Day to Day Advocacy by Pediatric Health Care Providers.

This manuscript highlights the impressive advocacy work that members of the American Academy of Pediatrics have achieved and serves to inspire pediatric health care providers of all specialties to pursue such efforts beyond the acute physical need of the child. This article represents one of the Symposia presented at the 2023 American Academy of Pediatrics Section on Surgery.

Does preoperative calcium and 1, 25 OH vitamin D supplementation impact postoperative hypocalcemia and length of stay following pediatric thyroidectomy?

OBJECTIVE: To investigate whether perioperative calcium and 1,25 OH vitamin D supplementation (PCDS) influences the rates of postoperative hypocalcemia and length of stay (LOS) following pediatric thyroidectomy. STUDY DESIGN: Retrospective Cohort Review. SETTING: Tertiary children's hospital. METHODS: 94 patients who underwent completion or total thyroidectomy with or without concomitant neck dissection from 2010 to 2020 at a single institution were included. Patients with pre-existing hypocalcemia or preoperative vitamin D insufficiency were excluded. Rates of postoperative hypocalcemia and LOS were compared for patients receiving PCDS to those receiving no supplementation. RESULTS: Thirty percent of patients with PCDS had documented postoperative hypocalcemia compared to 64% of patients without PCDS (p = 0.01). Patients with PCDS had a median LOS of 30 h compared to 36 h (p = 0.002). Multivariable analyses confirmed that patients with PCDS had lower odds of postoperative hypocalcemia (OR: 0.32, CI: 0.11, 0.89) and shorter LOS by 17 h (SE: 8, p = 0.04) after adjustment for confounders. CONCLUSION: PCDS is associated with significantly lower risk of hypocalcemia and shorter LOS. Standardizing preoperative care for pediatric patients undergoing thyroidectomy may decrease variability and improve outcomes following surgery.

The past, present and future of ACS NSQIP-Pediatric: Evolution from a quality registry to a comparative quality performance platform.

Quality and process improvement (QI/PI) in children's surgical care require reliable data across the care continuum. Since 2012, the American College of Surgeons' (ACS) National Surgical Quality Improvement Program-Pediatric (NSQIP-Pediatric) has supported QI/PI by providing participating hospitals with risk-adjusted, comparative data regarding postoperative outcomes for multiple surgical specialties. To advance this goal over the past decade, iterative changes have been introduced to case inclusion and data collection, analysis and reporting. New datasets for specific procedures, such as appendectomy, spinal fusion for scoliosis, vesicoureteral reflux procedures, and tracheostomy in children less than 2 years old, have incorporated additional risk factors and outcomes to enhance the clinical relevance of data, and resource utilization to consider healthcare value. Recently, process measures for urgent surgical diagnoses and surgical antibiotic prophylaxis variables have been developed to promote timely and appropriate care. While a mature program, NSQIP-Pediatric remains dynamic and responsive to meet the needs of the surgical community. Future directions include introduction of variables and analyses to address patient-centered care and healthcare equity.

Enhanced Recovery After Surgery: A Quality Improvement Approach.

Enhanced recovery after surgery (ERAS) protocols exist to optimize perioperative care for patients of all ages. The efficacy of ERAS protocols has been studied in various surgical specialties, including pediatric surgery and otolaryngology, but its role in pediatric otolaryngology has not been widely demonstrated in the literature. This review article attempts to assess the current state of ERAS within otolaryngology, pediatric surgery, and more specifically, pediatric otolaryngology to identify opportunities for future development and utilization.

Correlation Between Postoperative Antimicrobial Prophylaxis Use and Surgical Site Infection in Children Undergoing Nonemergent Surgery.

