Head and Neck Manifestations of Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review.

OBJECTIVE: To conduct the first and only systematic review of the existing literature on head and neck manifestations of eosinophilic granulomatosis with polyangiitis to guide clinical decision making for the otolaryngologist. DATA SOURCES: PubMed, Cochrane Library, Scopus, and LILACS. REVIEW METHODS: A systematic review of the aforementioned sources was conducted per the PRISMA guidelines. RESULTS: From an initial 574 studies, 28 trials and reports were included, accounting for a total of 1175 patients with eosinophilic granulomatosis with polyangiitis. Among clinical and cohort studies, 48.0% to 96.0% of all included patients presented with head and neck manifestations. In a distinct group of patients detailed in case reports describing patients presenting with head and neck manifestations, patients on average fulfilled 4.6 diagnostic criteria per the American College of Rheumatology. Furthermore, 95.8% of reported cases were responsive to steroids, and 60% required additional therapy. CONCLUSION: Otolaryngologists are in a unique position for the early diagnosis and prevention of late complications of eosinophilic granulomatosis with polyangiitis. The American College of Rheumatology criteria should be relied on in the diagnostic workup. Close surveillance of these patients in a multidisciplinary fashion and with baseline complete blood counts, chest radiographs, and autoimmune laboratory tests is often necessary. Such patients with head and neck manifestations of the disease are nearly always responsive to steroids and often require additional immunosuppressive therapy or surgical intervention in cases of cranial neuropathies, temporal bone involvement, and refractory symptoms

Orbital Considerations in Sinonasal Malignancy

Outline: Anatomy Epidemiology Presentation Pathology Orbital Invasion Evaluation Management Sequela Disease-specific management Research Presentation: 35:18 PowerPoint slides located at bottom of this page

Biphenotypic Sinonasal Sarcoma-Case Report and Review of Clinicopathological Features and Diagnostic Modalities.

Background Biphenotypic sinonasal sarcoma is a recently described malignancy showing dual differentiation with both myogenic and neural elements. Due to its histologic similarities to other sinonasal malignancies, it is a diagnostic challenge. Objective The main purpose of this article is to report a case of biphenotypic sinonasal sarcoma and to consolidate data and provide a comprehensive review regarding pathological differences between biphenotypic sarcoma and other sinonasal malignancies and diagnostic modalities used for biphenotypic sarcoma. Material and Methods A systematic review of all cases of biphenotypic sinonasal sarcoma was performed using electronic databases (PubMed and Medline). Data collected included age, gender, symptoms, sub-site of origin, immunophenotyping, metastasis, recurrence, treatment, duration of follow-up, and survival outcomes. Results Ninety-five cases of biphenotypic sarcoma were found with mean age at diagnosis of 52.36 years (range, 24-87 years). Female to male ratio was 2.27:1. Extra-sinonasal extension was present in 28%. Immunophenotyping revealed that S-100 and SMA (smooth muscle actin) were consistently positive, while SOX-10 was consistently negative. PAX3-MAML3 fusion [t (2; 4) (q35; q31.1)] was the most common genetic rearrangement. Surgical excision with or without adjuvant radiotherapy was the most frequent treatment modality used. Recurrence was observed in 32% of cases with follow-up. None of the cases reported metastasis. Three patients had died at the time of publication that included one case with intracranial extension. Conclusion Biphenotypic sarcoma is distinct sinonasal malignancy with unique clinicopathological features. Testing involving a battery of myogenic and neural immunomarkers is essential for diagnostic confirmation and is a clinically useful endeavor when clinical suspicion is high. © 2019 Georg Thieme Verlag KG Stuttgart. New York

Usage of sleep domain of SNOT-22 scores in screening for OSA in patients of chronic rhinosinusitis.

Introduction: SNOT-22 is a screening tool universally applied in perioperative settings for endoscopic sinus surgery (ESS), typically for chronic rhinosinusitis (CRS). Comorbid obstructive sleep apnea (OSA) increases risk of perioperative complications following ESS, yet pre-ESS OSA screening is not performed. This study sought to determine whether the sleep subdomain SNOT-22 (SD-SNOT) score could be useful for OSA screening. Methods: A retrospective study was performed by reviewing electronic records of 108 patients that underwent endoscopic sinus surgery between November 2019 – January 2020. Pre-operative and 2 months post-operative SD-SNOT scores were compared with the presence or absence of OSA. Results: Mean sleep pre-ESS scores was 16.04±11.95 while 2-month post-operative score was 8.84±10.55. SD-SNOT score preoperatively (22.4±12.9 vs. 14.7±11.4; p=0.02) and post-operatively (20.8±15.0 vs. 6.8±8.6; p=0.02) were significantly higher in the OSA cohort. The lowest SD-SNOT scores that were significantly associated with diagnosis of OSA were 21 for preoperative (OR: 3.5; CI:1.1-10.7; p=0.02) and 16 for postoperative (OR:10.5; CI:1.3-83.5; p=0.03). Discussion: SD-SNOT is a useful parameter in screening for OSA. Scores \u3e 20 preoperatively and \u3e15 at 2 months postoperatively may indicate comorbid OSA. These results allow the repurposing of SNOT-22, an already widespread perioperative tool, to be used to detect potential undiagnosed OSA among chronic rhinosinusitis patients

