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  Improving fluid management of extreme premature infants by providing a restrictive fluid management algorithm in the ICN

  Jacob S. Ward, Dena Hubbard, and Nicholas Clark

  Intro: Excessive fluid intake in extremely premature infants during the first week of life is known to increase risk of adverse events such as necrotizing enterocolitis (NEC), patent ductus arteriosus (PDA), and overall mortality. There also is some correlation with the need for mechanical ventilation and development bronchopulmonary dysplasia (BPD). While fluid restriction is recommended to decrease the likelihood of adverse outcomes, at our institution we found there was a wide variability in and lack of evidence-based standards on fluid management in this vulnerable population. We aimed to improve the fluid management of the extremely premature infants by standardizing a restrictive fluid algorithm. Methods: Children’s Mercy Hospital admitted a total of 107 premature infants <28 6>/7 weeks gestation to the ICN during 2021, and 25 of these infants were admitted during the timeframe of our study. The restrictive fluid management algorithm (Fig. 1) was initiated in April of 2021. During a 6-month period we monitored clinician compliance as outcome measures and patient clinical indicators of fluid status as balancing measures (percentage of birth weight lost, time to diuresis, and time back to birth weight). Results: Of 25 patients started on the algorithm, 3 patients were excluded due to admission after 12 hours of life or death on day of life 1 and 14 patients followed algorithm on all 3 days. The infrequent number of admissions during this period lead to presenting data in g-chart format. The process measure of clinician compliance with total fluid volume algorithm showed mild improvement over time after the education, initiation, and availability of the algorithm began in our ICN with overall mean of 1.89 patients outside protocol of total fluids between those patients on protocol (Fig. 2). This was again reflected in additional outcome measure of compliance with total fluid volume algorithm plus an additional 20ml/kg/day of fluid with overall mean was 0.63 patients outside protocol between patients on protocol (Fig. 3) showing a modest sustainability. The results of following the restrictive fluid algorithm did not result in excessive weight loss, absence of diuresis, or prolonged time back to birth weight based on the balancing measures tracked during this time period (Fig. 4-6). Conclusion: The fluid algorithm for premature infants did allow for a modest improvement in standardizing fluid management as compliance with a more restrictive plan improved without resulting in concerning short term consequences, such as excessive weight loss. The next step would be to provide continued education regarding the fluid algorithm to help sustain improvement. We are currently in process of obtaining baseline data prior to onset of current algorithm to further compare past and present fluid management to better help determine if changes have improved patient outcomes thus leading to a new standard in our ICN. In the future, we would like to follow up the current patients to determine if the fluid algorithm was helpful in avoiding adverse outcomes such as BPD, NEC, or PDA.

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  Malignancy masquerading as steroid resistant hypereosinophilic syndrome

  Sonya Parashar and Aarti Pandya

  Introduction: Hypereosinophilia syndrome (HES) is defined as 1500 eosinophils/mL or above with evidence of end-organ damage due to the eosinophils at least 4 weeks apart. Case description: A 3-year-old male was admitted for newly found anterior mediastinal mass and left pleural effusion. About 6 weeks prior to admission, he was seen in his primary care office for difficulty breathing. Despite the appropriate treatment for his symptoms, the patient continued to have significant respiratory symptoms and a chest x-ray showed a large left pleural effusion. He was immediately transferred to a tertiary care center and a CT chest showed large left pleural effusion, possible abscess, small pericardial effusion, and concern for an anterior mediastinal mass due to mediastinal shift. He underwent a thoracentesis, which showed eosinophilia within the pleural fluid. While his peripheral eosinophils had fluctuated at the beginning of his hospitalization, the patient’s absolute eosinophil count suddenly increased from 600 to 25,048 and remained elevated above 20,000. He developed recurrent eosinophilic pericardial effusions requiring multiple pericardiocentesis. Despite high dose steroids and inpatient use of mepolizumab, his hypereosinophilia persisted. After two inconclusive fine-needle aspirations of his mediastinal mass, the patient’s family agreed to a thoracotomy with removal of the mediastinal mass. Pathology eventually revealed myeloid sarcoma, a variant of acute myeloid leukemia. Discussion: Steroid resistant HES should raise concern for underlying malignancy especially when eosinophils are above 20,000. Educational objective: After reviewing this case, participants should be able to include myeloid malignancy in their differentials when evaluating a patient with hypereosinophilia.

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  Mobile Health Software Platform Implementation to Improve Pediatric Remote Patient Monitoring Outcomes

  Lori A. Erickson, Amy Ricketts, Jenny Marshall, Krista Nelson, Peter Churchill, Dawn Wolff, and Robert W. Steele

  Background: Children’s High Acuity Monitoring Program (CHAMP®) is a software platform for pediatric remote patient monitoring. It is used with a proactive, personalized, pediatric care model providing a link for communication with a specialized healthcare team to patients and families in their homes. Successful implementation since 2014 includes: adoption at 12 pediatric hospitals and over 870 infants with a survival of 96.5% (down from 20% in 2012). This work led our team to engage leadership to discuss a remote patient monitoring department with new monitoring devices and expanded access to pediatric conditions.

  Methods: Strategic Planning and Innovation facilitated navigation of an enterprise-wide endeavor over an 18-month period. Closed loop feedback with the administrative, clinical, information systems, and research teams ended with a well-rounded proposal for an innovative strategic initiative.

  Results: Remote Health Solutions department was founded in September 2021 with a focus on integration of digital health and mobile applications, hardware, software platforms and wearable sensors to improve the healthcare team’s ability to provide personalized, proactive patient care. The team has three active feasibility studies for pediatric monitoring devices, four analyses from the Cardiac CHAMP registry, performs consults on remote monitoring facilitation with care teams, and is expanding CHAMP application use into additional clinical pediatric areas.

  Conclusions: The forward movement of pediatric remote health stands at a precipice of opportunity within the landscape of mobile devices and applications in advancing pediatric medicine and outcomes. This project magnifies opportunities for evidence-based innovative pediatric care models, especially in the midst of the pandemic.

