Congenital Lobar Emphysema Diagnosed in Adult Age: A Case Report

Çalışkan, Tayfun; Okutan, Oğuzhan; Çiftçi, Faruk; Kartaloğlu, Zafer; Taş, Dilaver; Demirer, Ersin

doi:10.5152/ejp.2014.27136

Cited by 4 publications

(3 citation statements)

References 12 publications

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“…The etiology of CLE is unknown, and in 50% of cases no specific cause was found [7]. It is likely that abnormal interactions between embryonic endodermal and mesodermal components of the lung during bronchopulmonary development lead to progressive lobar hyperinflation [5].…”

Section: Discussionmentioning

confidence: 99%

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Adult congenital lobar emphysema treated with video-assisted thoracoscopic lobectomy

Bayram¹,

Kolbas²,

Vayvada³

et al. 2018

Curr Thorac Surg

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Congenital lobar emphysema (CLE) is a rare developmental anomaly of the lower respiratory tract, which generally presents during the neonatal/pediatric period. Lobectomy is the only curative treatment for CLE. An 18-year-old male with enlarging left anterior chest wall deformity and progressive breathlessness for the last 2 years admitted to our clinic.Physical examination revealed asymmetrical expansion of left hemithorax. Chest radiography and thoracic computed tomography demonstrated hyperinflation of the left upper lobe and a mediastinal shift to the right side. FEV1 was 1.70 L (50%). Ventilation-perfusion scintigraphy showed reduced perfusion and ventilation in the right lung to 33.4% (upper zone, 1.9%; middle zone, 10.0%; lower zone, 22.5%).Patient underwent a successful video-assisted thoracoscopic surgery (VATS) left upper lobectomy, and was discharged on postoperative day 5. He is still being followed-up on fifth year, asymptomatically, with normalized chest wall appearance. VATS lobectomy is a feasible, safe and effective treatment for congenital pulmonary disorders, even for delayed cases.

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Section: Discussionmentioning

confidence: 99%

“…In another 25%, the development of CLE is caused by bronchial obstruction. Reports showed an association of CLE with other congenital diseases as heart failure and various cardiac defects [7].…”

Section: Discussionmentioning

confidence: 99%

Adult congenital lobar emphysema treated with video-assisted thoracoscopic lobectomy

Bayram¹,

Kolbas²,

Vayvada³

et al. 2018

Curr Thorac Surg

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“…Adults with CLH can present with frequent respiratory infections, chest pain, and shortness of breath, or it may be diagnosed incidentally. Symptomatic CLH requires surgical resection and has an excellent prognosis [ 3 , 16 , 17 ].…”

Section: Bronchopulmonary Anomaliesmentioning

confidence: 99%

Imaging of congenital lung diseases presenting in the adulthood: a pictorial review

et al. 2021

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Congenital lung diseases in adults are rare diseases that can present with symptoms or be detected incidentally. Familiarity with the imaging features of different types of congenital lung diseases helps both in correct diagnosis and management of these diseases. Congenital lung diseases in adults are classified into three main categories as bronchopulmonary anomalies, vascular anomalies, and combined bronchopulmonary and vascular anomalies. Contrast-enhanced computed tomography, especially 3D reconstructions, CT, or MR angiography, can show vascular anomalies in detail. The tracheobronchial tree, parenchymal changes, and possible complications can also be defined on chest CT, and new applications such as quantitative 3D reconstruction CT images, dual-energy CT (DECT) can be helpful in imaging parenchymal changes. In addition to the morphological assessment of the lungs, novel MRI techniques such as ultra-short echo time (UTE), arterial spin labeling (ASL), and phase-resolved functional lung (PREFUL) can provide functional information. This pictorial review aims to comprehensively define the radiological characteristics of each congenital lung disease in adults and to highlight differential diagnoses and possible complications of these diseases.

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