Unique orbital biphenotypic tumour with neuroendocrine and sebaceous carcinoma features.

A 60-year-old female presented with a large, left upper eyelid mass that had rapidly expanded in the 3 months prior to presentation. She had a presumed chalazion excised from the same area 1.5 years ago, but no pathology was investigated. On examination, she had a palpebral mass measuring 4.5 cm x 3.5 cm that abutted the globe with extensive conjunctival involvement. Neuroimaging demonstrated lesions concerning for parotid gland metastases. An incisional biopsy demonstrated synaptophysin-positive small blue cells concerning for neuroendocrine carcinoma. The patient underwent orbital exenteration with parotidectomy and radical neck dissection. The excised mass was found to have distinct neuroendocrine carcinoma cells intermingled with sebaceous carcinoma cells, a combination not previously reported.

Aerosol and droplet generation from mandible and midface fixation: Surgical risk in the pandemic era.

PURPOSE: The COVID-19 pandemic has led to concerns over transmission risk from healthcare procedures, especially when operating in the head and neck such as during surgical repair of facial fractures. This study aims to quantify aerosol and droplet generation from mandibular and midface open fixation and measure mitigation of airborne particles by a smoke evacuating electrocautery hand piece. MATERIALS AND METHODS: The soft tissue of the bilateral mandible and midface of two fresh frozen cadaveric specimens was infiltrated using a 0.1% fluorescein solution. Surgical fixation via oral vestibular approach was performed on each of these sites. Droplet splatter on the surgeon's chest, facemask, and up to 198.12 cm (6.5 ft) away from each surgical site was measured against a blue background under ultraviolet-A (UV-A) light. Aerosol generation was measured using an optical particle sizer. RESULTS: No visible droplet contamination was observed for any trials of mandible or midface fixation. Total aerosolized particle counts from 0.300-10.000 µm were increased compared to baseline following each use of standard electrocautery (n = 4, p < 0.001) but not with use of a suction evacuating electrocautery hand piece (n = 4, p = 0.103). Total particle counts were also increased during use of the powered drill (n = 8, p < 0.001). CONCLUSIONS: Risk from visible droplets during mandible and midface fixation is low. However, significant increases in aerosolized particles were measured after electrocautery use and during powered drilling. Aerosol dispersion is significantly decreased with the use of a smoke evacuating electrocautery hand piece.

Aerosol and droplet generation from orbital repair: Surgical risk in the pandemic era.

INTRODUCTION: The highly contagious COVID-19 has resulted in millions of deaths worldwide. Physicians performing orbital procedures may be at increased risk of occupational exposure to the virus due to exposure to secretions. The goal of this study is to measure the droplet and aerosol production during repair of the inferior orbital rim and trial a smoke-evacuating electrocautery handpiece as a mitigation device. MATERIAL AND METHODS: The inferior rim of 6 cadaveric orbits was approached transconjunctivally using either standard or smoke-evacuator electrocautery and plated using a high-speed drill. Following fluorescein inoculation, droplet generation was measured by counting under ultraviolet-A (UV-A) light against a blue background. Aerosol generation from 0.300-10.000 µm was measured using an optical particle sizer. Droplet and aerosol generation was compared against retraction of the orbital soft tissue as a negative control. RESULTS: No droplets were observed following the orbital approach using electrocautery. Visible droplets were observed after plating with a high-speed drill for 3 of 6 orbits. Total aerosol generation was significantly higher than negative control following the use of standard electrocautery. Use of smoke-evacuator electrocautery was associated with significantly lower aerosol generation in 2 of 3 size groups and in total. There was no significant increase in total aerosols associated with high-speed drilling. DISCUSSION AND CONCLUSIONS: Droplet generation for orbital repair was present only following plating with high-speed drill. Aerosol generation during standard electrocautery was significantly reduced using a smoke-evacuating electrocautery handpiece. Aerosols were not significantly increased by high-speed drilling.

Evolving microbial patterns of acute mastoiditis in pediatric patients undergoing mastoidectomy.

