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Hypoplastic left heart syndrome (HLHS) is a congenital diagnosis that necessitates immediate intervention at the beginning of life to ensure survival past infancy and to optimize left-side cardiac function. Often, these required procedures can lead to deleterious side effects and resultant complications. In this case report, we present a 15-month-old patient with HLHS who underwent multiple procedures, including two aortic arch surgeries. After the interventions, the patient experienced left main pulmonary bronchus compression along with pulmonary artery stenosis. In this case, we outline an approach to performing vascular dilation without compromise of airway patency.

Squamous cell carcinoma predominates as the most common malignant lesion of the oropharynx with human papilloma virus-associated disease now predominant over tobacco-related oropharynx cancer. Other rare malignant pathologies can manifest as visible neoplasms in these anatomic sites with varying degrees of symptoms such as dysphagia, odynophagia, otalgia, aspiration, hemorrhage, weight loss and dyspnea. We present a case of a rarely encountered primary oropharyngeal sarcoma managed by single-port transoral robotic resection and a selective cervical lymph node dissection followed by adjuvant radiotherapy.

The allergic airway diseases chronic rhinosinusitis (CRS), allergic fungal rhinosinusitis (AFRS), asthma, allergic bronchopulmonary mycosis/aspergillosis (ABPM/A), and cystic fibrosis (CF) share a common immunological signature marked by T H 2 and TH 17 cell predominant immune responses, the production of IgE antibody, and atypical inflammatory cell infiltrate that includes eosinophils and other innate immune effector cells. Severe forms of these disorders have long been recognized as being related to hypersensitivity reactions to environmental fungi. Increasingly however,environmental fungi are assuming a more primary role in the etiology of these disorders, with airway mycosis, a type of non-invasive airway fungal infection,recognized as an essential driving factor in at least severe subsets of allergic airway diseases. In this review, we consider recent progress made in understanding the immune mechanisms that drive airway mycosis-related diseases, improvements in immune-based diagnostic strategies, and therapeutic approaches that target key immune pathways.

Although the vast majority of pediatric neck masses are benign, pediatric malignancies commonly present in the supraclavicular region. We present the case of a 4-year-old male who presented with a mass in the trapezius muscle with accompanying lymphadenopathy. An extensive work-up was performed to exclude malignancy, and the patient was ultimately diagnosed with a benign monocytic mass, which surgically excised. He has been doing well since surgery with no evidence of recurrence. A review of the literature revealed this case to be the first of its kind to be reported.

Machine learning is a rapidly growing subset of artificial intelligence (AI) which involves computer algorithms that automatically build mathematical models based on sample data. Systems can be taught to learn from patterns in existing data in order to make similar conclusions from new data. The use of AI in facial emotion recognition (FER) has become an area of increasing interest for providers who wish to quantify facial emotion before and after interventions such as facial reanimation surgery. While FER deep learning algorithms are less subjective when compared to layperson assessments, the databases used to train them can greatly alter their outputs. There are currently many well-established modalities for assessing facial paralysis, but there is also increasing interest in a more objective and universal measurement system to allow for consistent assessments between practitioners. The purpose of this article is to review the development of AI, examine its existing uses in facial paralysis assessment, and discuss the future directions of its implications.

