Granato F, Voltolini L, Spina D, Paladini P, Gallazzi M, Gotti G
Thoracic Surgery Unit, Department of Cardio-Thoracic and Vascular Surgery, University Hospital of Siena, Siena, Italy.
Minerva Chir. 2009 Feb;64(1):111-5.
Congenital lobar emphysema (CLE) is a rare congenital lung disease consisting in overinflation of a pulmonary lobe. Adult onset of CLE is therefore unusual, often presented with mild symptoms. The authors report a very uncommon case of congenital segmental emphysema diagnosed in a 21-year-old non-smoking man because of recurrent right pneumothorax. Indication to pulmonary resection was established according to functional limitation, radiological findings of right upper lobe segmental emphysema with corresponding bronchial agenesia, scintigraphic result of extremely reduced ventilation and perfusion of lung emphysematous area and recurrency of pneumothorax. The intervention was carried out by 3-portal video-assisted thoracic surgery (VATS) using single-lung ventilation leading to determine precisely how much lung to resect thanks to the obvious and clear-cut distinction between functioning and non functioning parenchyma of the upper lobe. A stapler wedge resection by VATS was thus obtained, that, as far as the author's knowledge, it is the first case of endoscopic parenchymal sparing resection in CLE. Even though congenital lobar emphysema is rare, clinical awareness of this condition is important for early diagnosis and effective surgical treatment that in this case led to favourable
The VATS procedure seems to be an advantageous approach.
先天性肺叶气肿（CLE）是一种罕见的先天性肺部疾病，表现为肺叶过度充气。因此，成人期发生CLE并不常见，通常症状较轻。作者报告了一例非常罕见的先天性节段性气肿病例，该病例发生在一名21岁的不吸烟男性身上，其因反复出现右侧气胸而被诊断。根据功能受限情况、右上叶节段性气肿伴相应支气管发育不全的影像学表现、肺气肿区域通气和灌注极度降低的闪烁扫描结果以及气胸复发情况，确定了肺切除的指征。手术通过三孔电视辅助胸腔镜手术（VATS）进行，采用单肺通气，由于上叶功能正常和功能异常的实质之间有明显且清晰的区分，从而能够精确确定需要切除多少肺组织。因此，通过VATS获得了吻合器楔形切除术，据作者所知，这是CLE中首例内镜下保留实质的切除术。尽管先天性肺叶气肿罕见，但对这种疾病的临床认识对于早期诊断和有效的手术治疗很重要，在本病例中取得了良好的结果：VATS手术似乎是一种有利的方法。
