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Extralobar pulmonary sequestration (EPS) in the mediastinum is rare, and preoperative diagnosis can be challenging. We report a case of EPS in the middle mediastinum, where a congenital pericardial defect became apparent on computed tomography (CT) imaging as pneumopericardium concurrent with spontaneous pneumothorax. The patient presented with a left spontaneous pneumothorax.

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Spontaneous pneumomediastinum is an infrequent condition typically secondary to smoking, illicit drug use, or asthma. The condition often follows barotrauma or bronchial hyperactivity, causing alveolar destruction and air trapping within the mediastinum. Rarely, it may present following strenuous exercise, particularly in tall, thin males, resembling the presentation of pneumothorax.

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Air leak syndrome (ALS) is described in human medicine as a constellation of clinical disorders including pneumomediastinum, pneumopericardium, pulmonary interstitial emphysema, pneumothorax, pneumoperitoneum, pneumoretroperitoneum and subcutaneous emphysema. The pathogenesis of ALS depends on the anatomy of the mediastinum and its associations with thoracic, abdominal and cervical connective tissues, as well as a physical phenomenon referred to as the Macklin effect. Various animal species develop diverse combinations of these lesions, although ALS has not been recognized in animals.

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