Congenital Pulmonary Lymphangiectasia, Associated with Total Anomalous Pulmonary Venous Return
황성욱, 김미선, 박보은, 박태인,
소속 상세정보
황성욱 ( Hwang Seong-Wook )
Kyungpook National University School of Medicine Department of Pathology
김미선 ( Kim Mee-Seon )
Kyungpook National University School of Medicine Department of Pathology
박보은 ( Park Po-Eun )
Kyungpook National University School of Medicine Department of Pathology
박태인 ( Park Tae-In )
Kyungpook National University School of Medicine Department of Pathology
KMID : 0357920110450060650
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.
키워드
Congenital pulmonary lymphangiectasia; Pulmonary venous return anomaly
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