No abstract available.
Chylothorax* ; Lymphangiectasis*

Lymphangioma circumscriptum is a rare disease and the lesions are indistinguishable, clinically and histologically, from lymphangiectasis. The only difference is that lymphangiectasis is usually bilateral and develops in adult or late life, ie, condary to irradiation, surgical interruption, or some malignancies, but lymphangioma circumscriptum is congenital disease or develops soon after birth.
Adult ; Humans ; Lymphangiectasis ; Lymphangioma* ; Parturition ; Rare Diseases

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.
Child ; Cough ; Dyspnea ; Extremities ; Humans ; Lung ; Lymphangiectasis* ; Lymphangiectasis, Intestinal ; Lymphedema ; Male ; Pleural Effusion ; Viscera

Adolescent ; Child, Preschool ; Female ; Humans ; Lymphangiectasis, Intestinal ; Male

Primary Intestinal lymphangiectasia (PIL) is a common cause of protein losing enteropathy (PLE). It will affect enter-hepatic circulation of lipid-soluble vitamin, and absorption of electrolytes, cause malnutrition related osteomalacia or osteoporosis. While seldom health care workers noted to assess and treat osteomalacia or osteoporosis in PIL. Here we report a related case. We found increased parathyroid hormone, decreased 25(OH)D3, low bone mineral density, which indicated that the PIL patient had osteomalacia and/or osteoporosis. Adequate calcium and vitamin D supply can relieve the condition efficaciously. We should pay attention to osteomalacia and osteoporosis in PIL patients.
Adolescent ; Female ; Humans ; Lymphangiectasis, Intestinal ; diagnosis ; Osteomalacia ; diagnosis ; Osteoporosis ; diagnosis

Female ; Humans ; Infant ; Lymphangiectasis, Intestinal ; diagnosis ; pathology ; therapy ; Male

Adult ; Aged ; Female ; Humans ; Intestine, Small ; surgery ; Lymphangiectasis, Intestinal ; pathology ; surgery ; Male ; Middle Aged

Congenital pulmonary lymphangiectasis is a rare cause of severe respiratory distress in the newborn period and most of these patient die, usually within the first 24 hours of life. The light microscopic characteristics of the lungs are a network of partly tubular, partly cystically enlarged lymph vessels within large areas of connective tissue and they have thin wall lined by endothelium. Also congenital pulmonary lymphangiectasis is closely associated with cardiovascular malformation, and most frequently occurs in the clinical setting of congenital heart disease with or without pulmonary venous obstruction(total anomalous venous return, atresia of large pulmonary veins) The authors describe three typical cases of congenital pulmonary lymphangiectasis.
Connective Tissue ; Endothelium ; Heart Defects, Congenital ; Humans ; Infant, Newborn ; Lung ; Lymphangiectasis* ; Lymphangioleiomyomatosis

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.
Humans ; Infant, Newborn ; Lung/pathology ; Lung Diseases/congenital/*pathology ; Lymphangiectasis/congenital/*pathology ; Lymphatic System/pathology ; Male

Adult ; Humans ; Intestine, Small ; pathology ; Liver Cirrhosis ; complications ; Lymphangiectasis, Intestinal ; etiology ; Male