RSI). Etomidate, ketamine, and propofol are safe in the trauma population-at-large but have not been assessed in patients with ongoing hemorrhage. We hypothesize that in hemorrhaging patients with penetrating injury, propofol deleteriously affects peri-induction hypotension compared to etomidate and ketamine. METHODS: Retrospective cohort study. Primary outcome was the effect of induction agent on peri-induction systolic blood pressure. Secondary outcomes were the incidence of peri-induction vasopressor use and quantity of peri-induction blood transfusion requirements. Linear multivariate regression modeling assessed the effect of induction agent on the variables of interest. RESULTS: 169 patients were included, 146 received propofol and 23 received etomidate or ketamine. Univariate analysis revealed no difference in peri-induction systolic blood pressure (P = .53), peri-induction vasopressor administration (P = .62), or transfusion requirements within the first hour after induction (PRBC P = .24, FFP P = .19, PLT P = .29). Choice of RSI agent did not independently predict peri-induction systolic blood pressure or blood product administration. Rather, only presenting shock index independently predicted peri-induction hypotension. CONCLUSIONS: This is the first study to directly assess the peri-induction effects of anesthetic induction agent choice in penetrating trauma patients undergoing emergent hemorrhage control surgery. Propofol does not appear to worsen peri-induction hypotension regardless of dose. Patient physiology is most predictive of peri-induction hypotension.

Transcystic Laparoscopic Common Bile Duct Exploration for Pediatric Patients with Choledocholithiasis: A Multi-Center Study.

BACKGROUND: Patients with choledocholithiasis are often treated with endoscopic retrograde cholangiopancreatography (ERCP) followed by laparoscopic cholecystectomy (LC). Upfront LC, intraoperative cholangiogram (IOC), and possible transcystic laparoscopic common bile duct exploration (LCBDE) could potentially avoid the need for ERCP. We hypothesized that upfront LC + IOC ± LCBDE will decrease length of stay (LOS) and the total number of interventions for children with suspected choledocholithiasis. METHODS: A multicenter, retrospective cohort study was performed on pediatric patients (<18 years) between 2018 and 2022 with suspected choledocholithiasis. Demographic and clinical data were compared for upfront LC + IOC ± LCBDE and possible postoperative ERCP (OR1st) versus preoperative ERCP prior to LC (OR2nd). Complications were defined as postoperative pancreatitis, recurrent choledocholithiasis, bleeding, or abscess. RESULTS: Across four centers, 252 children with suspected choledocholithiasis were treated with OR1st (n = 156) or OR2nd (n = 96). There were no differences in age, gender, or body mass index. Of the LCBDE patients (72/156), 86% had definitive intraoperative management with the remaining 14% requiring postoperative ERCP. Complications were fewer and LOS was shorter with OR1st (3/156 vs. 15/96; 2.39 vs 3.84 days, p < 0.05). CONCLUSION: Upfront LC + IOC ± LCBDE for children with choledocholithiasis is associated with fewer ERCPs, lower LOS, and decreased complications. Postoperative ERCP remains an essential adjunct for patients who fail LCBDE. Further educational efforts are needed to increase the skill level for IOC and LCBDE in pediatric patients with suspected choledocholithiasis. LEVEL OF EVIDENCE: Level III.

Enteric tube position on preoperative radiographs predicts clinical outcomes in neonatal congenital diaphragmatic hernia with and without prenatal diagnosis.

OBJECTIVE: Congenital Diaphragmatic Hernia (CDH) is diagnosed prenatally in ~60% of cases. Prenatal measures typically guide management and prognostication. Simple postnatal prognosticators are needed when prenatal diagnosis is lacking. We hypothesized that preoperative orogastric tube (OGT) tip position relative to the contralateral diaphragm correlates with defect severity, resource utilization, and clinical outcomes regardless of diagnostic status. STUDY DESIGN: 150 neonates with left-posterolateral CDH were analyzed. Impact of intrathoracic and intraabdominal preoperative tip position on clinical outcomes was compared. RESULTS: Ninety-nine neonates were prenatally diagnosed. Overall, intrathoracic position significantly correlated with larger diaphragmatic defects, advanced postnatal pulmonary support requirements (HFOV, pulmonary vasodilators, and ECMO), operative complexity, longer hospitalization, and poorer survival to discharge. These observations persisted when analyzing only cases lacking prenatal diagnosis. CONCLUSIONS: Preoperative OGT tip position predicts defect severity, resource utilization, and outcomes in CDH. This observation enhances postnatal prognostication and care planning for neonates without a prenatal diagnosis.