Castleman Disease in the Pediatric Neck: Case Report and Literature Review

Objective: To investigate the common features of cervical pediatric Castleman disease (CD). Study Design: Case report and literature review of pediatric patients with cervical CD. Methods: Online medical journal databases were searched for patients aged 18 years or younger. Eighteen published papers were found, comprising 29 cases. One case from our institution was also included for a total of 30 patients. Results: An asymptomatic mass in level V was the most common presentation. No gender differences were noted. Multiple forms of imaging were pursued, and no particular modality showed signs specific for CD. All cases were treated with complete surgical excision and diagnosed as hyaline-vascular type on histology, except for one case, where histologic type was not reported. No reports of multicentric disease, plasma cell, or mixed histology were found. No recurrences were reported. Conclusions: This poster provides the largest known literature review of pediatric patients with cervical CD. In our analysis, there is a higher propensity for level V than previously reported in small studies. While CD is rare, it should be considered on the differential for a pediatric neck mass, particularly when presenting with an asymptomatic posterior neck mass and equivocal work-up. Fortunately, our study suggests that, if diagnosed as CD, the most likely diagnosis is hyaline-vascular type for which the long-term prognosis is good. Surgical excision is both diagnostic and therapeutic

Endoscopic Ultrasonic Dacryocystorhinostomy for Recurrent Dacryocystitis Following Rhinoplasty

Abstract: The lacrimal sac is the structure most vulnerable to injury when performing osteotomies for rhinoplasty. When performed in a low lateral position or along the frontal process of the frontal-maxillary suture, osteotomies have the potential to tear the medial canthal ligament and injure the underlying lacrimal sac resulting in dacryocystitis. We report a case of dacryocystitis in a 19 year old male who presented with recurrent episodes of pain, tearing, and discharge from his left eye following primary rhinoplasty. He was found to have obstruction of the lacrimal system secondary to a low lateral osteotomy with an impinging bone fragment on imaging. Endoscopic dacryocystorhinostomy was performed using a Sonopet® ultrasonic bone aspirator under image guidance to remove the bone fragments posing risk to further injury to the lacrimal sac and orbit. Patency of the nasolacrimal duct was achieved and the patient remained symptom free at 6 month follow up. We describe the first case of recurrent dacryocystitis following rhinoplasty requiring treatment by an endoscopic dacryocystorhinostomy (DCR). Endoscopic DCR with the use of the ultrasonic bone aspirator provides several advantages over open DCR, including the lack of an external incision and decreased risk of injury to the adjacent orbital soft tissue anatomy including the lacrimal system

COVID Smell Tracker: A research-based mobile application to study smell loss in subjects with COVID-19

Introduction: Up to 60% of people infected with SARS-CoV-2 report anosmia or ageusia during their disease course. “COVID Smell Tracker” is a smart phone application (app) developed to elucidate the onset, duration and extent of anosmia and ageusia through questionnaires. Methods: “COVID Smell Tracker” is publicly available on smart phone devices (www.covidsmelltracker.org). Users complete surveys around demographics, medical history, COVID status and symptomology. Deidentified responses were collated and analyzed using descriptive statistics. Results: Of the 266 users included, the majority were located in Europe (43%) and North America (33%). Male, Caucasian users were most common (54.9% and 61.7% respectively), followed by Indian (10.5%) and Latino (9.4%). The majority of users reported no COVID testing (63%). 164 users reported COVID-related symptoms, and 57% of them reported olfactory dysfunction. Users who were younger age (p = 0.0003) and with type A and B blood types (p = 0.021) experienced smell loss at higher frequencies. Dysgeusia was associated with 28-34% of patients with concomitant smell loss, versus 6%-9% in users without. Smell loss was described as “sudden” (63%), occurring on average 3 days following the onset of any other symptom. Of those that reported resolution of their smell loss, 50% resolved in 1 week, with 75% resolution reported within 1 month. Conclusions: The results herein correlate with other established COVID-related studies, despite relying on purely volunteered data from participants from around the world. This is the first study to suggest an association of age and blood type with smell loss. Mobile app use offers a novel method for safe, remote and granular insight into those suffering from infectious diseases like COVID-19

Outcomes of Dupilumab treatment in patients presenting with severe asthma or chronic rhinosinusitis with nasal polyps, over the age of 65

