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A 20-year-old woman with recurrent left-sided pneumothoraces underwent video-assisted thoracoscopic pleurectomy and wedge resection of a left apical bulla. Medical history included ABCA3 pulmonary surfactant dysfunction and subsequent interstitial lung disease with diffuse ground glass changes suggestive of a desquamative interstitial pneumonitis (DIP). She was a lifelong non-smoker. Spirometry revealed forced vital capacity 0.97 L, 32% predicted and carbon monoxide transfer factor (TLCO) 27% predicted. The operation was uneventful but there was a persistent postoperative air leak requiring prolonged pleural drainage but no other intervention. …
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