Purpose of review
Over the past four decades, the average life expectancy for patients with cystic fibrosis (CF) has increased from 13 to 37 years of age. With increasing survival and improved pulmonary management, otolaryngologists are now seeing an increasing number of CF patients with chronic rhinosinusitis (CRS). Although CRS is a commonly treated disease process, there are a number of subtleties specific to CF. As the life expectancy of CF patients increases, quality of life issues gain importance. It is essential for otolaryngologists to understand the current therapeutic modalities to treat this challenging subset of CRS patients.
The sinonasal mucosa of CF patients has distinct differences including impaired mucociliary clearance and a predominantly neutrophilic polyp profile. Performing more aggressive surgical intervention, especially in the setting of revision cases may lead to improved outcomes. A recent study demonstrated that extensive sinus surgery with postoperative management can eradicate pathogenic bacteria from the sinuses of CF patients for up to 1 year.
With increasing life expectancy in CF, patients will require long-term follow-up with an otolaryngologist. Understanding the intricacies of the presentation of this disease in patients with CF is important for optimizing management.