小儿先天性肺囊性腺瘤样畸形的临床及影像学表现分析

目的通过对小儿先天性肺囊性腺瘤样畸形（CCAM）的临床特征、发病机制及鉴别诊断的分析，探讨患儿CCAM的影像学特点，以提高对该病的影像学认识。方法回顾性分析2011年3月-2013年3月3例经手术、病理证实CCAM患儿的临床资料、胸部CT影像学特点，复习相关文献，并对3例患儿随访2年。结果3例CCAM患儿均累及单侧单一肺叶，且位于下叶，其中左肺下叶2例，右肺下叶1例；2例可见多个含气大囊，1例见多发蜂窝样小囊。依照Stocker组织病理学分型，3例患儿中Ⅰ型2例，Ⅱ型1例。手术证实病变均为单侧，并侵犯一个肺叶，不与支气管相通。所有患儿治愈出院，术后随访2年结果显示恢复良好，无复发。结论影像学检查为发现和诊断CCAM的主要依据，其CT表现具有特征性，根据CT表现可推测其病理分型，熟悉其临床及CT特征有助于提高对该病的诊断。

Clinical and Imaging Manifestation Analysis of Congenital Cystic Adenomatoid Malformation of the Lung in Children

Objective To study the clinical features, pathogenesis and differential diagnosis, and explore the imaging characteristics of congenital cystic adenomatoid malformation of the lung （CCAM） in children, in order to improve the knowledge of imaging findings of the disease. Methods We retrospectively analyzed the clinical data and imaging characteristics of three patients proven to have CCAM of the lung both by surgery and pathology between March 2011 and March 2013. Related literature was also reviewed, and two out of the three patients were followed up for two years. Results All CCAM lesions in this study involved unilateral lower lobe, including two left lung and one right. Two patients had multiple large cysts containing gas, and one had multiple honeycomb-shaped vesicles. Histopathological examination showed that two cases were classified as Stocker type Ⅰ and one as type Ⅱ, without type Ⅲ cases. Surgeries proved that the malformation was unilateral in all patients without connection with the bronchia. All patients with CCAM got complete cure by operation. The two-year postoperative follow-up showed an excellent recovery in all the patients. Conclusions Imaging examination is the main basis for discovery and diagnosis of CCAM. The CT manifestations of CCAM are featured, and according to CT examination, pathological subtypes of the disease can be predicted. Being familiar with these characteristics is helpful for its diagnosis.