Abstract: Objective　to study the CT imaging features of paranasal sinuses in children with primary ciliary dyskinesia（PCD）. Methods　A retrospective study was performed on children with PCD receiving treatment from July 2018 to March 2021 in Children‘s Hospital of Fudan University. Data included medical history，paranasal sinus computed tomography（CT） scans，nasal endoscopy，chest radiography，chest CT，echocardiography，abdominal ultrasound and genetic tests，etc. Paranasal sinus CT scans were assessed and scored according to the Lund-Mackay staging system. The Lund-Mackay score was compared between PCD patients and patients receiving functional endoscopic sinus surgery（FESS） for chronic rhinosinusitis（CRS）. The correlation between age and the Lund-Mackay score was analyzed using Spearman’s correlation. Results　24 children with confirmed PCD visited in our ENT department. Paranasal sinus CT scans of 21 children were obtained and included in the final analysis，including 15 males and 6 females，aged from 1 to 17 yrs，with a mean age of（7.4±4.3） yrs. Among these patients，21 patients（100.0%） had paranasal sinusitis，4 patients（19.0%） had adenoid hypertropy，and 18 patients（85.7%） had otitis media. Five patients were given nasal endoscopy，but none had nasal polyps. 11 patients（52.4%） had situs inversus. Elevenpatients（52.4%） had lung consolidation and pulmonary atelectasis. eight patients（38.1%） had different degrees of bronchiectasis. Twelve patients（57.1%） had a history of neonatal respiratory distress，and only 1 patient was born prematurely. One patients（4.8%） had atrioventricular septal defect. Mean Lund-Mackay score in children with PCD was 15.1±3.8，while mean Lund-Mackay score in CRS patients was 12.1±6.0，the difference being statistically significant（P＜0.05）. The inflammation was most severe at the ostiomeatal complex. Compared with CRS patients，PCD patients had higher Lund-Mackay scores at the ostiomeatal complex，sphenoid sinus and posterior ethomoid sinus（P＜0.05）. There was a negative correlation between patients’age and the Lund-mackay score（r＝-0.5270，P＜0.05）. Conclusion　Most pediatric patients with PCD suffer from chronic paranasal sinusitis. Compared with ordinary CRS patients，PCD patients suffer from more severe sinusitis，especially at posterior ethomoid sinus.It is suggested clinicians pay attention to patients with intractable sinusitis and recurrent lower respiratory tract infections，or to those full-term neonates with a history of neonatal respiratory distress，PCD diagnosis should be taken into consideration.

Key words: primary ciliary dyskinesia, paranasal sinustis, Lund-Mackay score, respiratory distress

摘要： 目的　探讨儿童原发性纤毛运动障碍（primary ciliary dyskinesia，PCD）的鼻窦CT影像学特征。方法　回顾性分析2018年7月至2021年3月复旦大学附属儿科医院诊治的24例PCD患儿的临床资料，包括病史、鼻窦CT、鼻内镜、胸片、胸部CT、心脏超声、腹部B超及基因检测结果等。计算PCD患儿的Lund-Mackay鼻窦CT评分，与行功能性内窥镜鼻窦手术（FESS）的慢性鼻窦炎患儿的Lund-Mackay评分对比。 并应用Stata软件对PCD患儿的Lund-Mackay评分与年龄进行相关性分析。结果　24例患儿中21例行鼻窦CT检查，其中男15例，女6例，年龄1～17岁，平均年龄（7.4±4.3）岁。21例（100.0%）均伴鼻窦炎，4例（19.0%）伴腺样体肥大，18例（85.7%）伴中耳炎。5例行鼻内镜检查，未见鼻息肉。11例（52.4%）伴全内脏反位，11例（52.4%）伴肺实变肺不张，8例（38.1%）伴不同程度的支气管扩张。12例（57.1%）出生时有新生儿呼吸窘迫，其中仅1例为早产儿。1例（4.8%）伴房间隔室间隔缺损。PCD患儿Lund-Mackay评分平均（15.1±3.8）分，普通慢性鼻窦炎患儿Lund-Mackay评分平均（12.1±6.0）分，差异有统计学意义（P＜0.05）。PCD患儿鼻窦炎症在窦口鼻道复合体受累最重。与普通的慢性鼻窦炎患儿相比，PCD患儿在窦口鼻道复合体、蝶窦、后组筛窦的CT评分更高（P＜0.05）。PCD患儿年龄与Lund-Mackay评分呈负相关（r＝-0.5270，P＜0.05）。结论　绝大多数PCD患儿有慢性鼻窦炎。 与普通的慢性鼻窦炎患儿相比，PCD 患儿鼻窦炎症更重，后组鼻窦受累更重。临床遇到顽固性鼻窦炎伴反复下呼吸道感染或既往有新生儿呼吸窘迫的足月产患儿，要考虑PCD可能。

关键词: 纤毛运动障碍, 鼻窦炎, Lund-Mackay评分, 呼吸窘迫