Treatment of unilateral olfactory neuroblastoma: Appropriate extent of surgical resection and potential for olfactory preservation.

Historically, comprehensive surgical resection for olfactory neuroblastoma has included the bilateral olfactory epithelium, cribriform plate, overlying dura, olfactory bulbs and tracts. This results in postoperative anosmia that may significantly impact a patient's quality of life without definitive added benefit in survival. The prevalence of occult intracranial disease is low, especially for Hyams grade I and II tumors. A unilateral approach sparing the contralateral cribriform plate and olfactory system can be considered for select cases of early stage, low-grade tumors when the disease does not cross midline to involve the contralateral olfactory cleft or septal mucosa and when midline dural margins can be cleared with frozen pathology. Approximately half of patients who undergo unilateral resection may have residual olfaction even with adjuvant unilateral radiation. Early data suggest favorable disease-free survival and overall survival for patients who underwent the unilateral approach; however, larger sample studies are needed to confirm comparability to bilateral resections regarding oncologic outcomes.

Dysphagia in Pediatric Patients with Tracheostomy.

OBJECTIVE: Post-tracheotomy swallowing function has not been well described in the pediatric population. This study aims to (1) determine differences in swallowing functioning pre- and post-tracheotomy and (2) examine the association between postoperative dysphagia and indication for tracheotomy, age at the time of tracheotomy, and time between tracheotomy and modified barium swallow (MBS). METHODS: A retrospective chart review was performed on 752 patients who underwent a tracheotomy from 2003 to 2018 and had adequate documentation for review. Patients were included if they received a post-operative MBS. Descriptive statistics, logistic regression, and Fisher's exact test were used to analyze the data. RESULTS: The cohort included 233 patients. The mean age at the time of tracheotomy was 25 months (±50.5). The indications for the tracheotomy were upper airway obstruction (110/233, 47.2%), chronic respiratory failure (104/233, 44.6%), and neurologic disease (19/233, 8.2%). The mean time from tracheotomy to post-operative MBS was 224 days (±297.7). Of the patients who had documented pre- and post-tracheotomy diets, nearly half of patients had improvement in their swallowing function after tracheotomy placement (82/195; 42.1%). Post-tracheotomy MBS recommended thickened liquids in 30.9% of the patients (72/233) and 42.5% (99/233) were recommended thin liquids. The remainder (62/233, 26.6%) remained nothing by mouth (NPO). Patients with neurological disease as the indication for the tracheotomy were more likely to remain NPO (P = .039). CONCLUSION: A tracheotomy can functionally and anatomically affect swallowing in pediatric patients. The majority of our studied cohort was able to resume some form of an oral diet postoperatively based on MBS. This study highlights the need for objective measurements of swallowing in the postoperative tracheotomy patient to allow for safe and timely commencement of an oral diet. LEVEL OF EVIDENCE: Level 3.

Otolaryngology Consult Protocols in the Setting of COVID-19: The University of Pittsburgh Approach.

OBJECTIVE: To analyze trends in otolaryngology consultations and provide algorithms to guide management during the COVID-19 pandemic. METHODS: A retrospective cohort study at a single institution tertiary care hospital. A total of 95 otolaryngology consultations were performed from March 1, 2020 to April 26, 2020 (COVID-era) and 363 were performed from September 1, 2019 to February 29, 2020 (pre-COVID-era) at the UPMC Oakland campus. Data collected included patient demographics, COVID-19 status, reason for consult, location of consult, type of consult, procedures performed, need for surgical intervention, length of hospital stay and recommended follow up. RESULTS: Patient populations in the pre-COVID-era and COVID-era were similar in terms of their distribution of demographics and chief complaints. Craniofacial trauma was the most common reason for consultation in both periods, followed by vocal fold and airway-related consults. We saw a 21.5% decrease in the rate of consults seen per month during the COVID-era compared to the 6 months prior. Review of trends in the consult workflow allowed for development of several algorithms to safely approach otolaryngology consults during the COVID-19 pandemic. CONCLUSIONS: Otolaryngology consultations provide valuable services to inpatients and patients in the emergency department ranging from evaluation of routine symptoms to critical airways. Systematic otolaryngology consult service modifications are required in order to reduce risk of exposure to healthcare providers while providing comprehensive patient care.

