Rhinosinusitis in Pediatric Primary Ciliary Dyskinesia: Impact of Disease. (November 2019)

• Record Type: Journal Article
• Authors: Bhatt, Jay M.; Muhonen, Ethan G.; Meier, Maxene; Sagel, Scott D.; Chan, Kenny H.
• Is Part Of: Otolaryngology--head and neck surgery. Volume 161:Number 5(2019)
• Page Start: 877
• Page End: 880
• Publication Date: 2019-11
• Subjects: rhinosinusitis -- sinus disease -- sinus surgery -- primary ciliary dyskinesia; Head -- Surgery -- Periodicals; Neck -- Surgery -- Periodicals; Otolaryngology -- Periodicals; 617.51
• DOI: 10.1177/0194599819874842
• Languages: English
• ISSNs: 0194-5998

Abstract

Objectives: Primary ciliary dyskinesia (PCD) is a genetic disorder characterized by abnormal respiratory cilia ultrastructure and/or function causing defective mucociliary clearance. We investigated the extent and severity of rhinosinusitis in a large cohort of children with PCD and explored associations among risk factors, including genotype and sinus disease. Study Design: Retrospective chart review. Setting: Tertiary academic children's hospital. Subjects and Methods: A review was conducted with a patient registry at the PCD Foundation Center at our institution. Demographic, imaging, clinical, and operative data were reviewed through the institutional electronic health record system. Results: Fifty-four subjects were identified with mean and median age at diagnosis of 5.2 and 4.0 years. The male:female ratio was 35%:65%. Sinus symptoms were present in 46 (85%) subjects, 22 of whom had chronic rhinosinusitis. Nineteen (35%) subjects underwent operative intervention, consisting of endoscopic sinus surgery (ESS; 16 patients) and maxillary lavage (3 patients). Nineteen subjects underwent adenoidectomy for PCD-related indications. Five sinus-related admissions in 3 subjects were noted during the study period, and no complication of rhinosinusitis occurred in the cohort. Genetic test results were available in 27 subjects, in whom 23 (85%) had biallelic mutations in a PCD gene. Demographic factors, Lund-Mackay score, and PCD genotype were not found to be predictors for ESS or hospitalization in our cohort. Conclusion: While rhinosinusitis was common in our PCD cohort, most patients did not require ESS. Since complications of rhinosinusitis were uncommon, we recommend judicious surgical management tailored to the patient's symptoms.