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

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

Principles of Pituitary Surgery

Key Points 1. Understand the principles of pituitary surgery including the key-elements of surgical planning and decision-making 2. Identify the technical nuances distinguishing the endoscopic from the microscopic transsphenoidal approach 3. Understand the strategies utilized during the nasal, sphenoidal, and sellar stages of surgery that maximize tumor resection while minimizing complications and preserving sino- nasal anatomy/functio

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

Is Reconstruction of the Sella Necessary to Prevent Optic Chiasm Prolapse and Cerebrospinal Fluid Leakage Following Endoscopic Resection of Pituitary Macroadenomas?

Visual compromise is a common presentation of pituitary macroadenomas and is related to direct optic nerve and chiasm compression. Although the extent of visual recovery following treatment depends on the duration and severity of the visual compromise, the majority of patients experience gradual improvement in their vision. Delayed visual deterioration following treatment is typically related to either tumor recurrence or radiation-induced optic neuropathy, although visual worsening due to prolapse of the optic apparatus into a secondary empty sella has rarely been reported. In 1968, Guiot reported the first a case of reversible visual deterioration associated with optic chiasm prolapse following resection of a large pituitary macroadenoma (Guiot). Based on their observations, Guiot and collaborators recommended that a “prop” be placed in the sella at the time of transsphenoidal pituitary adenoma resection to prevent progressive herniation of the optic structures. Similarly, Hardy coined the term “preventive chiasmopexy” to describe filling of the sella cavity with autologous tissue such as muscle or fat following resection of large tumors to prevent this herniation phenomenon. While optic chiasm prolapse with associated visual deterioration appears to represent a rare occurrence, its true incidence and pathophysiological basis remain uncertain. Reconstruction of the sella with autologous tissues is also widely employed as a means to prevent postoperative cerebrospinal fluid leakage with these tissues typically harvested from a secondary operative site such as the abdomen. Although not frequently reported in the pituitary literature, complications of abdominal fat graft harvest include hematoma and seroma formation as well as infection with an incidence ranging from 1-7%. At our institution, we do not routinely perform dural reconstruction following transsphenoidal resection of pituitary macroadenomas using adipose tissue to prevent cerebrospinal fluid leakage or optic chiasm prolapse. In this study, we sought to determine the incidence of optic chiasm prolapse into the sellar defect by determining the radiographic position of the optic chiasm following surgery and incidence of delayed visual deterioration. Pages: 13-1

Prevention and Management of Bleeding During Endoscopic Approaches to Skull Base Pathologies

The rate of serious permanent morbidity and mortality with endonasal approaches has declined secondary to increased knowledge of the pertinent anatomy, advanced neuroimaging and navigation techniques, better surgical instruments, and improved exposure and reconstruction strategies.1-3 Although rare, vascular injury remains a potentially serious complication. However, with limited systematically-collected and reported data, the exact incidence rate of vascular injuries is difficult to determine. In terms of arterial injuries, the incidence based on reported series likely ranges from 0.3%-9% (Table 1),4-11 with higher rates most commonly associated with chordomas and chondrosarcomas involving the clivus. Venous injury is comparatively less severe and easier to manage. As a result, there is a comparatively lower impetus to publish epidemiological data and management strategies for these injuries. The consequences of arterial injury include fatal hemorrhage, vessel occlusion or thromboembolism causing infarction, development of a pseudoaneurysm (PA), carotid-cavernous fistula (CCF), subarachnoid hemorrhage (SAH), and vasospasm.6,7,9 Surgical expertise and detailed knowledge of the neurovascular anatomy is critical to the avoidance and management of vascular injuries. Pages: 20-2

Comprehensive Management of the Paranasal Sinuses in Patients Undergoing Endoscopic Endonasal Skull Base Surgery.

OBJECTIVE: The endonasal route often provides the most direct and safe approach to skull base pathology. In this article we review the literature with regard to management of the paranasal sinuses in the setting of skull base surgery. METHODS: We describe our institutional experience and review the literature of concurrent management of the sinusitis in patients undergoing endoscopic skull base surgery. RESULTS: Patients should be optimized preoperatively to ensure the endonasal route is a safe corridor to enter the intracranial cavity. Often the paranasal sinuses can be surgically addressed at the same time as endoscopic skull base surgery. We describe the technical details of management of the paranasal sinuses when addressing skull base pathology. CONCLUSIONS: Careful management of the paranasal sinuses throughout the peri-operative course is paramount to optimizing sinonasal function and safety

Endonasal Vascularized Flaps For Cranial Base Reconstruction

Since the introduction of extended endoscopic cranial base surgery, postoperative cerebrospinal fluid (CSF) leak has been a formidable and troublesome issue resulting in complications such meningitis, pneumocephalus, and the need for additional surgical interventions. Establishment of a watertight cranial base reconstruction is the most critical step in preventing postoperative CSF leakage. Historically, various free grafts, both synthetic and autologous, were utilized as repair materials for reconstruction of the cranial base defect often in combination with temporary CSF diversion. Free grafts are often sufficient for repair of small low flow, low pressure dural defects. High postoperative CSF leak rates reported in the initial endoscopic skull base literature are evidence that free grafts do not provide a reliably competent repair for large defects or direct high-flow CSF leaks. The introduction of the Hadad-Bassagasteguy vascularized nasoseptal flap has significantly reduced the reported CSF leak rate with a recent meta-analysis reporting that use of the vascularized flap is associated with a 7% rate of postoperative CSF leakage compared to 16% with free grafts alone for large dural defects.6 Since the initial description of the vascularized pedicled nasoseptal flap in 2006, many surgeons have developed a variety of alternative vascularized flaps for endonasal cranial base reconstruction. In this article, we summarize and compare several of the most clinically useful vascularized flaps including their harvest technique, indications and limitations, and potential complications. Pages: 28-3

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