OBJECTIVE: To investigate the microbial patterns and clinical outcomes of pediatric patients undergoing mastoidectomy for acute coalescent mastoiditis and to identify factors associated with poor outcomes and/or prolonged treatment. STUDY DESIGN: Monocentric retrospective cohort study. SETTING: Tertiary referral pediatric hospital in Indiana. METHODS: By cross-referencing database data from the Pediatric Health Information System (PHIS) querying for all inpatient stays (patients younger than eighteen) with a diagnostic code of mastoiditis between January 1st, 2010 and August 31, 2019, and the electronic health record (Cerner) for Riley Hospital for Children, 46 patients with mastoidectomy were included. A two-tailed T-test was used to evaluate continuous parametric data. Statistical significance was determined as P < 0.05. For continuous variables, data was analyzed using continuous logistic regression. A criteria of p > 0.1 was used for inclusion in the multivariate regression. RESULTS: Inclusion criteria was met by 46 patients. From 2010 to 2019, S. pyogenes and S. pneumoniae were the most common bacteria, each isolated in 11 of 42 bacterial isolates (26.2%). There was no growth in 35.4% (17/48) of intra-operative wound cultures. On univariate analysis, patients with negative cultures had longer length of hospital stay (LOS) (7.7 days [6.5] vs. 4.3 [2.8]; p = 0.018) as well as higher rates of PICC (peripherally inserted central catheter) placement (53.3% vs. 19.4%; p = 0.021). There was a statistically significant difference in terms of gender (p = 0.021), with 15 males and 16 females in the positive culture cohort and 13 males and 2 females in the negative culture cohort. On multivariate analysis, which included gender, PICC placement, both intracranial and extracranial complications, duration of antibiotics, and LOS, female gender was the only significant predictor of positive culture status (p = 0.039). CONCLUSION: S. pyogenes and S. pneumoniae were the predominant etiologic agents in acute coalescent mastoiditis between 2010 and 2019, and negative wound cultures were associated with worse clinical outcomes.

Droplet Exposure Risk to Providers From In-Office Flexible Laryngoscopy: A COVID-19 Simulation.

To provide data on risk of respiratory droplets from common otolaryngologic procedures during the COVID-19 pandemic, a novel simulation of droplet exposure from flexible laryngoscopy was performed. After completion of a nasal symptom questionnaire, topical fluorescein spray was administered into the nasal and oropharynx of 10 healthy volunteers, who then underwent flexible laryngoscopy under 2 conditions: routine without provoked response and with prompted sneeze/cough. After each, droplets on the proceduralist and participant were counted under ultraviolet A light. Droplets were observed on 1 of 10 volunteers after routine laryngoscopy and 4 of 10 during laryngoscopy with sneeze/cough. A nasal symptom score based on congestion and rhinorrhea was significantly elevated among droplet producers after sneeze/cough (P = .0164). No droplets were observed on the provider. Overall, with adequate personal protective equipment, flexible laryngoscopy poses minimal droplet risk to providers. Nasal symptoms can identify patients more likely to produce droplets after sneeze/cough.

Aerosol Generation During Myringotomy With Tympanostomy Tube Insertion: Implications for Otolaryngology in the COVID-19 Era.

The risk of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) transmission to health care workers during myringotomy and tympanostomy tube (MT) insertion is unknown. To determine the need for enhanced precautions to prevent potential spread via aerosolized particles, we used an optical particle sizer to measure aerosol generation intraoperatively during a case series of MT insertion. We also discuss our institutional experience with safe pandemic-era perioperative practices. There was no measured increase in aerosol particle number during the procedure at a distance of 30 cm from the external auditory canal. These initial data are reassuring regarding the risk of SARS-CoV-2 transmission to the operating room team due to aerosol generation, but further study is necessary before making definitive recommendations.

Aerosol generation during cadaveric simulation of otologic surgery and live cochlear implantation.

