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先天性肺囊性疾病是较少见的先天性肺部疾病,是肺组织胚胎发育异常所形成的畸形。囊性病变包括囊性腺瘤样畸形、支气管源性肺囊肿、先天性大叶性肺气肿、肺隔离症。此类疾病的临床表现相似,但发生机制及病理改变有各自的特点。囊性腺瘤样畸形是由于细支气管发育停滞,以肺泡不发育为代价引起的肺间质大量增生,目前根据病理类型分成5型。支气管源性肺囊肿是胚胎发育时期支气管树某段的异常出芽形成的。先天性大叶性肺气肿是支气管内阻外压以及支气管壁的病变造成的气体陷闭,肺泡腔扩张。肺隔离症是部分肺组织与正常的支气管肺组织无交通,供血来自体循环,病变肺无正常功能,分叶内型和叶外型。
Congenital pulmonary cystic disease is a rare congenital lung disease, abnormal formation of lung tissue embryo deformity. Cystic lesions include cystadenomatoid deformity, bronchogenic pulmonary cysts, congenital lobar emphysema, and pulmonary sequestration. The clinical manifestations of these diseases are similar, but the mechanism of occurrence and pathological changes have their own characteristics. Cystic adenomatoid deformity is due to bronchial development arrest, alveolar hypoplasia at the expense of pulmonary interstitial mass proliferation, according to the current pathological type is divided into 5 types. Bronchogenic pulmonary cysts are anomalous sprouting of a section of the bronchial tree during embryonic development. Congenital lobar emphysema is the bronchial resistance and external pressure caused by the bronchial wall lesions caused by gas trap, alveolar expansion. Pulmonary sequestration is part of the lung tissue and normal bronchial lung tissue without traffic, blood supply from the systemic circulation, no normal lung function, lobulation and leaf shape.