FOETAL PULMONARY LYMPHANGIECTASIS

Abstract

Less than 70 cases of congenital pulmonary lymphangiectasis in neonates are reported in the literature. Half of them are stillborn, however to our knowledge no such foetal cases have yet been reported. The occurrence of these lesions in 2nd term gestation and the absence of valves, would indicate that this disease is probably a malformation and not an acquired lesion. We report here 5 new cases of pulmonary lymphangiectasis with the classical microcystic honeycomb appearance on cut section. 3 of them are isolated pulmonary lymphangiectasis from 20 and 22 week old spontaneous abortions and a 31 week old male stillborn, with no cardiopathy, no pleural effusion, no lymph node hypertrophy. 2 other cases may be a part of hydrops foetalis with no erythroblastosis : one is a 31 week old still born boy, the other is a 24 week old male twin foetus with placental vascular anastomoses, presenting a cystic adenomatoid malformation of the left inferior lobe. In all cases the development of the elastic network appears normal. Except the last case Factor VIII staining using the immunoperoxydase method reveals' no Factor VIII related antigen in cystic lymphatic vascular endothelial cells.