To determine the effects of the COVID-19 pandemic on Adenotonsillectomies (TA), Tonsil Related Cases (TC), and Peritonsillar Abscess (PTA) Trends.Retrospective Cohort Study.This is a retrospective cohort study using the Pediatric Health Information System® (PHIS) database, which consists of 51 children's hospitals. Regions were defined according to PHIS rules with at least five children's hospitals per region. We compared monthly total TA, TC, TC as a proportion of all hospital visits, and PTA from all encounters at each hospital from January 1, 2019, through December 31, 2021.Compared to 2019, April 2020 saw mean TC drop significantly from 371.62 to 68.37 (p 0.001). Interestingly, June, September, and December 2020 had significantly higher mean TC compared to 2019. TC as a proportion of all hospital visits decreased significantly throughout the majority of 2021. Similarly, TA significantly decreased during 2020 and 2021 across all regions in the US, starting in March 2020 and this reduction in TA extended through the end of 2021 without any signs of recovery. PTA rates did not change significantly over the three years.The pandemic-plagued 2020 saw a noticeable decrease in overall TC and TA but then rebounded quickly to even higher than pre-pandemic levels. However, this rebound halted for the majority of 2021 and subsequently decreased to lower than pre-pandemic levels, which differs from other communicable pathologies such as otitis media which decreased initially then recovered to pre-pandemic levels by Summer of 2021.

Purpose The pandemic related to the novel coronavirus (COVID-19) has led to a decrease in communicable diseases due to social distancing and mask-wearing. How have the prevalence of otitis media (OM) and its associated procedures changed during the pandemic? Study design Retrospective Cohort Study. Methods This is a retrospective cohort study using the Pediatric Health Information System® (PHIS) database, which consists of 48 children's hospitals. Regions were defined according to PHIS rules. We compared proportion of OM to total diagnoses codes, and collected mastoiditis, and MT placements from all encounters through January 1, 2019-June 31, 2021. Results In April 2020, there was a decrease in mean proportion of OM cases per 100 hospital visits (7 v. 2, p

Postpartum psychosis (PPP) is a severe mood disorder following childbirth that rarely leads to injurious or suicidal behavior. This report illustrates otolaryngologic intervention for pharyngeal laceration and airway instability following traumatic foreign body ingestion in the setting of PPP. A 25-year-old woman with PPP presented with hemoptysis after attempting suicide by traumatically forcing tree branches into her oropharynx. Imaging revealed pneumomediastinum, and flexible laryngoscopy and esophagoscopy showed a large foreign body (tree branch) extending from the hypopharynx to the gastroesophageal junction. She was taken to the operating room for direct microlaryngoscopy, bronchoscopy and esophagoscopy with removal of the 25-cm tree branch. Panendoscopy revealed a mucosal laceration at the cricopharyngeus with supraglottic and hypopharyngeal edema but no injury to the larynx. Due to airway concerns, a cuffed tracheostomy was placed along with a gastrostomy tube for feeding access. She tolerated her postoperative course with successful decannulation and oral feeding prior to discharge.

BACKGROUND: Corrective nasal surgery has historically been avoided in the pediatric population out of concerns surrounding the potential disruption of nasal growth centers. There is a paucity of data on the rate of complications or revision surgery following septoplasty in this population. As such, the purpose of this study is to review the long-term outcomes of a large cohort of children who underwent nasal septoplasty and to compare outcomes of septoplasty patients under the age of 14 to those 14 years and older. METHODS: A retrospective review was performed on all patients who received nasal septoplasty at our tertiary care pediatric referral center between October 2009 and September 2016. All patients who underwent septoplasty for a deviated nasal septum and were 0–18 years of age at the time of surgery were included in this analysis. Outcomes were compared between patients under the age of 14 to those 14 years and older. Demographic, surgical, and follow-up data were collected including complications and the need for revision surgery. RESULTS: A total of 194 pediatric patients were identified as meeting inclusion criteria for the study. Mean age for the total cohort was 14.6 years (0–18 years), with a mean of 15.9 years in the older group and 10.6 years in the younger group. Revision septoplasty was performed more frequently in the younger group. However, no significant difference in the rate of complications was seen between the two groups. CONCLUSIONS: To the best of our knowledge, this is the largest retrospective study examining outcomes following septoplasty in pediatric patients. We also specifically examine outcomes of very young septoplasty patients, a population for which limited evidence exists. Further retrospective studies are needed to validate the use of nasal septoplasty in the pediatric population.

