The relationship between paranasal sinus pneumatization and chronic rhinosinusitis (CRS) without cystic fibrosis is not well understood. Previous investigations have confirmed sinus hypoplasia in cystic fibrosis (CF) patients. This study compares paranasal sinus pneumatization of CRS patients to unaffected controls to determine if there is an analogous effect to that seen in CF. 591 sinus computed tomography (CT) scans, comprised of 303 adolescents (age 13–18) and 288 adults (age > 18), were analyzed for Lund-MacKay and Assessment of Pneumatization of the Paranasal Sinuses (APPS) scores. The APPS score is validated for measuring the extent of sinus pneumatization. A diagnosis of CRS and CRS phenotype was determined from the medical record. The mean APPS score for patients with a diagnosis of CRS was 10.61 (n = 111) compared to 9.62 (n = 448) for unaffected controls (p = 0.001). This was significant in adult (p = 0.021) and adolescent subgroups (p = 0.035). Sinus pneumatization did not differ according to CRS phenotype (p = 0.699). This suggests that there is not analogous anatomical sinus variation between CRS and CF, and that the mechanisms underlying sinus hypoplasia in CF may not be universal in patients with other types of sinus inflammation.

Cystic fibrosis (CF) is the most frequent congenital lethal disease in Caucasians. Impaired mucociliary clearance causes chronic bacterial rhinosinusitis in up to 62% of patients, and almost all patients exhibit sinonasal pathology in CT scans. Pathogens like Pseudomonas aeruginosa (P.a.) chronically colonize about 70% of the CF adults’ lungs and are the major reason for pulmonary destruction and premature death. In our 34-year-old female CF patient, rhinosinusitis caused massive orbital hypertelorism despite three sinonasal operations. Her sputum samples had always been negative for P.a. Then, P.a. was primarily detected in her sputum and additionally in nasal lavage, which since then persisted in both, her upper and lower airways. The P.a. strains turned out to be genetically identical in both airway levels, indicating early colonization of the entire airway system with P.a. This first report on simultaneous primary P.a. detection in the sinonasal and pulmonary compartments highlights the need to include an assessment of upper airway colonization in the standards of CF care, particularly in patients without chronic P.a. colonization. Both airway levels need to be considered as one united system, and a strong cooperation between ENT and CF specialists should be established. Prospective longitudinal studies should assess the upper airways´ role in acquisition and persistence of pathogens and evaluate conservative and surgical therapeutic options.