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  Alterations in the Metabolic Comorbidities of Youth with Overweight and Obesity during the COVID-19 Pandemic

  Safa Farrukh, Erica Wee, Ashley Sherman, and Yun Yan

  Background: Childhood obesity rates have continued to increase with the COVID-19 pandemic. However, data are limited on the impact of increasing obesity on associated comorbidities. Methods: We evaluated the progression of overweight- or obesity-associated comorbidities by investigating change in laboratory results pre–COVID-19 pandemic and post–COVID-19 pandemic onset in youth with overweight or obesity. Results: There was no significant BMI progression. There was progression of metabolic comorbidities. HbA1c progression and LDL progression were significantly correlated. HbA1c and LDL progression were significantly associated with older age and Hispanics respectively. Conclusion: The results indicate that despite no significant increase in BMI, youths with overweight or obesity have experienced progression of comorbidities during the COVID-19 pandemic. This study emphasizes the importance of early detection of comorbidities among a high-risk pediatric population.

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  Endoscopic Removal of Safety Pin from Appendiceal Orifice

  Alex Biller, Moises Alatorre-Jimenez, Jeremy Stewart, and Ruba A. Abdelhadi

  Introduction: Foreign body ingestion in children is considered a common concern in every Emergency Department worldwide. In the United States, it is estimated that foreign body ingestions cause approximately 1,500 deaths yearly (1). Safety pins ingestions are estimated to be 1% of the foreign body ingestions in children (2). In literature, ingested safety pins have been reported to be lodged mostly in the upper gastrointestinal tract, but a few cases have been reported to be lodged in the appendix (2,3,4). Objective: Recommendations for safety pin ingestions when past the second portion of the duodenum. Case: We report the case of a 5-year-old previously healthy female who presented with an accidental ingestion of an open safety pin. Following ingestion, the patient visited a local Emergency Room where an abdominal X-ray revealed an open safety pin located in the stomach. The patient was asymptomatic and had vital signs within normal limits for age with a normal exam. The patient was then transferred to a Children’s Hospital for further management and care; after traveling three hours, a follow-up X-ray on arrival to our Children’s Hospital revealed the safety pin to be located in the left upper quadrant (fig.1). As the patient continue to be asymptomatic, it was decided to continue monitoring progression with serial abdominal X-rays and initiate a bowel cleanout with GoLytely. Serial abdominal X-rays initially showed progression of the safety pin through the digestive tract and the patient remained asymptomatic. On the third day of admission, a repeat abdominal X-ray did not show progression of the safety pin and it appeared to be lodged in the right lower quadrant. At that time it was decided that the patient should undergo a colonoscopy with foreign body removal. During the procedure, the open safety pin was found in the appendiceal orifice with the head inside the Bauhin’s valve (fig.2). Careful extraction with rat-tooth forceps was performed; a pediatric surgeon was at the bedside for emergency abdominal decompression in case of appendiceal perforation. The open safety pin was successfully removed endoscopically without further damage to the patient’s colon. Conclusion: As per the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN), sharp foreign bodies should be removed immediately when located in the stomach or intestines if the patient is symptomatic (5). In asymptomatic patients with sharp foreign bodies in the stomach or small intestine, it is recommended that these be removed endoscopically within 24 hours if possible (5). Surgical intervention should be considered if there is no spontaneous clearance of the sharp object after 3 days (5). It has been reported in the literature by Gün et al., that ingested safety pins passing through the duodenum can be excreted spontaneously (6). In our case, the ingested open safety pin failed to advance on serial abdominal X-rays and appeared to be lodged in the right lower quadrant, and the safety pin was then found to be in the appendiceal orifice during colonoscopy. A metallic foreign body lodging in the appendix is a rare finding. Benizri et al. reported a case of a 29-year-old with abdominal pain who was found to have a metallic foreign body in the RLQ; serial x-ray one month later showed unchanged position (4). In this case they were unable to retrieve the foreign body by endoscopy due to inability to enter Bauhin’s valve to extract the object (4). Compared to our case, early serial x-rays and measuring progression to determine timing of extraction could prevent possible perforation or inflammation of the appendix and may lead to improved ability to remove the object endoscopically.

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  Endoscopic Retrograde Cholangiography Using A Colonoscope In A Pediatric Liver Transplant Patient With Roux-en-y Biliary Anastomosis

  Nadia Ibrahimi, Thomas M. Attard, Ryan T. Fischer, Voytek Slowik, Richard J. Hendrickson, Bhargava Mullapudi, Moises Alatorre-Jimenez, and Syed Jafri

  Introduction: Biliary ducal sequelae including occlusions, strictures, leaks, or necrosis frequently complicate pediatric liver transplantation. The underlying etiology includes prolonged intraoperative ischemia or postoperative arterial inflow obstruction. In adult patients the role of endoscopic retrograde cholangiography (ERC) is well established in the management of biliary complications post-liver transplantation. In contrast, in the pediatric population, due to the combination of small patient size and the complexity of Roux-en-Y anastomotic techniques, ERC has been described as difficult or virtually impossible. This limits the options available for managing biliary duct complications to percutaneous techniques Endoscopic management of biliary complications in pediatric patients with Roux-en-Y anatomy has not been previously reported in the pediatric population. Case: We present the case of an 8-yr-old patient with history of unresectable hepatoblastoma who underwent liver transplantation with Roux-en-Y anastomosis at the age of 15 months. His transplant was initially complicated by hepatic artery spasm and developing biliary strictures requiring biliary internal/external drain placement by interventional radiology (IR) twice in the first year following transplantation. He then presented at the age of 7 with recurrence of his biliary stricture at the site of choledocho-jejunal anastomosis and initial attempt for placing an internal/external biliary drain by IR was unsuccessful having not been able to pass a guidewire through the stricture. Another attempt 2 months later was successful, and an 8.5 French drain was placed then upsized to a 12 French 2 months later and removed after a total of 4 months. His biliary stricture recurred once more, and IR drain placement was unsuccessful despite multiple attempts. The patient was therefore referred for an ERC. A Pentax EC-2990Li with a 2.8mm working channel was initially used and access to the hepatic duct, through anastomosis site was successful using a standard sphincterotome and the stricture was dilated. An attempt to place a 7 Fr stent was unsuccessful as it couldn’t be passed through the scope channel, so the scope was switched to a Pentax EC-3490LK with a 3.8mm working channel and a 7Fr stent was successfully placed. Discussion: Biliary complications post liver transplantation represent a considerable cause of morbidity and mortality. Biliary complication rate has been reported as 20-38% in pediatric liver transplantation with higher rate in duct-to-duct anastomosis compared to hepato-jujenal anastomosis . The most common complications are bile leaks and biliary strictures. Endoscopic management of biliary complications remains the preferred approach, due to its safety profile, as opposed to the percutaneous route and has been extensively reported in the adult population, and has been increasingly used in patients with Roux-en-Y reconstruction. Studies have shown that endoscopic management of biliary complications can minimize the need for post-transplant biliary surgery and is generally safe and effective The ideal endoscope and technique for endoscopic management in Roux-en-Y patients remains unclear. Nabeel et al. compared using a single-balloon enteroscope with variable stiffness colonoscopes in patient with Roux-en-Y anatomy in 199 procedures and NASPGHAN 1 1 1 1 2 2 1 1 (1). (2) (4). found that rates of biliary cannulation, therapeutic success and procedural success were higher with the use of single-balloon endoscopes and lowest with the use of pediatric colonoscopes . Herein we report the first pediatric case of performing ERC in a liver transplant patient with Roux-en-Y anatomy. Conclusions: Endoscopic management of biliary complications post liver transplantations in pediatric patients with Roux-en-Y anatomy is possible and considered as a safer alternative to percutaneous management.

