Medical Specialties Commons^™

Posterior Reversible Encephalopathy Syndrome After Dinutuximab, Kayeleigh Higgerson Do, Chandni Dargan Md

Research Days

Background: Posterior reversible encephalopathy syndrome (PRES) is a reversible disorder associated with edema of subcortical white matter and cortical gray matter that usually involves the occipital and parietal lobes. Endothelial injury secondary to abrupt blood pressure changes breaks down the blood brain barrier leading to brain edema. Per the ANBL0032 Fall 2015 Study Progress Report, Dinutuximab was the probable cause of PRES in two patients. There have been published reports of PRES with other anti-GD2 antibody therapies but very few reports of Dinutuximab as the causative agent.

Objectives/Goal: To report a case of PRES after Dinutuximab

Methods/Design: Case Report

Results: …

Levofloxacin Versus Ciprofloxacin Prophylaxis In Pediatric Cancer Patients At High Risk Of Infection, Chandni Dargan Md, Amy Johnson Md, Mba

Research Days

Background: Patients with cancer and those undergoing chemotherapy are at risk of developing bacterial infections due to myelosuppression. Patients undergoing the most intensive chemotherapy regimens are at a higher risk for morbidity and mortality due to profound neutropenia. Antibacterial prophylaxis is given to reduce the incidence of infection in those at highest risk. Starting March 1, 2016 our institution used ciprofloxacin for antibacterial prophylaxis however recent literature, including the COG trial ACCL0934, supports using levofloxacin in certain high risk (HR) populations due to greater efficacy in reducing neutropenic fever (NF) and bacteremia. Therefore, we switched to this April 1, 2019. …

A Pediatric Case Of Treatment-Related Myelodysplastic Syndrome While On Therapy For Pre-B All, Sara Mcelroy

Research Days

Background: Treatment-related myelodysplastic syndrome (t-MDS) is a known, but rare, late effect of cancer therapy, specifically radiation, alkylating agents or topoisomerase II inhibitors. When secondary to treatment with alkylating agents, t-MDS typically occurs 4 to 7 years after therapy, and common cytogenetics include chromosomes 5 and 7 abnormalities. Treatment in children with t-MDS is typically allogeneic stem cell transplant, but the prognosis remains poor.

Objectives/Goal: To describe a rare case of a pediatric patient who developed t-MDS while receiving treatment for Pre-B Cell Acute Lymphoblastic Leukemia (pre-B ALL) and to outline the treatment regimen that he received.

Methods/Design: Case Report …

Therapeutic Plasma Exchange In Critically Ill Pediatric Patients With Leukemia, Sarah E. Mc Dermott, Chandni Dargan

Research Days

Background: Therapeutic plasma exchange (TPE) has well-documented applications in the adult population, outlined by the American Society of Apheresis (ASFA) 2019 guidelines. Limited data exists regarding the use of TPE in critically ill pediatric patients, however these reports rarely include patients with oncological diseases. Care for these patients poses certain clinical considerations including the safety of continuing chemotherapy, delayed clearance of chemotherapy secondary to end organ damage and mechanical clearance (i.e., continuous renal replacement therapy (CRRT) circuitry), and ability to tolerate chemotherapy once recovered. We aim to highlight the potential benefits of TPE in their acute management, so that it …

Atrial Standstill In A Pediatric Patient With Scn5a Mutation Following Procainamide Challenge, Anmol Goyal

Research Days

Background: Atrial standstill (AS) is a rare arrhythmia characterized by absence of electrical and mechanical atrial activity associated with SCN5A channelopathy.

