Bale P M
Am J Clin Pathol. 1979 Apr;71(4):411-20. doi: 10.1093/ajcp/71.4.411.
Of 41 cases of cystic lungs in children, 21 were found by microscopy to be due to congenital cystic malformation. In most of the remainder, chronic inflammation and fibrosis precluded differentiation from postinflammatory pneumatocele. A few were intermediate between cystic malformation and congenital lobar emphysema. The cystic malformations were 17 surgical and four necropsy specimens, and two thirds of the patients were under 1 year old. The condition was unilobar, and the cysts were thin-walled, up to 8 cm in diameter, multiple or multilocular, and microscopically resembled proliferated, dilated bronchioles communicating with alveoli. There was a wide range of size, shape, and number of cysts, and no sharp demarcation from adenomatoid malformation in stillborn infants. Thus, bronchiolar malformations fall into two overlapping clinicopathologic groups: (1) adenomatoid malformation in edematous stillborn and premature infants with perdominantly solid lobes showing more epithelial proliferation and immature terminal airways and (2) cystic malformation in term infants and children with predominantly cystic lobes and interspresed mature alveoli.
在41例儿童肺囊肿病例中，经显微镜检查发现21例是由先天性囊肿性畸形所致。在其余大多数病例中，慢性炎症和纤维化使得难以与炎症后肺膨出相鉴别。少数病例介于囊肿性畸形和先天性大叶性肺气肿之间。囊肿性畸形有17例为手术标本，4例为尸检标本，三分之二的患者年龄在1岁以下。病变为单叶，囊肿壁薄，直径达8厘米，可为多个或多房性，显微镜下类似于增生、扩张并与肺泡相通的细支气管。囊肿的大小、形状和数量差异很大，与死产婴儿的腺样畸形无明显界限。因此，细支气管畸形可分为两个重叠的临床病理组：（1）水肿的死产和早产婴儿的腺样畸形，主要为实性肺叶，上皮增生较多，终末气道不成熟；（2）足月儿和儿童的囊肿性畸形，主要为囊性肺叶，散在有成熟肺泡。