OBJECTIVE: The risk of SARS-CoV-2 transmission to healthcare workers through airborne aerosolization during otologic surgery has not been characterized. The objective of this study was to describe and quantify the aerosol generation during common otologic procedures in both cadaveric surgical simulation and live patient surgery. METHODS: The number concentrations of generated aerosols in the particle size range of 0.30 to 10.0 µm were quantified using an optical particle sizer during both a cadaveric simulation of routine otologic procedures as well as cochlear implant surgery on live patients in the operating room. RESULTS: In the cadaveric simulation, temporalis fascia graft harvest using cold techniques (without electrocautery) (n = 4) did not generate aerosols above baseline concentrations. Tympanoplasty (n = 3) and mastoidectomy (n = 3) both produced statistically significant increases in concentrations of aerosols (P < 0.05), predominantly submicron particles (< 1.0 µm). High-speed, powered drilling of the temporal bone during mastoidectomy with a Multi Flute cutting burr resulted in higher peak concentrations and greater number of spikes in aerosols than with a diamond burr. In the operating room, spikes in aerosols occurred during both cochlear implant surgeries. CONCLUSION: In the cadaveric simulation, temporalis fascia graft harvest without electrocautery did not generate aerosol levels above baseline, while significant aerosol levels were generated during mastoidectomy and to a much less degree during tympanoplasty. Aerosol spikes were appreciated during cochlear implantation surgery in live patients. LEVEL OF EVIDENCE: 2.

Aerosol and Droplet Generation from Open Rhinoplasty: Surgical Risk in the Pandemic Era.

Introduction: The coronavirus disease 2019 pandemic has led to concerns over transmission risk from head and neck operations including facial cosmetic surgeries. Objectives: To quantify droplet and aerosol generation from rhinoplasty techniques in a human anatomic specimen model using fluorescein staining and an optical particle sizer. Methods: Noses of human anatomic specimens were infiltrated using 0.1% fluorescein. Droplets and aerosols were measured during rhinoplasty techniques including opening the skin-soft tissue envelope, monopolar electrocautery, endonasal rasping, endonasal osteotomy, and percutaneous osteotomy. Results: No visible droplet contamination was observed for any rhinoplasty techniques investigated. Compared with the negative control of anterior rhinoscopy, total 0.300-10.000 µm aerosols were increased after monopolar electrocautery (p < 0.001) and endonasal rasp (p = 0.003). Opening the skin-soft tissue envelope, endonasal osteotomies, and percutaneous osteotomies did not generate a detectable increase in aerosols (p > 0.15). Discussion and Conclusions: In this investigation, droplets were not observed under ultraviolet light, and aerosol generation was noted only with cautery and endonasal rasping.

Mitigation of Aerosols Generated During Rhinologic Surgery: A Pandemic-Era Cadaveric Simulation.

OBJECTIVE: After significant restrictions initially due to the COVID-19 pandemic, otolaryngologists have begun resuming normal clinical practice. However, the risk of SARS-CoV-2 transmission to health care workers through aerosolization and airborne transmission during rhinologic surgery remains incompletely characterized. The objective of this study was to quantify the number concentrations of aerosols generated during rhinologic surgery with and without interventions involving 3 passive suction devices. STUDY DESIGN: Cadaver simulation. SETTING: Dedicated surgical laboratory. SUBJECTS AND METHODS: In a simulation of rhinologic procedures with and without different passive suction interventions, the concentrations of generated aerosols in the particle size range of 0.30 to 10.0 µm were quantified with an optical particle sizer. RESULTS: Functional endoscopic sinus surgery with and without microdebrider, high-speed powered drilling, use of an ultrasonic aspirator, and electrocautery all produced statistically significant increases in concentrations of aerosols of various sizes (P < .05). Powered drilling, ultrasonic aspirator, and electrocautery generated the highest concentration of aerosols, predominantly submicroparticles <1 µm. All interventions with a suction device were effective in reducing aerosols, though the surgical smoke evacuation system was the most effective passive suction method in 2 of the 5 surgical conditions with statistical significance (P < .05). CONCLUSION: Significant aerosol concentrations were produced in the range of 0.30 to 10.0 µm during all rhinologic procedures in this cadaver simulation. Rhinologic surgery with a passive suction device results in significant mitigation of generated aerosols.

Long-term neck and shoulder function among survivors of oropharyngeal squamous cell carcinoma treated with chemoradiation as assessed with the neck dissection impairment index.