Importance: Use of postoperative antimicrobial prophylaxis is common in pediatric surgery despite consensus guidelines recommending discontinuation following incision closure. The association between postoperative prophylaxis use and surgical site infection (SSI) in children undergoing surgical procedures remains poorly characterized. Objective: To evaluate whether use of postoperative surgical prophylaxis is correlated with SSI rates in children undergoing nonemergent surgery. Design, Setting, and Participants: This is a multicenter cohort study using 30-day postoperative SSI data from the American College of Surgeons' Pediatric National Surgical Quality Improvement Program (ACS NSQIP-Pediatric) augmented with antibiotic-use data obtained through supplemental medical record review from June 2019 to June 2021. This study took place at 93 hospitals participating in the ACS NSQIP-Pediatric Surgical Antibiotic Prophylaxis Stewardship Collaborative. Participants were children (<18 years of age) undergoing nonemergent surgical procedures. Exclusion criteria included antibiotic allergies, conditions associated with impaired immune function, and preexisting infections requiring intravenous antibiotics at time of surgery. Exposures: Continuation of antimicrobial prophylaxis beyond time of incision closure. Main Outcomes and Measures: Thirty-day postoperative rate of incisional or organ space SSI. Hierarchical regression was used to estimate hospital-level odds ratios (ORs) for SSI rates and postoperative prophylaxis use. SSI measures were adjusted for differences in procedure mix, patient characteristics, and comorbidity profiles, while use measures were adjusted for clinically related procedure groups. Pearson correlations were used to examine the associations between hospital-level postoperative prophylaxis use and SSI measures. Results: Forty thousand six hundred eleven patients (47.3% female; median age, 7 years) were included, of which 41.6% received postoperative prophylaxis (hospital range, 0%-71.2%). Odds ratios (ORs) for postoperative prophylaxis use ranged 190-fold across hospitals (OR, 0.10-19.30) and ORs for SSI rates ranged 4-fold (OR, 0.55-1.90). No correlation was found between use of postoperative prophylaxis and SSI rates overall (r = 0.13; P = .20), and when stratified by SSI type (incisional SSI, r = 0.08; P = .43 and organ space SSI, r = 0.13; P = .23), and surgical specialty (general surgery, r = 0.02; P = .83; urology, r = 0.05; P = .64; plastic surgery, r = 0.11; P = .35; otolaryngology, r = -0.13; P = .25; orthopedic surgery, r = 0.05; P = .61; and neurosurgery, r = 0.02; P = .85). Conclusions and Relevance: Use of postoperative surgical antimicrobial prophylaxis was not correlated with SSI rates at the hospital level after adjusting for differences in procedure mix and patient characteristics.

Pediatric Battery-Related Emergency Department Visits in the United States: 2010-2019.

OBJECTIVES: To describe the epidemiology of battery-related emergency department (ED) visits among children aged <18 years in the United States from 2010 to 2019 and compare with previous study findings. METHODS: Data on ED visits were obtained from the National Electronic Injury Surveillance System. Using narrative descriptions and diagnosis codes, battery-related cases were coded into four exposure routes: (1) ingestion, (2) mouth exposure, (3) ear insertion, and (4) nasal insertion. RESULTS: An estimated 70 322 (95% confidence interval: 51 275-89 369) battery-related ED visits among children aged <18 years occurred during the study period, or 9.5 per 100 000 children annually. Button batteries were implicated in 84.7% of visits where battery type was described. A statistically significant increase in the ED visit rate occurred from 2010 to 2017 (P = .03), followed by a nonstatistically significant decrease from 2017 to 2019. The ED visit rate was highest among children aged ≤5 years compared with those 6 to 17 years (24.5 and 2.2 per 100 000 children, respectively). The mean patient age was 3.2 years (95% confidence interval: 2.9-3.4). Ingestions accounted for 90.0% of ED visits, followed by nasal insertions (5.7%), ear insertions (2.5%), and mouth exposures (1.8%). CONCLUSIONS: Pediatric battery-related ED visit rates continued to significantly increase from 2010 to 2017, with children aged ≤5 years having the highest rates. Prevention efforts have not significantly reduced injury rates; therefore, regulatory efforts are needed. Ultimately, hazard reduction or elimination through safer button battery design is critical and should be adopted by the battery industry.

Adverse Events in Infants Less Than 6 Months of Age After Ambulatory Surgery and Diagnostic Imaging Requiring Anesthesia.

AAP guidelines recommend infants less than 6 months of age are monitored for at least 2 hours following surgery. This retrospective study evaluated if adherence to the 2-hour monitoring guideline decreased the risk of adverse events associated with ambulatory procedures in infants younger than 6 months. Methods: We queried the hospital's electronic medical record to identify patients younger than 6 months of age who received anesthetic care from January 2015 to March 2020. Demographic data, intraoperative adverse events, and returns to the emergency department (ED) or urgent care within 7 days were captured for each patient. We calculated the number and frequency for categorical data and median and interquartile range (IQR) for continuous data. Chi-square or Fisher's exact test were used to compare patients who experienced an adverse event to those that did not. Results: One thousand one hundred seventy-seven patients who had 1,261 unique anesthetic encounters were analyzed. Forty-four adverse events were identified, 20 (1.6%) before discharge, including 3 unplanned admissions, and 24 (1.9%) returns to the ED/UC within 7 days postoperatively. We did not observe differences in postoperative recovery time in patients who experienced an adverse event and those who did not (88 min vs. 77 min, respectively, P = 0.078). None of the ED/UC returns would have been avoided by a longer PACU stay. Conclusions: With the appropriate patient selection, once physiological discharge readiness is met, adherence to a strict 2-hour time-based discharge criteria does not increase safety for infants younger than 6 months of age after ambulatory procedures.