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  Intraductal Papillary Mucinous Neoplasm In An 18-year-old With Recurrent Pancreatitis

  Nadia Ibrahimi, Thomas M. Attard, Moises Alatorre-Jimenez, Karie Robinson, and Syed Jafri

  Introduction: Intraductal Papillary Mucinous Neoplasms (IPMNs) of the pancreas are premalignant, intraductal, mucin secreting cystic lesions. IPMNs are a rare entity in the pediatric population and commonly seen in men in their 6 to 7 decade. Pediatric IPMN has been previously reported in a 14-year-old and a neonate, who also had hyperinsulinism and a de novo germline SKIL gene mutation. . Kim et al. did a ten-year single centered pediatric pancreatic malignancy study discovering IPMN in one patient of 35 with pancreatic mass removals . Case: Our patient is an 18-year-old male who initially presented at the age of 15 with acute, then recurrent mild pancreatitis. His routine screening investigations including calcium, triglycerides, and IgG4 levels were normal. No mutations were detected upon pancreatitis gene panel including PRSS1, SPINK1, CFTR, CASR, CTRC, and CPA1 genes. The gallbladder was normal on abdominal ultrasonography, however Magnetic Resonance Cholangio Pancreatography (MRCP) showed focal irregular dilation of the pancreatic duct with a concern of santorinocele, which is a cystic dilation of the dorsal pancreatic duct.[ATM1] The patient underwent Endoscopic Retrograde Cholangio-Pancreatography (ERCP), and Endoscopic Ultrasound (EUS) with findings of mucin seen in the major papilla with a “fisheye” appearance (fig 1) and a cystic lesion measuring 7x22mm communicating with the pancreatic duct in the head of the pancreas (fig 2), respectively. Cytology was obtained through EUS guided fine-needle aspiration as well as aspirating mucus from the pancreatic duct during ERCP and both samples confirmed IPMN[ATM2] . Discussion: IPMN was first described in the 1980s but is increasingly recognized with the current incidence estimated as 0.48-2.04 per 100,000 . Pediatric IPMN is a premalignant lesion and typically progresses from IPMN adenoma, borderline dysplastic, carcinoma-in-situ and eventually to invasive carcinoma, usually within 5 years. IPMNs lesions are classified as main duct (MD-IPMN), branch duct (BD-IPMN) or mixed type with MTIPMN harboring a higher risk of malignant transformation. IPMNs are often diagnosed incidentally on imaging studies but can also present with abdominal pain or recurrent pancreatitis. It has been reported that patients presenting with pancreatitis are at high risk of harboring a malignancy . Worrisome Clinicopathologic features include cyst size ≥ 3 cm, thickened or enhancing cyst walls, main duct size 5–9 mm, non-enhancing mural nodules, an abrupt change in pancreatic duct caliber with distal pancreatic atrophy and lymphadenopathy. In addition, the incidence of extra-pancreatic neoplasms is higher in patients with IPMN, with reported rates of 25% to 50% Conclusion: Despite the rarity and low incidence of pediatric pancreatic tumors, especially IPMNs, the broad differential diagnosis of pediatric pancreatic lesions associated with pancreatitis has to include IPMNs especially in view of the potential malignant transformation.

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  Inverted Appendix Presenting As Abdominal Pain And Lower Gastrointestinal Bleeding

  Moises Alatorre-Jimenez and Thomas M. Attard

  Background: Cecal polyps are challenging insofar as the region represents the highest risk of bleeding and perforation during polypectomy . In addition, polypoid lesions in the cecum may represent a morphologically abnormal ileocecal valve or an inverted appendix. Inadvertent polypectomy in those scenarios would lead to serious sequelae including perforation. Case report: Our patient is a 6-year-old male with a background history of prematurity, lactose intolerance, chronic esophagitis, gastritis, constipation, and painless rectal bleeding. He presented with a acute onset diffuse, severe abdominal pain associated with diarrhea that evolved to include hematochezia. At the time of presentation, he required aggressive resuscitation. His initial hemoglobin was 10mg/dl trending down to 7g/dl over 24 hours. His basic metabolic panel, coagulation panel, infectious stool studies, and calprotectin were negative or within normal limits. The patient stabilized following transfusion and octreotide infusion. Following a negative Meckel’s scan it decided to proceed to endoscopy. Upper endoscopy revealed normal findings whereas colonoscopy showed a pedunculated cecal polypoid lesion estimated at 3 cms diameter, with normal mucosal appearance, streaking erythema with superficial erosions (Fig1) but not actively bleeding. The lesion was recognized as likely protruding from the appendix consistent with appendiceal inversion (Type V; McSwain Classification) . Endoloop assisted appendectomy was not available and urgent surgery consult was obtained. The patient underwent uneventful laparoscopic appendectomy the next day. Histopathology report from the appendix was reported as eosinophilic infiltration with chronic active colitis consistent with presumed ischemic injury. The patient subsequently recovered uneventfully and was discharged home with no residual or recurrent symptoms at 4 month follow up. Discussion: Inversion of the appendix is a rare finding, with an estimated incidence of < 0.01% among all ages . It is theorized that inversion of the appendix may result from appendix peristalsis due to local inflammation or by appendix endometriosis . When symptomatic, inversion presents as abdominal pain, obstruction from intussusception, hematochezia, or diarrhea . Endoscopically an inverted appendix can be easily confused with polyp or neoplasia. Endoscopic removal of inverted appendix, when symptomatic can be achieved safely through endoloop ligation followed by distal (above endoloop) snare polypectomy.

