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

Cystic fibrosis is an autosomal recessive genetic disorder that causes dysf unction of exocrine glands, and has several clinical manifestations. Among those, sinonasal involvement is almost universal, with or without chronic s inusitis and/or nasal polyposis. This review will detail the pathophysiolog ic changes of the sinonasal mucosa, and the clinical manifestations, diagno sis, and treatment. Developmental anatomic abnormalities, which are identif ied 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 he avily on surgery. In the past, nasal polyposis was the main indication for surgery, and consisted mostly of polypectomy alone. This procedure was asso ciated with a high recurrence rate. The development of functional endoscopi c sinus surgery has contributed to decreasing the morbidity of sinonasal su rgery and the recurrence of nasal polyposis in cystic fibrosis. The evoluti on of the surgical techniques will be discussed and a review of the literat ure will be provided. Pediatr Pulmonol, 2000; 30:481-489, (C) 2000 Wiley-Li ss, Inc.