Improving emergency airway cart efficiency in compliance with Joint Commission standards.

BACKGROUND AND OBJECTIVE: Emergency Airway Carts (EAC) are essential for pediatric otolaryngologists to provide rapid bedside care for emergent airway scenarios. At many institutions, EAC bronchoscopy equipment is individually peal-packaged due to Joint Commission (JC) standards, creating significant inefficiency in equipment assembly during time sensitive clinical settings. The objective of this quality improvement initiative was to improve the efficiency of use of our emergency airway cart equipment. METHODS: Individually peel-packaged bronchoscope equipment was replaced with JC compliant sets. Otolaryngology trainees (N = 8) and pediatric otolaryngology attending physicians (N = 11) were tested in a simulated airway emergency scenario, requiring bronchoscope assembly. Complete bronchoscope assembly and time to tracheal visualization (TTV) was measured for each participant, which started with initial clinical scenario presentation and ended with successful visualization of the trachea using the rigid bronchoscope. RESULTS: Pre-airway cart interventions, 68.4% of participants built a complete bronchoscope with no missing pieces, which improved to 100% with the new cart organization. Post-EAC interventions, all 19 participants reduced TTV significantly by a mean of 177.7 s (p < 0.001). Trainees reduced TTV by a mean of 251.2 s (46.2%, p < 0.0001) and attendings by 124.2 s (31.5%, p < 0.0022). All participants found the new airway cart sets easier to use and improved equipment setup efficiency. CONCLUSIONS: Compiling bronchoscope equipment into sterile sets allowed for improved TTV and bronchoscope quality for trainees and attending physicians while maintaining JC standards. Simulation improved confidence among both trainees and attending surgeons in providing optimal patient care in airway emergencies.

Examining the Utility of Preoperative Telemedicine Care Across Multiple Pediatric Surgery Disciplines.

INTRODUCTION: Telemedicine use within pediatric surgery fields has been growing, but research on the utility of remote evaluation in the perioperative period remains scarce. The objective of this study was to examine the utility of perioperative telemedicine care for the pediatric patient by evaluating the outcomes following completion of an outpatient appointment with a surgical provider. MATERIALS AND METHODS: We performed a retrospective chart review of all patients who completed a telemedicine appointment with a provider across nine pediatric surgery divisions, without a limitation based on patient-specific characteristics or telemedicine platform. We examined the result of the initial telemedicine appointment and the outcome of any surgical procedure that was performed as a result. RESULTS: A total of 803 patients were evaluated by telemedicine during the study period. Of the 164 encounters (20.2%) that were followed by a surgery, nearly 70% were performed using a video. There was no discordance in the preoperative and postoperative diagnoses for more than 98% of patients. Nearly 25% of operations were followed by at least a 1-night hospital stay and 6.7% of patients developed a postoperative complication. CONCLUSIONS: Telemedicine is a safe tool for evaluating pediatric patients in the preoperative and postoperative phases of care and offers potential value for families seeking an alternative to the traditional in-person appointment. Ongoing support will require permanent legislative changes aimed at ensuring comparable compensation and the development of strategies to adapt the outpatient healthcare model to better accommodate the evolving requirements of remotely evaluating and treating pediatric patients.

Button battery taping and disposal: Risk reduction strategies for the household setting.