Case: An 18 year old male with structurally normal heart, frequent sinus pauses, nonsustained atrial tachycardia and high-grade block was found to have SCN5A mutation c.3823G>A (p.Asp1275Asn). An electrophysiology study (EPS) with high density voltage mapping of the right atrium was done (Fig 1a). Nonsustained multifocal atrial tachycardia was induced without ablative targets (Fib 1b). Procainamide challenge was negative for Brugada, however induced AS (Fig 1c-d). No atrial capture could be achieved at maximal output. Empiric atrial lead …

Reconsidering Perioperative Antibiotic Use In Elective Laparoscopic Cholecystectomy, Kayla B. Briggs

Research Days

Background: As rates of antimicrobial resistance increase, the use of prophylactic perioperative antibiotics (PPA) has been questioned in cases with a low risk of surgical site infection (SSI). In laparoscopic cholecystectomy performed for biliary dyskinesia, hyperkinetic gallbladder, and gallbladder polyps, the use of PPA varies with no widely accepted practice pattern.

Objectives/Goal: We examined institutional PPA usage and SSI rates for elective laparoscopic cholecystectomy to determine if PPAs are indicated.

Methods/Design: Following IRB approval, children/2010 and 8/2020 for symptomatic cholelithiasis, biliary dyskinesia, hyperkinetic gallbladder, and/or gallbladder polyps were included. Several surgeons changed practice to selective PPA use in 2016, decreasing …

Do Males With 45,X/46,Xy Mosaicism Have Turner Syndrome, Michelle Knoll

Research Days

Background: Patients with 45,X/46,XY mosaicism can have genitalia spanning the range from typical female to typical male, and the sex assigned at birth is often based on the appearance of the genitalia. According to current nomenclature, only the subgroup assigned female are diagnosed with Turner syndrome (TS) (Gravholt et al, 2017). Clinical practice guidelines recommend that girls with TS be screened for several comorbidities, including short stature, cardiac and renal anomalies, and autoimmune conditions, but there are no recommendations for screening of boys with 45,X/46,XY mosaicism.

Objectives/Goal: We sought to determine if boys with 45,X/46,XY mosaicism exhibited a similar rate …

Changes In Family Functioning During The First Year Of Pediatric Cancer Treatment: A Mixed-Method Pilot Study, Carolyn Bates

Research Days

Background: A new diagnosis of pediatric cancer presents a sudden and tremendous stressor for families of all backgrounds and circumstances (Long & Marsland, 2011; Bemis et al., 2015). Although many families are resilient in the context of new cancer diagnosis, approximately 33% are at risk for negative outcomes due to psychosocial distress and difficulties with effective coping (Kazak et al., 2020; Pai et al., 2008), supporting the need for systematic assessment of psychosocial health as a standard of care in pediatric oncology (Kazak et al., 2015). Alongside heightened stress within the first year of diagnosis, parents may relax family rules …

Impact Of Covid-19 On Families With A Child In Cancer Treatment, Mirae J. Fornander

Research Days

Background: A new diagnosis of pediatric cancer diagnosis is a sudden and tremendous stressor to families (Long & Marsland, 2011). The COVID-19 pandemic presented an acute, universal stressor that impacted daily life around the world (Brooks et al., 2020). The impact of the COVID-19 pandemic on children with cancer and their families is not yet known.

Objectives/Goal: The current study is a pilot investigation of COVID-19 exposure and impact among a sample of families with a child being treated for cancer.

Methods/Design: Primary caregivers (N=22) of a child (M age=7.4, SD=4.6) who was diagnosed with cancer within the last 18 …

Pharmacogenetic Testing In Patients With Autism Spectrum Disorder Evaluated In The Children’S Mercy Hospital Goldiloks© Clinic, Rachel Goodson

Research Days

Background: Autism Spectrum Disorder (ASD) affects 1 in 54 children in the United States. Children with ASD are more likely to be diagnosed with co-occurring mental health disorders. There is currently little research guiding medication choice and dosing in patients with ASD. Children with ASD are at increased risk for reduced clinical response and adverse reaction (including due to polypharmacy). Pharmacogenomics is an approach for medication decision making that leverages an individual’s genetic information and clinical presentation to make informed choices, but no studies have specifically investigated the outcomes of PGX for patients with ASD. Understanding the clinical and genetic …

Outcomes Of Infants With Severe Rop At Risk Of Treatment With Avastin Compared To Laser Surgery, Stephen Doxey

