CONGENITAL PULMONARY LYMPHANGIECTASIA AND CHYLOTHORAX – A CASE SERIES
- G Rocha
- P Soares
- I Azevedo
- MJ Baptista
- J Casanova
- CS Moura
- H Guimaraes
Abstract
Congenital pulmonary lymphangiectasia (CPL) and chylothorax (CC) are rare lymphatic developmental disorders. We report six clinical cases of CPL and CC that were admitted to our level III neonatal intensive care unit over the last 20 years. One case of unilateral CC was successfully treated with pleuro-amniotic shunt; three cases of bilateral CC were associated to lung hypoplasia, hydrops fetalis, and generalized lymphangiectasias; one case of CPL was associated with obstructive congenital heart defect; one case of unilateral CC was successfully treated with thoracocentesis and medium-chain triglyceride diet. Mortality was high (66.6%).
Keywords: congenital pulmonary lymphangiectasia, chylothorax, lymphangiectasia, lung hypoplasia, lydrops fetalis, congenital heart defect
How to Cite:
Rocha, G., Soares, P., Azevedo, I., Baptista, M., Casanova, J., Moura, C. & Guimaraes, H., (2018) “CONGENITAL PULMONARY LYMPHANGIECTASIA AND CHYLOTHORAX – A CASE SERIES”, Lymphology 50(4), 188-196.
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