[Congenital pulmonary lymphangiectasis].

Congenital pulmonary lymphangiectasis is a rare cause of severe respiratory distress in the newborn period and most of these patients die, usually within the first 24 hours of life. It may present as an isolated anomaly or it may be associated with other congenital abnormalities, most commonly cardiac in origin, as is the case of total obstruction of the pulmonary venous drainage. Congenital pulmonary lymphangiectasis may be also associated with lymphatic anomalies of other portions of the body or with other non-lymphatic, non-cardiac congenital malformations. Chest radiographs show more commonly that the lungs are hyperexpanded and present a diffuse interstitial reticulonodular pattern. The differential diagnosis from the radiological standpoint should include total anomalous pulmonary venous drainage and pneumonia. We present the case of a newborn infant with congenital pulmonary lymphangiectasis who died within the first 24 hours of life, emphasizing clinical, radiological and pathological findings.