BACKGROUND: Of interest is the long-term neck and shoulder impairment of patients treated with primary chemoradiotherapy (CRT). This is important for counseling patients regarding treatment decisions when discussing primary CRT. METHODS: A cross-sectional study to identify factors that contribute to neck and shoulder dysfunction in patients treated with primary CRT. We utilized the neck dissection impairment index (NDII). Eighty-seven patients treated between 2003 and 2010, who were free of disease, responded; 24 of these 87 underwent post-CRT neck dissection. Mean interval since completion of CRT was over 5 years (62.7 months). Mean age, 63.5 years, male:female 75:12. RESULTS: Mean NDII score was 87.4 (SD 22.1, range 5-100). Multiple linear regression revealed worse NDII scores for patients with larger pre-CRT gross tumor nodal volume (GTVnodal), controlled for age, sex, body mass index (BMI), and the presence of neck dissection (p = 0.02). There were significant associations with increasing GTVnodal and "low" scores for components of the NDII that assessed neck pain (p = 0.02), neck stiffness (p = 0.01), lifting heavy objects (p = 0.02), reaching overhead (p = 0.02), and ability to do work (p = 0.02). Physical therapy (PT) was evaluated as an "anchor" but it was prescribed "as needed." Regression revealed participation in PT was associated with higher GTVnodal, lower BMI, presence of neck dissection, and female sex (p = 0.00007). CONCLUSION: GTVnodal was an independent predictor of neck and shoulder impairment. High GTVnodal was associated with increased pain and stiffness, and increased difficulty lifting heavy objects, reaching overhead, overall ability to perform work-related tasks and was associated with participation in post-treatment PT.

Aerosol generation during routine rhinologic surgeries and in-office procedures.

OBJECTIVE: Cadaveric simulations have shown endonasal drilling and cautery generate aerosols, which is a significant concern for otolaryngologists during the COVID-19 era. This study quantifies aerosol generation during routine rhinologic surgeries and in-office procedures in live patients. METHODS: Aerosols ranging from 0.30 to 10.0 µm were measured in real-time using an optical particle sizer during surgeries and in-office procedures. Various mask conditions were tested during rigid nasal endoscopy (RNE) and postoperative debridement (POD). RESULTS: Higher aerosol concentrations (AC) ranging from 2.69 to 10.0 µm were measured during RNE (n = 9) with no mask vs two mask conditions (P = .002 and P = .017). Mean AC (0.30-10.0 µm) were significantly higher during POD (n = 9) for no mask vs a mask covering the patient's mouth condition (mean difference = 0.16 ± 0.03 particles/cm3, 95% CI 0.10-0.22, P < .001). There were no discernible spikes in aerosol levels during endoscopic septoplasty (n = 3). Aerosol spikes were measured in two of three functional endoscopic sinus surgeries (FESS) with microdebrider. Using suction mitigation, there were no discernible spikes during powered drilling in two anterior skull base surgeries (ASBS). CONCLUSION: Use of a surgical mask over the patient's mouth during in-office procedures or a mask with a slit for an endoscope during RNE significantly diminished aerosol generation. However, whether this reduction in aerosol generation is sufficient to prevent transmission of communicable diseases via aerosols was beyond the scope of this study. There were several spikes in aerosols during FESS and ASBS, though none were associated with endonasal drilling with the use of suction mitigation. LEVEL OF EVIDENCE: 4.

Pak1 regulates multiple c-Kit mediated Ras-MAPK gain-in-function phenotypes in Nf1+/- mast cells.

Neurofibromatosis type 1 (NF1) is a common genetic disorder caused by mutations in the NF1 locus, which encodes neurofibromin, a negative regulator of Ras. Patients with NF1 develop numerous neurofibromas, which contain many inflammatory mast cells that contribute to tumor formation. Subsequent to c-Kit stimulation, signaling from Ras to Rac1/2 to the MAPK pathway appears to be responsible for multiple hyperactive mast cell phenotypes; however, the specific effectors that mediate these functions remain uncertain. p21-activated kinase 1 (Pak1) is a downstream mediator of Rac1/2 that has been implicated as a positive regulator of MAPK pathway members and is a modulator of cell growth and cytoskeletal dynamics. Using an intercross of Pak 1(-/-) mice with Nf1(+/-) mice, we determined that Pak1 regulates hyperactive Ras-dependent proliferation via a Pak1/Erk pathway, whereas a Pak1/p38 pathway is required for the increased migration in Nf1(+/-) mast cells. Furthermore, we confirmed that loss of Pak1 corrects the dermal accumulation of Nf1(+/-) mast cells in vivo to levels found in wild-type mice. Thus, Pak1 is a novel mast cell mediator that functions as a key node in the MAPK signaling network and potential therapeutic target in NF1 patients.

