Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia

Pulmonary lymphangiectasia (PL) is a rare congenital disorder of pulmonary lymphatic development. Although it was traditionally a fatal disorder of infancy, some cases in later childhood have been reported, suggesting a spectrum of severity. We present an unusual case of unilateral, congenital pulmo...

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Main Authors: Dionne Adair (Author), Raja Rabah (Author), Maria Ladino-Torres (Author), Thomas G. Saba (Author)
Format: Book
Published: Frontiers Media S.A., 2021-04-01T00:00:00Z.
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100 1 0 |a Dionne Adair  |e author 
700 1 0 |a Raja Rabah  |e author 
700 1 0 |a Maria Ladino-Torres  |e author 
700 1 0 |a Thomas G. Saba  |e author 
245 0 0 |a Case Report: A Relatively Mild Presentation of Unilateral Congenital Pulmonary Lymphangiectasia 
260 |b Frontiers Media S.A.,   |c 2021-04-01T00:00:00Z. 
500 |a 2296-2360 
500 |a 10.3389/fped.2021.657473 
520 |a Pulmonary lymphangiectasia (PL) is a rare congenital disorder of pulmonary lymphatic development. Although it was traditionally a fatal disorder of infancy, some cases in later childhood have been reported, suggesting a spectrum of severity. We present an unusual case of unilateral, congenital pulmonary lymphangiectasia. Our patient presented with neonatal respiratory distress, a chronic wet cough and recurrent episodes of bronchitis. Chest CT revealed thickening of the interlobular septae of the right lung. A lung biopsy confirmed the diagnosis of lymphangiectasia. His clinical course is characterized by chronic coughing and recurrent bronchitis but normal growth and development. This case illustrates a relatively mild presentation of unilateral PL, which, along with other reports, suggests variability in the presentation and severity of this disorder. In the absence of imaging and histological examination, mild presentations may go undiagnosed. 
546 |a EN 
690 |a lymphangiectasia 
690 |a pediatric 
690 |a histology 
690 |a computed tomography 
690 |a case report 
690 |a Pediatrics 
690 |a RJ1-570 
655 7 |a article  |2 local 
786 0 |n Frontiers in Pediatrics, Vol 9 (2021) 
787 0 |n https://www.frontiersin.org/articles/10.3389/fped.2021.657473/full 
787 0 |n https://doaj.org/toc/2296-2360 
856 4 1 |u https://doaj.org/article/02d157e686f54e7098b02d36322bca02  |z Connect to this object online.