气管支气管弥漫性狭窄少见病变的CT诊断

CT diagnosis of rarerare disease on widely narrowing of trachea and bronchi

目的探讨复发性多软骨炎(RPC)、气管淀粉样变性、骨化性气管支气管病(TO)的CT表现及鉴别特点,以提高诊断的正确率。方法分析13例经病理证实的三种病变,其中RPC3例,气管淀粉样变4例,TO6例。结果三种病变均表现为气管支气管弥漫性狭窄、管壁增厚及钙化。但病变累及的部位有所不同,RPC以气管全段为主,气管淀粉样变以气管全段或中下段为主,TO累及气管中下段前及两侧壁。除气管外,左右主支气管均可受累,部分病例累及叶和段支气管。气管壁增厚狭窄程度以RPC和气管淀粉样变明显,TO较轻。结论气管前及侧壁广泛多发钙化小结节向腔内突出,是TO较具特征性的CT表现;合并全身多部位软骨炎有助于RPC的诊断;而气管淀粉样变的诊断,需根据CT表现特点,并结合临床做出。

Objective To study the CT appearances of relapsing polychondritis （PRC）, amyloidosis of the trachea and tracheobronchopathia （TO）. Find the critical points for diagnosis and differential diagnosis. Methods The CT findings of 13 cases of RPC, 4cases of amyloidosis of trachea and 6 cases of TO were retrospectively analyzed and all of them were confirmed by pathology. Results These diseases all performed widely narrowing of trachea and bronchi, thickness and calcification in the wall of lumen. But they had distinctive distribution, PRC usually affected the whole trachea, amyloidisis of the trachea often localized in the middle and inferior parts of the trachea, TO mainly affected the anterior and lateral wall of the middle and inferior parts of the trachea. Except the trachea, right and left main bronchi could be affected also. Amyloidosis of the trachea could influence, the lobar and segmental bronchi. The thickness of wall was marked in PRC and amyloidosis of the trachea, while mild in TO. Conclusion ＂ Muctiple calcification in anterior and lateral wall is the special demonstration of TO, the diagnosis of PRC and amyloidosis of the trachea should combine CT performances with clinical appearances. Pdychondritis in several sites may indicate the diagnosis of PRC.