Prevalence of cystic fibrosis in children who present with nasal polyposis

Abstract

RATIONALE: Children with cystic fibrosis have a higher incidence of nasal polyposis than unaffected children. Additionally, children with nasal polyposis are commonly screened for cystic fibrosis, whether or not other co-morbid features of cystic fibrosis are evident. The actual prevalence of cystic fibrosis diagnosed solely on the basis of isolated nasal polyposis disease has not been reported. METHODS: A medical records search identified all patients with an ICD-9 diagnosis code for cystic fibrosis and nasal polyps at Children's Medical Center from 1999-2004. The order of each diagnosis was determined by chart review. RESULTS: We report 893 children with cystic fibrosis and 216 children with nasal polyps were seen at our institution over this five year period. Forty-five children were confirmed to have both diagnoses. Only 2 of 45 children developed nasal polyps as the initial manifestation that lead to the subsequent diagnosis of cystic fibrosis. Both of these subjects were otherwise healthy 6 year old Caucasian females, however one had chronic sinusitis with hypoplastic sinuses while the other was believed to have nasal allergies. We find a prevalence of 45/893 or 5% of cystic fibrosis patients with nasal polyps, but only 2/893 or 0.2% of the cystic fibrosis patients had their disease discovered because of nasal polyps. CONCLUSION: It is quite rare to diagnose cystic fibrosis secondary to the presence of nasal polyps. Furthermore, a suspicion for other etiologies aside from cystic fibrosis should be maintained in children who present with isolated nasal polyposis.