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A Journal on Otorhinolaryngology, Head and Neck Surgery,
Plastic Reconstructive Surgery, Otoneurosurgery
Indexed/Abstracted in: EMBASE, Scopus
Otorinolaringologia 2009 June;59(2):75-87
Management of sinus disease in patients with cystic fibrosis: a review of the state of the art
Guy C. W., Russell P. T., Becker S. S.
Beker Nose and Sinus Center Voorhees, NJ, USA
Cystic fibrosis (CF) is a lethal autosomal recessive disease that mainly affects Caucasians. The disease is a multisystem disease caused by an alteration in a transmembrane chloride transporter, CF transmembrane conductance regulator (CFTR). Most of its mortality stems from its effects on the lungs, however these patients can have significant morbidity associated with their nearly universal sinus disease. Sinus disease in the CF patient develops from different pathophysiologic mechanism compared to the non-CF patient. This has widespread effects on the disease process including bacteriology and development of the sinuses. Treatment of their sinus disease remains medical using regimens to decrease the viscosity of the airway surface liquid and antibiotics, both topical and systemic, directed at the most common organisms. Surgical treatment of sinus disease in the CF patient has unique considerations that must be addressed by the surgeon including goals, timing of surgery, perioperative care, anesthesia risks, anatomic alterations and amount of surgery to be performed. This article critically reviews the literature concerning sinus disease in the CF patient. The pathophysiology and bacteriology of sinus disease and the anatomic variations that are characteristic of the disease are discussed. Medical treatments that are pertinent to the CF patient are reviewed along with discussion of the surgical management.