Diffuse pulmonary lymphangiectasis with heart defect discovered 4 months post-natally

Congenital pulmonary lymphangiectasia is a rare abnormality with dilatation of pulmonary lymphatics and the radiological pattern of a pulmonary interstitial syndrome. It is usually symptomatic at birth and is almost always lethal. We report the case of an infant with congenital pulmonary lymphangiectasis and congenital heart disease who is still alive at 11 years. This case is interesting from the pathogenic, nosologic and prognostic point of view.