[Congenital pulmonary lymphangiectasis. One new case with complete blockage of the return venous pulmonary circulation and marked interstitial emphysema].
In this unusual case of bilateral congenital pulmonary lymphangiectasia with complete block of pulmonary venous return, the radiological diagnosis was made difficult by the association of the lymphangiectasia with bilateral interstitial emphysema. Routine dissection of the heart, considered to be normal since the cardio-thoracic index was only 0.45, relealed the true cause of death. The heart was the site of extreme hypoplasia of the left heart with complete mitral and aortic atresia. The ostium secundum being completely closed, the left atrium, after receiving the pulmonary veins, was completely blocked. It communicated neither with the right atrium, nor with the non-existent left ventricule.
D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000000981:diagnostic imaging*; D008200:Lymphangiectasis / Q000150:complications / Q000151:congenital* / Q000000981:diagnostic imaging; D011652:Pulmonary Circulation; D011656:Pulmonary Emphysema / Q000150:complications*; D011859:Radiography
Lymphangiectasie pulmonaire congénitale. Une nouvelle observation avec blocage complet de la circulation veineuse pulmonaire de retour et important emphysème interstitiel.