Introduction: Dupilumab is an IL-4/13 inhibitor that has been successfully implemented in the treatment of severe asthma and chronic rhinosinusitis with nasal polyps (CRSwNP). The safety and efficacy of Dupilumab has not been reported for these indications in patients over the age of 65. This study aims to determine the long-term benefits of dupilumab while detailing adverse effects. Methods: This study is a single institution retrospective review of patients receiving dupilumab for severe asthma and/or CRSwNP from 2018 to present. Charts were reviewed for patient demographics, Sino-nasal Outcome Test (SNOT-22) scores, asthma control test (ACT) scores, polyp scores, and adverse effects. We reported the findings of patients over the age of 65 whose first biologic was dupilumab. Results: Thirty-one patients over the age of 65 were identified (mean = 72.2), 18 (58.1%) of whom are female. Twelve (38.7%) presented with severe asthma, 7 (22.6%) with CRSwNP and 12 (38.7%) with both severe asthma and CRSwNP. Compared to baseline, there was a significant difference observed within patients in mean ACT (17.8 to 20.3; n=13, p=0.002), SNOT-22 (40.5 to 23.4; n=10, p= 0.015), and polyp scores (3.06 to 1.13; n=8, p=0.010) post-treatment initiation. Seven patients (22.6%) reported adverse reactions that may or may not be related to dupilumab. Adverse reactions included ophthalmologic (1), musculoskeletal (1), dermatologic (2) and other (2) which included lower extremity edema, tinnitus, hair loss, and TMJ pain. Zero patients reported allergic reactions. Four of the 7 patients who experienced adverse reactions chose to continue dupilumab therapy, while 3 discontinued. Conclusions: Patients over 65 on dupilumab showed improvement in asthma and CRSwNP symptoms. There were significant improvements in ACT, SNOT-22, and nasal polyp scores withing 9 months of therapy initiation. The majority did not experience any adverse effects, demonstrating the value of dupilumab treatment in this patient cohort

Avoidance of Maxillary Swing for Nasopharyngectomy via Combined Open Lateral and Endoscopic Approach

Objectives: Nasopharyngectomy performed via a solely endoscopic approach has limitations in access and feasibility, particularly regarding management of the carotid artery. To address these limitations, we report three cases with one cadaver dissection where nasopharyngectomy was performed via a combined open lateral an endoscopic approach. We highlight the benefits and technical considerations for this operative technique. Study Design: Case Series Methods: Patients diagnosed with recurrent nasopharyngeal carcinoma (NPC) that underwent combined open lateral and endoscopic nasopharyngectomy from 2016-2020 were analyzed. A cadaver dissection was also performed. Results: We present the details of the approach and follow-up in three patients with recurrent nasopharyngeal carcinoma. Briefly, a preauricular incision is extended down to the neck. The zygoma and mandibular ramus can be removed and replaced if required. V3, the pterygoid plates and the eustachian tube can be resected or mobilized. The carotid artery may be identified proximally in the neck and traced to the skull base, where the carotid canal may be drilled to the level of the foramen lacerum and protected with a pledget. Then, tumor mucosal cuts are made via an endoscopic endonasal approach and connected to the lateral exposure. No carotid artery injuries occurred. Conclusion: The combined open lateral approach and endoscopic nasopharyngectomy technique is a useful technique in salvage patients. It provides excellent control of major vessels, adequate access to the carotid canal, V3, and remainder of the skull base, and cervical protection.https://jdc.jefferson.edu/otoposters/1009/thumbnail.jp

Free Tissue Transfer for Central Skull Base Defect Reconstruction: Case Series and Surgical Technique

Objectives: Local reconstruction of central skull base defects may be inadequate for large defects or reoperative cases; free tissue transfer may be necessary. Inset of the flap and management of the pedicle can be challenging. We report our experience and approach. Methods: Retrospective review identifying seven patients with central skull base defects who underwent free flap reconstruction from 2016-2020. Results: Four patients with recurrent nasopharyngeal carcinoma, one with recurrent craniopharyngioma, one with clival-cervical chordoma, and one with meningioma of the middle cranial fossa were analyzed. Six defects were closed with an anterolateral thigh free flap and one with a radial forearm free flap. In two patients, the flap was secured in an onlay fashion to the defect via a Caldwell-Luc transmaxillary approach. In one patient, the flap was passed transorally, and the pedicle was delivered into the neck via Penrose drain. In two patients, a parapharyngeal technique and in two others, a retropharyngeal was used for nasopharyngeal inset with endoscopic assistance. There were no flap failures, with an average follow-up time of 20.1 (range 3.2 - 47.1) months. One patient required flap repositioning on postoperative day three due to midline shift and intracranial contents compression. The transoral inset flap necessitated flap repositioning on postoperative day 13 to improve the nasopharyngeal airway. Conclusion: Free flap reconstruction of the central skull base is challenging, but transmaxillary, transoral, parapharyngeal, and retropharyngeal approaches can be used with endoscopic assistance to ensure secure inset flap and avoid airway obstruction