Congenital pulmonary lymphangiectasia in an extremely low birth weight: a case report

Abstract

Abstract Objectives Congenital pulmonary lymphangiectasia (CPL) is a rare but fatal disorder of the lung. Case presentation We 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. Conclusions We intend to underline that CPL, albeit rare, must be one of the differential diagnoses in infants with severe neonatal respiratory distress not responding to intensive care. Chronic interstitial lung diseases are a challenging diagnostic clinical problem, which requires a multidisciplinary approach. Histological lung examination may be useful to demonstrate the presence of CPL.