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PURPOSE OF REVIEW: To summarize the evidence surrounding diagnosis, treatment, prognosis, and surveillance of patients with acute invasive fungal sinusitis (AIFS) and discuss future research needs. RECENT FINDINGS: New risk factors for AIFS such as COVID have been identified, and a new prognostic staging system has been developed. SUMMARY: Most patients who develop AIFS are immunocompromised, with the majority having a history of diabetes or a hematologic malignancy. Unfortunately, there are not any highly sensitive and specific diagnostic tools. Therefore, a combination of signs and symptoms, imaging, endoscopy, biopsy, and labs should be used to diagnosis AIFS. Although surgery and systemic antifungals are known to improve outcomes, there is limited data on time to intervention, duration of antifungals, and surveillance patterns. There is also limited information on factors that can predict outcomes in AIFS patients. However, sensory/perceptual changes, prolonged neutropenia duration, and comorbidity burden may be associated with a poor prognosis.
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Nasal obstruction is the subjective perception and objective state of insufficient airflow through the nose. Nasal congestion, conversely, describes a state of not just inadequate airflow or obstructive phenomena but also pressure- and mucus-related states to the patient. Nasal receptors belonging to the transient receptor potential (TRP) protein family mediate the sense of nasal patency via the trigeminal nerve. The transient receptor potential melastatin-8 (TRPM8) responds to temperatures around 8°C to 22°C, and is stimulated by menthol and other cooling agents. The radiant effects of airflow create heat loss to activate these receptors and humans perceive this as nasal patency rather than the direct detection of airflow. The thermovascular state of the mucosa, in conditions such as rhinitis, influence TRPM8 activation. Nasal endoscopy can show signs of rhinitis and should be considered an essential part of the workup of nasal congestion. Efforts to relieve nasal congestion need to manage the mucosal state and surgery needs to ensures that the nasal cavity mucosa is exposed to the cooling effects of airflow rather than simply creating a passage to the nasopharynx.
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Mucosa Nasal , Obstrucción Nasal , Humanos , Obstrucción Nasal/diagnóstico , Mucosa Nasal/metabolismo , Canales Catiónicos TRPM/metabolismo , Endoscopía , Rinitis/diagnóstico , Rinitis/fisiopatologíaRESUMEN
Importance: Despite the aggressive progression of fulminant acute invasive fungal sinusitis (AIFS), data on prognostic factors have been disparate, hindering the development of a staging system. A composite staging system may improve prognostication for patient counseling and conduct of clinical research. Objective: To identify prognostically important factors in AIFS and to incorporate the factors into a comprehensive Functional Severity Staging System and Clinical Severity Staging System. Design, Setting, and Participants: This retrospective cohort study included adult patients diagnosed with pathology-proven AIFS from June 1, 1992, to December 31, 2022, at Washington University Medical Center and Barnes-Jewish Hospital, a tertiary care center in St Louis, Missouri. Data were analyzed from April to July 2023. Main Outcome and Measures: Sequential sequestration and conjunctive consolidation was used to develop a composite staging system to predict 6-month overall survival. Results: Of 71 patients with pathology-proven AIFS over the 30-year period, the median (range) age of the cohort was 56 (19-63) years, and there were 47 (66%) male patients. The median (range) follow-up time was 2 (0-251) months. There were 28 patients alive within 6 months, for a 39% survival rate. Symptoms, comorbidity burden, and presence and duration of severe neutropenia were associated with 6-month survival and were consolidated into a 3-category Clinical Severity Staging System with 6-month survival of 75% for stage A (n = 16), 41% for stage B (n = 27), and 18% for stage C (n = 28). The discriminative power of the composite staging system was moderate (C statistic, 0.63). Conclusion and Relevance: This cohort study supports the clinical importance of symptomatology, comorbidity burden, and prolonged severe neutropenia at the time of AIFS presentation. The composite clinical staging system may be useful for clinicians when counseling patients with AIFS and conducting clinical research.
