Cystic Fibrosis Chronic Rhinosinusitis

Chronic rhinosinusitis (CRS) is a pervasive and challenging entity to treat in patients with cystic fibrosis (CF). CF CRS develops as a direct consequence of mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene, which causes defective anion transport across cell membranes, leading to viscous sinonasal secretions, impaired mucociliary clearance, and subsequent colonization with microorganisms including Pseudomonas aeruginosa, Staphylococcus aureus, and fungi. Nasal polyposis in CF patients reflects a more neutrophilic than eosinophilic inflammatory cascade, as compared to non-CF nasal polyposis. Conservative management with systemic and topical sinonasal medical therapies constitutes first-line treatment. Surgical management has been demonstrated to improve sinonasal symptoms and quality of life, and it may also improve lung function. Surgical management entails comprehensive endoscopic sinus surgery with the creation of large sinonasal cavities to optimize topical drug delivery and gravity-dependent drainage and requires careful postoperative management. New CFTR modulator therapies appear to significantly improve CF CRS.