Research Days

Background: Retinopathy of prematurity (ROP) is a well-known complication occurring in the most premature infants leading to visual impairment and in the most severe cases associated with retinal detachment and childhood blindness. Factors contributing the development of ROP include chronic hypoxia and disordered vascular genesis primarily through the vascular endothelial growth factor (VEGF) signaling pathway. Infants at risk of developing ROP are screened with serial eye exam and treated with either laser surgery or intraocular injects of bevacizumab (Avastin) to prevent progression of ROP and preserve vision. Bevacizumab, an inhibitor of VEGF signaling, may have positive effects on preventing ROP …

Hyperglycemia Requiring Insulin Among Pediatric Patients Diagnosed With Hemophagocytic Lymphohistiocytosis, Cintya Schweisberger

Research Days

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder marked by massive cytokine release due to macrophage and T-cell activation. Hallmarks of the diagnosis include fever, splenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogemia, and elevations in ferritin and soluble IL-2 receptor. Given HLH is associated with critical illness, elevation in inflammatory markers, and treated with glucocorticoids, the development of hyperglycemia during its course is not unexpected. However, detailed descriptions of the severity of hyperglycemia and strategies in insulin management among HLH patients are lacking. We describe 10 years’ experience at a single tertiary pediatric health center with HLH patients who developed insulin dependent …

The Power Of Outpatient Botulinum Injections In Preventing Hirschsprung Associated Enterocolitis, Wendy Jo Svetanoff

Research Days

Background: Hirschsprung associated enterocolitis (HAEC) is a potentially life-threatening complication, where the child often presents with obstructive symptoms. Failure of the internal anal sphincter (IAS) to relax is thought to play a role in these symptoms. Botulinum toxin (BT) injections assist in temporarily relieving obstructive symptoms due to IAS achalasia in patients with Hirschsprung’s disease following surgical pull-through. The use of BT may play a role in preventing HAEC episodes related to IAS dysfunction.

Objectives/Goal: Our aim was to determine the effect of scheduled outpatient Botulinum injections on the development of HAEC.

Methods/Design: A retrospective review between July 2010 – …

Iv Amiodarone Vs Iv Sotalol Use In Postoperative Junctional Ectopic Tachycardia (Jet): A Randomized Study, Tanner Isaacson

Research Days

Background: Junctional ectopic tachycardia (JET) is one of the most common post-operative arrhythmias encountered following congenital heart surgery. For many, amiodarone is the pharmacologic treatment of choice. While effective, amiodarone has been associated with dose-related adverse effects. Sotalol has been approved for intravenous use in pediatric patients in the United States to treat both supraventricular and ventricular arrhythmias, although pediatric data remains limited. Sotalol has class III antiarrhythmic properties with some mild β-adrenergic blocking effects, similar to amiodarone. Data shows a lower rate of cardiovascular collapse and adverse events with sotalol in patients with congenital heart disease. At present, it …

Long-Term Outcomes And Satisfaction Rates After Costal Cartilage Resection For Slipping Rib Syndrome, James Fraser

Research Days

Background/Objectives/Goal: Slipping rib syndrome (SRS) is a challenging and underdiagnosed condition that has proven to be an elusive diagnosis due to an extensive differential with presenting symptoms that mimic a myriad of alternative diagnoses of both thoracic and abdominal pathology. The diagnosis of SRS is recognized in a patient with consistent, progressive, often debilitating unilateral or bilateral chest wall and/or upper abdominal pain with intermittent clicking or rib slipping. SRS frequently does not resolve with conservative management, including physical therapy, rest, and NSAIDs, as well as intercostal injections, and even opioid medications. Workup can be extensive including multiple subspecialty referrals …

Multimodal Pain Control In Common Neonatal Surgeries: Post-Operative Pain Protocol Reduces Opiate Exposure And Side Effects, Jamesia Donato

Research Days

Background: Opioids are the primary post-operative (post-op) analgesic in neonates. Side effects include hypotension, apnea and ileus. Previous studies show IV acetaminophen decreases opiate need in specific populations including cleft palate repair, cardiac surgery. The effect of IV acetaminophen as part of a post-op pain algorithm is not known in common neonatal surgical procedures.

