Vanishing Lung Syndrome (Giant Bullous Emphysema): CT Findings in 7 Patients and a Literature Review

Vanishing Lung Syndrome (Giant Bullous Emphysema): CT Findings in 7 Patients and a Literature Review
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DOI:
10.1097/rti.0b013e31819b9f2a
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发表时间:
2009-08-01
影响因子:
3.3
通讯作者:
Mohammed, Tan-Lucien H.
Mohammed, Tan-Lucien H.
中科院分区:
医学3区
文献类型:
--
作者:
Sharma, Nidhi;Justaniah, Al Mamoon;Mohammed, Tan-Lucien H.

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目的:回顾性分析7例特发性巨大肺气肿的影像学表现。这是一种慢性进行性疾病,通常影响年轻男性吸烟者,其特征是巨大的肺气肿性大疱,通常发生在肺上叶。广泛的间隔旁肺气肿合并形成巨大的肺大疱,压迫正常的肺实质并经常使其向中央移位。这些大疱占据至少三分之一的hemithorac.Materials和方法:7例胸部影像学证据的大疱或大疱占据至少三分之一的hemithoracic,谁也进行了检查,高分辨率计算机断层扫描(H RCT),被列入本回顾性研究。在HRCT扫描,大小,位置和分布的大疱被记录和分类为无论是胸膜下或central.Results:在所有7个研究患者的HRCT扫描结果包括许多大疱的大小范围从几厘米的直径巨大的大疱几乎填充整个半胸,模仿气胸。7例患者中有5例有广泛的上叶为主的大疱,7例患者中有4例显示严重的双侧疾病,累及不对称,7例患者中有2例显示左肺为主,而1例显示右肺为主的疾病。我们所有的患者都有胸膜下大疱,有实质纤维化,另一个有广泛的皮下气肿,我有伴随bronchiectasis.Conclusions:HRCT扫描的主要结果是广泛的间隔旁肺气肿合并成巨大的大疱。HRCT有助于明确VLS的诊断,评估病变程度,并提供指导治疗的信息。
Purpose: we reviewed the imaging findings in 7 patients with idiopathic giant bullous emphysema. This is a chronic, progressive condition usually affecting young male smokers and is characterized by giant emphysematous bullae, which commonly develop in the upper lobes. Extensive paraseptal emphysema coalesces to form giant bullae, compressing the normal lung parenchyma and often displacing it centrally. These bullae occupy at least one-third of a hemithorax.Materials and Methods: Seven patients with chest radiographic evidence of a bulla or bullae occupying at least one-third of a hemithorax, who had also been examined with high-resolution computed tomography (H RCT), were included in this retrospective study. On HRCT scans, the size, location, and distribution of the bullae were documented and categorized as either subpleural or central.Results: The HRCT scan findings in all 7 study patients included numerous bullae ranging in size from a few centimeters in diameter to giant bullae nearly filling an entire hemithorax, mimicking a pneumothorax. Five of the 7 patients had extensive upper lobe predominant bullae, 4 of the 7 patients showed severe bilateral disease with asymmetric involvement, 2 of the 7 patients demonstrated left lung predominance and whereas I patient showed right lung predominant disease. All of our patients had subpleural bullae, had parenchymal fibrosis, another had extensive Subcutaneous emphysema, and I had accompanying bronchiectasis.Conclusions: The predominant findings on HRCT scans are extensive paraseptal emphysema coalescing into giant bullae. HRCT is helpful in confirming the diagnosis of VLS, assessing the degree of the disease, and providing information to guide treatment.