Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases

Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.

A Case of Pulmonary Lymphangiectasis

Pulmonary lymphangiectasis is relatively a rare disorder. This disorder can be divided into three groups. First, pulmonary abnormality is associated with lymphangiectasia in other viscera (especilly intestine) and extremities. In the second group, patients have a concomitant cardiac anomaly, which appears to be associated with obstructed pulmonary venous return. The third group comprises of patients whose lymphangicetasia is not associated with cardiac anomalies. This form is thought to result from abnormal development of the lung. In this case, a 6-year-old male who was previously diagnosed as lymphedema of extremities and intestinal lymphangiectasis, suffered from coughing and dyspnea repeatedly. High resolution computed tomography showed thickening of bronchovascular bundle and interlobular septa, pneumonic consolidation and pleural effusion. These findings were compatible with pulmonary lymphangiectasis. We report a case of pulmonary lymphangiectasis with brief review of literatures.