Rose Paul, Rana Saim, Abbey Cassie, Deskins Seth, Al-Qatarneh Saif
Division of Medical Education, School of Medicine, West Virginia University, Morgantown, West Virginia, USA.
Department of Internal Medicine/Pediatrics, West Virginia University, Morgantown, West Virginia, USA.
Pediatr Pulmonol. 2023 Jun;58(6):1821-1823. doi: 10.1002/ppul.26376. Epub 2023 Mar 8.
Congenital lobar overinflation, also known as congenital lobar emphysema (CLE), is an uncommon (1/20,000-30,000 live births) abnormality characterized by hyperinflation of one or more pulmonary lobes, usually with contralateral displacement of the mediastinum. While the etiology of most cases of CLE is poorly understood and labeled idiopathic, some cases are thought to be due to an intrinsic or extrinsic bronchial wall abnormality causing a ball valve mechanism with resultant hyperinflation of the affected lobe. CLE tends to have a predilection for males presenting insidiously in the first 6 months of life and have respiratory distress and progressive failure, with 50% of cases being asymptomatic at birth. Acquired forms of lobar emphysema are similar but are secondary to prolonged exposure to oxygen and positive pressure ventilation in premature infants. Clinical presentation is variable, ranging from wheezing, increased respiratory effort, cyanosis, feeding difficulties, and reflux or respiratory failure. Chest radiography (CXR) is the initial imaging obtained for any neonate with respiratory distress, which can aid in diagnosis showing overinflation, while computerized tomography (CT) remains the gold standard for confirmatory diagnosis. Treatment is variable and based on clinical severity. Some cases can be managed conservatively, while more severe cases require surgical intervention with lobectomy. Here, we present a case of a neonate in respiratory distress soon after birth, had initial improvement with supportive care, and was found to be secondary to CLE.
先天性肺叶过度充气，也称为先天性肺叶气肿（CLE），是一种罕见的疾病（每20000 - 30000例活产中有1例），其特征是一个或多个肺叶过度充气，通常伴有纵隔向对侧移位。虽然大多数CLE病例的病因尚不清楚，被标记为特发性，但一些病例被认为是由于内在或外在的支气管壁异常导致球瓣机制，从而使受影响的肺叶过度充气。CLE往往好发于男性，在出生后的前6个月隐匿出现，伴有呼吸窘迫和进行性衰竭，50%的病例在出生时无症状。获得性肺叶气肿形式相似，但继发于早产儿长期暴露于氧气和正压通气。临床表现多样，包括喘息、呼吸费力增加、发绀、喂养困难、反流或呼吸衰竭。胸部X线摄影（CXR）是对任何有呼吸窘迫的新生儿进行的初始影像学检查，有助于诊断显示过度充气，而计算机断层扫描（CT）仍然是确诊的金标准。治疗方法因人而异，取决于临床严重程度。一些病例可以保守治疗，而更严重的病例需要进行肺叶切除术的手术干预。在此，我们报告一例新生儿出生后不久出现呼吸窘迫，经支持治疗后最初有所改善，最终发现是由CLE引起的病例。
