Treatment and outcomes of chronic rhinosinusitis in children with primary ciliary dyskinesia: where is the evidence? A qualitative systematic review

Background: Consensus on treatment of chronic rhinosinusitis (CRS) in children with primary ciliary dyskinesia (PCD) is limited. We sought to synthesize the best available evidence pertaining to treatment and outcomes of CRS in children with PCD.

Methods: This work is a systematic review of PubMed and EMBASE for studies pertaining to treatment and outcomes of CRS in children with PCD in a 20-year period. Two reviewers independently extracted information on study design, setting, medical or surgical therapy for CRS, and treatment outcomes.

Results: Twelve studies from 8 countries were included for analysis: (3 retrospective cohorts, 2 prospective cohorts, 2 cross-sectional, 1 case series, and 4 case reports). The number of patients with PCD ranged from 1 to 78. Medical treatment included oral antibiotics, intranasal steroids, and oral steroids. Surgical treatment was mentioned in 8 studies, with endoscopic sinus surgery (ESS) described in 3 of 8. Outcome measures were rarely defined but included the 20-item Sino-Nasal Outcome Test (SNOT-20) questionnaire (1/12), symptom questionnaire (1/12), decreased recurrent pneumonia (1/12), and subjective report of CRS symptoms. No randomized, controlled, or long-term prospective cohort studies were identified, and no study employed a disease-specific, pediatric-validated instrument to measure outcomes of therapy.

Conclusion: There is a paucity of evidence pertaining to CRS treatment or outcomes in children with PCD. Given the rare prevalence of PCD and the insufficient evidence pertaining to treatment effectiveness for CRS, we propose an international, multicenter database to prospectively track data pertaining to diagnosis, treatment, and outcomes of CRS for children with PCD. This effort would encourage implementation of validated and standardized outcome measures.