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  Management of Primary Spontaneous Pneumothorax: A Single Institution Protocol Analysis

  Shai Stewart MD, James A. Fraser, Rebecca M. Rentea, Pablo Aguayo, David Juang, Jason D. Fraser, Charles L. Snyder, Richard J. Hendrickson, Tolulope A. Oyetunji, and Shawn D. St.Peter

  Management of Primary Spontaneous Pneumothorax in Children: A Single Institution Protocol Analysis

  Background: The Midwest Pediatric Surgery Consortium (MWPSC) has suggested a management algorithm to include simple aspiration of primary spontaneous pneumothorax (PSP), failing which, Video-Assisted Thoracoscopic Surgery (VATS) should be considered. We describe our outcomes in patients who have been managed with this suggested protocol.

  Methods: An Institutional Review Board (IRB) approved, single institution retrospective analysis was conducted on all patients between 12 and 18 years who were diagnosed with PSP from 2016 to 2022. Initial management consisted of aspiration alone with a 12F Thal-Quick chest tube followed by clamping of the tube and chest radiograph at 6 hours. Success was defined asfailed.

  Results: Fifty-nine patients were initially managed with aspiration, with a median age of 16.8 years (IQR 15.9, 17.3). Most of the patients were male (n=45, 76.2%) and white (n=50, 85%). Left sided pneumothorax was more common (n=38, 64%). Overall median LOS was 2.7 days (IQR 1, 3.5).

  Aspiration was successful in 33% (20) of the patients and were subsequently discharged home, while 66% (39) required VATS. The median LOS for those with successful aspiration during the index admission was 20.4 hours (IQR 16.8, 34.8), while those who underwent VATS had a median LOS of 3.1 days (IQR 2.6, 4). In comparison, in the MWPSC study of which our site was a participant, the mean LOS for those managed with a chest tube after failed aspiration without progressing directly to VATS was 6.0 days (± 5.5).

  Of those who had successful aspiration, 45% (9) had recurrent PSP, of which 1 had another successful aspiration and 8 required VATS after failed aspiration.

  Of the 39 patients who initially underwent VATS after a failed aspiration, 25% (10) had recurrent PSP, of which 1 went on to have successful aspiration and 90% (9) required repeat VATS after another failed aspiration. The median time to recurrence for the successful aspiration group was shorter than that of the VATS group [66.6 days +/- 41.3 vs. 546.5 days +/- 166.5 (p=0.02)].

  Conclusion: Simple aspiration is safe and effective for children with PSP as an initial managment, although most will require VATS either due to failed aspiration or recurrence of PSP. Early VATS appears to reduce overall length of stay.

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  Repeat Tracheal Aspirates in Pediatric Intensive Care Patients: Frequency, Resistance and Antimicrobial Use

  Edward Lyon, Jennifer Goldman, Brian Lee, Margaret Campbell, Rangaraj Selvarangan, and Elizabeth Monsees

  Background: Tracheal aspirates (TA) are frequently obtained in the pediatric intensive care unit (PICU); however, no data exists on the frequency or clinical management of multiple TA cultures on the same PICU patient. In this study, we describe the frequency of repeat TAs in PICU patients, the emergence of multidrug resistant organisms (MDRO), frequently cultured pathogens of TAs, and associated antibiotic prescribing patterns. Methods: We identified 70 PICU patients between 2018-2019 who met our criteria for retrospective chart review with ≥2 TAs obtained during their hospitalization. The following information was collected: patient demographics, number of TAs per patient, microbiology with susceptibilities, antibiotic use, and clinical data summarizing patient condition. Descriptive statistics established the frequency and time between initial and repeat TAs, reason for collection, antibiotic exposure, and frequency of MDRO development. Findings: Preliminary data on 15 patients showed 90 total TA cultures, with a median of 4 [IQR 2.5, 8] cultures per patient during their PICU stay (Figure 1). The median days between cultures was 10 [IQR 4, 26]. Most patients were < 5 years of age (n=14; 94%), male (n=13; 87%), and were admitted to the medical ICU service (n=8, 53%). Fever (46%) was the most common reason for TA collection followed by vital sign changes and secretion burden (34% and 32% respectively). A total of 133 organisms were isolated, with Pseudomonas aeruginosa (n=32), Methicillin-susceptible Staphylococcus aureus (n=16), and Klebsiella oxytoca (n=11) comprising the top pathogens (Figure 2). Eleven of the 15 patients (73.3%) had the same organism detected on ≥2 separate cultures. A total of 616 antibiotic days were prescribed with 149 (24%) antibiotic days prescribed for the TA result specifically. Six (40%) patients developed a MDRO after a median of 14.5 antibiotic days [IQR 11.75, 37.5]. Conclusions: Repeat TAs are performed in the PICU, often identifying the same pathogen repeatedly, likely representing colonization. Development of resistance is common and only one-fourth of TAs are directly treated with antibiotics. These data provide an opportunity to further explore clinical criteria to maximize the impact of TA cultures in the PICU.

