Abstract
Background: Primary ciliary dyskinesia (PCD) may be an underlying factor in some cases of refractory chronic rhinosinusitis (CRS). However, clinical management of this condition is not well defined. This systematic review examines the available evidence for the diagnosis and management of CRS in adults with PCD. Methods: A systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Pubmed, EMBASE, and Cochrane database were queried for studies pertinent to treatment of PCD in adults. Two investigators performed eligibility assessment for inclusion or exclusion in a standardized manner. Results: Of the 278 articles identified, six studies met the criteria for analysis. These studies had a predominately low level of evidence. Medical therapy included oral antibiotics and nasal saline rinses. Endoscopic sinus surgery (ESS) was described in three of six studies. Outcomes measures were limited and included non-validated questionnaires, subjective reporting of CRS symptoms, and decreased preciptins against pseudomonas following ESS. Recommendation for a standardized therapeutic strategy was not possible with the available literature. Conclusion: A paucity of evidence is available to guide the treatment of PCD in the adult population. Further prospective studies are needed to determine the optimal diagnostic and management strategy for this condition.
Highlights
Primary ciliary dyskinesia (PCD) is a heterogeneous disorder of ciliary ultrastructure resulting in decreased mucociliary clearance
Sino-nasal symptoms vary but it is estimated that chronic rhinosinusitis (CRS) affects over 50% of patients with PCD, with 15%–40% suffering from nasal polyposis [7,8]
Nasal saline is recommended for patients with CRS, and may improve symptoms in patients with PCD, no studies exist examining their direct benefit
Summary
Primary ciliary dyskinesia (PCD) is a heterogeneous disorder of ciliary ultrastructure resulting in decreased mucociliary clearance. Ciliary immotility and defective ciliary ultrastructure were initially described by Afzelius in 1976 [1], with subsequent studies demonstrating uncoordinated or ineffective ciliary beat. These ciliary abnormalities result in chronic oto-sino-pulmonary disease including bronchiectasis, rhinitis, sinusitis, bronchitis, pneumonia, and chronic otitis media [2]. Sino-nasal symptoms vary but it is estimated that chronic rhinosinusitis (CRS) affects over 50% of patients with PCD, with 15%–40% suffering from nasal polyposis [7,8]. This systematic review examines the available evidence for the diagnosis and management of CRS in adults with PCD. Results: Of the 278 articles identified, six studies met the criteria for analysis. Further prospective studies are needed to determine the optimal diagnostic and management strategy for this condition
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