Journal of Pathology and Translational Medicine

Case Report

Congenital Pulmonary Lymphangiectasia, Associated with Total Anomalous Pulmonary Venous Return.: Seong Wook Hwang, Mee Seon Kim, Po Eun Park, Tae In Park; Korean J Pathol. 2011;45(6):650-653.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.6.650

Abstract PDF: 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.

Original Article

Idiopathic Intestinal Lymphangiectasia.: Kun Chang Song, Chan Il Park; Korean J Pathol. 1994;28(3):319-321.

Abstract: This is a case report of idiopathic intestinal lymphangiectasia occurring in a 3-year-old boy. Idiopathic intestinal lymphangiectasia is believed to be a part of the generalized congenital disorder of lymphatic system. The present case revealed markedly ectatic lymphatics in the lamina propria of duodenal villi with lymphorrhagia. Clinical features were typical of the protein-losing enteropathy; hypoalbuminemia, hypocalcemia, chylous ascites, edema of the lower extremities and mild lymphocytopenia. This case indicates that serious protein loss may ensue, even in cases of intestinal lymphangiectasia without abnormalities of extraintestinal lymphatics.

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