Floating Harbor Syndrome (FHS) is a rare genetic disease with few reports detailing its potential otologic manifestations. The objective of this paper is to discuss two FHS patients with otologic manifestations. The first patient presented with delayed speech, middle ear effusions and conductive hearing loss (CHL) in addition to abnormal tympanograms. Her hearing improved after routine tympanostomy tube placement. The second patient presented with cleft palate, retracted ear drums and bilateral tympanic membrane who required multiple sets of T-tubes and eventually developed tympanic membrane perforations. She ultimately required hearing aids, however her speech and language development improved dramatically with time.

Oral surgeons remove third molars (wisdom teeth) to prevent impaction. Given the close anatomical relationship to the maxillary sinus, perforation and displacement of third molars into the maxillary sinus is a well-known phenomenon that is typically removed with a buccal mucoperiosteal flap or through a Caldwell–Luc approach. However, a less invasive route of endoscopic removal has been utilized to success in one report. A literature review shows few reports of displaced molars into the maxillary sinus and no reported patient below the age of 18. A 14-year-old patient presented with a displaced third molar into her maxillary sinus following third molar extraction with her oral surgeon. The tooth was removed successfully, utilizing endoscopic sinus surgery (ESS) with a transition to transoral retrieval to prevent nasal trauma. This is the first reported case of displaced third molar into the right maxillary sinus in a pediatric patient that was removed via ESS.

Background Nasopharyngeal carcinoma (NPC) is a cancer of epithelial origin with a high incidence in certain populations. While NPC has a high remission rate with concomitant chemoradiation, recurrences are frequent, and the downstream morbidity of treatment is significant. Thus, it is imperative to find alternative therapies. Methods We employed a Search Tag Analyze Resource (STARGEO) platform to conduct a meta-analysis using the National Center for Biotechnology’s (NCBI) Gene Expression Omnibus (GEO) to define NPC pathogenesis. We identified 111 tumor samples and 43 healthy nasopharyngeal epithelium samples from NPC public patient data. We analyzed associated signatures in Ingenuity Pathway Analysis (IPA), restricting genes that showed statistical significance (p Results Our meta-analysis identified activation of lipopolysaccharide (LPS)-induced tissue injury in NPC tissue. Additionally, interleukin-1 (IL-1) and SB203580 were the top upstream regulators. Tumorigenesis-related genes such as homeobox A10 (HOXA10) and prostaglandin-endoperoxide synthase 2 (PTGS2 or COX-2) as well as those associated with extracellular matrix degradation, such as matrix metalloproteinases 1 and 3 (MMP-1, MMP-3) were also upregulated. Decreased expression of genes that encode proteins associated with maintaining healthy nasal respiratory epithelium structural integrity, including sentan-cilia apical structure protein (SNTN) and lactotransferrin (LTF) was documented. Importantly, we found that etanercept inhibits targets upregulated in NPC and LPS induction, such as MMP-1, PTGS2, and possibly MMP-3. Conclusions Our analysis illustrates that nasal epithelial barrier dysregulation and maladaptive immune responses are key components of NPC pathogenesis along with LPS-induced tissue damage.

