ORIGINAL ARTICLE Treatment and outcomes of chronic rhinosinusitis in children with primary ciliary dyskinesia: where is the evidence? A qualitative systematic review David J. Mener, MD, MPH, Sandra Y. Lin, MD, Stacey L. Ishman, MD, MPH and Emily F. Boss, MD, MPH Background: Consensus on treatment of chronic rhinos- inusitis (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 re- viewers independently extracted information on study de- sign, seing, medical or surgical therapy for CRS, and treat- ment 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. Medi- cal 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) question- naire (1/12), symptom questionnaire (1/12), decreased recur- rent pneumonia (1/12), and subjective report of CRS symp- toms. No randomized, controlled, or long-term prospec- tive cohort studies were identified, and no study employed a disease-specific, pediatric-validated instrument to mea- sure 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 pro- pose an international, multicenter database to prospec- tively 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. C  2013 ARS-AAOA, LLC. Key Words: Chronic Rhinosinusitis; Primary Ciliary Dyskinesia; Karta- gener’s Syndrome How to Cite this Article: Mener DJ, Lin SY, Ishman SL, Boss EF. Treatment and outcomes of chronic rhinosinusitis in children with pri- mary ciliary dyskinesia: where is the evidence? A qualita- tive systematic review. Int Forum Allergy Rhinol. 2013;3: 986–991. P rimary ciliary dyskinesia (PCD) is a rare autosomal re- cessive disorder with an estimated incidence of 1 in 15,000 to 20,000 births. PCD consists of defects in cil- iary bending resulting in decreased mucociliary clearance. Physical examination signs of PCD include chronic otitis Department of Otolaryngology–Head and Neck Surgery, Division of Pediatric Otolaryngology, Johns Hopkins University School of Medicine, Baltimore, MD Correspondence to: Emily F. Boss, MD, 601 N. Caroline St., 6th Floor, Baltimore, MD 21287; e-mail: erudnic2@jhmi.edu Potential conflict of interest: The authors have no financial or corporate interests to disclose associated with this work. Presented at the European Society for Pediatric Otorhinolaryngology, 11th International Congress, May 2012, Amsterdam, the Netherlands. Received: 6 June 2013; Revised: 30 July 2013; Accepted: 20 August 2013 DOI: 10.1002/alr.21227 View this article online at wileyonlinelibrary.com. media, chronic productive cough, and a history of recurrent respiratory infections and bronchiectasis, rhinitis, sinusitis, bronchitis, and pneumonia. Specifically, chronic nasal con- gestion and abundant watery to mucoid nasal secretions starting from the first day of life and rhinitis are common. Nasal polyps occur in approximately 18% to 33% of pa- tients with PCD, 1, 2 most often starting in adolescence. 3 Kartagener’s syndrome, a triad of rhinosinusitis, chronic bronchitis with bronchiectasis, and situs inversus occurs in approximately 50% of PCD patients. Chronic rhinosinusitis (CRS), as its own entity, signifi- cantly impacts physical health and quality of life in children. Symptoms of CRS in children with complex mucociliary disorders such as PCD or cystic fibrosis may be debilitat- ing and result in respiratory compromise with decreased pulmonary function. Purulent nasal secretions, typically transported by gravity or airflow due to decreased ciliary International Forum of Allergy & Rhinology, Vol. 3, No. 12, December 2013 986