OBJECTIVES: Pediatric esophageal button battery (BB) injury occurs rapidly and continues to be a significant source of morbidity and mortality. Unfortunately, a BB that no longer supplies power to a device can still have enough residual voltage to cause injury within the body. Development of additional prevention strategies for consumers may reduce esophageal injury risk. METHODS: In this study, 24 commercially available button batteries (BBs) were horizontally and vertically wrapped (2 layers, full circumferential coverage, 90° apart) with 6 different types of common household tapes (Scotch®/clear, Scotch®/Magic, masking tape, packing tape/clear, packing tape/brown, black electrical tape) and left at room temperature for 30 days. In addition, 6 of the CR2032 batteries covered with each type of tape were placed in a cadaveric piglet esophageal model for a 4-h period and then compared to controls without tape for tissue pH changes and visible tissue injury. RESULTS: None of the tape-wrapped batteries showed voltage changes nor presented any hazard stemming from BB ingestion. All 6 tape covered batteries placed in the cadaveric piglet esophageal tissue model demonstrated no visible tissue injury and no change in tissue pH in contrast to the controls. Review of BB packaging language from various brands of commercially available CR2032 batteries showed that none had specific disposal recommendations. CONCLUSION: Both BB and electronics manufacturers should consider instructing the use of common household tape options to cover these BB immediately after removal from a device for either recycling or disposal. Such precautions may help to reduce related ingestion injuries in children.

Comparison of the Fitbit® charge and polysomnography for measuring sleep quality in children with sleep disordered breathing.

BACKGROUND: Due to the cost and inconvenience of polysomnography (PSG), wrist-worn accelerometers have been explored as an alternative method to measure sleep efficacy in children with obstructive sleep apnea (OSA) or sleep-disordered breathing (SDB). We compared the measurement of sleep quality with the Fitbit® charge (Fitbit Inc., San Francisco, CA, USA) compared to PSG in children presenting for sleep studies. METHODS: Children ages 3 to 18 years presenting for PSG with persistent SDB symptoms were enrolled. During PSG, the Fitbit® charge (Fitbit Inc.) was placed on the wrist and time-synchronized with sleep laboratory devices, which were worn while a single-night PSG was performed. Bias and concordance in measurements of total sleep time (TST), total wake time (TWT), and number of awakenings were assessed using paired t-tests, sign-rank tests, and Lin's concordance coefficient. RESULTS: The study cohort included 22 patients (9 boys and 13 girls; 9±3 years). TST was significantly overestimated using the Fitbit® charge (Fitbit Inc.) (difference in means: 30 min; 95% confidence interval [CI] of difference: 3-58; P=0.031), while TWT was underestimated (difference: 23 min; 95% CI: 4-42; P=0.018). All measures showed a lack of concordance between the Fitbit® charge (Fitbit Inc.) and PSG. CONCLUSIONS: The current prospective study confirms that the Fitbit® charge (Fitbit Inc.) overestimates time spent asleep compared to PSG in children with OSA/SDB symptoms, limiting the validity of sleep monitoring with wearable activity trackers appears in these patients.

Survival of Toddler with Aortoesophageal Fistula after Button Battery Ingestion.

Button batteries (BBs) are found in many households and are a source of esophageal foreign body in the pediatric population. Upon ingestion, significant caustic injury can occur within 2 hours leading to tissue damage and severe, potentially fatal sequelae. Aortoesophageal fistula (AEF) is a rare complication that nearly always results in mortality. We report a rare case of a toddler who developed an AEF after BB ingestion and survived following staged aortic repair. There should be a high index of suspicion for this complication with the history of BB ingestion and presence of hematemesis, hemoptysis, or melena.

Topical Nasal Decongestant Oxymetazoline: Safety Considerations for Perioperative Pediatric Use.

The over-the-counter nasal decongestant oxymetazoline (eg, Afrin) is used in the pediatric population for a variety of conditions in the operating room setting. Given its vasoconstrictive properties, it can have cardiovascular adverse effects when systemically absorbed. There have been several reports of cardiac and respiratory complications related to use of oxymetazoline in the pediatric population. Current US Food and Drug Administration approval for oxymetazoline is for patients ≥6 years of age, but medical professionals may elect to use it short-term and off label for younger children in particular clinical scenarios in which the potential benefit may outweigh risks (eg, active bleeding, acute respiratory distress from nasal obstruction, acute complicated sinusitis, improved surgical visualization, nasal decongestion for scope examination, other conditions, etc). To date, there have not been adequate pediatric pharmacokinetic studies of oxymetazoline, so caution should be exercised with both the quantity of dosing and the technique of administration. In the urgent care setting, emergency department, or inpatient setting, to avoid excessive administration of the medication, medical professionals should use the spray bottle in an upright position with the child upright. In addition, in the operating room setting, both monitoring the quantity used and effective communication between the surgeon and anesthesia team are important. Further studies are needed to understand the systemic absorption and effects in children in both nonsurgical and surgical nasal use of oxymetazoline.

Current management of button battery injuries.

