Pulmonary lymphangiectasia

Last revised by Dr Rohit Sharma on 07 Mar 2018

Pulmonary lymphangiectasia (PL) refers to a rare, fatal congenital abnormality of the lungs characterized by grossly dilated lymphatic channels in the sub pleural, interlobar, perivascular and peribronchial areas 3.

It is divided into two main types 1:

Clinical features depend on the type:

Abnormal development of the lungs and associated lymphatics during 14th to 20th week of gestation 1.

During the intrauterine life, in this phase of gestation, the lymphatics in the sub pleural space and interlobular areas are large and decrease in caliber by the 20th week. However, continued development of the lung without decrease in size of lymphatics results in this abnormality.

  • hydrops fetalis (associated feature)
  • perihilar infiltrates
  • pleural effusion
  • varying degrees of hyperinflation
  • perihilar infiltrates with air bronchograms
  • interstitial and interlobular septal thickening
  • pleural effusion (chylothorax)
  • T1 (especially coronal): 
    • interstitial thickening
    • pleural effusion
    • atelectasis
  • T2 (especially axial)
    • usually shows high-signal material within the pulmonary interstitium 3
  • radiotracer accumulation in the lungs
  • asymmetric visualization of lymphatic channels

Cardiac associated and the non-cardiac associated early onset types are more fatal leading to death. The late onset type does not have a poor prognosis as symptoms and clinical findings improve after the first year of life 5.

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Cases and figures

  • Case 1 from venous obstruction
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