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目的 描述小儿先天性肺囊性腺瘤样畸形的影像学表现 ,以提高对该病的认识。方法男 7例 ,女 1例 ,平均年龄 3岁 10个月。均以反复呼吸道感染就诊 ,病史 4d至 8年。 8例均经手术、病理证实 ,术前摄胸片及胸部CT平扫 ,3例病变区加作高分辨率CT扫描。结果 (1)胸片见单个或多个毗邻含气大囊 (囊径 >3cm) 4例 ,多发蜂窝样小囊 (囊径 <3cm)的 3例 ,1例表现为肺纹理模糊 ,6例合并纵隔肺疝 ,患侧肺气肿样改变 8例。 (2 )CT见左右肺受累各 4例 ,2例表现为巨大囊腔 (囊腔最大径约 9cm) ,2例呈类圆形薄壁囊腔 (囊径 3 8～ 5 6cm) ,4例表现为多发蜂窝样小囊 (囊径 0 2～3 2cm) ,囊内以含气为主 ,3例囊内含少量液体 ,病变均有不同程度的占位效应。 (3)病理检查见囊腔为异常增生的管腔或腺样结构 ,壁内被覆假复层纤毛柱状上皮 3例 ,纤毛柱状及立方上皮 5例 ;囊周可见平滑肌及弹性纤维环绕 ,8例囊壁内均未见软骨成分及腺体。结论 影像学检查为诊断先天性肺囊性腺瘤样畸形的可靠方法 ,它可以提出定位、定性诊断 ,CT能明显提高病变的检出率。
Objective To describe the imaging findings of congenital cystic adenomatoid malformation in children in order to improve the understanding of the disease. Methods Male 7 cases, female 1 case, average age 3 years and 10 months. Respiratory infections were repeated treatment, history 4d to 8 years. All the 8 cases were confirmed by operation, pathology, preoperative chest radiography and chest CT scan, and 3 cases of high resolution CT scan. Results (1) The chest radiographs were found in 4 cases of single or multiple large air sacs (> 3 cm in diameter), 3 cases of multiple honeycomb vesicles (<3 cm in diameter), 1 case of obscure lung appearance and 6 cases Combined mediastinal pulmonary hernia, ipsilateral emphysema-like changes in 8 cases. (2) In CT, there were 4 cases of left and right lung involvement, 2 cases showed huge cysts (maximal diameter of cysts about 9cm), 2 cases were round-like thin cysts (38 ~ 56cm in diameter) and 4 The performance of multiple cellular vesicles (pocket diameter 0 2 ~ 3 2cm), the main gas-containing capsules, 3 cases of small amount of fluid containing the capsule, lesions have varying degrees of mass effect. (3) pathological examination showed that the cyst was abnormally hyperplastic lumen or adenoid structure, the wall was covered with pseudostratified cilia columnar epithelium in 3 cases, cilia columnar and cubic epithelium in 5 cases; smooth muscle and elastic fibers around the cyst can be seen in 8 cases None of the cartilage within the cartilage composition and glands. Conclusion Radiographic examination is a reliable method to diagnose congenital cystic adenomatoid malformation of the lung. It can provide localization and qualitative diagnosis. CT can significantly improve the detection rate of lesions.