Congenital pulmonary lymphangiectasis.

  title={Congenital pulmonary lymphangiectasis.},
  author={Friedrich Reiterer and Karin Grossauer and Nicholas Morris and Sabine Uhrig and Bernhard Resch},
  journal={Paediatric respiratory reviews},
  volume={15 3},
Congenital pulmonary lymphangiectasis (CPL) is a rare vascular malformation causing dilated lymph vessels and disturbed drainage of lymph fluid. Based on the pathogenesis and clinical phenotype it can be classified as primary or secondary CPL. Associated genetic syndromes with or without lymphedema, familial occurrence and gene mutations have been described. In utero, it may present as non-immune hydrops with pleural effusions. At birth neonates may have respiratory failure due to chylothorax… CONTINUE READING