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  Stewardship Opportunities for Cervical Lymphadenitis and Deep Neck Space Infections

  Aaron Shaw, Brian R. Lee, Lauren Kazmaier, Emily Baker, Tina Dao, Sandra Arnold, and Angela Myers

  Background: Cervical lymphadenitis (LAD) and deep neck space abscesses (DNSA) are common pediatric infections caused by similar bacteria. We sought to determine differences in presentation, diagnosis, and treatment between LAD and DNSA to identify antimicrobial and diagnostic stewardship opportunities. Methods: Charts were obtained using ICD9/10 codes for retropharyngeal and parapharyngeal abscessed (DNSA), and LAD between 1/1/10-12/31/20 from two pediatric centers. 1981 charts were identified. Charts were excluded if the diagnosis was not a bacterial infection (e.g. Kawasaki disease), if the LAD was not in the neck, or if caused by less common bacteria (e.g. tuberculosis). Data on presenting signs, symptoms, imaging, microbiology results, antibiotics, and surgical interventions were collected. Statistical analyses between LAD and DNSA groups were performed with Fisher’s exact and Wilcoxon rank-sum tests. Results: 1432 patients met inclusion criteria; 767 patients with LAD, 665 with DNSA. Median age and gender differed between LAD and DNSA, but no difference was seen in race between groups (Table). Presenting symptoms were different between groups (p <0.0001) (Figure). Almost all patients in the DNSA group received a CT scan, compared to 69% in the LAD group. Only 8% in the DNSA group had an ultrasound, in contrast to 50% in the LAD group. Most patients in both groups had a blood culture obtained; these were negative in ≥95% of all cases. Abscess cultures in the LAD group more commonly grew S. aureus, while S. pyogenes was more common in the DNSA group. Clindamycin was the most common antibiotic utilized; 91% of LAD, 87% of DNSA. Most patients received ≥2 antibiotics; 81% of DNSA and 63% of LAD (Table). Conclusions: These data may be used to help target diagnostic and treatment stewardship interventions for DNSA and LAD. Ultrasound alone may be sufficient for some cases of LAD, and CT reserved for DNSA when suspected based on presenting signs and symptoms. Routine blood cultures appear to be unnecessary for these patients. Multiple antibiotics are used to treat LAD when a single antibiotic may be more appropriate, given the prevalence of Gram positive organisms that would likely respond to clindamycin.

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  Two Siblings With Microvillous Inclusion Disease

  Moises Alatorre-Jimenez, Brandi Weller, Meike Orlick, and William San Pablo

  Introduction: Microvillous Inclusion Disease (MVID) (OMIM#251850) is a rare autosomal recessive condition caused by mutations or deletions mostly in the MYH6 gene but also STX3 and STXBP2 genes. It is characterized by protracted diarrhea with severe congenital alteration of the intestinal epithelium resulting in watery diarrhea, metabolic acidosis, failure to thrive, and permanent malabsorption that normally leads to a lifelong dependency on total parenteral nutrition (TPN) and the eventual possibility of bowel transplantation . Histological hallmarks of MVID in small intestinal biopsies are hypoplastic, atrophic, or disorganized villi without crypt hypertrophy, or immune cell infiltrate and, at the intestinal cellular level, microvillus atrophy, intracellular accumulation of brush border enzymes, and microvillus inclusions in the cytoplasm. It is caused by a dysfunction of myosin Vb protein encoded by the MYO5B gene; at least 41 different mutations have been discovered . It is thought that these mutations can cause intracellular trafficking defects causing impairment of the apical enterocytes recycling and reduced expression of the apical proteins at the brush border membrane; also, this defect can cause fusion of transport vesicles of brush border proteins in the cytoplasm causing the Microvillous inclusions . Objective: Present a case of MVID with confirmed clinical features and histology with a novel mutation in MYH6 and RYR2 genes. Case report: We report two siblings, the oldest sibling is four years old, and the youngest is two years old; both are TPN-dependent and diagnosed as neonates with MVID both of whom presented with severe congenital diarrhea. Histopathology revealed epithelial cells with poorly formed brush borders and many cells with intracytoplasmic inclusions. Electron microscopy showed microvilli-containing inclusions within the apical cytoplasm in the enterocytes. Genetic results revealed that both siblings have maternally inherited heterozygous deletion of 2.62 kb in exons 32-33 of the MYO5B gene. Conclusions: There are very few MVID cases reported in the literature. To date, 188 patients are registered in the International Microvillous Inclusion Disease Patient Registry . None of those patients were reported to have a heterozygous deletion involving exons 32-33 of the MYO5B gene.

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  Impact of Remote Monitoring During the Interstage Period on Outcomes in Single Ventricle Patients Across Socioeconomic Groups

  Bianca Cherestal, Lori Erickson, Janelle R. Noel-Macdonnell, Girish S. Shirali, Hayley S. Hancock, Doaa Aly, and Natalie Jayaram

  Background: Despite improvements in outcomes over time, morbidity and mortality for infants with single ventricle (SV) heart disease remains high. Among other risk factors for mortality, infants of low socioeconomic status (SES) are known to be particularly vulnerable following stage 1 palliation. We sought to determine whether use of a novel remote monitoring program, CHAMP (Cardiac High Acuity Monitoring Program), mitigates differences in outcomes by SES for infants with SV during the interstage period. Methods: Using the CHAMP database, we identified 607infants across 11 institutions enrolled in the program between 2014–2021. All enrolled patients’ families were provided with a Windows-based tablet with built in cellular capability, at no cost to them, and video capability allowing for instantaneous transfer of patient information to the care team. Patients were divided into SES tertiles based upon six unique variables relating to SES status. Hierarchical logistic regression, adjusted for potential confounding characteristics, was used to determine the association between SES and mortality or transplant during the time between the first and second palliative procedures (interstage period). Results: Of 607 infants in the study cohort, 44 (7.2%) died or were listed for transplant during the interstage period. In univariate analysis, non-hispanic/non-latino patients, patients with renal failure following stage 1 palliation, patients with ventricular dysfunction prior to discharge, and patients with AV-valve regurgitation prior to discharge were at higher risk for mortality or need for transplant during the interstage period. The rate of mortality or need for transplant between SES tertiles was not statistically significant (p=0.18). Even after multivariable adjustment, the odds of mortality or transplant were no different for those in the middle [odds ratio (OR) 2.170, 95% confidence interval (CI) 0.789, 5.971, p=0.133] or highest (OR 1.003, 95% CI 0.297, 3.388, p=0.996) SES tertile compared to patients in the lowest SES tertile. Conclusion: In a large cohort of infants with SV heart disease enrolled in a digital remote monitoring program during the interstage period, we found no difference in outcomes based upon SES. The findings of this study are in contrast to findings from prior studies showing worse outcomes for SV patients of low SES. Our study suggests this novel technology could help mitigate differences in outcomes for this fragile population of patients.

