Management of the upper airway in cystic fibrosis

Abstract

Purpose of review: Upper airway disease engenders significant morbidity for patients with cystic fibrosis and is increasingly recognized as having a much greater role in pulmonary outcomes and quality of life than originally believed. Widespread disparate therapeutic strategies for cystic fibrosis chronic rhinosinusitis underscore the absence of a standardized treatment paradigm. This review outlines the most recent evidence-based trends in the management of upper airway disease in cystic fibrosis.

Recent findings: The unified airway theory proposes that the sinuses are a focus of initial bacterial colonization which seeds the lower airway and may play a large role in maintaining lung infections. Mounting evidence suggests more aggressive treatment of the sinuses may confer significant improvement in pulmonary disease and quality of life outcomes in cystic fibrosis patients. However, there is a lack of high-level evidence regarding medical and surgical management of cystic fibrosis chronic rhinosinusitis that makes generalizations difficult.

Summary: Well designed clinical trials with long-term follow-up concerning medical and surgical interventions for cystic fibrosis sinus disease are required to establish standardized treatment protocols, but increased interest in the sinuses as a bacterial reservoir for pulmonary infections has generated considerable attention.

Figure 1

Sequential coronal CT scans of the posterior nasal cavity revealing the tracking of sinus secretions from the right sinuses to the nasopharynx (left to right, top to bottom). Draining purulence frequently exacerbates cough and likely seeds the lower airway. CT, computed tomography.

Figure 2

Transnasal endoscopic views of the right (a) and left (b) nasal cavities of a CF patient. Note the extensive polyposis on both sides that would be difficult to discern without nasal endoscopic visualization. Culture of mucopurulent secretions is easily accomplished using rigid endoscopy as well. CF, cystic fibrosis.

Figure 3

Our suggested treatment algorithm based on the available evidence. CT, computed tomography.

Figure 4

Coronal CT scan demonstrating the preoperative appearance of a patient with CF after traditional maxillary antrostomies with completely opacified maxillary sinuses (Left). Postoperative appearance after endoscopic sinus surgery and bilateral modified endoscopic medial maxillectomies reveals complete marsupialization (arrow) of the maxillary sinus (Right). The coronal CT image is posterior to the anterior 1/3 of the inferior turbinate. CF, cystic fibrosis; CT, computed tomography. Reproduced with permission from [75].

Figure 5

Transnasal endoscopic view of the left nasal cavity before (left) and after (right) endoscopic sinus surgery with modified endoscopic medial maxillectomy. Note the nasal polyps () within the middle meatus and thick secretions preoperatively. A 30-degree endoscope is inserted past the anterior 1/3 of the inferior turbinate postoperatively revealing a well healed maxillary cavity with no secretions retained in the floor of the sinus (arrow). Reproduced with permission from [75].