Adenotonsillectomy (AT) is the most common surgical procedure for the treatment of sleep related breathing issues in children. While overnight observation in the hospital setting is utilized frequently in children after a AT, ICU setting is commonly used for patients with sleep apnea. This objective of this study is to examine factors associated with the preoperative decision to admit patients to PICU following AT as well as co-morbidities that may justify necessity for higher level of care.This is a retrospective chart review from the years of 2009-2016. All patients who underwent AT for known sleep-related breathing issues at Nationwide Children's Hospital were eligible for inclusion. A complication was defined as an adverse event such as pulmonary edema, re-intubation, or a bleeding event. Respiratory support was defined as utilizing supplementary oxygen for more than one day, positive pressure ventilation, or intubation. Proportions and medians were used to describe the overall rate of complications/complexities in care, and bivariate statistics were used to evaluate the relationship between patient characteristics and outcomes. Similar methods were used to evaluate factors associated with preoperative referral to the PICU.There were 180 patients admitted to hospital in non-ICU setting and 158 patients with a planned PICU stay. The patients with planned PICU stays had higher rates of technological dependence (13% vs. 3%; p = 0.0006), perioperative sleep studies (80% vs. 29%; p 0.0001), and more severe classifications of OSA (p 0.0001). Patients with planned ICU placement also had higher rates of apneas, hypopneas, respiratory disturbance indexes, apnea hypopnea indexes, lower oxygen saturation nadirs, and a longer time spent below 90% oxygenation in sleep studies (p 0.0001). Nearly 45% of the patients with planned ICU stays required respiratory support compared to just 8% of non-PICU patients. Additionally, 32% of the patients with planned ICU stays experienced complications compared to just 8% of the floor population. Complications were associated with younger ages, gastrointestinal comorbidities, technological dependence, viral infections, and a history of reflux. Interestingly, there were no differences in the complication rate by sleep studies findings. Similarly, there were no population level differences between patients who required respiratory support in the ICU and those that did not. Unplanned PICU placement was a rare but significant adverse event (n = 24). None of the hypothesized risk factors were associated with unplanned PICU placement.This study suggest that while our pre-operative referral program for PICU placement is effective in identifying patients needing higher levels of care, the program places many patients in the PICU who did not utilize respiratory support or suffer from complications. We observed some misalignment between characteristics associated with planned ICU stays and actual complications. This suggests that patients with specific clinical histories, not findings on their sleep studies, should be prepared to receive higher levels of care.

Peritonsillar abscess (PTA) in pediatric patients is a challenging condition to diagnose and treat, and recurrence can occur in up to 15% of patients. This condition can be managed in various settings and there may be outcome differences depending on the choice of inpatient/outpatient management. The objective of this study was to evaluate immediate and long-term outcomes, specifically recurrence, after PTA management in the inpatient versus outpatient setting as well as specific patient characteristics leading to hospital management decisions.We conducted a retrospective cohort study of all suspected cases of PTA seen at a tertiary pediatric hospital from 2008 to 2017, and 566 confirmed cases of PTA were included in this study. Demographics, PTA management, recurrence, and subsequent tonsillectomy (immediate or within 1 year of diagnosis) were ascertained from patient medical records. We compared characteristics of patients seen in the inpatient and outpatient setting using Chi-squared and Wilcoxon-Mann-Whitney tests.Patients treated in the outpatient setting were more likely to be older and have a lower rate of recurrent ear infections (p 0.0001 and p = 0.01 respectively). Additionally, no differences in gender, Down Syndrome, or autism were found. Patients who were admitted were more likely to undergo immediate tonsillectomy within a month or tonsillectomy within one year (p 0.0001 and p = 0.02 respectively), whereas patients in the outpatient setting were more likely to receive antibiotics alone (p 0.0001). Outpatient management was associated with no differences in recurrence rate within 30 days (p = 0.56). However, recurrence was associated with older age, a history of recurrent tonsillitis, but not a history of ear infections (p = 0.005, p 0.0001, and p = 0.49 respectively). A history of recurrent ear infections, recurrent tonsillitis and recurrent PTAs were associated with the decision to pursue a tonsillectomy (p = 0.003, p = 0.03, and p 0.0001 respectively).Patients seen in the outpatient setting are more likely to receive antibiotics alone as their initial treatment, likely a result of certain clinical characteristics and presenting features as well as uncertain diagnosis. Inpatient status was associated with a younger age and a history of ear infections while recurrence was associated with a history of recurrent tonsillitis and older age. The recurrence rates for outpatient management were not statistically different than inpatient, suggesting that triaging and treatment of patients was occurring appropriately. The association of tonsillectomies in the inpatient group within 12 months is likely due to the higher rate of preexisting tonsil issues considering that recurrent tonsillitis, ear infections and a higher rate of PTA recurrence were all statistically correlated.