The Ethics of Discharge Planning After Violent Injury.

INTRODUCTION: Victims of violence (VoV) are at disproportionate risk for future violence, making consideration of patient safety by Emergency Medicine (EM) physicians and Trauma Surgeons (TS) essential when discharge planning (DP) for VoV. Practice patterns and ethical perspectives in DP for VoV, and their respective scenario- and specialty-specific variations, are unknown. METHODS: We surveyed 118 EM and 37 TS physicians at a level 1 trauma center. Three clinical scenarios were presented (intimate partner violence, elder abuse, gun violence), each followed by four questions assessing practices and ethical dilemmas in DP. Responses were compared using Chi-Square testing. RESULTS: Response rate was 51.6%. EM physicians more frequently supported patient autonomy to proceed with a potentially unsafe discharge plan after an episode of Intimate Partner Violence (P = .013) and believed that admission could facilitate change in the victim's social situation after an episode of Elder Abuse (P = .026). TS physicians were more likely to offer social admission, providing additional time to navigate safe discharge planning (P = .003), less likely to see social admission as an inappropriate use of limited resources (P = .030) and less likely to support patient autonomy to proceed with a potentially unsafe discharge (P = .003) after gun-related violence. CONCLUSION: There appears to exist scenario- and specialty-specific variability in the practice patterns and ethical perspectives of EM and TS physicians when discharge planning for victims of violence. These findings highlight the need for further evaluation of specific factors underlying variability by situation and specialty, and their implications for patient-centered outcomes.

Infectious Complications and Antibiotic Use in Gastroschisis.

Background: Gastroschisis is a challenging neonatal condition often with prolonged hospitalizations, need for parenteral nutrition, infectious complications, and can even result in death. Infection is reported to occur in up to two-thirds of patients with gastroschisis and is a strong risk factor for increased morbidity and mortality. Increased days with a central venous catheter, complex gastroschisis, and delayed abdominal wall closure have been consistently found to be associated with increased risk of infection, whereas sutureless gastroschisis closure has been associated with fewer infections. Although one of the most common complications of gastroschisis is infection, the use of antibiotic agents varies widely with variability in the literature to guide management. Antibiotic usage should be selective and short-term, especially in neonates with simple gastroschisis regardless of method for abdominal wall closure. Conclusions: Future initiatives should focus on development of evidence-based guidelines on the care of these patients with the goal of reducing variability and improve outcomes within and across institutions.

Distribution and Surgical Management of Visceral Ewing Sarcoma Among Children and Adolescents.

INTRODUCTION: Ewing sarcoma (EWS) is a highly malignant tumor of bone and soft tissue that occasionally arises from viscera. Visceral EWS (V-EWS) is challenging to manage given its varied organ distribution and often late-stage presentation. We aimed to characterize our institutional experience with V-EWS, focusing on its surgical management, and to compare V-EWS outcomes against those with osseous (O-EWS) and soft tissue EWS (ST-EWS). METHODS: Retrospective review of all EWS patients ≤21 years presenting to a single institution between 2000 and 2022. Patient- and disease-specific characteristics were compared. Overall and relapse-free survival were estimated using Kaplan Meier methods and log-rank test. RESULTS: 156 EWS patients were identified: 117 O-EWS, 20 ST-EWS, and 19 V-EWS. V-EWS arose in the kidney (n = 5), lung (n = 5), intestine (n = 2), esophagus (n = 1), liver (n = 1), pancreas (n = 1), adrenal gland (n = 1), vagina (n = 1), brain (n = 1), and spinal cord (n = 1). No significant demographic differences were detected between EWS groups. V-EWS was more frequently metastatic at presentation (63.2%; p = 0.005), yet no significant overall or relapse-free survival differences emerged between EWS groups, with similar follow-up intervals. While V-EWS required multiple unique operative strategies to gain primary control, no significant difference in treatment strategies appeared between groups. Surgery-only primary control was associated with improved overall and relapse-free survival in all groups. CONCLUSIONS: V-EWS presents unique management challenges in children and adolescents given its variable sites of origin. This large cohort is the first to describe the surgical management and outcomes of V-EWS, demonstrating more frequent metastatic presentation, while achieving similar survival across groups. LEVEL OF EVIDENCE: Level 2 - Cohort Study.