Outcomes of dupilumab treatment versus endoscopic sinus surgery for chronic rhinosinusitis with nasal polyps.

BACKGROUND: Outside of SINUS-24 (A Controlled Clinical Study of Dupilumab in Patients With Bilateral Nasal Polyps) and SINUS-52 (Controlled Clinical Study of Dupilumab in Patients With Nasal Polyps), there are limited data on the efficacy of dupilumab in patients with chronic rhinosinusitis with nasal polyps (CRSwNP). The objective was to compare dupilumab with functional endoscopic sinus surgery (FESS) in patients with CRSwNP by assessing the change in nasal polyp and Sino-Nasal Outcome Test (SNOT-22) scores postintervention. METHODS: A retrospective matched cohort study compared 54 patients who had CRSwNP treated with dupilumab with 54 patients who had CRSwNP treated with FESS; both cohorts were treated with topical corticosteroids. The primary end points were change in nasal polyp score and overall SNOT-22 score. Secondary end points were change in SNOT-22 domain scores and SNOT-22 olfaction score. RESULTS: Patients who underwent FESS had a greater improvement in nasal polyp score (5.18 ± 2.01) compared with patients treated with dupilumab (4.27 ± 1.98, p = 0.02). There was no significant difference in terms of the change in overall SNOT-22 score. Patients treated with dupilumab had greater improvement in the extranasal rhinologic SNOT-22 domain scores (4.87 ± 3.91) compared with patients treated with FESS (2.93 ± 4.32, p = 0.02). There was a greater improvement in the SNOT-22 olfaction scores for patients treated with dupilumab (2.35 ± 2.17) compared with patients treated with FESS (1.48 ± 2.24, p = 0.04). Patients taking dupilumab were followed on average for 12.20 months and patients treated with FESS were followed for 17.90 months. CONCLUSION: Overall, both therapies are effective at reducing symptoms in patients with CRSwNP according to SNOT-22. Patients treated with dupilumab reported improved olfaction and decreased cough, postnasal drainage, and thick nasal drainage as compared with patients treated with FESS, while patients treated with FESS had a greater reduction in polyp burden.

Aerosol and Droplet Risk of Common Otolaryngology Clinic Procedures.

OBJECTIVES: Define aerosol and droplet risks associated with routine otolaryngology clinic procedures during the COVID-19 era. METHODS: Clinical procedures were simulated in cadaveric heads whose oral and nasal cavities were coated with fluorescent tracer (vitamin B2) and breathing was manually simulated through retrograde intubation. A cascade impactor placed adjacent to the nares collected generated particles with aerodynamic diameters ≤14.1 µm. The 3D printed models and syringes were used to simulate middle and external ear suctioning as well as open suctioning, respectively. Provider's personal protective equipment (PPE) and procedural field contamination were also recorded for all trials using vitamin B2 fluorescent tracer. RESULTS: The positive controls of nebulized vitamin B2 produced aerosol particles ≤3.30 µm and endonasal drilling of a 3D model generated particles ≤14.1 µm. As compared with positive controls, aerosols and small droplets with aerodynamic diameter ≤14.1 µm were not detected during rigid nasal endoscopy, flexible fiberoptic laryngoscopy, and rigid nasal suction of cadavers with simulated breathing. There was minimal to no field contamination in all 3 scenarios. Middle and external ear suctioning and open container suctioning did not result in any detectable droplet contamination. The clinic suction unit contained all fluorescent material without surrounding environmental contamination. CONCLUSION: While patients' coughing and sneezing may create a baseline risk for providers, this study demonstrates that nasal endoscopy, flexible laryngoscopy, and suctioning inherently do not pose an additional risk in terms of aerosol and small droplet generation. An overarching generalization cannot be made about endoscopy or suctioning being an aerosol generating procedure. LEVEL OF EVIDENCE: 3.

