Northeast Georgia Health Systems, Gainesville, GA, USA.
Am Surg. 2023 Jul;89(7):3284-3285. doi: 10.1177/00031348231160837. Epub 2023 Feb 28.
Congenital lobar emphysema (CLE) is a rare developmental lung disorder characterized by lobar hyperinflation secondary to bronchopulmonary obstruction. Half of patients are symptomatic at birth, with many requiring urgent or emergent surgical resection to treat respiratory distress. Meanwhile, patients achieving late childhood or adolescence without symptoms usually never require surgery. We present a case of a 26 year old otherwise healthy female with known CLE who developed massive hemoptysis and required urgent videoscopic (VATS) resection of her right lung upper lobe. We know of no other report of CLE causing life-threatening bleeding at any age, and herein review pathology and pathophysiology of the condition.
先天性肺大疱(CLE)是一种罕见的肺发育障碍性疾病,其特征为支气管肺阻塞导致的肺叶过度充气。一半的患者在出生时就有症状,许多患者需要紧急或紧急手术切除以治疗呼吸窘迫。同时,在儿童后期或青春期无症状的患者通常不需要手术。我们报告了一例 26 岁的健康女性,患有已知的 CLE,她发生了大量咯血,需要紧急行电视辅助胸腔镜手术(VATS)切除其右上肺叶。我们不知道在任何年龄 CLE 导致危及生命的出血的其他报告,在此我们回顾了该疾病的病理学和病理生理学。