Refractory Rhinosinusitis in Primary Ciliary Dyskinesia

Abstract

Abstract: Primary ciliary dyskinesia (PCD) a rare disease with abnormalities of the ciliary structure and function, which causes impaired mucociliary clearance. Patients with PCD present with rhinosinusitis, otitis media with effusion, recurrent lower respiratory tract infections, and bronchiectasis. The diagnosis of PCD is sometimes difficult due to the absence of the specific clinical and laboratory signs. Herein, we described two cases of PCD, which clinically presented with refractory sinusitis to various antibiotics treatment. Our cases of PCD were treated by endoscopic sinus surgery (ESS). The diagnosis of PCD was confirmed by the demonstration of ciliary structure anomalies with electron microscopy. The incidence of lower respiratory infections decreased postoperatively. To date, fundamental treatment for PCD has not been established yet. Early diagnosis and appropriate management for lower respiratory infections are important to prevent irreversible lung damage and the progression of PCD.