Medical and Surgical Advancements in the Management of Cystic Fibrosis Chronic Rhinosinusitis

Abstract

Purpose of review: The purpose of this review is to provide otolaryngologists with the most up-to-date advancements in both the medical and surgical management of CF-related sinus disease.

Recent findings: Recent studies have supported more aggressive CRS management, often with a combination of both medical and surgical therapies. Comprehensive treatment strategies have been shown to reduce hospital admissions secondary to pulmonary exacerbations in addition to improving CRS symptoms. Still, current management strategies are lacking in both high-level evidence and standardized guidelines.

Summary: The unified airway model describes the bi-directional relationship between the upper and lower airways as a single functional unit and suggests that CRS may play a pivotal role in both the development and progression of lower airway disease. Current strategies for CF CRS focus primarily on amelioration of symptoms with antibiotics, nasal saline and/or topical medicated irrigations, and surgery. However, there are no definitive management guidelines and there remains a persistent need for additional studies. Nevertheless, otolaryngologists have a significant role in the overall management of CF, which requires a multi-disciplinary approach and a combination of both surgical and medical interventions for optimal outcomes of airway disease. Here we present a review of currently available literature and summarize medical and surgical therapies best suited for the management of CF-related sinus disease.

Keywords: CFTR; chronic sinusitis; cystic fibrosis; drug therapy; endoscopic sinus surgery; ivacaftor; modified medial maxillectomy; sinusitis; surgical therapy.

Figure 1

Transnasal endoscopic views of a cystic fibrosis patient with mucopurulence emanating from the left sphenoethmoidal recess (A) and left maxillary antrostomy (B) extending to the nasopharynx (C). The sinuses may serve as a bacterial reservoir where overflowing mucopurulence may lead to recurrent pulmonary exacerbations.

Figure 2

Coronal computed tomography scan (A) through the mid-maxillary sinuses demonstrates complete filling of the dependent portion of the sinus bilaterally. A 3 month postoperative transnasal endoscopic view of the left maxillary sinus (B) where a modified endoscopic medial maxillectomy has been performed shows no mucopurulence. This patient had a comprehensive approach to their disease including “large hole” surgery, culture-directed antibiotics, topical tobramycin/mupirocin/mometasone irrigations BID, and aggressive postoperative debridement.