Rhinosinusitis in Pediatric Primary Ciliary Dyskinesia: Impact of Disease
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Jay M. Bhatt, MD, Ethan G. Muhonen, MD, Maxene Meier, MS, Scott D. Sagel, MD, PhD, Kenny H. Chan, MDFirst Published September 10, 2019 Research Article
https://doi.org/10.1177/0194599819874842
Article information
Article has an altmetric score of 1 No Access
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.
Keywords rhinosinusitis, sinus disease, sinus surgery, primary ciliary dyskinesia
Show less
Jay M. Bhatt, MD, Ethan G. Muhonen, MD, Maxene Meier, MS, Scott D. Sagel, MD, PhD, Kenny H. Chan, MDFirst Published September 10, 2019 Research Article
https://doi.org/10.1177/0194599819874842
Article information
Article has an altmetric score of 1 No Access
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.
Keywords rhinosinusitis, sinus disease, sinus surgery, primary ciliary dyskinesia
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