累及鼻腔鼻窦的Rosai-Dorfman病临床特征分析  

Analysis of the clinical charateristics of Rosai-Dorfman disease in nasal cavity and paranasal sinuses

作　　者：郑铭[1] 司马宇彤 庄梦岩 王向东[1,2,3] 张罗 ZHENG Ming;SIMA Yutong;ZHUANG Mengyan;WANG Xiangdong;ZHANG Luo(Department of Otolaryngology Head and Neck Surgery,Beijing Tongren Hospital,Capital Medical University,Key Laboratory of Otolaryngology Head and Neck Science,Ministry of Education(Capital Medical University),Beijing,100730,China;Beijing Institute of Otolaryngology,Beijing Laboratory of Allergic Diseases,Beijing Municipal Education Commission,Beijing Key Laboratory of Nasal Diseases,Beijing,100005,China;Research Unit of Diagnosis and Treatment of Chronic Nasal Diseases,Chinese Academy of Medical Sciences,Beijing,100005,China)

机构地区：[1]首都医科大学附属北京同仁医院耳鼻咽喉头颈外科,耳鼻咽喉头颈科学教育部重点实验室(首都医科大学),北京100730 [2]北京市耳鼻咽喉科研究所,过敏性疾病北京实验室(北京市教育委员会),鼻病研究北京市重点实验室,北京100005 [3]中国医学科学院慢性鼻病诊疗策略研究创新单元,北京100005

出　　处：《中国耳鼻咽喉头颈外科》2024年第4期236-241,共6页Chinese Archives of Otolaryngology-Head and Neck Surgery

基　　金：国家重点研发计划(2022YFC2504100);教育部长江学者创新团队(IRT13082);中国医学科学院医学与健康科技创新工程项目(2019-I2M-5-022);首都卫生发展科研专项重点攻关项目(2022-1-1091);国家自然科学基金(81970852、82171110);北京市自然科学基金(7222024);首都医科大学科研培育基金(2020-1210020214)。

摘　　要：目的探讨累及鼻腔鼻窦的Rosai-Dorfman病(Rosai-Dorfman disease,RDD)的临床特征性表现。方法对2016年12月~2023年8月在首都医科大学附属北京同仁医院鼻科接受诊治的16例累及鼻腔鼻窦的RDD患者的临床资料回顾性分析。结果男女比例1∶3,平均年龄47.4岁,平均病程22.34个月。主诉鼻塞、鼻干、嗅觉减退、头面部痛和突眼/复视的患者,分别有14、3、5、6和1例,9例存在外鼻肿胀。14例至少累及鼻中隔,12例单纯鼻腔受累,3例累及鼻窦,1例为混合型。10例行快速冰冻病理检查,8例结果误诊或漏诊。鼻内镜检查9例患者的鼻中隔呈现双侧膨隆,7例鼻底和下鼻甲表面有结节状隆起或者颗粒状新生物。鼻窦CT发现9例患者鼻中隔区域呈现类圆形均匀软组织影或对称性软组织增厚影,5例鼻底、下鼻甲呈现明显的软组织增厚影。结论累及鼻腔鼻窦的RDD在外鼻表现、内镜和影像学检查中有一定的典型特征,综合判断有助于提高临床诊断RDD的可行性。OBJECTIVE To explore the clinical characteristic manifestation of Rosai-Dorfman disease(RDD)involving the nasal cavity and paranasal sinuses.METHODS The clinical data of 16 patients with RDD involving the nasal cavity and paranasal sinuses who received treatment in Department of Rhinology of Beijing Tongren Hospital Affiliated to Capital Medical University from December 2016 to December 2023,were retrospective analyzed.RESULTS The male to female ratio was 1:3,with an average age of 47.4 years and an average disease duration of 22.34 months.There were 14,3,5,6 and 1 patients who complained of nasal congestion,dry nose,decreased sense of smell,head and face pain,and bulging/diplopia,respectively.There were 9 patients who had external nasal swelling.There were 14 RDD patients who were involved at least the nasal septum,12 patients who were simply affected in the nasal cavity,and 3 cases who involved the paranasal sinuses.There was 1 case belonged to a mixed type of RDD.There were 8 of 10 cases who were misdiagnosed or missed due to intraoperative rapid freezing.Endoscopic examination showed bilateral bulging in 9 patients’nasal septum,and nodular or granular new growth on 7 patients’nasal floor and inferior turbinate surface.Sinus CT found 9 patients’the nasal septum area showed a circular uniform soft tissue shadow or symmetrical soft tissue thickening shadow,5 patients’nasal floor and inferior turbinate showed obvious soft tissue thickening shadow.CONCLUSION RDD involving the nasal cavity and paranasal sinuses has certain typical features in external nasal manifestations,endoscopic and imaging examinations.Comprehensive judgment can help improve the feasibility of clinical diagnosis of RDD.

关 键 词：鼻腔 鼻窦疾病 诊断 计算机辅助 ROSAI-DORFMAN病 临床特征 影像学 

分 类 号：R765[医药卫生—耳鼻咽喉科]