Dyskeratosis Congenita and Oral Cavity Squamous Cell Carcinoma: Report of a Case and Literature Review.

Dyskeratosis congenita is a rare genetic condition of telomerase dysfunction in which patients are at an increased risk of squamous cell carcinoma (SCCa) of the oral cavity. We present here the youngest patient in the literature with a diagnosis of SCCa. We discuss the literature and management of this advanced presentation of SCCa in a child, stressing the importance of palliative care involvement in facilitating medical decision making.

Implementation of a Web-Based Provider Care Team System at the VA North Texas Health Care System.

Concern that our Department of Veterans Affairs (VA) suffers low relational coordination led us to seek a system that might improve domains of relational coordination. The lack of an accurate system to identify the physician caring for a patient at any given time was a ready target. We conducted forward effect analysis, a novel methodology we adapted from our shared experience in software development and entrepreneurship. This study sought to implement a Web-based patient care team system in the critical care units at the VA North Texas Health System. Surveys were distributed to nurses before and after the intervention. Nursing confidence in the system improved from a median of 40% to 70%, and median satisfaction improved from 20% to 80%. Forty percent of nurses believed the old paging system resulted in harm to patients, compared with none with the new paging system. This study demonstrates technical and organizational implementation of a paging system as a two-sided platform with the ability to improve relational coordination between these sides.

Otitis Media Practice During the COVID-19 Pandemic.

The global coronavirus disease-2019 (COVID-19) pandemic has changed the prevalence and management of many pediatric infectious diseases, including acute otitis media (AOM). Coronaviruses are a group of RNA viruses that cause respiratory tract infections in humans. Before the COVID-19 pandemic, coronavirus serotypes OC43, 229E, HKU1, and NL63 were infrequently detected in middle ear fluid (MEF) specimens and nasopharyngeal aspirates in children with AOM during the 1990s and 2000s and were associated with a mild course of the disease. At times when CoV was detected in OM cases, the overall viral load was relatively low. The new severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is the causative pathogen responsible for the eruption of the COVID-19 global pandemic. Following the pandemic declaration in many countries and by the World Health Organization in March 2020, preventive proactive measures were imposed to limit COVID-19. These included social distancing; lockdowns; closure of workplaces; kindergartens and schools; increased hygiene; use of antiseptics and alcohol-based gels; frequent temperature measurements and wearing masks. These measures were not the only ones taken, as hospitals and clinics tried to minimize treating non-urgent medical referrals such as OM, and elective surgical procedures were canceled, such as ventilating tube insertion (VTI). These changes and regulations altered the way OM is practiced during the COVID-19 pandemic. Advents in technology allowed a vast use of telemedicine technologies for OM, however, the accuracy of AOM diagnosis in those encounters was in doubt, and antibiotic prescription rates were still reported to be high. There was an overall decrease in AOM episodes and admissions rates and with high spontaneous resolution rates of MEF in children, and a reduction in VTI surgeries. Despite an initial fear regarding viral shedding during myringotomy, the procedure was shown to be safe. Special draping techniques for otologic surgery were suggested. Other aspects of OM practice included the presentation of adult patients with AOM who tested positive for SARS-2-CoV and its detection in MEF samples in living patients and in the mucosa of the middle ear and mastoid in post-mortem specimens.

Competency-Based Assessment Tool for Pediatric Esophagoscopy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal. STUDY DESIGN: Blinded modified Delphi consensus process. SETTING: Tertiary care center. METHODS: A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items. RESULTS: The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus. CONCLUSIONS: It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated. LEVEL OF EVIDENCE: 5. Laryngoscope, 131:1168-1174, 2021.

Case report: Langerhans cell histiocytosis presenting as massive cervical adenopathy and retropharyngeal thickening: importance of chest radiography and coordinated airway and anesthetic management.

OBJECTIVE: To demonstrate the importance of chest radiography and special anesthetic management in children with massive cervical adenopathy. METHOD: Case report of an 8-month-old infant who presented to the emergency department with fever, drooling, and massive cervical and mediastinal adenopathy. Safe anesthetic management allowed for cervical lymph node biopsy, which showed Langerhans cell histiocytosis. RESULTS: Chest imaging and special anesthetic techniques (intravenous anesthesia and positioning in the lateral decubitus position) should be considered for children with massive cervical and mediastinal adenopathy. CONCLUSIONS: Interdisciplinary airway, radiographic, and anesthetic management is recommended for children who present with massive and cervical and mediastinal adenopathy.

Acute pediatric tracheitis: Distinguishing the disease by tracheostomy status.

