A Case of Pulmonary Lymphangiomatosis.

Jang, Eun Kyung; Kim, Chang Rae; Choi, Chang Min; Oh, Yeon Mok; Shim, Tae Sun; Lee, Sang Do; Kim, Woo Sung; Kim, Dong Soon; Song, Jin Woo

doi:2011.70.5.428

Tuberc Respir Dis > Volume 70(5); 2011 > Article

Case Report

Tuberculosis and Respiratory Diseases 2011;70(5):428-432.
DOI: https://doi.org/10.4046/trd.2011.70.5.428 Published online May 1, 2011.

A Case of Pulmonary Lymphangiomatosis.

Eun Kyung Jang, Chang Rae Kim, Chang Min Choi, Yeon Mok Oh, Tae Sun Shim, Sang Do Lee, Woo Sung Kim, Dong Soon Kim, Jin Woo Song

Department 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.

Key Words: Lymphangiomatosis, pulmonary, Chylothorax, Lung diseases, Interstitial