Schneider J R, St Cyr J A, Thompson T R, Johnson D E, Burke B A, Foker J E
J Thorac Cardiovasc Surg. 1985 Mar;89(3):332-9.
Congenital labor emphysema and cystic adenomatoid malformation have been the most common surgically treatable cystic lesions of the lung. With the successful treatment of newborn respiratory distress syndrome, an increased frequency of an acquired form of cystic disease, interstitial pulmonary emphysema, has been observed. Some degree of interstitial pulmonary emphysema is relatively common, and during the years 1980 to 1983 this disease was diagnosed in 372 infants. Seven of these infants, all premature, underwent resection of relatively localized areas of persistent cystic interstitial pulmonary emphysema. Lobectomy or wedge resection was performed in five patients because of their inability to be weaned from the ventilator. A sixth patient with this disease underwent lobectomy for recurrent pneumothoraces. A seventh patient underwent lobectomy because the cystic interstitial pulmonary emphysema produced atelectasis and recurrent infections. All seven patients were extubated by the fourth postoperative day, have been discharged, and are showing respiratory improvement. Within the same period, four infants had congenital lobar emphysema and two had congenital cystic adenomatoid malformation. They were gestational age 36 weeks or older and, although respiratory distress was present to some degree in all six, only one was ventilator dependent at operation. All underwent lobectomy and one infant had a left upper lobe resection and right middle lobectomy on separate occasions for bilateral congenital cystic adenomatoid malformation. All patients with congenital labor emphysema and congenital cystic adenomatoid malformation have been discharged and are doing well. Our results suggest the following conclusions: Persistent interstitial pulmonary emphysema is now the most common indication for pulmonary resection in the newborn period. The anatomic distribution of resected interstitial pulmonary emphysema is similar to that of congenital lobar emphysema. Although only a small fraction (less than 2%) of patients with interstitial pulmonary emphysema require operation, the development of relatively large discrete cystic areas that (1) significantly decrease effective lung volume and produce respirator dependence, (2) produce atelectasis and recurrent infections, or (3) lead to pneumothoraces may make pulmonary resection beneficial. Finally, despite the presence of generalized lung disease in patients with interstitial pulmonary emphysema, these patients can be expected to improve significantly after resection, and the long-term outcome is generally good.
先天性肺叶气肿和囊性腺瘤样畸形一直是最常见的可手术治疗的肺部囊性病变。随着新生儿呼吸窘迫综合征治疗的成功，一种后天性囊性疾病——间质性肺气肿的发病率有所增加。一定程度的间质性肺气肿相对常见，在1980年至1983年期间，有372名婴儿被诊断出患有此病。其中7名婴儿均为早产儿，接受了相对局限的持续性间质性肺气肿区域的切除术。5名患者因无法脱离呼吸机而接受了肺叶切除术或楔形切除术。第六名患有此病的患者因复发性气胸接受了肺叶切除术。第七名患者接受肺叶切除术是因为间质性肺气肿导致肺不张和反复感染。所有7名患者术后第4天均拔除气管插管，已出院，呼吸状况正在改善。在同一时期，有4名婴儿患有先天性肺叶气肿，2名患有先天性囊性腺瘤样畸形。他们的孕周为36周或更大，尽管这6名婴儿都有一定程度的呼吸窘迫，但只有1名在手术时依赖呼吸机。所有患者均接受了肺叶切除术，1名婴儿因双侧先天性囊性腺瘤样畸形，先后接受了左上叶切除术和右中叶切除术。所有患有先天性肺叶气肿和先天性囊性腺瘤样畸形的患者均已出院，情况良好。我们的结果提示以下结论：持续性间质性肺气肿现在是新生儿期肺切除最常见的指征。切除的间质性肺气肿的解剖分布与先天性肺叶气肿相似。虽然只有一小部分（不到2%）间质性肺气肿患者需要手术，但相对较大的离散囊性区域的形成，（1）显著减少有效肺容积并导致呼吸依赖，（2）导致肺不张和反复感染，或（3）导致气胸，可能使肺切除有利。最后，尽管间质性肺气肿患者存在全身性肺部疾病，但这些患者在切除术后有望显著改善，长期预后通常良好。
