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JPTM > Volume 45(6); 2011 > Article
The Korean Journal of Pathology 2011;45(6): 650-653.
doi: https://doi.org/10.4132/KoreanJPathol.2011.45.6.650
Congenital Pulmonary Lymphangiectasia, Associated with Total Anomalous Pulmonary Venous Return.
Seong Wook Hwang, Mee Seon Kim, Po Eun Park, Tae In Park
Department of Pathology, Kyungpook National University School of Medicine, Daegu, Korea. tipark@knu.ac.kr
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.
Key Words: Congenital pulmonary lymphangiectasia; Pulmonary venous return anomaly