Dept of Paediatrics, University of Pisa, Via Roma 67 56126, Pisa, Italy.
Eur Respir J. 2011 Mar;37(3):566-71. doi: 10.1183/09031936.00068810. Epub 2010 Jul 22.
Agenesis of paranasal sinuses has only been described in case reports of patients with primary ciliary dyskinesia (PCD). As agenesis of paranasal sinuses may contribute to low nasal nitric oxide levels, a common finding in PCD, we speculated that this condition might frequently occur in PCD patients. Patients referred for PCD evaluation were consecutively recruited for 30 months. In addition to standard diagnostic testing for PCD, a computed tomography (CT) scan of paranasal sinuses was performed in all subjects. 86 patients (46 children aged 8-17 yrs) were studied. PCD was diagnosed in 41 subjects and secondary ciliary dyskinesia (SCD) was diagnosed in the remaining 45 subjects. Frontal and/or sphenoidal sinuses were either aplastic or hypoplastic on CT scans in 30 (73%) out of 41 PCD patients, but in only 17 (38%) out of 45 with SCD (p = 0.002). There was a significant inverse correlation between the score for aplasia/hypoplasia of each paranasal sinus and nasal NO values in the PCD patients (p = 0.008, r = -0.432) but not in SCD (p = 0.07, r = -0.271). The findings of aplasia/hypoplasia of the frontal and or sphenoidal sinuses may be part of the spectrum of PCD and this finding should prompt exclusion of this condition.
鼻窦发育不全仅在原发性纤毛运动障碍（PCD）患者的病例报告中被描述过。由于鼻窦发育不全可能导致 PCD 常见的低鼻一氧化氮水平，我们推测这种情况可能在 PCD 患者中经常发生。在 30 个月的时间里，我们连续招募了前来接受 PCD 评估的患者。除了对 PCD 进行标准诊断性测试外，我们还对所有受试者进行了鼻窦计算机断层扫描（CT）。共研究了 86 名患者（46 名 8-17 岁的儿童）。41 名患者被诊断为 PCD，其余 45 名患者被诊断为继发性纤毛运动障碍（SCD）。41 名 PCD 患者中有 30 名（73%）的 CT 扫描显示额窦和/或蝶窦发育不全或发育不良，但 45 名 SCD 患者中只有 17 名（38%）（p = 0.002）。在 PCD 患者中，每个鼻窦发育不全/发育不良的评分与鼻一氧化氮值呈显著负相关（p = 0.008，r = -0.432），但在 SCD 患者中则没有相关性（p = 0.07，r = -0.271）。额窦和/或蝶窦发育不全/发育不良的发现可能是 PCD 谱系的一部分，这一发现应促使排除这种情况。