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Neutropenia , Sinusitis , Adulto , Humanos , Masculino , Persona de Mediana Edad , Femenino , Pronóstico , Estudios de Cohortes , Estudios Retrospectivos , Sinusitis/diagnóstico , Estadificación de NeoplasiasRESUMEN
BACKGROUND: Chronic rhinosinusitis is a very common condition. Granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (eGPA) are systemic diseases which can contribute to the development of chronic rhinosinusitis in select patients. OBJECTIVE: Characterize the presenting features, diagnostic criteria, workup, and management of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis as they are encountered in otolaryngology clinics. METHODS: Full length manuscripts published 2000 or later were reviewed. A separate search was conducted for each disease. Pertinent clinical features related to sinonasal manifestations of GPA and eGPA were collected and reported in this review. RESULTS: 467 references were discovered during literature review process. In total, 42 references for GPA and 35 references for eGPA were included in this review. CONCLUSION: GPA and eGPA are vasculitis syndromes which commonly present in the context of multisystem disease. For GPA, pulmonary and renal disease are common; for eGPA a history of asthma is nearly ubiquitous. Sinonasal disease is a very common feature for both disease processes and may precede the development of systemic symptoms in many patients. Clinical work up and diagnosis is complex and generally requires multidisciplinary care. Treatment primarily consists of immunosuppressive agents, and a number of steroids, steroid sparing agents, and biologics have been shown to be effective. The role of sinus surgery includes tissue biopsy for diagnosis, functional surgery for symptom management in select cases, and reconstruction of cosmetic and functional defects.
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Síndrome de Churg-Strauss , Granulomatosis con Poliangitis , Rinitis , Sinusitis , Humanos , Granulomatosis con Poliangitis/complicaciones , Granulomatosis con Poliangitis/diagnóstico , Sinusitis/etiología , Sinusitis/diagnóstico , Sinusitis/terapia , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/complicaciones , Rinitis/etiología , Rinitis/diagnóstico , Rinitis/terapia , Enfermedad Crónica , Inflamación , MasculinoRESUMEN
BACKGROUND: Chronic rhinosinusitis is a very common condition. IgG4-related disease (IgG4-RD) and sarcoidosis are systemic diseases which can contribute to the development of chronic rhinosinusitis in select patients. OBJECTIVE: Characterize the presenting features, diagnostic criteria, workup, and management of sinonasal IgG4-RD and sarcoidosis as they are encountered in otolaryngology clinics. METHODS: Full length manuscripts published 2000 or later were reviewed. A separate search was conducted for each disease. Pertinent clinical features related to sinonasal manifestations of IgG4-RD and sarcoidosis were collected and reported in this review. RESULTS: 404 references were discovered during literature review process. In total, 42 references for IgG4-RD and 34 references for sarcoidosis were included in this review. CONCLUSION: IgG4-RD and sarcoidosis are autoimmune inflammatory conditions that can affect many systems of the body. For both disease entities, sinonasal disease is a less common presentation which can lead to delayed diagnosis. Sinonasal IgG4-RD commonly presents in the setting of multisystem disease. All with other clinical features, biopsy plays a key role in the diagnosis for both diseases. Treatment for IgG4-RD consists primarily of steroids and rituximab which can lead to excellent and durable remission. A variety of immunosuppressive agents are used in the management of sarcoidosis. Surgery for IgG4-RD is primarily utilized for tissue biopsy, although resection or debulking may be considered. For sarcoidosis, surgery can be used for tissue biopsy and functional sinus surgery can offer symptomatic relief in many patients.
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Enfermedad Relacionada con Inmunoglobulina G4 , Sarcoidosis , Sinusitis , Humanos , Sarcoidosis/diagnóstico , Sarcoidosis/inmunología , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/terapia , Enfermedad Relacionada con Inmunoglobulina G4/complicaciones , Sinusitis/inmunología , Sinusitis/diagnóstico , Rinitis/inmunología , Rinitis/diagnóstico , Rinitis/terapia , Enfermedad Crónica , Inflamación/inmunología , Inflamación/diagnóstico , Inmunoglobulina G/inmunología , Inmunoglobulina G/sangre , Rituximab/uso terapéutico , Inmunosupresores/uso terapéutico , Femenino , MasculinoRESUMEN
OBJECTIVE: The objective of this study was to explore the safety and feasibility of stellate ganglion blocks (SGBs) to treat persistent COVID-19-induced olfactory dysfunction (OD). Secondarily, the goal was to determine effect sizes to plan a future randomized clinical trial. STUDY DESIGN: Prospective case series. SETTING: Quaternary Care Academic Medical Center. METHODS: In this single-arm pilot trial, adult participants with a COVID-19 diagnosis ≥ 12 months prior to enrollment with OD underwent bilateral SGBs. Subjects were followed for 1 month after completion of SGB. The primary outcome measure was the change in the Clinical Global Impression-Improvement Scale for smell loss. Secondary outcome measures included changes in the University of Pennsylvania Smell Identification Test (UPSIT) and Olfactory Dysfunction Outcomes Rating (ODOR). RESULTS: Twenty participants were enrolled with a mean (SD) age of 46 (11) years and a mean (SD) duration of OD of 21 (5) months. At 1 month, 10 (50%) participants experienced at least slight subjective improvement in their OD, 11 (55%) attained a clinically meaningful improvement in smell identification using the UPSIT, and 7 (35%) achieved a clinically meaningful improvement in olfactory-specific quality of life (QoL) measured by the ODOR. The median difference between UPSIT scores at baseline and 1 month was 6 (95% confidence interval: 3-11), exceeding the minimal clinically important difference of 4. There were no serious adverse events. CONCLUSION: Sequential SGBs for COVID-19-associated OD were safe and associated with modest improvements in subjective olfaction, odor identification, and olfactory-specific QoL. A placebo-controlled trial is warranted to determine the efficacy of SGBs for COVID-19-associated OD.
