A 30-year-old male chronic smoker presents with progressive breathlessness for 1 month. Chest X-ray is shown. What is the most probable diagnosis?
A i.e. Vanishing lung syndromeRef: RadiopediaExplanation:Vanishing Lung Syndrome (Idiopathic Giant Bullous Emphysema)Characterized by giant emphysematous bullae, which commonly develop in the upper lobes and occupy at least one-third of a hemithorax.Progressive condition.Associated with several forms of emphysema.Commonly affects young men 1-2.Associated with:Cigarette smoking.Marfan syndrome,Ehlers-Danlos syndrome,Alpha-1-antitrypsin deficiency.Affected patients may be asymptomatic or experience hypoxia, severe dyspnea and/or chest pain.The bullae commonly involve the upper lobes with an asymmetric distribution and paraseptal location.Plain radiographNon-specific features.Bullae occupy more than one-third of the affected hemithorax (vary in size 1-20 cm),Upper lobes have greater involvement,There is bilateral and asymmetric lung involvement.May have compress effect to adjacent structures (lung parenchyma atelectasis, invert the ipsilateral diaphragm or contralateral displacement of the mediastinum and displacement of junction lines).CTBullae predominant in the subpleural location,Size of bullae ranges from 1 to 20 cm in diameter,Usually asymmetric.Presence of concomitant foci of paraseptal and centrilobular emphysema.Treatment and prognosisThe condition tends to be progressive.Criteria for bullectomy include large bullae with significant symptoms (reduced lung function or infection).ComplicationsCompression of surrounding lung (atelectasis),Pneumothorax,Bullae infection,Higher risk of lung cancer.Other Options:Emphysema: Lungs are large and hyper inflated.Signs of hyperinflation are: o Low set diaphragm.Flat diaphragm best determined by lateral chest,Hyper lucent lung fields,Increased AP diameter,Increased retro-sternal air.Vertical heart.BullaAir-filled space in the lung parenchyma due to destruction of alveolar tissue, distal to terminal bronchiole.Larger than 2 cm in distended state.Bullae + emphysema = bullous emphysema (can be congenital or complication of COPD).Giant bulla = bulla larger than one third of the hemithorax size and compression of adjacent lungparenchyma.Distinguish Giant Bulla from PneumothoraxImportant for treatment plan (bulla - no tube thoracostomy; pneumothorax - may need tube thoracostomy if large or symptomatic).Differentiation can be difficult on conventional radiography; they can coexist.Helpful signs for pneumothorax: visible visceral pleural line.Expiratory chest radiograph may help delineating a visceral pleural line of pneumothorax.CT scan is the most accurate mean to differentiate the two diagnoses."Double wall" sign described in cases with ruptured bulla causing pneumothorax (air outlining both sides of the bulla wall parallel to the chest wall).
Vanishing lung syndrome
Pneumothorax
Panacinar emphysema
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Respiratory System