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Tuberc Respir Dis.  2011 May;70(5):428-432.

A Case of Pulmonary Lymphangiomatosis

Affiliations
  • 1Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. skysong3@hanmail.net

Abstract

Pulmonary lymphangiomatosis is a rare disorder involving the entire intrathoracic lymphatic system from the mediastinum to the pleura. Pulmonary lymphangiomatosis mostly occurs in children and young adults without gender predilection. Although it is pathologically benign, it shows a progressive and fatal course with variable initial presentation. We now report a case of pulmonary lymphangiomatosis in a 35-year-old man. He presented with hemoptysis 6 months previously. Chest x-ray and a chest computed tomography scan showed diffuse interstitial thickening with left pleural effusion. Chylothorax was confirmed by thoracentesis. Lymphangiography showed dilated and tortuous lymphatic channels. Surgical lung biopsy revealed proliferation of complex anastomosing lymphatic channels. He was diagnosed with pulmonary lymophangiomatosis. Closed thoracostomy and chemical pleurodesis were done and the dyspnea was reduced.

Keyword

Lymphangiomatosis, pulmonary; Chylothorax; Lung diseases, Interstitial

MeSH Terms

Adult
Biopsy
Child
Chylothorax
Dyspnea
Hemoptysis
Humans
Lung
Lung Diseases
Lung Diseases, Interstitial
Lymphangiectasis
Lymphatic System
Lymphography
Mediastinum
Pleura
Pleural Effusion
Pleurodesis
Thoracostomy
Thorax
Young Adult
Lung Diseases
Lymphangiectasis
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