COVID-19 Status Differentially Affects Olfaction: A Prospective Case-Control Study.

OBJECTIVE: The symptoms and long-term sequelae of SARS-CoV-2 infection have yet to be determined, and evaluating possible early signs is critical to determine which patients should be tested and treated. The objective of this ongoing study is to evaluate initial and short-term rhinologic symptoms, olfactory ability, and general quality of life in patients undergoing SARS-CoV-2 testing. STUDY DESIGN: Prospective case-control. SETTING: Academic institute. METHODS: Adult patients tested for SARS-CoV-2 were prospectively enrolled and separated into positive and negative groups. Each participant completed 4 validated patient-reported outcome measures. The UPSIT (University of Pennsylvania Smell Identification Test) was distributed to patients who were SARS-CoV-2 positive. RESULTS: The positive group reported significantly decreased sense of smell and taste on the 22-item Sinonasal Outcome Test (SNOT-22) as compared with the negative group (mean ± SD: 3.4 ± 1.7 vs 1.2 ± 1.4, P < .001). The positive group had a much higher probability of reporting a decrease in smell/taste as "severe" or "as bad as it can be" (63.3% vs 5.8%) with an odds ratio of 27.6 (95% CI, 5.9-128.8). There were no differences between groups for overall SNOT-22 domain scores, PHQ-4 depression/anxiety (Patient Health Questionnaire-4), and 5-Level EQ-5D quality-of-life scores. Mean Self-MOQ (Self-reported Mini Olfactory Questionnaire) scores were 7.0 ± 5.6 for the positive group and 1.8 ± 4.0 for the negative group (P < .001). The mean UPSIT score was 28.8 ± 7.2 in the positive group. CONCLUSION: Symptomatic patients who are SARS-CoV-2 positive report severe olfactory and gustatory dysfunction via the Self-MOQ and SNOT-22 as compared with symptomatic patients testing negative.

Cadaveric Simulation of Endoscopic Endonasal Procedures: Analysis of Droplet Splatter Patterns During the COVID-19 Pandemic.

OBJECTIVE: The primary mode of viral transmission of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is thought to occur through the spread of respiratory droplets. The objective of this study was to investigate droplet and splatter patterns resulting from common endoscopic endonasal procedures. STUDY DESIGN: Cadaver simulation series. SETTING: Dedicated surgical laboratory. SUBJECTS AND METHODS: After instilling cadaver head specimens (n = 2) with fluorescein solution, endoscopic endonasal procedures were systematically performed to evaluate the quantity, size, and distance of droplets and splatter following each experimental condition. RESULTS: There were no observable fluorescein droplets or splatter noted in the measured surgical field in any direction after nasal endoscopy, septoplasty with microdebrider-assisted turbinoplasty, cold-steel functional endoscopic sinus surgery (FESS), and all experimental conditions using an ultrasonic aspirator. Limited droplet spread was noted with microdebrider FESS (2 droplets, <1 mm in size, within 10 cm), drilling of the sphenoid rostrum with a diamond burr (8, <1 mm, 12 cm), and drilling of the frontal beak with a cutting burr (5, <1 mm, 9 cm); however, the use of concurrent suction while drilling resulted in no droplets or splatter. The control condition of external activation of the drill resulted in gross contamination (11, 2 cm, 13 cm). CONCLUSION: Our results indicate that there is very little droplet generation from routine rhinologic procedures. The droplet generation from drilling was mitigated with the use of concurrent suction. Extreme caution should be used to avoid activating powered instrumentation outside of the nasal cavity, which was found to cause droplet contamination.

Cadaveric Simulation of Otologic Procedures: An Analysis of Droplet Splatter Patterns During the COVID-19 Pandemic.