Button batteries (BB) are found in common household items and can lead to significant morbidity and mortality in the pediatric population when ingested. BBs are made of various chemistries and have a unique size and shape that yield significant injury when lodged in the pediatric esophagus. BBs create a local tissue pH environment of 10 to 13 and can induce liquefactive necrosis at the negative pole. This initial injury can progress with further tissue breakdown even after removal. Unfortunately, patients may present with vague symptoms similar to viral illnesses and there is not always a known history of ingestion. Plain film X-ray can be diagnostic. Exposure can lead to caustic injury within 2 hours. Thus, timely endoscopic removal is the mainstay of treatment. Novel mitigation and neutralization strategies have been implemented into treatment guidelines. These include the preremoval ingestion of honey or sucralfate and intraoperative irrigation with acetic acid. Depending on the severity of injury following removal, careful consideration should be given for potential delayed complications including fistulization into major vessels which often leads to death. The National Button Battery Taskforce and several industry members have implemented prevention strategies such as educational safety outreach campaigns, child-resistant packaging changes, and warning labels. Governmental regulation and industry changes are key to limit not only the amount of BB ingestions, but also the devastating consequences that can result. Anonymous reporting of BB injuries through the Global Injury Research Collaborative has been made convenient and centralized through the advent of a user-friendly smartphone iOS/App Store and Android/GooglePlay application called the "GIRC App"; all specialists who manage foreign body cases should contribute their cases to help prevent future injuries. BB ingestion must be recognized and treated promptly using a multidisciplinary approach to optimize outcomes for these patients. Ultimately, a safer BB technology is critically needed to reduce or eliminate the severe and life-threatening injuries in children. LEVEL OF EVIDENCE: 5.

Recognizing the Benefit of Telemedicine Before and After COVID-19: A Survey of Pediatric Surgery Providers.

BACKGROUND: Prior to the COVID-19 pandemic, the use of telemedicine to evaluate pediatric surgery patients was uncommon. Due in part to restrictions imposed to mitigate the spread of the virus, the use of telemedicine within pediatric surgery has significantly expanded. METHODS: Prior to the use of telemedicine within surgery divisions at our institution, pediatric surgeons were surveyed to determine their perspectives on the use of telemedicine. Following the expanded use of telemedicine in response to the COVID-19 pandemic, a follow up survey was distributed to determine the impact of telemedicine and the perceived benefits and barriers of continuing its use going forward. RESULTS: The pre-COVID survey was completed by 37 surgeons and the post-COVID survey by 36 surgeons and advanced practice providers across 10 pediatric surgical divisions. General surgeons were the most represented division for both the pre- (25%) and post-COVID (33.3%) survey. Less than 25% of providers reported use of telemedicine at any point in their career prior to COVID-19; but following the expanded use of telemedicine 95% of respondents reported interest in continuing its use. After expansion, 25% of respondents were concerned with the possibility of inaccurate diagnoses when using telemedicine compared to nearly 50% prior to expanded use. CONCLUSION: Following the expanded use of telemedicine within pediatric surgery, there was a decrease in the concern for inaccurate diagnoses and a near uniform desire to continue its use. Going forward, it will be imperative for pediatric surgeons to take an active role in creating a process for implementing telemedicine that best fits their needs and the needs of their patients and patients' families.

Current management of aerodigestive foreign bodies in children.

Foreign bodies ingestion or aspiration events in children can be a source of morbidity and mortality. In most cases, removal by a coordinated endoscopy can be performed when there is the availability of equipment and support staff for pediatric care. Close communication with the anesthesia, surgeon and operating room support staff is essential and rehearsing with a facsimile of the foreign body is useful. In certain cases, such as sharp foreign bodies or button batteries the endoscopy should be performed immediately.

The value of telemedicine for the pediatric surgery patient in the time of COVID-19 and beyond.