Respiratory Distress: Three Patient Cases.

As mentioned in the January 2022 Pediatrics in Review Commentary, we now present three patients who have a common chief complaint followed by 5 questions for CME credit. All three cases have discussions on presentation, the differential diagnosis, and management that collectively serve as a Review article. The common theme here is that all three patients have difficulty breathing. We hope you will enjoy this review format.

Congenital Diaphragmatic Hernia Repair at the Bedside or Operating Theater.

BACKGROUND: For critically ill congenital diaphragmatic hernia (CDH) patients on high frequency oscillatory ventilation (HFOV), extracorporeal membrane oxygenation (ECMO), and/or inhaled nitric oxide (iNO), operative repair in the neonatal intensive care unit (NICU) has been proposed to avoid complications during transport to an operating room (OR). This study compared neonates with CDH who received herniorrhaphy in the NICU or OR, with a subgroup analysis considering only patients supported with ECMO. METHODS: Patients admitted to the NICU in the first 2 weeks of life at a free-standing children's hospital between July 2004 and September 2021 were examined. Patients were categorized according to location of CDH repair, and impact on operative complications and survival was compared. RESULTS: 185 patients were admitted to the NICU with posterolateral CDH and received operative repair. 48 cases were operated on at the bedside in the NICU and 137 in the OR. Patients repaired in the NICU had higher use of HFOV, pulmonary vasodilators, and ECMO (all P < .001). Children repaired in the NICU experienced significantly higher in-hospital death and overall mortality (P < .001). However, in multivariate analysis, repair location was not a significant predictor of survival to discharge in patients receiving ECMO. No significant difference in surgical site infection was detected for operative location (P = .773). DISCUSSION: Congenital diaphragmatic hernia repair in the NICU occurred more frequently among higher risk patients who experienced worse survival. The rate of surgical site infection appeared similar overall and across subgroups suggesting adequate sterility and technique for bedside procedures, when necessary, despite restricted access to advanced operative equipment.

A multicenter study evaluating the sensitization potential of enamel matrix derivative after treatment of two infrabony defects.

BACKGROUND: Several studies reported some success toward regeneration in infrabony defects using enamel matrix derivative (EMD). Clinically and statistically significant improvements in probing depth reduction, clinical attachment levels, and bone fill have been demonstrated. This multi-center study evaluated the potential for sensitization to EMD in a subgroup of periodontal patients treated at least twice with at least 2 months between treatments. METHODS: Three hundred seventy-six (376) patients in 11 university-based postgraduate periodontics programs and five private practices were selected. Surgeries were performed on infrabony defects. Following reflection of mucoperiosteal flaps and debridement of the root surface and defect, root conditioning (either citric acid pH = 1 or 24% EDTA) was performed and the site was irrigated with sterile saline. Enamel matrix derivative was reconstituted and applied to the exposed root surface and the bony defect. Flaps were sutured and pressure applied for 5 minutes. The second test defect was treated in a similar manner at least 8 weeks after the first surgery. The patient was given a diary card where any subjective adverse events (erythema, swelling, itching, headache, root hypersensitivity, or pain) were recorded at weeks 1 and 2 post-surgery. In addition, objective adverse events (gingival inflammation, ulcers, abscess, cratering, and lesions) were recorded by the investigator on an adverse event form. RESULTS: No clinical adverse reactions to multiple applications of EMD were noted. Of 376 patients, two were referred to a dermatologist for evaluation, but neither had signs indicating any adverse events due to EMD treatment. Instead their reactions were classified as a small local abscess and tinea cruris. The single immunoassay performed (on the patient with a small local abscess) did not demonstrate any EMD-reactive antibodies, neither IgE nor IgG. Other subjective/objective reactions that occurred during this study were of the type that are commonly experienced by patients immediately following periodontal surgery, but were not related to EMD. They included headache, swelling, itching, pain, and root hypersensitivity. CONCLUSIONS: This study demonstrated a lack of clinical adverse reactions following two separate applications of EMD. Any subjective/objective adverse reactions experienced by the patient were typical complications following routine periodontal surgery and were not directly related to the use of enamel matrix derivative.