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

A Case of Pulmonary Lymphangiomatosis

• Jang EK
• Kim CR
• Choi CM
• Oh YM
• Shim TS
• Lee SD
• Kim WS
• Kim DS
• Song JW

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

Figure

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  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.

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