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  Safety, Timing, and Outcomes of Early Postoperative Cardiac Catheterization Following Congenital Heart Surgery

  Karoline Krzywda, Jeremy Affolter, Darcie Al-Hassan, William Gibson, Ryan Romans, and Kelly S. Tieves

  Intro: Historically, early postoperative (<6weeks) cardiac catheterization (EPOCC) was considered high risk and often delayed. Recently, the safety of early postoperative cardiac catheterization has been demonstrated. The timing of catheterization in the struggling postoperative patient remains variable and optimal timing remains uncertain. There is limited data on the impact of EPOCC on outcomes such as length of stay, duration of mechanical ventilatory or extracorporeal support, and surgical or catheter- based interventions. The aim of this study was to describe our experience with EPOCC and its impact on management, length of stay, and duration of mechanical ventilatory support. Methods: This is a descriptive retrospective cohort study of patients who underwent cardiac surgery between 01/01/2010 and 12/31/2019 and cardiac catheterization within 30 days after surgery. Patients with catheterization for LA decompression on ECMO, endomyocardial biopsy, or BAS post hybrid procedure were excluded. We collected information surrounding timing, indication, and complications of catheterization, duration of mechanical ventilation/ECMO, and ICU/hospital LOS and compared these differences in patients who underwent EPOCC < 72hours (earlier) versus >72hours (later). Data was analyzed using IBM SPSS, version 28. Chi-square and Student’s t-test, or appropriate nonparametric tests were used to compare categorical and continuous variables, respectively. Results: There were 2542 surgeries performed during the study period with 167 patients undergoing cardiac catheterization within the defined 30 days. Twenty-six patients met exclusion criteria, leaving 141 patients for analyses. Median time from surgery to EPOCC was 10 days (1-30), with 26 patients(18.4%) having an earlier EPOCC. Interventions were performed in 70 patients (49.6%), not statistically different in those with earlier vs later EPOCC [n=9 (12.9%) vs 61 (87.1%), p=0.09]. Most common interventions included arch (n=9), atrial septum (n=9), collateral (n=9), and combination procedures (n=13). Complications of catheterization occurred in 10 patients (7%), not statistically different in the earlier vs later groups [n=6 (60% ) vs 4 (40%), p= 0.09]. Majority of complications were related to arrhythmia/ST segment depression, none included death or stroke. Patients with earlier EPOCC were statistically older (3 mos vs 1.5 mos, p=0.02) and weighed more (5.4kg vs 3.9kg, p=0.006) than those in the later EPOCC group. There were no statistically significant differences in median ICU length of stay (17.9d vs 28d, p=0.09), hospital length of stay (29.6d vs 49.9d, p=0.16), duration of mechanical ventilation (11.4d vs 13.4d, p=0.11) or duration of extracorporeal support (7.0d vs 15d, p=0.06) in the earlier vs later EPOCC groups. Discussion: The occurrence of complications related to EPOCC in our cohort was low, consistent with previously published work. While we did not demonstrate statistically significant differences in outcomes in patients who had catheterization performed earlier vs later after surgery, there is a trend towards significance with a higher number of catheter- based interventions, longer length of stay and duration of extracorporeal support in those patients who underwent later EPOCC. EPOCC appears to be safe when done < 72hours following surgery and we speculate that earlier catheterization may result in earlier intervention and a resultant decrease in extracorporeal support, ICU, and hospital length of stay. Our study is limited by the retrospective nature and small sample size. Further investigation is warranted.

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  Hemoglobin A1c Increase in Youth at Risk for Developing Type 2 Diabetes Mellitus During the COVID-19 Pandemic

  Erica Wee, Ashley Sherman, Mark A. Clements, Kelsee Halpin, and Yun Yan

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  Hyperinsulinemic Hypoglycemia and Growth Hormone Deficiency secondary to 20p11 deletion

  Erica Wee, Kavitha Dileepan, Sarah Tsai, and Emily Paprocki

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  Myocardial work by strain echocardiography: a predictor of exercise capacity in the Fontan population

  Amulya Buddhavarapu, Suma Goudar, David A. White, Daniel Forsha, and Doaa Aly

  INTRODUCTION: Global myocardial work index (GWI) obtained by non-invasive pressure-strain loops is a fast-emerging tool to assess myocardial contractility, independent of loading conditions. Data about the feasibility and utility of this modality in single ventricles are still lacking. AIM: We aimed to explore the relationship between the non-invasive myocardial work indices and exercise parameters in the Fontan population. METHOD: Fontan patients were prospectively evaluated by exercise testing and echocardiography. The systemic ventricle was tracked in 3 orthogonal apical planes for global longitudinal strain (GLS). Pressure-strain loops were generated against brachial blood pressure (surrogate for ventricular systolic pressure in the absence of outflow obstruction). GWI, global work efficiency (GWE), global constructive work (GCW) and global wasted work (GWW) were calculated. Peak VO2 and percentage of predicted peak VO2 were recorded during the same visit. RESULTS: Seventeen Fontan patients were included, 10 (58%) of whom had systemic left ventricle. Mean age was 13.8 y (range 9-21 y). Peak VO2 was 24.7 ± 5.9 ml/kg/min and predicted VO2% was 57.4 ± 11.1%. GLS was -14% ± 2.5%. Mean GWI was 979.9 ± 232 mmHg% which showed a strong correlation with the predicted VO2% (r=0.78, p < 0.001). Similarly, there was a strong correlation between GWE (mean= 87 ± 4.8%) and predicted VO2% (r=0.76, p=0.001). Less correlation was found between GLS and predicted VO2% (r=-0.58, p=0.019). GCW was 1293 ± 399 mmHg% and GWW was 161.1 ± 88 mmHg%. GWW had a moderate negative correlation with peak VO2 and predicted VO2% (r= -0.45, p=0.07 and r=-0.48, p= 0.06 respectively). CONCLUSION: Peak exercise capacity- a proven prognostic marker in the Fontan population, correlates strongly with GWI and GWE. Therefore, indices of myocardial work by non-invasive pressure strain loops are feasible measures in Fontan patients that may provide new insights into risk stratification.