Droplet and Aerosol Generation With Mastoidectomy During the COVID-19 Pandemic: Assessment of Baseline Risk and Mitigation Measures With a High-performance Cascade Impactor.

HYPOTHESIS: Aerosols are generated during mastoidectomy and mitigation strategies may effectively reduce aerosol spread. BACKGROUND: An objective understanding of aerosol generation and the effectiveness of mitigation strategies can inform interventions to reduce aerosol risk from mastoidectomy and other open surgeries involving drilling. METHODS: Cadaveric and fluorescent three-dimensional printed temporal bone models were drilled under variable conditions and mitigation methods. Aerosol production was measured with a cascade impactor set to detect particle sizes under 14.1 µm. Field contamination was determined with examination under UV light. RESULTS: Drilling of cadaveric bones and three-dimensional models resulted in strongly positive aerosol production, measuring positive in all eight impactor stages for the cadaver trials. This occurred regardless of using coarse or cutting burs, irrigation, a handheld suction, or an additional parked suction. The only mitigation factor that led to a completely negative aerosol result in all eight stages was placing an additional microscope drape to surround the field. Bone dust was scattered in all directions from the drill, including on the microscope, the surgeon, and visually suspended in the air for all but the drape trial. CONCLUSIONS: Aerosols are generated with drilling the mastoid. Using an additional microscope drape to cover the surgical field was an effective mitigation strategy to prevent fine aerosol dispersion while drilling.

Novel Use of Vitamin B2 as a Fluorescent Tracer in Aerosol and Droplet Contamination Models in Otolaryngology.

OBJECTIVE: During the COVID-19 era, a reliable method for tracing aerosols and droplets generated during otolaryngology procedures is needed to accurately assess contamination risk and to develop mitigation measures. Prior studies have not investigated the reliability of different fluorescent tracers for the purpose of studying aerosols and small droplets. Objectives include (1) comparing vitamin B2, fluorescein, and a commercial fluorescent green dye in terms of particle dispersion pattern, suspension into aerosols and small droplets, and fluorescence in aerosolized form and (2) determining the utility of vitamin B2 as a fluorescent tracer coating the aerodigestive tract mucosa in otolaryngology contamination models. METHODS: Vitamin B2, fluorescein, and a commercial fluorescent dye were aerosolized using a nebulizer and passed through the nasal cavity from the trachea in a retrograde-intubated cadaveric head. In another scenario, vitamin B2 was irrigated to coat the nasal cavity and nasopharyngeal mucosa of a cadaveric head for assessment of aerosol and droplet generation from endonasal drilling. A cascade impactor was used to collect aerosols and small droplets ≤14.1 µm based on average aerodynamic diameter, and the collection chambers were visualized under UV light. RESULTS: When vitamin B2 was nebulized, aerosols ≤5.4 µm were generated and the collected particles were fluorescent. When fluorescein and the commercial water tracer dye were nebulized, aerosols ≤8.61 µm and ≤2.08 µm respectively were generated, but the collected aerosols did not appear visibly fluorescent. Endonasal drilling in the nasopharynx coated with vitamin B2 irrigation yielded aerosols ≤3.30 µm that were fluorescent under UV light. CONCLUSION: Vitamin B2's reliability as a fluorescent tracer when suspended in aerosols and small droplets ≤14.1 µm and known mucosal safety profile make it an ideal compound compared to fluorescein and commercial water-based fluorescent dyes for use as a safe fluorescent tracer in healthcare contamination models especially with human subjects.

Droplet and Aerosol Generation With Endonasal Surgery: Methods to Mitigate Risk During the COVID-19 Pandemic.

