Rhinosinusitis, symptomatology & absence of polyposis in children with primary ciliary dyskinesia
Primary Ciliary Dyskinesia (PCD) describes a group of inherited disorders which result in functional ciliary defects leading to mucous stasis. Clinical manifestations include otitis media with effusion and chronic rhinosinusitis. Nasal polyposis has previously been thought to be linked to PCD, and current theories of 'polypogenesis' suggest that early and severe polyp formation could be expected among sufferers of this condition. Cross-sectional observational review of all children attending the multi-disciplinary clinic at a national tertiary-referral centre for PCD across a 3-month period. Careful examination was undertaken, and the SNOT-20 questionnaire administered. Thirty patients were included. No nasal polyps were found, despite children clearly suffering rhinosinusitis and being debilitated by their symptoms. The rhinologically orientated questions of the SNOT-20 produced the most positive responses; however some other questions were found not to be useful in a paediatric population. Nasal polyps do not occur in children with PCD, despite the presence of rhinosinusitis. Given that many current theories of polyp pathogenesis hinge on prolongation of proinflammatory stimuli, further investigations are needed into why this should not occur in the situation of chronic mucous stasis which is the hallmark of PCD.