BACKGROUND: Prior to COVID-19, the use of telemedicine within pediatric surgery was uncommon. To curb the spread of the virus many institutions restricted non-emergent clinic appointments, resulting in an increase in telemedicine use. We examined the value of telemedicine for patients presenting to a pediatric surgery clinic before and after COVID-19 METHODS: Perspectives and the potential value of telemedicine were assessed by surveying patients or caregivers of patients being evaluated by a general pediatric surgeon in-person prior to COVID-19 and by patients or caregivers of patients who completed a telemedicine appointment with a pediatric surgical provider during the COVID-19 period. RESULTS: The pre-COVID survey was completed by 57 respondents and the post-COVID survey by 123. Most respondents were white and were caregivers 31-40 years of age. Prior to COVID-19, only 26% were familiar with telemedicine, 25% reported traveling more than 100 miles and >50% traveled more than 40 miles for their appointment. More than 25% estimated additional travel costs of at least $30 and in 43% of households, at least one adult had to miss time from work. Following a telemedicine appointment during the COVID-19 period, 76% reported the care received as excellent, 86% were very satisfied with their care, 87% reported the appointment was less stressful for their child than an in-person appointment, and 57% would choose a telemedicine appointment in the future. CONCLUSION: For families seeking an alternative to the in-person encounter, telemedicine can provide added value over the traditional in-person encounter by reducing the burden of travel without compromising the quality of care. Telemedicine should be viewed as a viable option for pediatric surgery patients and future research directed toward optimizing the experience for patients and providers. LEVEL OF EVIDENCE: III.

Opioid-Sparing Anesthetic Technique for Pediatric Patients Undergoing Adenoidectomy: A Pilot Study.

INTRODUCTION: An opioid-sparing anesthetic involves a multi-modal technique with non-opioid medications targeting different analgesic pathways. Such techniques may decrease adverse effects related to opioids. These techniques may be considered in patients at higher risk for opioid-related adverse effects including obstructive sleep apnea or sleep disordered breathing. METHODS: A prospective, pilot study was performed in 10 patients (3-8 years of age), presenting for adenoidectomy. The perioperative regimen included oral dextromethorphan (1 mg/kg) and acetaminophen (15 mg/kg) plus single boluses of intraoperative dexmedetomidine (0.5 µg/kg) and ketamine (0.5 mg/kg). Pain scores were assessed in the post anesthesia care unit (PACU) using the FLACC (Face, Legs, Activity, Cry, Consolability) scale. Patients with a pain score >4 received fentanyl as needed. PACU time, pain scores, and parent satisfaction were recorded. Postoperatively, patients were instructed to use oral acetaminophen or ibuprofen every 6 hours as needed for pain. RESULTS: The study cohort included 10 patients, 3-8 years of age. All patients had opioid-free anesthetic care. PACU time ranged from 24 to 102 minutes (median: 56 minutes). FLACC pain scores were 0 for all PACU assessments. Nine patients were discharged home and 1 patient had a planned overnight admission. Following hospital discharge, the pain scores were satisfactory during the 72-hour study period and 90% of the patients' guardians were satisfied or highly satisfied with their child's pain control. CONCLUSION: This opioid-sparing approach provided safe and effective pain control as well as parental satisfaction following adenoidectomy in children. Additional prospective studies are needed to determine whether this regimen is effective in a larger cohort of patients with and for other otolaryngology procedures.

Infantile fibrosarcoma-like tumor driven by novel RBPMS-MET fusion consolidated with cabozantinib.

Infantile fibrosarcoma (IFS) is nearly universally driven by gene fusions involving the NTRK family. ETV6-NTRK3 fusions account for ∼85% of alterations; the remainder are attributed to NTRK-variant fusions. Rarely, other genomic aberrations have been described in association with tumors identified as IFS or IFS-like. We describe the utility of genomic characterization of an IFS-like tumor. We also describe the successful treatment combination of VAC (vincristine, actinomycin, cyclophosphamide) with tyrosine kinase inhibitor (TKI) maintenance in this entity. This patient presented at birth with a right facial mass, enlarging at 1 mo to 4.9 × 4.5 × 6.3 cm. Biopsy demonstrated hypercellular fascicles of spindle cells with patchy positivity for smooth muscle actin (SMA) and negativity for S100, desmin, myogenin, and MyoD1. Targeted RNA sequencing identified a novel RBPMS-MET fusion with confirmed absence of ETV6-NTRK3, and the patient was diagnosed with an IFS-like tumor. A positron emission tomography (PET) scan was negative for metastatic disease. VAC was given for a duration of 10 mo. Resection at 13 mo of age demonstrated positive margins. Cabozantinib, a MET-targeting TKI, was initiated. The patient tolerated cabozantinib well and has no evidence of disease at 24 mo of age. We describe a novel RBPMS-MET driver fusion in association with a locally aggressive IFS-like tumor. MET functions as an oncogene and, when associated with the RNA binding protein RBPMS, forms an in-frame fusion product that retains the MET kinase domain. This fusion is associated with aberrant cell signaling pathway expression and subsequent malignancy. We describe treatment with cabozantinib in a patient with an IFS-like neoplasm.