Congenital Pulmonary Lymphangiectasia, Associated with Total Anomalous Pulmonary Venous Return

Congenital pulmonary lymphangiectasia (CPL) is a rare pathological diagnosis. It was first reported by Virchow in 1856. CPL is histologically characterized by pulmonary lymphatic dilatation without lymphatic proliferation in the subpleural, peribronchovascular and interlobular areas. The prognosis of CPL is very poor where approximately 50% of all infants are stillborn and most others usually die within the first day of life. CPL can occur as a primary disorder or arise due to other diseases. CPL is often related to congenital heart diseases such as total anomalous pulmonary venous return (TAPVR), obstructed pulmonary venous return and hypoplastic left heart syndrome. Most cases of CPL are usually diagnosed by clinical and radiological exams, while some cases are confirmed by postmortem examinations. However, our patient was an extremely rare case, diagnosed by an antemortem lung biopsy. We report a case of CPL with TAPVR, confirmed by an antemortem lung biopsy.