Table 1 Classification of Pulmonary Lymphangiectasia
Primary Pulmonary Lymphangiectasia | • Primary pulmonary developmental defect • Failure of normal regression process of lymphatic channels of the fetal lung (20 weeks' gestation) • Dilated pulmonary lymphatics • Hemihypertrophy and lymphedema may be present • Syndromic (See table II) |
Secondary Pulmonary Lymphangiectasia | • Hypoplastic left heart syndrome, pulmonary vein atresia, congenital mitral stenosis, cor triatum • Thoracic duct agenesis • Obstructive forms • Infections |