Objectives The objective of this study is to examine the impact that an institution-wide policy to reduce radiation exposure in pediatric patients had on the use of computed tomography (CT) imaging in children presenting with suspected peritonsillar abscess (PTA)1. Methods A single institution retrospective review was conducted for all pediatric patients under the age of 18 who presented to the emergency department with suspected PTA over a ten-year period. Patients were divided into two groups; those that presented during the years prior to policy implementation and those that presented during the years following. Main outcome measures were the frequency of patients who underwent CT scanning as a part of their workup, the specialty of the ordering physician, and the treatment for suspected PTA. Results A total of 317 patients with suspected PTA were seen prior to policy implementation, with an additional 275 patients seen in the years after. The frequency of CT use was 41% (131/317) in pre-policy patients, and 28% (77/275) in post-policy patients (p = 0.004). The most common specialty of ordering physician was Pediatrics in the pre-policy patients (45%), and Otolaryngology in the post-policy patients (41%) (p = 0.0003). Of the 131 patient who underwent CT scanning before policy implementation, 36 patients (27%) required incision and drainage (I&D) of their PTA in the OR. This is in comparison to 42/77 patients (55%) in the post-policy group who underwent I&D in the OR (p Conclusion An institution-wide policy to decrease unnecessary imaging studies in pediatric patients resulted in a significant reduction in the use of CT imaging in the work-up for suspected PTA. Additionally, there was increase in the frequency of patients who underwent surgical intervention for their PTA after CT imaging, suggesting a more scrutinized approach to CT usage since policy implementation.

Orbital abscess and subperiosteal abscess are pathologies which may require surgical treatment in the pediatric patient. Though rare, orbital cerebrospinal fluid (CSF) leak is a serious complication of abscess drainage. This paper presents a unique 5-month-old male with transorbital abscess drainage complicated by CSF leak. An endoscope was used to repair the defect through the orbitotomy incision. There was no evidence of persistent leak at follow-up. The surgical approach likely contributed to the complication, and the otolaryngologist played a key role in the leak repair. This case should serve to raise the awareness regarding considerations to avoid orbital CSF leak.

Introduction The clinical implications of single-sided deafness (SSD) in children has historically been underappreciated by patients and providers alike, despite a large body of literature on the wide-ranging neurocognitive, language, scholastic, and functional impairments that occur. Conventional amplification options are marked by variable results and frequent loss of follow-up. Methods Retrospective case series for pediatric SSD from 2008 to 2018. Results 88 children with congenital SSD were identified. Seventeen (N = 17/88, 23.9%) passed their newborn hearing screen. Median age at first otolaryngology evaluation was 0.65 years (range 0.1–16.9 years). Most common etiologies included cochlear nerve deficiency (N = 39, CND, 44.3%), unknown (N = 30, 35.2%), inner ear malformation (N = 7, 8.0%), and congenital cytomegalovirus (N = 6, 6.8%). 32.5% of patients elected for continued observation only, followed by bone conduction hearing aid (27.7%), contralateral routing of sound aid (20.5%), conventional hearing aid (13.3%), or cochlear implant (6%). Lack of follow-up at ≥1 year was common (39.8%). Of those with device use data (N = 39), 84.7% reported either discontinued or Conclusions Despite early diagnosis and evaluation, the pediatric SSD cohort is characterized by high rates of loss of follow-up and amplification discontinuation. Cochlear nerve deficiency is commonly seen in congenital SSD. Early specialist referral is critical for habilitation evaluation. Patients and caregivers should be educated on the significant implications of unilateral hearing loss.