OBJECTIVE: To define the aerosol and droplet risks associated with endonasal drilling and to identify mitigation strategies. STUDY DESIGN: Simulation series with fluorescent 3-dimensional (3D) printed sinonasal models and deidentified cadaveric heads. SETTINGS: Dedicated surgical laboratory. SUBJECTS AND METHODS: Cadaveric specimens irrigated with fluorescent tracer and fluorescent 3D-printed models were drilled. A cascade impactor was used to collect aerosols and small droplets of various aerodynamic diameters under 15 µm. Large droplet generation was measured by evaluating the field for fluorescent debris. Aerosol plumes through the nares were generated via nebulizer, and mitigation measures, including suction and SPIWay devices, nasal sheaths, were evaluated regarding reduction of aerosol escape from the nose. RESULTS: The drilling of cadaveric specimens without flexible suction generated aerosols ≤3.30 µm, and drilling of 3D sinonasal models consistently produced aerosols ≤14.1 µm. Mitigation with SPIWay or diameter-restricted SPIWay produced same results. There was minimal field contamination in the cadaveric models, 0% to 2.77% field tarp area, regardless of drill burr type or drilling location; cutting burr drilling without suction in the 3D model yielded the worst contamination field (36.1%), followed by coarse diamond drilling without suction (19.4%). The simple placement of a flexible suction instrument in the nasal cavity or nasopharynx led to complete elimination of all aerosols ≤14.1 µm, as evaluated by a cascade impactor positioned immediately at the nares. CONCLUSION: Given the findings regarding aerosol risk reduction, we strongly recommend that physicians use a suction instrument in the nasal cavity or nasopharynx during endonasal surgery in the COVID-19 era.

Peri-procedural Anticoagulation in Patients with Head and Neck Versus Extremity Venous Malformations.

OBJECTIVE: (1) Review a multidisciplinary vascular anomalies center's practice regarding periprocedural anticoagulation for venous malformations (VM) and the associated risk of thromboembolic and disseminated intravascular coagulation (DIC) events. (2) Compare the risk of thromboembolic events and DIC post-procedure between head and neck (H&N) and extremity VM patients. METHODS: An Institutional Review Board (IRB)-approved, retrospective chart review was performed on 120 VM patients. A thromboembolic event was defined as a thrombus formation post-sclerotherapy or post-surgery within 2 months in a distant or local venous structure not directly addressed by the procedure. RESULTS: There were 39 cases involving the H&N and 81 cases based at the extremities. There were eight cases of post-procedure thrombus formation within the extremity VM group (8/71; 11.3%) as opposed to 0 cases in the H&N group (OR: 0, 95% CI .00-.09), p = .049. There was no difference in incidence of post-procedure thromboembolic events between those with elevated D-dimer (H&N: 0%, extremity: 22.7%, 5/22) and normal D-dimer values (H&N: 0%, extremity: 6.3% [1/16], P = .370). There was no difference in incidence of post-procedure thromboembolic events between those who received periprocedural anticoagulation (H&N: 0%, extremity: 21%, 4/19) and those who did not (H&N: 0%, extremity: 8.2%, 4/49), (Extremity: OR: 3.00, .67-13.50, P = .206). CONCLUSION: Post-procedure thromboembolism is rare in the treatment of venous malformations, especially in the head and neck subsite. Regardless of anticoagulation use, there were no thromboembolic events for H&N VM patients. Such events are rare, and the odds may approach zero, especially with small sample size. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1163-1167, 2021.

Regulation of Iron Homeostasis through Parkin-Mediated Lactoferrin Ubiquitylation.

