xRead - Nasal Obstruction (September 2024) Full Articles

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Orlandi et al.

GPA can affect any organ system with classic manifes tations of systemic illness, otitis media, subglottic steno sis, nodular infiltrates on chest radiograph, and renal dis ease. From the rhinologic perspective, sinonasal disease is the most common manifestation of GPA. 2399 Progressive ischemic necrosis of the nasal mucosa and internal struc tures can occur, resulting in epistaxis, crusting, septal per foration, and saddle nose deformity. 2399 Churg-Strauss syn drome is associated with both ANCA-positive testing and 4 of 6 of the following clinical findings: refractory CRSwNP, peripheral eosinophilia, asthma, neuropathy, pulmonary infiltrates and systemic vasculitis. 2400 It is important that rhinologic symptoms, such as nasal obstruction or epis taxis, tend to appear at an early stage in GPA and EGPA. Therefore, otorhinolaryngologists should maintain a high index of suspicion to not overlook these rare entities. 2400 Sarcoidosis is a systemic non-caseating granulomatous inflammatory process, which is typified by nodular, infil trative submucosal lesions in the nasal mucosa. However, patients may develop friable mucosa with nasal crusting and structural deformities similar to GPA. Management of CGD in general includes systemic con trol of disease via immunosuppression, with individual ized medical and/or surgical rhinologic care. Recently, anti-IL-5 monoclonal antibody therapy has proven to be useful in some settings. 2401 Medical therapy remains the cornerstone of management of sinonasal involvement in CGD, including INCS and saline irrigations. Surgery for mucocele formation, nasolacrimal stenosis, and CRS in general may be beneficial to control sequelae of GPA in appropriately selected patients, 2401 although persis tent or recurrent disease is common. 2399 Systemic man ifestations of both sarcoidosis and EGPA are managed with chemotherapeutic agents, oral corticosteroids + / − immune modulators. Similar to GPA, the literature sup ports use of medical management, while reserving surgical intervention for persistent rhinologic symptoms in select patients. 2400,2402–2405,2406 Given the epithelial abnormali ties present in CGD patients, patients should be counseled regarding suboptimal and/or delayed healing that can fol low intranasal procedures. XIV.C Primary Ciliary Dyskinesia Primary ciliary dyskinesia (PCD) is a rare, genetically het erogeneous disease. 2407 Prevalence of the disease is esti mated to be approximately 1 in 20,000 individuals. Situs inversus is present in 50% of patients. Dysfunction of motile cilia leads to oto-sino-pulmonary manifestations. Classic Kartagener’s syndrome is comprised of situs inver sus, CRS and bronchiectasis. Cardiovascular abnormalities and infertility are also commonly noted.

Symptoms of PCD are often non-specific. Evaluation for PCD is recommended when chronic wet cough and 6 of the following 7 predictive parameters are present: full term gestation, neonatal chest symptoms, neonatal inten sive care admittance, chronic rhinitis, ear symptoms, situs inversus and congenital cardiac defect. 2408 For patients with supportive clinical symptoms as mentioned above, the following results are confirmatory of a positive diag nosis of PCD: 1) hallmark ciliary ultrastructure defects assessed by transmission electron microscopy, and 2) non ambiguous bi-allelic mutations in PCD-causing genes. 2409 For patients with compatible clinical symptoms of PCD, the following results make the diagnosis of PCD highly likely; 1) Very low nasal nitric oxide plus high-speed video microscopy analysis findings consistently suggestive of PCD on 3 occasions, and 2) Very low nasal nitric oxide plus high-speed video microscopy findings consistent with PCD following cell culture. 2409 At present, treatment of PCD is not standardized, and there are no validated PCD-specific therapies. 2410 ThePCD Foundation recommends 1) daily airway clearance, 2) daily nasal sinus lavage, 3) standard vaccinations, 4) Influenza, Pneumococcal and RSV vaccine, 5) cessation of smoking, and 6) prompt antibiotics therapy at the time of respira tory tract infection. 2411 Although the effectiveness of ESS is controversial, combined ESS and adjuvant therapy can decrease sinus bacteria, reduce pulmonary infections and improve QoL of PCD. 2412 Diagnosis in the early stages is important to prevent progression of bronchiectasis and deterioration of lung function. 1 One recent study reports PCD affects lung func tion early in life, which emphasizes the importance of early standardized care for all patients. 2413 XIV.D Invasive Fungal Rhinosinusitis Fungi are ubiquitous and contribute to the diverse micro biome of the paranasal sinuses. 2414 However, in immuno compromised states such as diabetes mellitus (DM), hema tologic disorders, HIV/AIDS, and organ transplantation, immunological defenses are disrupted and hyphae may invade mucosa, vasculature or bone, thereby causing inva sive fungal sinusitis (IFS). Classification of IFS exists along a continuum deter mined by host factors and symptom duration. Acute inva sive fungal rhinosinusitis (AIFS) is defined by histopatho logic evidence of fungal invasion into tissue with less than 4 weeks of symptoms, whereas chronic inva sive fungal sinusitis (CIFS) is defined by symptoms beyond this period. 1709,2415,2416 Further distinction is based on presence of non-caseating granulomas, as seen in chronic granulomatous invasive fungal sinusitis (GIFS).