Management of Sinusitis in Adult Cystic Fibrosis

Abstract

Until recently, cystic fibrosis was frequently fatal during childhood. However, with current medical management, many patients are living into adulthood. This has created a new population of patients with chronic sinusitis and severe medical problems. In this report, experience with 22 patients, eight of whom have undergone sinus surgery, is presented, and recommendations for management are proposed. Presenting symptoms are typical of sinusitis, but in a few patients, severe debilitating headaches predominate. Oral antibiotics are often of little use due to the numerous courses of high dose intravenous antibiotics used for resistant pulmonary infections. Topical nasal steroids and mucolytics have been of some benefit. Fourteen operative procedures were performed on eight patients. These procedures included 12 endoscopic sphenoethmoidectomies, four Caldwell-Luc procedures, two frontal sinus obliterations, and one transseptal sphenoidotomy (many of these were in combination.) Results from this experience indicate 1) Failure of endoscopic surgery to control frontal and maxillary sinus disease; 2) Delayed healing of the ethmoid cavity with persistent crusting; and 3) Significant, albeit short term, symptomatic relief following surgical intervention. Based on this limited series, we conclude that surgery should be delayed until absolutely necessary and that an aggressive approach should be adopted when surgery is performed. In our hands this included initial endoscopic sphenoethmoidectomy with open surgical techniques used for removal of trapped inspissated secretions. We recommend long term intravenous antibiotics postoperatively and frequent cleaning of the ethmoid cavity after surgery, continuing indefinitely, to optimize the benefit of surgery.