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COVID-19 , Trastornos del Olfato , Adulto , Humanos , Persona de Mediana Edad , Olfato , COVID-19/complicaciones , Proyectos Piloto , Calidad de Vida , Trastornos del Olfato/etiología , Trastornos del Olfato/terapia , Trastornos del Olfato/diagnóstico , Ganglio Estrellado , Prueba de COVID-19RESUMEN
Novel diagnostic tests may help diagnose patients with local allergic rhinitis (AR) when systemic testing is negative or inconclusive. Surgical approaches including septoplasty, inferior turbinate reduction, nasal swell body reduction, and posterior nasal nerve ablation may improve symptoms in patients whose symptoms are refractory to medical therapy, though high-quality evidence is lacking in the AR population. Intralymphatic and epicutaneous immunotherapy have the potential to improve adherence to allergen immunotherapy, though comparisons with current gold standard treatments are lacking and studies reporting long-term outcomes are needed. Immunomodulatory agents in combination with subcutaneous immunotherapy (SCIT) may improve tolerance of SCIT but reports to date do not demonstrate a clear benefit in symptom alleviation. Future work in these areas may support these options as beneficial for testing and treatment of AR.
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Rinitis Alérgica , Rinoplastia , Humanos , Rinitis Alérgica/diagnóstico , Rinitis Alérgica/terapia , Desensibilización Inmunológica , Nariz , AlérgenosRESUMEN
KEY POINTS: Acute invasive fungal sinusitis (IFS) is a rare disease with high mortality There is no designated International Classification of Diseases code for IFS We propose a novel method to identify IFS using optimized codes complemented by medications.
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Infecciones Fúngicas Invasoras , Sinusitis , Humanos , Estudios Retrospectivos , Infecciones Fúngicas Invasoras/diagnóstico , Sinusitis/diagnóstico , Sinusitis/epidemiología , Sinusitis/microbiologíaRESUMEN
OBJECTIVE: Develop and validate a quality-of-life (QoL) outcome measure for patients with dysosmia. STUDY DESIGN: Cross-sectional survey study. SETTING: Otolaryngology clinics, research registries, and Facebook support groups. METHODS: A 59-item pilot survey with questions addressing parosmia concerns was developed using input from subjects with parosmia and clinical expertise from Otolaryngologists. After item reduction, the Parosmia Olfactory Dysfunction Outcomes Rating (DisODOR) was reduced to its final 29 items. DisODOR maximum score is 116 (each item score 0-4) with higher scores indicating a higher degree of dysfunction from smell distortion. DisODOR was validated using participants with parosmia persisting >3 months after severe acute respiratory syndrome coronavirus 2 (cases) and healthy controls. Reliability, face and content validity, internal consistency, convergent validity, discriminative validity, sensitivity to change, and the minimal clinically important difference (MCID) were assessed. RESULTS: A total of 134 cases and 20 controls completed DisODOR. The mean (SD) age was 45.9 (12.2) for cases and 29.6 (8.9) for controls. The mean score difference between cases and controls was 45.0 (95% confidence interval, 40.5-49.5) displaying good discriminative validity. DisODOR showed strong test-retest reliability (r = .942) with high internal consistency (Cronbach's α = .971). DisODOR had a moderate correlation with SNOT-22 scores (r = .619) indicating good convergent validity. There is an excellent association with the global impression of severity categories (η2 = 0.447). Based on the distribution method, the MCID is 15. CONCLUSION: DisODOR is a valid, reliable QoL instrument for parosmia that can be used to measure the functional impact and QoL impairment for parosmia patients. DisODOR is sensitive to change and thus can be used in studies investigating treatments for parosmia.