OBJECTIVE: The otolaryngology community has significant concerns regarding the spread of SARS-CoV-2 through droplet contamination and viral aerosolization during head and neck examinations and procedures. The objective of this study was to investigate the droplet and splatter contamination from common otologic procedures. STUDY DESIGN: Cadaver simulation series. SETTING: Dedicated surgical laboratory. METHODS: Two cadaver heads were prepped via bilateral middle cranial fossa approaches to the tegmen (n = 4). Fluorescein was instilled through a 4-mm burr hole drilled into the middle cranial fossa floor, and presence in the middle ear was confirmed via microscopic ear examination. Myringotomy with ventilation tube placement and mastoidectomy were performed, and the distribution and distance of resulting droplet splatter patterns were systematically evaluated. RESULTS: There were no fluorescein droplets or splatter contamination observed in the measured surgical field in any direction after myringotomy and insertion of ventilation tube. Gross contamination from the surgical site to 6 ft was noted after complete mastoidectomy, though, when performed in standard fashion. CONCLUSION: Our results show that there is no droplet generation during myringotomy with ventilation tube placement in an operating room setting. Mastoidectomy, however, showed gross contamination 3 to 6 ft away in all directions measured. Additionally, there was significantly more droplet and splatter generation to the left of the surgeon when measured at 1 and 3 ft as compared with all other measured directions.

Pathogenetic Analysis of Sinonasal Teratocarcinosarcomas Reveal Actionable ß-catenin Overexpression and a ß-catenin Mutation.

Objective Sinonasal teratocarcinosarcomas are rare, aggressive tumors of the skull base. Treatment options are limited and outcomes are poor. Little is known in regard to the genetic factors regulating these tumors. Characterization of actionable molecular alterations in these tumors could provide potentially successful therapeutic options. Methods We performed targeted exome sequencing on an index sinonasal teratocarcinosarcoma specimen to identify potential driver mutations. We performed immunohistochemical stains for ß-catenin on paraffin-embedded tissue on the index tumor and a subsequent teratocarcinosarcoma. Online databases of cancer mutations (Catalogue of Somatic Mutations in Cancer and The Cancer Genome Atlas) were accessed. Results We identified an activating p.S45F mutation in ß-catenin in our index sinonasal teratocarcinosarcoma. This mutation results in constitutive signaling in the Wnt/ß-catenin pathway. We confirmed ß-catenin overexpression and nuclear localization via immunohistochemistry in the index tumor and a second patient. The p.S45F activating mutation was found in a variety of solid tumors, and accounts for 3.3 to 10.4% of all known ß-catenin mutations. Conclusion We identified a potential driver mutation in ß-catenin in a sinonasal teratocarcinosarcoma, resulting in ß-catenin overexpression. These findings suggest a role for the Wnt/ß-catenin pathway in sinonasal teratocarcinosarcoma tumorigenesis and a role for anti-ß-catenin targeted therapy.

A rare case of Kussmaul Disease (Sialodochitis Fibrinosa).

Sialodochitis fibrinosa (or commonly known as Kussmaul Disease) is a rare salivary gland disease characterized by recurrent salivary gland swelling and pain as a result of mucofibrinous plugs. Typically patients have a history of multiply recurrent glandular swelling, dehydration and/or decreased salivary flow, thick secretions from Stensen's or Wharton's duct, and/or history of allergic diseases. Retention of mucofibrinous plugs may lead to acute suppurative parotitis and chronic sialadenitis ultimately. The diagnosis is one of exclusion, and treatment is based on symptomatology and largely supportive.

The incidence of postoperative aspiration among children undergoing supraglottoplasty for laryngomalacia.

We conducted a retrospective study to determine the incidence of aspiration after supraglottoplasty at Riley Hospital for Children in Indianapolis. We reviewed the charts of 468 patients-281 males and 187 females, aged 2 days to 20 years-who had undergone supraglottoplasty for the treatment of laryngomalacia; most patients (69.9%) were aged 28 days to 2 years. A total of 47 patients (10.0%) experienced aspiration after supraglottoplasty; the overall association between supraglottoplasty and aspiration was not statistically significant (p = 0.25). Aspiration was positively correlated with age younger than 18 months, the performance of a revision procedure, the presence of an underlying neuromuscular disorder (n = 20), and the need for a postoperative gastrostomy tube (p < 0.001 for all). When the 20 patients with a neuromuscular disorder were excluded from our data analysis, the incidence of aspiration after supraglottoplasty fell to only 5.8% (27/468). We conclude that supraglottoplasty is a safe and effective procedure for the treatment of laryngomalacia. It does not significantly increase the overall incidence of aspiration in children, and thus the risk of aspiration should not be considered a deterrent to surgery, even in children with neuromuscular problems.