Abstract:ObjectivesCongenital pulmonary lymphangiectasia (CPL) is a rare but fatal disorder of the lung.Case presentationWe describe the case of an extremely low birth weight (ELBW) infant who presented with severe respiratory distress and recurrent bilateral pneumothorax. He died on day 17. The post-mortem examination of the lungs showed numerous cystic spaces, ranging from 1 to 2 mm in size, in the visceral pleura, in the thickened interlobular septum and hilum. A diagnosis of primary CPL was made.ConclusionsWe inten… Show more
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