Objectives/Goal: To determine if (a) a post-op pain algorithm including IV acetaminophen and (b) IV acetaminophen exposure reduces cumulative opioid requirements in neonates undergoing common surgeries in the NICU.

Methods/Design: This is a retrospective cohort study comparing cumulative post-operative opiate exposure between infants with common surgeries …

The Effect Of Covid-19 On Pediatric Orthopaedic Trauma Volume At A Level 1 Pediatric Trauma Center In The Midwestern United States, Kirsten Norrell

Research Days

Background: School and playground closures and the cancellation of extracurricular activities in the Kansas City region on March 17, 2020 due to the COVID-19 pandemic created an unprecedented situation for children and parents. Early reports in the literature on adult and pediatric trauma volume suggested a decrease in orthopaedic injuries during the pandemic. This is the first study to report how the pandemic affected orthopaedic trauma volume at a Level 1 pediatric trauma center during the entire five months since school and activity closures went into effect.

Objectives/Goal: The aim of this study was to compare the volume and type …

Pre-Hospital Traumatic Cardiopulmonary Arrest In Children At A Level 1 Pediatric Trauma Center, Kayla B. Briggs

Research Days

Background: The survival of traumatic cardiopulmonary arrest (TCA) requiring pre-hospital cardiopulmonary resuscitation (P-CPR) is abysmal across age groups.

Objectives/Goal: We aim to describe the patterns of injury, pre-hospital interventions, and outcomes of children suffering from TCA leading to P-CPR at our institution to support standardized guidelines addressing the termination of prolonged resuscitation attempts in children.

Methods/Design: Following IRB approval, retrospective review was conducted to identify children ages 0-17 years who suffered TCA leading to P-CPR at our institution between 5/1/2009–3/1/2020. For analysis, patients were stratified into those still undergoing CPR at arrival and those who attained pre-hospital return of spontaneous …

Improving Thromboprophylaxis In Hospitalized Pediatric Inflammatory Bowel Disease Patients- A Quality Improvement Project, Amy Issa, Panam Kaur

Research Days

Background/Project Intent (Aim Statement): Pediatric inflammatory bowel disease (IBD) patients in acute flare have 1.5 to 3-fold higher risk of thromboembolism compared to the general population. Anticoagulation therapy is recommended for pediatric IBD patients who have ≥ 1 risk factors for venous thromboembolic events. At Children’s Mercy Hospital (CMH), approximately 80 patients/year are admitted for acute flare, of which, roughly 30% receive appropriate thromboprophylaxis. Lack of standardization of thromboprophylaxis and an educational gap amongst providers are significant barriers. Our goal is to standardize and improve the rates of thromboprophylaxis in IBD patients admitted at CMH from 30% to 60% by …

Mini-Ace® Low-Profile Appendicostomy Button, Joseph Lopez

Research Days

Purpose: Malone antegrade continence enemas (MACE) provide a conduit in which the patient can achieve improved continence, be clean of stool, and gain independence in maintaining bowel function. The Mini-ACE® is a low-profile balloon button that is used to facilitate administration of antegrade enemas. We sought to describe our practice and short-term outcomes.

Methods: We present our experience using the Mini-ACE® at Children’s Mercy – Kansas City from April 2019 to September 2020. Patient demographics, colorectal diagnoses, tube sizes, flush regimens, patient experience and outcomes were examined.

Results: Thirty patients were included; 18 (60%) were male. The average age at …

Umbilical Access In Laparoscopic Surgery In Infants Less Than 3 Months: Single Institution Retrospective Review, James Fraser

Research Days

Background/Objectives/Goal: Umbilical access in laparoscopic surgery has been cited as a potential factor for increased complications in low-birth-weight infants and those less than three months old. Previous series noted a self-reported complication rate of 10.6% among 329 pediatric surgeons via anonymous survey, citing carbon dioxide (CO2) embolism as the most common complication. We report four-year outcomes with blunt transumbilical laparoscopic access to examine the safety of this technique.

