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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.
摘要：
介绍原发性纤毛运动障碍是一种罕见的遗传性疾病，可导致粘液纤毛清除和鼻窦不适。额叶和蝶窦的发育不全/发育不全在该人群中更为常见。然而,到目前为止,没有研究对诊断为这种疾病的成年患者的计算机断层扫描结果进行了详细描述.目的描述成人原发性纤毛运动障碍的CT表现。方法回顾性观察成人原发性纤毛运动障碍患者行CT检查。结果21名成人纳入研究。38．1％的额窦和14.3％的蝶窦发生发育不全。同样，发育不全发生在47.6%的额窦,54.8%的蝶窦和40.5%的上颌窦。此外，在61.9％的筛窦中发现了小梁丢失。Lund-Mackay的平均得分为13.5。此外,9.5%的患者患有大疱性外耳，47.6％有明显的双侧下鼻甲肥大，38.1%有明显的中鼻甲肥大,47.6%有明显的间隔偏离。最后,我们发现了暗示真菌球的图像，粘液囊肿,骨瘤,可能是后鼻孔息肉,和额骨侵蚀。结论本研究提供了原发性纤毛运动障碍患者的CT表现。我们还描述了为了更安全的手术计划而必须识别的异常，并且如果在具有一致临床表现的患者中发现，则建议诊断为原发性纤毛运动障碍。
