• triad of chronic sinusitis, bronchiecctasis, and situs inversus
• recurrent acute otitis media and nasl polyps also common
• syndrome is due to an ultrastructural abnormality of the cilia in which dynein arms are absent resulting in ciliary dysfunction and mucus stasis and frequent sinopulmonary infections
• situs inversus is due to the absence of the influence of cilia function on viscera development
• diagnosed by bronchoscopy for cilia biopsy

References

Haddad GG. Primary ciliary dyskinesia. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa: WB SaundersCo; 2000:1327-1328