Somatic mutations that perturb Parkin ubiquitin ligase activity and the misregulation of iron homeostasis have both been linked to Parkinson's disease. Lactotransferrin (LTF) is a member of the family of transferrin iron binding proteins that regulate iron homeostasis, and increased levels of LTF and its receptor have been observed in neurodegenerative disorders like Parkinson's disease. Here, we report that Parkin binds to LTF and ubiquitylates LTF to influence iron homeostasis. Parkin-dependent ubiquitylation of LTF occurred most often on lysines (K) 182 and 649. Substitution of K182 or K649 with alanine (K182A or K649A, respectively) led to a decrease in the level of LTF ubiquitylation, and substitution at both sites led to a major decrease in the level of LTF ubiquitylation. Importantly, Parkin-mediated ubiquitylation of LTF was critical for regulating intracellular iron levels as overexpression of LTF ubiquitylation site point mutants (K649A or K182A/K649A) led to an increase in intracellular iron levels measured by ICP-MS/MS. Consistently, RNAi-mediated depletion of Parkin led to an increase in intracellular iron levels in contrast to overexpression of Parkin that led to a decrease in intracellular iron levels. Together, these results indicate that Parkin binds to and ubiquitylates LTF to regulate intracellular iron levels. These results expand our understanding of the cellular processes that are perturbed when Parkin activity is disrupted and more broadly the mechanisms that contribute to Parkinson's disease.

Tracheostomy time-out: New safety tool in the setting of COVID-19.

Tracheostomy procedures have a high risk of aerosol generation. Airway providers have reflected on ways to mitigate the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) transmission risks when approaching a surgical airway. To standardize institutional safety measures with tracheostomy, we advocate using a dedicated tracheostomy time-out applicable to all patients including those suspected of having COVID-19. The aim of the tracheostomy time-out is to reduce preventable errors that may increase the risk of transmission of SARS-CoV-2.

Transition to a virtual multidisciplinary tumor board during the COVID-19 pandemic: University of Pittsburgh experience.

Multidisciplinary conferences (MDC) are an important component of head and neck oncologic care including diagnosis, treatment, and survivorship. Virtual MDC allows for improved collaboration between providers at distant sites and proper allocation of health care resources in a time of crisis. When approached systematically, a virtual MDC is feasible to design and implement in a large academic medical center with multiple satellite hospitals.

Difficult airway intubation simulation using Bonfils fiberscope and rigid fiberscope for surgical training.

INTRODUCTION: Pediatric otolaryngologists are frequently called to assist in difficult airway management in newborns with Pierre Robin Sequence (PRS) who have microretrognathia, glossoptosis, and an anterior larynx. The Bonfils fiberscope (BF) is a curved rigid scope designed to provide superior visualization in the anterior larynx. OBJECTIVE: (1) to assess whether BF provides an improvement in intubation success rate, time to intubation, or airway visualization as compared to rigid fiberscope (RF) in a difficult airway simulation setting and (2) to determine whether a training program for BF can improve time to intubation through practice trials. METHODS: Six right-handed trainees completed five trials on each of the three following airway models using the BF and RF: normal anatomy, anterior larynx and PRS. The normal larynx model was intubated only with RF. Main outcome measures were the time needed for tracheal intubation and Cormack-Lehane classification (1-4). RESULTS: The majority of the intubation trials showed a statistically significant difference between first and last completion times (p < .05) suggesting construct validity. Only the anterior larynx trials with BF did not demonstrate an improvement in time to intubation between first and last attempts (p < .3125). For the PRS retrognathia model, there was no statistically significant difference in time to intubation between using the BF and the RF (p < .44); in the anterior larynx model, the RF yielded a faster time to intubation than the BF on the final trial attempts (p < .0313). By Cormack-Lehane classification measures, laryngeal visualization by the BF was better than RF in the PRS manikin (p < .0022) while there was no significant difference in grade scores for the anterior larynx manikin (p < .45). All six trainees reported an improved visualization of the larynx with the BF compared to the RF for both the anterior larynx and PRS manikins; at the end of the trial runs, all participants noted an improvement in comfort level using the BF. CONCLUSION: The difficult airway simulation model is feasible for surgical training. BF adds superior visualization of the anterior larynx in PRS. Otolaryngology training programs may include BF as a supplemental tool in addition to RF as a part of the airway equipment training since there is significant improvement in time to intubation with consecutive practice trials and superior laryngeal visualization.