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Trastornos del Olfato , Olfato , Humanos , Calidad de Vida , Reproducibilidad de los Resultados , Estudios Transversales , Trastornos del Olfato/diagnósticoRESUMEN
KEY POINTS: Invasive fungal sinusitis (IFS) rate and risk factors in transplant recipients were explored IFS rate is higher in allogeneic recipients with prior transplants and worse comorbidity scores The at-risk timeframes for IFS development were identified.
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Trasplante de Células Madre Hematopoyéticas , Infecciones Fúngicas Invasoras , Sinusitis , Humanos , Receptores de Trasplantes , Infecciones Fúngicas Invasoras/epidemiología , Sinusitis/microbiología , Factores de Riesgo , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Estudios RetrospectivosRESUMEN
KEY POINTS: HRCT-MRI fusion is a new imaging modality that can be used to diagnose spontaneous CSF leaks. HRCT-MRI fusion has better accuracy in localizing CSF leaks compared to MRI and HRCT alone.
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An updated edition of the International Consensus Statement on Allergy and Rhinology: Allergic Rhinitis (ICAR:AR) has recently been published. This consensus document, which included the participation of 87 primary authors and 40 additional consultant authors, who critically appraised evidence on 144 individual topics concerning allergic rhinitis, provides guidance for health care providers using the evidence-based review with recommendations (EBRR) methodology. This synopsis highlights topical areas including pathophysiology, epidemiology, disease burden, risk and protective factors, evaluation and diagnosis, aeroallergen avoidance and environmental controls, single and combination pharmacotherapy options, allergen immunotherapy (subcutaneous, sublingual, rush, cluster), pediatric considerations, alternative and emerging therapies, and unmet needs. Based on the EBRR methodology, ICAR:AR includes strong recommendations for the treatment of allergic rhinitis: (1) for the use of newer generation antihistamines compared with first-generation alternatives, intranasal corticosteroid, intranasal saline, combination therapy with intranasal corticosteroid plus intranasal antihistamine for patients not responding to monotherapy, and subcutaneous immunotherapy and sublingual tablet immunotherapy in properly selected patients; (2) against the use of oral decongestant monotherapy and routine use of oral corticosteroids.
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Complejo Hierro-Dextran , Rinitis Alérgica , Humanos , Niño , Complejo Hierro-Dextran/uso terapéutico , Rinitis Alérgica/diagnóstico , Rinitis Alérgica/terapia , Rinitis Alérgica/epidemiología , Antagonistas de los Receptores Histamínicos/uso terapéutico , Alérgenos/uso terapéutico , Corticoesteroides/uso terapéuticoRESUMEN
BACKGROUND: In the 5 years that have passed since the publication of the 2018 International Consensus Statement on Allergy and Rhinology: Allergic Rhinitis (ICAR-Allergic Rhinitis 2018), the literature has expanded substantially. The ICAR-Allergic Rhinitis 2023 update presents 144 individual topics on allergic rhinitis (AR), expanded by over 40 topics from the 2018 document. Originally presented topics from 2018 have also been reviewed and updated. The executive summary highlights key evidence-based findings and recommendation from the full document. METHODS: ICAR-Allergic Rhinitis 2023 employed established evidence-based review with recommendation (EBRR) methodology to individually evaluate each topic. Stepwise iterative peer review and consensus was performed for each topic. The final document was then collated and includes the results of this work. RESULTS: ICAR-Allergic Rhinitis 2023 includes 10 major content areas and 144 individual topics related to AR. For a substantial proportion of topics included, an aggregate grade of evidence is presented, which is determined by collating the levels of evidence for each available study identified in the literature. For topics in which a diagnostic or therapeutic intervention is considered, a recommendation summary is presented, which considers the aggregate grade of evidence, benefit, harm, and cost. CONCLUSION: The ICAR-Allergic Rhinitis 2023 update provides a comprehensive evaluation of AR and the currently available evidence. It is this evidence that contributes to our current knowledge base and recommendations for patient evaluation and treatment.