OBJECTIVES: Tracheitis is an upper airway infection that often presents in patients with tracheostomies and can potentially cause airway obstruction. This study aims to use a nationwide database to identify a large cohort of pediatric patients admitted with tracheitis to elucidate the management and resource utilization associated with the disease both with and without tracheostomies. METHODS: The Kids' Inpatient Database (KID) 2012 was used to identify 2394 weighted discharges with acute tracheitis, with or without obstruction, as the primary diagnosis. Data on prior tracheostomy status, demographics, hospital characteristics, management, and resource utilization were obtained. Two groups of interest, based on presence of prior tracheostomy, were studied. Linear regression was performed to determine independent predictors of total charges. RESULTS: The mean age was 5.52 years (SD: 5.54), mean length of stay (LOS) was 6.37 days (SD: 10.18), and mean total charges were $60,996.61 (SD: 107,798.41). Patients with prior tracheostomy had lower rates of endoscopy and endotracheal intubation than patients without (p < 0.0005). There was no significant difference in LOS (p = 0.076) or total charges (p = 0.210) between the groups based on prior tracheostomy status. CONCLUSION: Pediatric tracheitis should be differentiated on the basis of tracheostomy status. We propose that tracheitis diagnosis codes should be distinguished by the presence of tracheostomy as "open" and the absence of tracheostomy as "closed."

General anesthesia risk across pediatric surgical specialties: Low in otolaryngology.

PURPOSE: To determine the relevance of the Food and Drug Administration (FDA) warning regarding general anesthesia (GA) in children under 3 years of age for procedures lasting longer than 3 h, by surgical specialty and for otolaryngology specifically. METHODS: A one-year retrospective review was conducted at a tertiary-care medical center for all children younger than 3 years undergoing surgical procedures with durations greater than 3 h. De-identified data related to age, surgical service, procedure types, American Society of Anesthesiologists (ASA) physical status classification, and general anesthesia time were collected and examined. RESULTS: During 2017, 430 of 11,757 patients (3.7%) met the age and duration of anesthesia criteria. Procedures performed by the cardiothoracic surgery service were mostly likely to result in duration of surgery greater than 3 h (46.6%), followed by neurosurgery (12.9%), cardiology (9.3%), plastic surgery (7.1%), general surgery (6.6%), and urology (5.1%). Less than 2% of patients undergoing ophthalmology (1.9%), orthopedic surgery (1.7%), and otolaryngology (0.5%) procedures required anesthesia greater than 3 h. CONCLUSION: Less than 4% of patients younger than 3 years undergoing surgery required general anesthesia for longer than 3 h. The theoretical risks of general anesthesia per the FDA warning are discussed and must be balanced against the known functional and neurodevelopmental consequences of not performing critical and time-sensitive surgery on children in this age group. A strategy for addressing parental and provider concerns is discussed.

Advanced practice providers and children's hospital-based pediatric otolarynology practices.

INTRODUCTION: Advanced practice providers (APPs), including nurse practitioners and physician assistants, have been deployed in children's hospital-based academic pediatric otolaryngology practices for many years. However, this relationship in terms of prevalence, roles, financial consequences and satisfaction has not been examined. The objective of this study is to explore how APPs impact healthcare delivery in this setting. METHODS: Pediatric otolaryngology chiefs of all academic children's hospitals in the US were electronically surveyed about the ways APPs intersected clinically and financially in their respective practice. RESULTS: A total of 29 of 36 children's hospital-based pediatric otolaryngology practices completed the survey, of which 26 practices (90%) utilized APP. There were large variances within the APP practice cohort in faculty size (mean/median/range = 9.4/8.5/3-29); annual patient visits (mean/median = 18,373/17,600); number of practice site (mean/median/range = 4.3/4/2-9) and number of outpatient APP (mean/median/range = 6.3/5/1-30). No factors (faculty size, annual visits and number of practice sites) differentiated between the APP and non-APP practices. Among APP practices, significant correlation (p<.00001) was observed between size of APP cohort to faculty size and annual visits. 69% of the practices did not differentiate job functions of nurse practitioners and physician assistants. 85% of the practices utilized APPs in all practice sites and 19% utilized APPs in the operating room. 77% of APPs billed independently and 46% had on-site supervision. The most prevalent APP salary bracket based on 0-5, 6-10 and > 11 years of tenure were $76-100K (65%), $100-150K (77%) and $100-150K (86%), respectively. In 46% of the practices, APPs were able to generate enough revenue to cover more than 75% of their salary and 23% of practices generated a profit. 81% of the chiefs ranked the effectiveness of APPs as high (4 and 5) on a 5-point Likert scale. DISCUSSION: The majority of academic pediatric otolaryngology practices employed APPs. Despite the diversity seen in practice complexity, APP functionality and financial impact, most found the APP model to be beneficial in improving patient care, patient access and faculty productivity.

