LAUSR

A healthy 25 year-old male presented to our emergency with a 1-day history of left pleuritic chest pain. He denied dyspnoea, haemoptysis or any risk factors for PE. He migrated to Australia from Fiji 2-years ago. He currently plays competitive rugby and has never smoked. He had no significant past antenatal or medical history. His observations were normal. On gross inspection, he had no structural chest wall deformities. There was dullness to percussion on the left lower zone with heart sounds audible on the left laterally. His abdomen was soft and non-tender. His inflammatory markers, troponin, LFT and lipase were normal. His ECG showed right axis deviation. A CXR showed marked left lung volume loss with multiple cystic spaces and left mediastinal shift. His right lung was hyperinflated. A CT-chest showed reduced left lung volume with multiple cysts upto 3cm in diameter, suggestive of CPAM-1. PFT was performed revealing restrictive spirometry without significant bronchodilator response with mild reduction in lung volume (TLC 63.3%). DLCO was 61.3% predicted and corrects for alveolar volume (98.5%). A bronchoscopy showed abnormal extrinsic compression of the left lower lobe with negative cytology and growth of haemophilus influenzae. Cardiothoracics were consulted for resection given the risk of pleuropulmonary blastoma who are monitoring annually. CPAM-1 presenting at adulthood without prior symptoms are very rare. In the literature most adult patients undergo lobectomy due to the 1% risk of malignant transformation. However, management of asymptomatic CPAM-1 is not well defined.