A Study of 5 Patients who Underwent Endoscopic Sinus Surgery for Primary Ciliary Dyskinesia

Urabe Masashi, Tateyama Kaori, Umemoto Shingo, Hirano Takashi, Suzuki Masashi

doi:10.7248/jjrhi.62.310

<p>Primary ciliary dyskinesia (PCD) is an otorhinolaryngological and respiratory condition characterized by motile ciliary dysfunction, which results in intractable recurrent sinusitis, otitis media, bronchiectasis, and pneumonia. Currently, there is no evidence-based therapeutic strategy for management of chronic sinusitis in patients with PCD. We retrospectively investigated the clinical features of five patients with PCD who underwent endoscopic sinus surgery. These patients included 4 men and 1 woman, and had a mean age of 24 years (range 12–33 years). Recurrent pneumonia and bronchitis occurred in 3 patients, inversion of the internal organs in 2, and male infertility in one. Electron microscopy revealed abnormalities in 4 patients: an inner dynein arm defect in 2, and inner and outer dynein arm defects in 2. Nasal endoscopy revealed polyps in 3 patients, and purulent rhinorrhea in all patients. Three patients had hypoplastic frontal and sphenoid sinuses. All patients received long-term low-dose macrolide therapy, but symptoms persisted and endoscopic sinus surgery was required in all cases. Postoperatively, nasal suction of the posterior nasal cavity and middle nasal meatus was easier, and nasal irrigation with saline reduced the frequency of pneumonia in 2 patients. These findings may suggest that endoscopic sinus surgery followed by nasal irrigation may improve upper airway secretion clearance and reduce the risk of lower respiratory tract infection.</p>

A Study of 5 Patients who Underwent Endoscopic Sinus Surgery for Primary Ciliary Dyskinesia

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