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  Use of CGM in Monitoring Neonatal Diabetes

  Emily Metzinger and Kelsee Halpin

  Introduction Although continuous glucose monitoring (CGM) has increased in patients with Type 1 diabetes, its use in the neonatal population has remained limited. Neonates, particularly preterm infants, are at risk of impaired glucose control. Several studies have looked at the use of these devices in improving care for premature infants without diabetes. We describe a case of a very low birth infant with neonatal diabetes secondary to a GATA-6 mutation. Clinical Case This is a late preterm infant with IUGR, truncus arteriosus, and neonatal diabetes. Exome genetic panel showed a mutation in GATA-6. Her neonatal diabetes was challenging to control due to variable blood sugars and sensitivity to insulin. Weight gain remained poor. A CGM (Dexcom G6) was placed when the infant was 1.59kg to optimize her diabetes management and improve weight gain. An insulin titration chart was used to adjust her IV regular insulin infusion according to the CGM glucose levels and trend arrows. The following outcomes were reviewed and analyzed fourteen days before and after CGM placement: glucose levels (238 vs. 177 mg/dL), percentage of glucose levels within target range of 70-180mg/dL (31% vs 51%), average number of daily POC glucose checks (10.2 vs. 6.9), average amount of insulin received per day (0.42 vs 0.52 units/day), and average weight gain (11.4 vs. 20.7 grams/day). The discrepancy between POC and CGM glucose was also analyzed. CGM was within 20% of POC values 44% of the time and within 30% POC values 70% of the time. Adverse side effects of CGM were also noted. The patient was started on antibiotics following a small abscess identified underneath the CGM site. Bruising and skin breakdown were noted at the site, and the patient completed a course of antibiotics for the cutaneous infection. There were no other concerns of infection related to CGM for the first 2.5 months following CGM placement. Conclusion This case highlights that CGM can effectively be used to optimize glucose management in neonatal diabetes in a very low birth weight infant. However, the area where the CGM is placed needs to be monitored for signs of infection. The use of continuous glucose monitoring (CGM) in newborn infants has remained limited even though newborn infants are at risk of impaired glucose control. We describe a case of a very low birth weight infant with neonatal diabetes in which a CGM was placed. Following placement of a CGM, average glucose levels were lower, the average amount of insulin received per day was increased, and average weight gain per day improved. Adverse side effects included infection at the site of the CGM. This case highlights that CGM can be used to optimize glucose management in neonatal diabetes.

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  A Rare Case of Suspected Generalized Arterial Calcification of Infancy (GACI) in An Infant Presenting with Respiratory Failure in An Infant Presenting with Respiratory Failure and Arterial Calcification

  Lilah Melzer, Catharine Kral, Bonnie Sullivan, Eric T. Rush, and Erin Khan

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  Case presentation: Pulpal Obliteration in Primary Dentition of an 8-year-old, Differential Diagnosis Dental Dysplasia Type II.

  Tara Craven, Amy Burleson, Brenda S. Bohaty, Jenna Sparks, Neena Patel, and Aparna Naidu

  Tooth development anomalies can happen at many stages of cell development and are relatively common. These anomalies can be isolated findings however, they may also indicate minor or major associated syndromes and identification of these findings are essential to providing comprehensive care for patients. Dentin Dysplasia Type II is a rare autosomal dominant disease resulting from a disruption in the apposition stage of cell development. This disease affects the primary teeth and clinical manifestations can include bulbous crowns, cervical constriction, mild discoloration (amber color) and pulp obliteration. Permanent teeth tend to look typical but can demonstrate thistle-tube shaped pulps and/or multiple pulp stones. This case presentation discusses an 8-year-old male patient who presented with chronic decay and enlarged pulps noted at first clinical exam and radiographic interpretation. Following restorative treatment, the patient experienced loss of Stainless-Steel Crown (SSC) with remaining coronal tooth structure due to pulpal obliteration. A differential diagnosis of Dentin Dysplasia Type II was determined with the aid of a local oral pathologist. Patient was advised to seek genetic testing to improve treatment planning capabilities and further recommendations.

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  Characteristics of High-Acuity Patient Transfers to the PICU for Status Asthmaticus

  Lilah Melzer, Marc Sycip, and Jade B. Tam-Williams

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  Concurrent COVID19 pneumonia and systemic inflammatory syndrome in a two-week-old requiring ECMO

  Jennie Godwin, Ayan Rajgarhia, Johanna I. Orrick, Jenna Miller, and John M. Daniel IV

  Objectives: * Initially, newborns seemed to be relatively spared from severe COVID-19. However, with the emergence of the B.1.617.2 (Delta) variant hospitalization rates among children aged 0-4 years increased by 10-fold by the middle of August 2021. We present a case of a 2-week-old previously healthy preterm, twin, male infant who developed severe COVID-19 pneumonia, subsequent acute respiratory distress syndrome (ARDS), and an inflammatory syndrome requiring VA – ECMO support for 22 days. Methods: * A retrospective chart review was performed after successful weaning from ECMO. Parental permission for a case report was obtained in compliance with institutional standards. Results: * Multiple challenges were encountered during this patient’s 22 days on ECMO. Rising markers of inflammation (LDH, Ferritin, D-dimer, CRP, ESR, troponin) made us concerned for multi-system inflammatory syndrome (MIS) in the setting of pneumonia. This complicated our treatment plan and led to a limited trial of immunomodulating agents in a baby with concurrent viral pneumonia. Dexamethasone, IVIg, and Anakinra were used. After treatment with these agents, clinical improvement began with a slowly clearing chest x-ray (Table 1 and 2) and weaning of ECMO flows. He developed seizures concerning for stroke in the setting of stable markers of coagulation, necessitating faster weaning and earlier ECMO decannulation than desired. Imaging studies showing extensive change to the lung architecture and ultimately required tracheostomy placement. Conclusions: * Severe COVID19 in infants remains a rare condition. Most infants have mild symptoms and recover completely. Unfortunately, some develop severe illness requiring extracorporeal life support. These infants can be successfully managed on ECMO during their acute crisis. The presents of a what appears to be a concurrent hyperinflammatory syndrome presents additional management challenges, but use of immunomodulating agents should be considered in infants.

