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Jang, Kim, Choi, Oh, Shim, Lee, Kim, Kim, and Song: A Case of Pulmonary Lymphangiomatosis
Case Report
Tuberculosis and Respiratory Diseases

Tuberculosis and Respiratory Diseases 2011; 70(5): 428-432.

Published online: 31 May 2011

DOI: https://doi.org/10.4046/trd.2011.70.5.428

A Case of Pulmonary Lymphangiomatosis

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

Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Address for correspondence: Jin Woo Song, M.D. Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 388-1, Pungnap 2-dong, Songpa-gu, Seoul 138-736, Korea. Phone: 82-2-3010-3993, Fax: 82-2-3010-6968, skysong3@hanmail.net

Received 22 January 2011       Accepted 6 April 2011

Copyright © 2011. The Korean Academy of Tuberculosis and Respiratory Diseases. All rights reserved.

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.

Keywords: Lymphangiomatosis, pulmonary, Chylothorax, Lung diseases, Interstitial

Figures and Tables

Figure 1
Chest X-ray shows peripheral and axial interstitial thickening in both lungs with left pleural effusion.
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Figure 2
(A, B) Chest CT shows peripheral and axial interstitial thickening in both lungs (1st admission). (C, D) Chest CT shows newly appeared large amount of pleural effusion and passive atelectasis in left lung. Peripheral and axial interstitial thickening in both lungs shows no significant interval changes since 1st admission (2nd admission).
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Figure 3
Surgical lung biopsy shows proliferation of complex anastomosing lymphatic channels in the interstitium (H&E stain, ×40).
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Figure 4
Lymphangiogram shows tortuous lymphatic channels and lymphatic leakage (arrow) idenitified at the medial aspect of the descending thoracic aorta.
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Figure 5
Chest CT after lymphangiography shows multifocal areas of residual contrast material within abnormal lymphatic spaces in bilateral lower mediastinum and left lower lobe. CT: computed tomography.
trd-70-428-g005

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