Competency-Based Assessment Tool for Pediatric Tracheotomy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric tracheotomy. STUDY DESIGN: Blinded, modified, Delphi consensus process. METHODS: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items. RESULTS: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus. CONCLUSIONS: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure. LEVEL OF EVIDENCE: 5 Laryngoscope, 130:2700-2707, 2020.

Corporate-physician relationships: a need for education.

Knowledge is lacking among Otolaryngologist-Head and Neck Surgeons (ORL-HNS) regarding basic ethical situations in corporate-provider relationships. A pilot educational program demonstrates the need and potential for improvement by structured intervention. "At risk" areas specifically identified regard acceptable gifts, and payments for meetings and travel. Recommendations are made to educate otolaryngologists in standards for compliant behavior in corporate-physician relationships. Further work to formalize and tailor education to the needs of ORL-HNS is warranted, including continued education through the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF). A checklist is provided here as a first step in enabling more compliant behavior as surgeons engage in corporate relationships.

Penalties for health care fraud and abuse: January 2007-March 2008.

This commentary details the providers, penalties, and affected regions resulting from US health care fraud and abuse prosecutions from January 2007 to March 2008. Database review found that over $3 billion in fines as well as incarceration in some cases were ordered for 21 convicted providers, 68 percent of whom were physicians, and to 41 nonproviders, most of whom were vendors of durable medical goods (36%), individual citizens (18%) and health care corporations (17%). Fewer claims were found against pharmaceutical firms (7%) and medical equipment manufacturers (4%). Most verdicts were in the state of Florida. False claims accounted for most of the violations for both providers and nonproviders. These severe repercussions of malfeasance should promote careful consideration and construction of the terms of engagement between health care providers, corporations, and payers.

Case studies in corporate compliance: putting the rubber to the road.

An interdisciplinary, proactive perspective allows providers to engage in productive, long-term collaborative relationships with corporations, while 1) maintaining patient care improvements; 2) maintaining legality; 3) enhancing technical and clinical innovation; and 4) providing fair compensation for work done. The case study approach is used to demonstrate an effective approach to compliant behavior.

Post-transplant lymphoproliferative disease of the larynx.

Laryngeal post-transplant lymphoproliferative disease (PTLD) is rare. Here, we describe two pediatric cases. The first, a 15-month-old who underwent liver transplantation at 5 weeks, presented with airway distress. Airway evaluation identified epiglottic and arytenoid infiltrate, and biopsy was consistent with polymorphic PTLD. The second, a 23-month-old who underwent liver transplantation at 13 months, presented with progressive stridor. Airway evaluation revealed sub-mucosal infiltrate of the epiglottis, arytenoids, post-cricoid region, and uvula. Biopsy was consistent with monomorphic PTLD. Airway findings and symptoms resolved for both after immunosuppression reduction. PTLD diagnosis requires a high index of suspicion in post-transplant patients with airway obstruction.

A warning to physicians: changing landscape of the corporate-provider relationship.

In 2007, the United States federal government recovered $1.54 billion for fraud in the health care industry. The government's unwavering and continuing commitment to enforcement and criminal actions compel the need for a stronger provider understanding of the regulatory framework within which corporate-provider relations must be structured. This commentary aims to review briefly the need for such education in order to protect providers from legal action and offers a straightforward, proactive compliance approach to optimize patient care and technological innovation while maintaining appropriate remuneration.

Palliation in pediatric otorhinolaryngology.

Palliation in pediatric otorhinolaryngology is a rarely discussed but important aspect of care. This review encapsulates current thinking on pediatric palliative care (PC) and demonstrates, through one case, the impact of integrating PC into clinical care. We encourage early consideration of pediatric palliative care approaches for children with complex otorhinolaryngologic disorders.

Risk factor analysis for mortality among infants requiring tracheostomy.

INTRODUCTION: An increasing number of tracheostomies are performed in infants with complex comorbidities including bronchopulmonary dysplasia (BPD) and congenital heart disease (CHD). With this shift in indications, there is an urgent need to characterize outcomes in this population. METHODS: This 5-year retrospective chart review assessed rates of 12-month mortality in infants who were ≤12 months of age at the time of tracheostomy at a tertiary care pediatric hospital and risk factors associated with death. Patient characteristics evaluated included chronologic age and post-menstrual age at tracheostomy placement, gestational age and weight, sex, ethnicity, indication for tracheostomy, and comorbidities including BPD, CHD, subglottic stenosis (SGS), craniofacial syndromes, and chromosomal trisomy syndromes. Subgroup analysis was performed in infants with CHD. RESULTS: One hundred thirty-two tracheostomies were performed during the study period with an overall 12-month mortality of 14.4% (19/132). Mortality was increased in patients with CHD (35%) and decreased in patients with SGS (3.7%). No other patient characteristics were associated with differences in mortality. There was a trend towards improved mortality outcomes among patients born at earlier gestational ages. CONCLUSIONS: Among infants with tracheostomy in this cohort, overall mortality rates were relatively low but not insignificant. CHD was associated with increased mortality; however, children with SGS showed more favorable outcomes. Other patient characteristics were not associated with differences in mortality. These data clarify outcomes in a group of infants with tracheostomy.