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  CVST As A Rare Complication of Acute Pediatric COVID-19

  Erin Bolen, Jonathan Ermer, and Mukta Sharma

  A case report and literature-review derived case series of pediatric central venous sinus thrombosis as a late complication of COVID-19.

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  Improvement in Follow-up Communication for Resident-Identified Patient Safety and Hospital Process Issues

  Ross Newman, Angela Etzenhouser, Emily Killough, Danielle Reed, Allison Adam, Michelle Brown, Kelly Meyer, Denise Bratcher, and Nicholas Clark

  BACKGROUND: Lean concepts, including Daily Management Systems (DMS), have been adopted by health systems to prioritize high-quality and efficient care. Since 2018, our residency program has utilized a daily readiness huddle (key component of DMS) to prepare inpatient residents for their workday. This is a time when residents can raise administrative and/or clinical patient safety concerns. Concerns unable to be immediately resolved locally are designated as Quick Hits (QH) and escalated through residency and hospital leadership with resolutions cascaded back to frontline providers. With <10% of residents on inpatient services each month, a gap in overall resident awareness of findings from resolved QH exists, posing potential patient safety risks. OBJECTIVE/AIM: We aimed to improve the weekly percentage of findings from resolved QH communicated to every resident (rather than only those in attendance at the daily readiness huddle) from 0% to >95% within 6 months. METHODS: A core team was created consisting of residency program leadership (DIO, Program Directors, Chief Residents). Root causes were identified and guided interventions. Outcome measure was percentage of weekly QH communicated to all residents. Process measure was weekly QH identified for escalation. Findings of resolved QH were shared at bi-weekly residency program meetings/minutes. Plan-Do-Study-Act Cycles included: 1) Standardizing process of identifying QH requiring report-back to all residents, and 2) Audit and feedback to program leadership regarding transmission of resolved QH with adjustment in standard work when needed. Due to rarity of patient safety events, these were unable to be tracked. Run charts assessed improvement over time. RESULTS: Over 20 weeks, 83 QH were identified with 45 requiring escalation beyond residency program leadership for resolution including to physician safety officer and CEO huddles. Outcome measure showed sustained, special-cause improvement from 0% to 100% by Week 15 following Cycle #2 (Fig 1). Process measure remained unchanged at 4.2 QH averaged per week. CONCLUSIONS: Using improvement methodology, we were able to achieve sustained improvement above our goal of communicating findings of resolved QH to all residents. Audit and feedback appeared to have the greatest impact on our outcome measure. Further study is needed, but this closed-loop communication process may also provide beneficial impacts to patient safety.

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  Memory T Cell Populations in Human Leukemia

  Sara McElroy, Fang Tao, John Szarejko, and John M. Perry

  Background Incorporating the immune system into cancer management is an area of robust research. Treatment modalities aimed at activating cytotoxic T cells against malignancies include immune checkpoint inhibitors, bispecific T-cell engagers, and chimeric antigen receptor (CAR) tumor-specific T cells. Most anti-cancer T cell research is narrowly focused, but knowledge about the nature of diverse sub-populations of T cells in cancer, particularly memory T cells, is vital prior to potential incorporation into therapies. T memory stem cells (Tscm) are of interest due to their longevity and powerful abilities of self-renewal and creating the full spectrum of memory CD8+ T cells, including central memory (Tcm) and effector memory cells (Tem). Objective To determine potential presence and frequency of memory T cell populations in human leukemia at diagnosis and after induction chemotherapy. Design/Method Children’s Mercy Cancer Center Biorepository provided samples from 16 patients with premature B cell acute lymphoblastic leukemia (pre-B ALL) from peripheral blood (PB) and/or bone marrow (BM). Timepoints were diagnosis and day 29 (D29) of induction chemotherapy. Flow cytometric analysis of cytotoxic memory T cell populations was performed and analyzed using descriptive statistics and the t-test. Results Despite sample variability, the absolute cell counts were not significantly different. The live cell percent was lower at diagnosis than D29 (PB- 57.2% vs 79.4%, p=0.001; BM- 41.7% vs 77.0%, p<0.001). T cell frequencies were lower at D29 (PB- 14.8% vs 5.74%, p=0.014; BM- 9.5% vs 5.9%, p=0.217). CD4+ and CD8+ T cells were not significantly different between diagnosis and D29. Among CD8+ T cells, naïve cells markedly increased from diagnosis to D29 (PB- 53.6% vs 82.2%, p < 0.001; BM- 38.8% vs 80.3%, p < 0.001) with a corresponding significant decrease in Tem (PB- 15.1% vs 4.2%, p=0.002; BM- 21.6% vs 4.7%, p < 0.001). Tscm (reported as a % of CD8+ T cells) were detected in all samples at diagnosis (PB range- 0.49-12.2%; BM range- 0.12-4.4%). After induction, two patients had no Tscm detected. The remainder demonstrated varying numbers of Tscm (PB range- 0.038-23.4%; BM range- 0.013-17.4%). Differences between diagnosis and D29 were not significant; however, percentages of Tscm were lower in 9 of 12 patients after chemotherapy. Conclusion Induction chemotherapy led to decreased differentiated CD8+ T cells with recovery of mostly naïve cells by D29. Furthermore, we established the presence of Tscm in most pediatric pre-B ALL samples. The decline of Tscm in most patients requires functional analyses to determine their role in leukemia and potential for use in immunotherapy.