7531 Background: Chronic lymphocytic leukemia (CLL) is the most common leukemia in adults and has a heterogenous presentation. CLL is known to shape the immune response to survive. Studying these processes will help gauge the potential success of immunotherapy and point to therapeutic targets. Methods: We used our Search, Tag, Analyze, Resource platform to meta-analyze patient samples from Gene Expression Omnibus. We tagged peripheral B cells from 741 CLL patients and peripheral B cell samples from 150 healthy donors as a control. We also tagged and compared B cell samples from 84 CLL progressors to 91 patients with stable CLL. Lastly, we tagged peripheral T cells from 70 CLL patients and T cells from 35 healthy donors as a control. We then analyzed the signature in Ingenuity Pathway Analysis. Results: Analysis of CLL cell samples identified T cell exhaustion signaling as our top canonical pathway. IL2, IL5, and TGFB1 were top upstream regulators. We found upregulation of PDL1, CTLA4, and Lag3, known markers for immunosuppressive B cells. FMOD, which sequesters TGFB, was also upregulated along with molecules that modulate BCR signaling such as MIR155HG. EBF1, required for B cell differentiation, and the co-stimulatory molecule CD80 were downregulated. Analysis of progressing CLL versus stable CLL highlighted metabolic changes. S-adenosyl-L-methionine biosynthesis, methionine degradation to homocysteine, cysteine biosynthesis, and acetate conversion to acetyl-CoA were top canonical pathways. No difference was seen in PDL1, CTLA4, and Lag3 expression but EBF1 was upregulated. Lastly, our T cell analysis demonstrated NFAT in regulation of the immune response as the top canonical pathway. Conclusions: Our results reinforce the promise immunotherapy can have in treatment of CLL and suggests more aggressive cases of CLL are a function of metabolic changes as opposed to differences in immune escape. We also suggest a role of NFAT in T cell exhaustion in the context of CLL.

e13048 Background: Hurthle Cell Carcinoma (HCC), a variant of follicular thyroid carcinoma, is a rare form of thyroid cancer. Though it accounts for 3-10% of thyroid cancer cases, very little is known about its pathogenesis. While HCC holds good survival rates, it is still vital to understand the pathogenesis of HCC in order to further optimize treatment protocols. Methods: We tagged 46 HCC samples and 90 healthy thyroid samples as a control. Gene signatures were analyzed in Ingenuity Pathway Analysis, using statistical significance p < 0.05 and absolute log ratio > 0.5 between disease and controls. Methods: Search Tag Analyze Resource was employed to conduct meta-analysis using the National Center for Biotechnologys Gene Expression Omnibus to define HCC pathogenesis. Results: Hepatic fibrosis, hepatic stellate cell activation and retinoic acid receptor (RAR) activation were the top canonical activators associated with HCC. Of the molecules involved, Defensin Beta 1 (DEFB1), Lipocalin-2 (LCN2), Aldehyde Dehydrogenase 1 Family Member A3 (ALDH1A3), and CDC28 Protein Kinase Regulatory Subunit 2 (CKS2) had the highest associations with HCC. The most down-regulated molecules were Hemoglobin Subunit Beta (HBB), Hemoglobin Subunit Alpha 1 and 2 (HBA1/HBA2), Cellular Retinoic Acid Binding Protein 1 (CRABP1), and BH3-Interacting Domain-Containing Protein 3 (HRK). Conclusions: These results suggest that there are a variety of factors at play regarding the development of HCC. Defensins are peptides made by neutrophils, however DEFB1 is specifically known for its ability to resist bacterial growth on epithelial surfaces, playing a major role in innate immunity. LCN2 is also known for resisting growth on surfaces and does so by sequestering iron-containing siderophores, thus effectively stopping growth of microorganisms. Additionally, ALDH1A3 and CRABP1 are associated with RAR activation. While the exact pathogenesis of HCC is not well known yet, we demonstrate that the two most up-regulated factors in HCC are strongly involved in innate immunity. This suggests that the dysfunction of innate immunity may play a vital role in the neoplastic pathogenesis of HCC.