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CC BY-NC-ND 4.0 · Int Arch Otorhinolaryngol 2023; 27(01): e130-e137
DOI: 10.1055/s-0042-1749392
Original Research

Computed Tomography Evaluation of the Paranasal Sinuses in Adults with Primary Ciliary Dyskinesia

Diogo Barreto Plantier 
1   Department of Otorhinolaringology and Ophthalmology, Faculdade de Medicina, Universidade de São Paulo, SP, Brazil
,
Renata R. M. Pilan 
1   Department of Otorhinolaringology and Ophthalmology, Faculdade de Medicina, Universidade de São Paulo, SP, Brazil
,
Rodrigo Athanazio
2   Pulmonary Division, Heart Institute, Faculdade de Medicina, Universidade de São Paulo, SP, Brazil
,
Mary Anne K. Olm 
3   Department of Pathology, Faculdade de Medicina, Universidade de São Paulo, SP, Brazil
,
Eloisa M. S. Gebrim 
4   Department of Radiology, Faculdade de Medicina, Universidade de São Paulo, SP, Brazil
,
Richard Louis Voegels 
1   Department of Otorhinolaringology and Ophthalmology, Faculdade de Medicina, Universidade de São Paulo, SP, Brazil
› Author Affiliations
Funding D. B. Plantier was supported by the National Council for Scientific and Technological Development (CNPq, Brazil) during this research.
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Abstract

Introduction Primary ciliary dyskinesia is a rare inherited disease that results in a malfunction of mucociliary clearance and sinonasal complaints. Aplasia/hypoplasia of the frontal and sphenoid sinuses has been described as more frequent in this population. However, to date, no studies have provided a detailed description of computed tomography findings in adult patients with a diagnosis of this condition.

Objective To describe the computed tomography (CT) findings of adult patients with primary ciliary dyskinesia.

Methods Retrospective observational study of adult patients with primary ciliary dyskinesia who underwent CT.

Results Twenty-one adults were included in the study. Aplasia occurred in 38.1% of frontal sinuses and in 14.3% of sphenoid sinuses. Likewise, hypoplasia occurred in 47.6% of the frontal sinuses, in 54.8% of the sphenoid sinuses and in 40.5% of the maxillary sinuses. Furthermore, trabecular loss was identified in 61.9% ethmoidal sinuses. The mean Lund-Mackay score was 13.5. In addition, 9.5% of the patients had concha bullosa, 47.6% had marked bilateral inferior turbinate hypertrophy, 38.1% had marked middle turbinate hypertrophy, and 47.6% had marked septal deviation. Finally, we identified images suggestive of fungus ball, mucocele, osteoma, a possible antrochoanal polyp, and frontal bone erosions.

Conclusion The present study provides a detailed description of CT findings in patients with primary ciliary dyskinesia. We also describe abnormalities that must be identified for safer surgical planning and that suggest a diagnosis of primary ciliary dyskinesia if found in patients with a consistent clinical picture.

Keywords

aplasia - computed tomography scanner - Kartagener syndrome - paranasal sinuses - primary ciliary dyskinesia

Authors' Contribution

All authors contributed to the study conception and design. Material preparation and data collection were performed by Diogo Plantier. Computed tomography scans analyses were performed by Diogo Plantier, Renata Pilan, and Eloisa Gebrim. The first draft of the manuscript was written by Diogo Plantier, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.




Publication History

Received: 14 December 2021

Accepted: 01 March 2022

Article published online:
01 August 2022

© 2023. Fundação Otorrinolaringologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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