Pulmonary lymphangiectasia

Dr Amir Rezaee and Dr Aditya Shetty et al.

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

It is divided into two main types 1:

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.

Cardiac-associated lymphangiectasia
Clinical presentation

Cyanosis and respiratory depression

Associated conditions

There is variable configuration of the heart depending on the underlying  cardiac malformation. Most commonly:

Non-cardiac associated lymphangiectasia (early-type)
Clinical presentation

Abnormal pattern of respiration.

Associated conditions 
Non-cardiac associated lymphangiectasia (late-type)
Clinical presentation

Progressive pattern of lung disease starting at 10 days1.

Associated conditions 
Antenatal ultrasound
  • hydrops fetalis (associated feature)
CXR
  • perihilar infiltrates
  • pleural effusion
  • varying degrees of hyperinflation
HRCT
  • perihilar infiltrates with air bronchograms
  • interstitial and interlobular septal thickening
  • pleural effusion (chylothorax)
MRI
Coronal T1 
  • interstitial thickening
  • pleural effusion
  • atelectasis
Axial T2

Usually shows high-signal material within the pulmonary interstitium 3.

Lymphoscintigraphy
  • 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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Article information

rID: 28822
Systems: Paediatrics, Chest
Section: Pathology
Tags: shetty, cases
Synonyms or Alternate Spellings:
  • Pulmonary cystic lymphangiectasis
  • Pulmonary lymphangiomatosis

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