Korean J Pathol.  2011 Dec;45(6):650-653.

Congenital Pulmonary Lymphangiectasia, Associated with Total Anomalous Pulmonary Venous Return

• Hwang SW
• Kim MS
• Park PE
• Park TI

Abstract

Congenital pulmonary lymphangiectasia (CPL) is very rare. It shows diffuse pulmonary lymphatic dilatation without lymphatic proliferation. CPL can occur as a primary disorder or arise secondarily from other diseases such as the obstruction of pulmonary veins or lymphatics. The prognosis of CPL is very poor. Approximately 50% of infants are stillborn and most others usually die within the first day of life. The present case showed diffuse lymphangiectasia in the subpleural, interlobular, and peribronchovascular areas. The flat lining cells were immunohistochemically positive for D2-40 and CD31. CPL is usually diagnosed by clinicoradiological or postmortem examinations. However, our case was diagnosed by an antemortem lung biopsy. We report a case of CPL with total anomalous pulmonary venous return.

Keyword

Congenital pulmonary lymphangiectasia; Pulmonary venous return anomaly

MeSH Terms

Autopsy
Biopsy
Dilatation
Humans
Infant
Lung
Prognosis
Pulmonary Veins
Scimitar Syndrome