小儿先天性肺囊性腺瘤样畸形的影像学表现

The imaging manifestation of congenital cystic adenomatoid malformation of the lung in children

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 manifestation in 8 cases of congenital cystic adenomatoid malformation of the lung in children (CCAM) in order to improve the recognition. Methods Seven males and 1 female were reported, and the mean age was 3 years 10 months. The complaints were cough, fever, and chest distress repeated for 4 days to 8 years. Chest films and CT scan were performed in all cases before operation and three of them were examined by high-resolution CT. All cases were confirmed by operation and pathology as CCAM. Results (1)On chest films, 4 cases showed single or multiple large air cystic lesions (>3 cm in diameter), one case only showed localized hazy and curl-like lung markings, and 3 cases showed honeycomb-like small cystic lesions (<3 cm in diameter). All cases were complicated with emphysema on affected side.6 cases showed pulmonary hernia of mediastinum. (2)On CT scan, each lung were involved in 4 cases, 2 cases showed large air cyst (almost 9 cm in diameter), 2 cases showed roundness thin-wall air cystic lesions (3.8～5.6 cm in diameter), and 4 cases showed multiple irregular small air cystic lesions (0.2～3.2 cm in diameter) and adenoid change. The cysts were filled with air but with small amount of liquid in only 3 cases. All lesions showed obvious space occupying sign. (3)The pathologic findings, the cyst was paraplasmic glandular or bronchiolar structures, cyst wall was lined with ciliated pseudostratified columnar epithelium in 3 cases and lined by cuboids to columnar epithelium in 5 cases. The cyst wall contained smooth muscle and elastic tissue. Cartilage plates were not present in all cases. Conclusion Imaging examination is a reliable method in diagnosing CCAM. It can provide the diagnosis in location and quality. CT scan can improve the detection rate of CCAM.