Methods/Design: Following IRB approval, a retrospective database of patients less than three months of age who underwent laparoscopic pyloromyotomy or inguinal hernia repair from 2016-2019 at a tertiary care academic center was …

Pediatric Pulmonary Artery Aneurysms Causing Pulmonary Hemorrhage: A Case Of Transcatheter Intervention While On Va Ecmo Support, Igor Areinamo

Research Days

Background: The incidence of pulmonary artery aneurysm (PAA) in children remains unknown. There are few reports of a transcutaneous interventional approach to address this type of pathology. Management of PAAs while on VA ECMO has not been described in pediatrics.

Objectives/Goal:

Methods/Design:

Case: 9-year-old previously healthy male presented with MRSA associated ARDS and sepsis requiring urgent VA ECMO cannulation. Chest CT done on ECMO day 22 showed development of right sided PAAs. Patient had an episode of significant pulmonary hemorrhage. Angiogram demonstrated two fusiform right PAAs, one of which had ruptured and was bleeding into the lung parenchyma. Patient had …

Congenital Mitral Valve Regurgitation, The Dilemma Of Repair Vs Replacement., Bianca Cherestal

Research Days

Background: Congenital mitral regurgitation is a rare condition and can be challenging to manage when presenting in the neonatal period

Objectives/Goal: Two week old male presented with poor weight gain, murmur and cardiomegaly on chest X-ray. Echocardiogram showed moderate to severe mitral regurgitation (MR) and suprasystemic pulmonary hypertension (PHN) (fig 1 a, b). The mitral valve (MV) leaflets were thickened and tethered with failure of central coaptation. PHN was classified as WHO I and II (due to persistent PHN of newborn and MR respectively). Inhaled nitric oxide, Enalapril and Furosemide were initiated. Cardiac catheterization revealed PVRi of 8.9 WU x …

Online Echocardiography Modules For Pediatric Cardiology Fellows, Sarah Studyvin, Doaa Aly, Tyler Johnson, Laura Kuzava, Alison Samrany, Nitin Madan, Sanket Shah

Research Days

Background: Adequate echocardiographic (echo) education is integral to successful cardiology training. Online education is proven to be effective in other specialties, but pediatric echo training data are lacking.

Objectives/Goal: Our aim was to design and assess the role of a novel online module-based curriculum to enhance pediatric cardiology fellows’ echo education.

Methods/Design: Four interactive modules, focused on principles and interpretation of a normal echo, were delivered to fellows. Content included high quality 2D and 3D echo clips as well as interactive quizzes and could be accessed from a phone, tablet, or computer. All fellows completed pre- and post-tests, validated by …

The Impact Of Early Tracheostomy On Neurodevelopmental Outcome Of Infants With Severe Bronchopulmonary Dysplasia Exposed To Postnatal Steroids, Amjad Taha

Research Days

Background: BPD is associated with long-term neurodevelopmental impairment. Tracheostomy is performed in 5-12% of severe BPD for prolonged ventilation. There is evidence that chronic ventilation with tracheostomy in severe BPD may facilitate neurodevelopment and lead to improved outcome. However, there is no consensus on the optimal timing of tracheostomy. A large multicenter study of infants with tracheostomy performed at <120 days of life had better neurodevelopmental outcomes at 18-22 months of age. Use of steroids postnatally to ameliorate the severity of BPD had been controversial due to its negative effect on neurodevelopment. To date, no data has specifically evaluated the impact of early tracheostomy on neurodevelopmental outcome of infants with severe BPD who are exposed to postnatal steroids.

Objectives/Goal: To compare the cognitive, language and motor scores among 3 groups of severe BPD infants who received early vs late vs no tracheostomy. Secondly, evaluate if postnatal steroids had an additive negative effect on neurodevelopmental outcomes.

