Sinonasal disease in cystic fibrosis: clinical characteristics, diagnosis, and management

Abstract

Cystic fibrosis is an autosomal recessive genetic disorder that causes dysfunction of exocrine glands, and has several clinical manifestations. Among those, sinonasal involvement is almost universal, with or without chronic sinusitis and/or nasal polyposis. This review will detail the pathophysiologic changes of the sinonasal mucosa, and the clinical manifestations, diagnosis, and treatment. Developmental anatomic abnormalities, which are identified radiologically, will also be demonstrated. Medical management is the first treatment for patients with cystic fibrosis, but effective treatment of sinonasal disease in cystic fibrosis relies heavily on surgery. In the past, nasal polyposis was the main indication for surgery, and consisted mostly of polypectomy alone. This procedure was associated with a high recurrence rate. The development of functional endoscopic sinus surgery has contributed to decreasing the morbidity of sinonasal surgery and the recurrence of nasal polyposis in cystic fibrosis. The evolution of the surgical techniques will be discussed and a review of the literature will be provided.