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Complejo Hierro-Dextran , Rinitis Alérgica , Humanos , Rinitis Alérgica/diagnóstico , Rinitis Alérgica/terapia , AlérgenosRESUMEN
BACKGROUND: Acute invasive fungal sinusitis (AIFS) is an aggressive disease that requires prompt diagnosis and multidisciplinary treatment given its rapid progression. However, there is currently no consensus on diagnosis, prognosis, and management strategies for AIFS, with multiple modalities routinely employed. The purpose of this multi-institutional and multidisciplinary evidence-based review with recommendations (EBRR) is to thoroughly review the literature on AIFS, summarize the existing evidence, and provide recommendations on the management of AIFS. METHODS: The PubMed, EMBASE, and Cochrane databases were systematically reviewed from inception through January 2022. Studies evaluating management for orbital, non-sinonasal head and neck, and intracranial manifestations of AIFS were included. An iterative review process was utilized in accordance with EBRR guidelines. Levels of evidence and recommendations on management principles for AIFS were generated. RESULTS: A review and evaluation of published literature was performed on 12 topics surrounding AIFS (signs and symptoms, laboratory and microbiology diagnostics, endoscopy, imaging, pathology, surgery, medical therapy, management of extrasinus extension, reversing immunosuppression, and outcomes and survival). The aggregate quality of evidence was varied across reviewed domains. CONCLUSION: Based on the currently available evidence, judicious utilization of a combination of history and physical examination, laboratory and histopathologic techniques, and endoscopy provide the cornerstone for accurate diagnosis of AIFS. In addition, AIFS is optimally managed by a multidisciplinary team via a combination of surgery (including resection whenever possible), antifungal therapy, and correcting sources of immunosuppression. Higher quality (i.e., prospective) studies are needed to better define the roles of each modality and determine diagnosis and treatment algorithms.
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Infecciones Fúngicas Invasoras , Sinusitis , Humanos , Estudios Prospectivos , Infecciones Fúngicas Invasoras/diagnóstico , Enfermedad Aguda , Pronóstico , Sinusitis/diagnóstico , Sinusitis/terapia , Sinusitis/microbiologíaRESUMEN
BACKGROUND: Acute invasive fungal sinusitis (AIFS) is an aggressive and dangerous disease of the paranasal sinuses with high morbidity and mortality. The immune response at the level of the nasal mucosa, the site of entry, has not been previously evaluated. OBJECTIVE: To evaluate differential gene expression in the sinonasal mucosa of AIFS patients as compared to control patients using RNA sequencing. METHODS: Sinonasal tissue samples were prospectively obtained from consenting patients undergoing surgery between November, 2020 and November, 2021. RNA extraction and sequencing were performed and differential expression was analyzed to detect transcriptional differences between patient groups. RESULTS: Tissue samples were collected from 4 patients with active AIFS diagnoses, 2 patients with recovered AIFS, 1 patient with a diagnosis of non-invasive fungal ball, and 4 healthy controls. 255 genes were differentially expressed in AIFS patients as compared to control patients. Specific Gene Ontology (GO) biological processes that were identified as differentially expressed in AIFS patients as compared to controls included the following: 1. GO:0007155 (cell adhesion), 2. GO:0030199 (collagen fibril organization) and 3. GO:0001525 (angiogenesis). CONCLUSION: Transcriptional differences were noted between AIFS and control patients in sinonasal tissue samples. Future work is necessary to determine causes of the differential gene expressions between AIFS and control patients, specifically those who are immunosuppressed, or with preexisting non-invasive forms of fungal sinusitis, to guide treatment and prevention strategies.
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Senos Paranasales , Sinusitis , Humanos , Ontología de Genes , Sinusitis/diagnóstico , Senos Paranasales/cirugía , Mucosa Nasal , Análisis de Secuencia de ARNRESUMEN
BACKGROUND: The escalating negative impact of climate change on our environment has the potential to result in significant morbidity of rhinologic diseases. METHODS: Evidence based review of examples of rhinologic diseases including allergic and nonallergic rhinitis, chronic rhinosinusitis, and allergic fungal rhinosinusitis was performed. RESULTS: The lower socioeconomic population, including historically oppressed groups, will be disproportionately affected. CONCLUSIONS: We need a systematic approach to improve healthcare database infrastructure and funding to promote diverse scientific collaboration to address these healthcare needs.