A caution regarding the use of low-molecular weight heparin in pediatric otogenic lateral sinus thrombosis.

Lateral sinus thrombosis (LST), a rare complication of otitis media, is managed by antibiotics, surgery and anticoagulation. Traditionally, post-operative anticoagulation has been achieved by intravenous unfractionated heparin followed by oral warfarin. Fractionated, or low-molecular weight heparin derivatives (LMWH) have been introduced recently. There has been minimal literature to date regarding their use for the management of LST. We present use of the LMWH enoxaparin (Lovenox) for otogenic LST in two children, both of whom experienced hemorrhagic complications. On this basis and in the context of a literature review, we urge caution when using LMWH for pediatric otogenic LST.

Coblation for tonsillectomy: an evidence-based review.

Coblation technology (Arthrocare Corp., Sunnyvale, Calif., USA) allows for tonsillectomy using plasma-mediated ablation. This review presents the evidence to date with respect to surgical and recovery measures, in order to promote an objective discussion of this surgical technology.

Contact diode laser myringotomy for medium-duration middle ear ventilation in children.

OBJECTIVE: To prospectively compare contact diode laser for myringotomy (CDLM) to myringotomy with tube insertion (M&T) for the surgical management of otitis media with effusion (OME) in children. STUDY DESIGN AND SETTING: Prospective randomized controlled study at a tertiary care pediatric institution. METHODS: Thirty children for whom PE tube insertion was indicated were randomized for treatment by CDLM or by M&T, in order to investigate length of time for middle ear ventilation, and complication rates. Surgical time was measured, post-operative parental questionnaires were reviewed, and follow-up examinations were performed. INTERVENTION: Middle ear ventilation by CDLM or M&T. RESULTS: Middle ear ventilation by CDLM took an average of 4.1min, and was maintained for an average of 3.5 months, compared to surgical time of 10.2min for M&T, and duration of middle ear ventilation of 6.3 months. These differences were statistically significant (p=0.005 for surgical time, and p<0.001 for duration of ventilation). Complication rates were equivalent. CONCLUSION: CDLM allowed for medium-duration middle ear ventilation in children compared to M&T. Further study to clarify the role of CDLM in the management of OME in children is indicated.

Complications Following Pediatric Tracheotomy.

IMPORTANCE: Pediatric tracheotomy is a complex procedure with significant postoperative complications. Wound-related complications are increasingly reported and can have considerable impact on clinical course and health care costs to tracheotomy-dependent children. OBJECTIVE: The primary objective of this study was to identify the type and rate of complications arising from pediatric tracheotomy. DESIGN, SETTING, AND PARTICIPANTS: A retrospective review of medical records of 302 children who underwent tracheotomy between December 1, 2000, and February 28, 2014, at a tertiary care pediatric referral center. Records were reviewed for preoperative diagnoses, gestational age, age at tracheotomy, tracheotomy technique, and incidence of complication. MAIN OUTCOMES AND MEASURES: Main outcome measures included incidence, type, and timing of complications. Secondary measures included medical diagnoses and surgical technique. RESULTS: Of the 302 children who underwent tracheotomy, the median (SD) age at time of tracheotomy was 5 months (64 months) and the range was birth to 21 years. The most frequent diagnosis associated with performance of a tracheotomy was ventilator-associated respiratory failure (61.9%), followed by airway anomaly or underdevelopment (25.2%), such as subglottic or tracheal stenosis, laryngotracheomalacia, or bronchopulmonary dysplasia. The remaining indications for tracheotomy included airway obstruction (11.6% [35 of 302]) and vocal fold dysfunction (1.3% [4 of 302]). No statistical significance was found associated with diagnosis and incidence of complications. Sixty children (19.9%) had a tracheotomy-related complication. Major complications, such as accidental decannulation (1.0% [3 of 302]). There were no deaths associated with tracheotomy. Minor complications, such as peristomal wound breakdown or granuloma (12.9% [39 of 302]) and bleeding from stoma (1.7% [5 of 302]), were more common. Of all complications, 70% (42 of 60) occurred early (≤7 days postoperatively) and 20% (12 of 60) were late (>7 days postoperatively). CONCLUSIONS AND RELEVANCE: Pediatric tracheotomy at our institution is associated with an overall 19.9% incidence of complications. Although the rate of major complications such as accidental decannulation or death is low, rates of peristomal skin breakdown and development of granuloma are more frequently reported and can occur at any point following tracheotomy. Further work is necessary to understand and mitigate wound care issues in post-tracheotomy care.