Methods/Design: IRB approved retrospective …

Efficacy Of Using Antibiotic Cocktail In Medically Refractory Colitis/Inflammatory Bowel Disease, David Aaron Simon

Research Days

Background: Antibiotic cocktail targeting intestinal bacteria may offer a promising approach in certain patients with medically refractory IBD. It may find a place in serving as a bridge to more effective long-term medical therapies and in some cases help in achieving clinical remission in patients with refractory disease as an adjunctive therapy. Its main attractions include efficacy in about 50% of medically refractory patients and a non-immunosuppressive adjunct in treatment of children who may otherwise suffer from complications of untreated disease or heavy immunosuppression. The main drawbacks are lack of understanding of true mechanism of action, potential development of drug …

Bar Removal Following Minimally Invasive Pectus Excavatum Repair – Does Removal At 2 Years Affect Recurrence Or Satisfaction Rates?, Wendy Jo Svetanoff

Research Days

Background: Some patients with pectus excavatum require bar removal despite having the bar in for less than three years after minimally invasive placement (MIRPE). It is unknown whether early removal is associated with a higher recurrence rate or lower cosmetic satisfaction.

Objectives/Goal: The aim of this study was to review post-operative outcomes, specifically recurrence rate, and patient satisfaction in patients who underwent bar removal prior to three years.

Methods/Design: A retrospective review was performed of patients who underwent MIRPE between October 2006 and June 2017 and had bar removal less than 3 years after repair. Demographics, reason for bar removal, …

Constrictive Pericarditis After Repair Of A Ruptured Sinus Of Valsalva, Sarah Studyvin, Laura Kuzava

Research Days

Background: Constrictive pericarditis is an uncommon complication of cardiac surgery. We report a patient who developed constrictive pericarditis after ruptured sinus of Valsalva (RSOV) repair.

Methods: A 23-year-old male presented with exertional dyspnea one year after RSOV repair. TTE showed a small, circumferential effusion with thickened pericardium, ventricular septal bounce, left atrial enlargement, diastolic hepatic flow reversal, and trivial mitral regurgitation without stenosis. He underwent cardiac catheterization, which revealed elevated filling pressures (RVEDP 16 mmHg, LVEDP 18 mmHg), RVEDP/RVSP ratio < 0.5, and a low cardiac index (1.65 L/min/m2). Cardiac MRI confirmed pericardial thickening with paradoxic septal motion, dilated pulmonary veins and retrograde flow in the SVC.

Results: Pericardiectomy of thickened and adherent pericardium was performed. The central venous pressure decreased from 23 to 7 mmHg and …

Prenatal Diagnosis Of An Uncommon Form Of A Hypoplastic Left Heart Syndrome Variant., Anmol Goyal

Research Days

Background: Mitral valve dysplasia syndrome (MVDS) is a rare form of congenital heart disease, similar to hypoplastic left heart syndrome (HLHS). Prenatal identification is important for counseling, delivery planning and postnatal management.

Case: A 39 year-old woman underwent fetal echocardiography at ~34 weeks gestation for evaluation of fetal cardiomegaly and hydrops revealing: biatrial enlargement, mild-to-moderate mitral and tricuspid valve insufficiency, echobright mitral valve apparatus, biventricular dilation, severe LV fibroelastosis (EFE) and systolic dysfunction, mild hypoplasia of aortic valve annulus and aortic arch, and a thick restrictive atrial septum (RAS) with left to right flow (Fig 1a-c).

Decision‐making: Although critical aortic …

The Role Of Fractionated Exhaled Nitric Oxide (Feno) In Eosinophilic Esophagitis And The Relationship With Downstream Eosinophils, Panamdeep Kaur

Research Days

Background: Eosinophilic Esophagitis (EoE) is a chronic immune mediated disease of the esophagus characterized by symptoms of esophageal dysfunction and eosinophilic-predominant inflammation of the esophagus. It can lead to feeding difficulties, failure to thrive, and esophageal stricturing. Currently, the gold standard for diagnosis and assessing response to therapy is upper endoscopy and histopathological analysis of biopsies. A noninvasive, cost-effective, and low risk alternative that can aid in the management of EoE is not currently available. Previous studies assessing correlation of fractionated exhaled nitric oxide (FeNO) with degree of esophageal eosinophilic inflammation were low powered but noted a trend for association. …