Korean J Leg Med.  1999 May;23(1):82-87.

Congenital Pulmonary Lymphangiectasis

• Lee BW
• Seo JS

Abstract

Congenital pulmonary lymphangiectasis is a rare cause of severe respiratory distress in the newborn period and most of these patient die, usually within the first 24 hours of life. The light microscopic characteristics of the lungs are a network of partly tubular, partly cystically enlarged lymph vessels within large areas of connective tissue and they have thin wall lined by endothelium. Also congenital pulmonary lymphangiectasis is closely associated with cardiovascular malformation, and most frequently occurs in the clinical setting of congenital heart disease with or without pulmonary venous obstruction(total anomalous venous return, atresia of large pulmonary veins) The authors describe three typical cases of congenital pulmonary lymphangiectasis.

Keyword

Lymphangioleiomyomatosis; cardiovascular malformation

MeSH Terms

Connective Tissue
Endothelium
Heart Defects, Congenital
Humans
Infant, Newborn
Lung
Lymphangiectasis*
Lymphangioleiomyomatosis