Lacquet L K, Lacquet A M
Prog Pediatr Surg. 1977;10:307-20.
The aetiology of congenital lobar emphysema is not always evident. In the group with demonstrable check-valve mechanism, which allows the air to enter but not to leave the lung, there is either internal stenosis or external compression of the bronchus. When no cause can be found, the condition is called idiopathic, although in some cases alveolar fibrosis has been demonstrated, the check-valve mechanism being in these cases at an alveolar level. In the small group of rare cases of bronchial atresia, air which enters through a collateral ventilation cannot be removed by the same route; in these case too, the check-valve mechanism exists at the alveolar level. Five cases of "congenital lobar emphysema" are presented. One case showed no bronchial anomaly; another case showed an increase in interstitial connective tissue in the lung; tow cases showed hypoplasia or absence of bronchial cartilage; in one case, bronchial atresia was found at operation. Infants show a typical symptomatology of dyspnoea and cyanosis, and a typical chest X-ray with unilateral radiolucency and a delicate lung pattern, collapse of surrounding lung tissue, and mediastinal hernia. In older children, the diagnosis is made either incidently or following a complication. The condition is usually found in the left upper and the right middle lobe. Treatment is surgical and consists of resection of the emphysematous segments.
先天性大叶性肺气肿的病因并不总是很明确。在具有可证实的单向活瓣机制（即允许空气进入但不能离开肺）的病例组中，存在支气管内部狭窄或外部受压情况。当找不到病因时，这种情况被称为特发性，尽管在某些病例中已证实存在肺泡纤维化，在这些病例中单向活瓣机制存在于肺泡水平。在一小部分罕见的支气管闭锁病例中，通过侧支通气进入的空气无法通过相同途径排出；在这些病例中，单向活瓣机制也存在于肺泡水平。本文介绍了5例“先天性大叶性肺气肿”病例。1例未显示支气管异常；另1例显示肺间质结缔组织增多；2例显示支气管软骨发育不全或缺失；1例在手术中发现支气管闭锁。婴儿表现出典型的呼吸困难和发绀症状，胸部X线表现典型，为单侧透亮区及纤细的肺纹理、周围肺组织萎陷和纵隔疝。在大龄儿童中，诊断要么是偶然做出的，要么是在出现并发症后做出的。这种情况通常见于左上叶和右中叶。治疗方法是手术，包括切除气肿段。
