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:
- cardiac-associated lymphangiectasia (secondary type)
non-cardiac associated lymphangiectasia (primary type)
- early onset type
- late onset 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.
Cyanosis and respiratory depression
There is variable configuration of the heart depending on the underlying cardiac malformation. Most commonly:
Non-cardiac associated lymphangiectasia (early-type)
Abnormal pattern of respiration.
- congenital ichthyosis
- Ehlers-Danlos syndrome
- polycystic kidney disease
- dysplastic kidneys
- uretero-pelvic obstruction
- urethral stenosis
- left ventricular hypertrophy
Non-cardiac associated lymphangiectasia (late-type)
Progressive pattern of lung disease starting at 10 days1.
- obstructive uropathy
- cystic fibrosis
Radiographic features of pulmonary lymphangiectasia
- 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)
- interstitial thickening
- pleural effusion
Usually shows high-signal material within the pulmonary interstitium 3.
- radiotracer accumulation in the lungs
- asymmetric visualization of lymphatic channels
Treatment and prognosis
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.
- 1.FELMAN A, RHATIGAN R, PIERSON K. American Journal of Roentgenology. 1972;116 (3): . doi:10.2214/ajr.116.3.548
- 2. Yao LC, Testini C, Tvorogov D et-al. Pulmonary lymphangiectasia resulting from vascular endothelial growth factor-C overexpression during a critical period. Circ. Res. 2014;114 (5): 806-22. doi:10.1161/CIRCRESAHA.114.303119 - Free text at pubmed - Pubmed citation
- 3. Bellini C, Boccardo F, Campisi C et-al. Congenital pulmonary lymphangiectasia. Orphanet J Rare Dis. 2009;1 (1): 43. doi:10.1186/1750-1172-1-43 - Free text at pubmed - Pubmed citation
- 4. Chung CJ, Fordham LA, Barker P et-al. Children with congenital pulmonary lymphangiectasia: after infancy. AJR Am J Roentgenol. 1999;173 (6): 1583-8. doi:10.2214/ajr.173.6.10584805 - Pubmed citation
- 5. Li YW, Snow J, Smith WL et-al. Localized pulmonary lymphangiectasia. AJR Am J Roentgenol. 1985;145 (2): 269-70. doi:10.2214/ajr.145.2.269 - Pubmed citation