Patterns of Sinusitis in Cystic Fibrosis

Abstract

It has been pediatric dogma that most children with cystic fibrosis (CF) have pansinusitis and few are symptomatic. To reassess this premise, we compared symptoms, clinical examinations, radiographic and surgical findings, and results of treatment in 19 children with cystic fibrosis who had undergone computed tomography at St. Christopher's Hospital (Philadelphia) from 1991 to 1994. We found two distinct patterns of sinus disease: chronic sinusitis (n = 3) and polyposis (n = 12). Two of the patients with polyposis had ethmoid mucoceles and 1 child had no sinus disease at all. Children with chronic sinusitis had headache as a major complaint, while those with polyposis suffered nasal obstruction alone unless a mucocele was present. Surgery provided marked and lasting improvement in the 14 patients who were operated on.

International Consensus Statement on Allergy and Rhinology: Rhinosinusitis

“…Secretions in general, and the air-surface liquid in particular, become viscous and stagnant in CF patients, impairing MCC, and contributing to frequent bouts of CRS in the upper airway and bronchopulmonary disease in the lower airway. CF patients have an incidence of RS approaching 100% 1458 and 44% to 58% have NPs. 1459,1460 Medical management in adult and pediatric CF includes nasal saline, INCS, topical antibiotics, dornase alfa, and oral macrolides.…”

Extensive Surgical and Comprehensive Postoperative Medical Management for Cystic Fibrosis Chronic Rhinosinusitis

“…1 CF patients have a high prevalence (approaching 100%) of severe chronic rhinosinusitis (CRS), which is often associated with nasal polyposis (6-48%). [2][3][4][5] The pathophysiology of CRS in CF patients is because of, in large part, impaired mucociliary clearance (MCC)-a critical host defense mechanism for maintaining healthy sinuses in normal individuals. 6 Although pulmonary failure leads to the ultimate demise of most CF patients, CRS has a significant impact on their overall quality of life and may contribute to lung disease pathogenesis.…”

Use of the SNOT-22 and UPSIT to Appropriately Select Pediatric Patients With Cystic Fibrosis Who Should Be Referred to an Otolaryngologist

“…3 Theoretic explanations for patients with CF underreporting their sinus symptoms include the early onset of CRS in childhood, causing a lack of awareness of a symptom-free state; or masking of their sinus symptoms by the severity of their other medical issues. 3,4 Despite limited subjective reporting of clinical symptoms, some studies [5][6][7][8] have demonstrated relationships between clinical indicators and the severity of sinus disease in these children. There has historically been limited interdisciplinary coordination between physicians who primarily treat patients with CF and otolaryngologists at our institution, owing to the relative underreporting of symptoms.…”