Mature and immature pediatric head and neck teratomas: A 15-year review at a large tertiary center.

INTRODUCTION: Pediatric head and neck teratomas account for less than 4% of congenital teratomas. The distinct presentations and outcomes of mature and immature head and neck teratomas have not been well established. OBJECTIVES: To review the management and outcomes of pediatric head and neck teratomas. To distinguish differences between mature and immature tumors with respect to the age at presentation and surgery, tumor size and location, alpha fetoprotein (AFP) levels, airway management, and recurrence. METHODS: A 15-year retrospective chart review of patients treated for head and neck teratomas at Texas Children's Hospital was performed. A total of 20 patients were included. Wilcoxon rank and Fisher's exact tests were used for statistical analysis. RESULTS: Immature teratomas were associated with both higher AFP levels (80800 ng/ml, p = 0.02) and maximum tumor dimensions (14.4 cm, p = 0.0034) than mature teratomas (24400 ng/ml and 6.44 cm). Patients with immature tumors were younger at the time of surgical resection (19.8 days, p = 0.025) compared to those with mature tumors (348 days). 89% of immature teratomas involved anterior neck localization compared to 27% for mature teratomas (p = 0.0098); 88% of the immature teratomas required an EXIT (Ex Utero Intrapartum Treatment) procedure compared with 40% of the mature teratomas (p = 0.0656). Recurrence was noted in only two cases: an immature teratoma 51 months after incomplete resection and a mature teratoma 33 months after complete resection. Long-term consequences of surgical resection included cleft palate (38.9%), dysphagia (33.3%), facial nerve paresis/paralysis (16.7%) and tracheotomy (16.7%). CONCLUSION: Immature teratomas had higher AFP levels, tumor dimensions, frequency of anterior neck localization, and requirement of EXIT than mature teratomas. Given that there was no significant difference between the recurrence rates of immature and mature teratomas, follow-up vigilance should be maintained equally regardless of tumor maturity.

Chondrosarcoma of the Epiglottis: A Case Report and Literature Review.

BACKGROUND: Laryngeal chondrosarcoma (LCS) is an exceedingly rare malignancy and least frequently reported in the epiglottis. METHODS AND RESULTS: We report a case of a 71-year-old male smoker who presented with sore throat, dysphagia, and odynophagia for 3 months. Computed tomography scan revealed an inhomogeneously enhancing necrotic mass emanating from the right epiglottis and traveling along the aryepiglottic fold. The mass was removed via endoscopic en bloc laser resection. Histopathological examination of the biopsied specimen confirmed low-grade LCS of the epiglottis. CONCLUSION: Differentiating low-grade LCS from laryngeal chondroma is a challenging task with an increased risk of potential misdiagnosis. This case study reinforces the need for a histopathological examination in forming the basis for ongoing management and follow-up observations. Timely surgery remains the optimal, primary means of treatment notwithstanding the rarity of LCS.

Tctex1d2 associates with short-rib polydactyly syndrome proteins and is required for ciliogenesis.

Short-rib polydactyly syndromes (SRPS) arise from mutations in genes involved in retrograde intraflagellar transport (IFT) and basal body homeostasis, which are critical for cilia assembly and function. Recently, mutations in WDR34 or WDR60 (candidate dynein intermediate chains) were identified in SRPS. We have identified and characterized Tctex1d2, which associates with Wdr34, Wdr60 and other dynein complex 1 and 2 subunits. Tctex1d2 and Wdr60 localize to the base of the cilium and their depletion causes defects in ciliogenesis. We propose that Tctex1d2 is a novel dynein light chain important for trafficking to the cilium and potentially retrograde IFT and is a new molecular link to understanding SRPS pathology.