Magnetic Resonance Lymphangiography and Lymphatic Embolization in the Treatment of Pulmonary Complication of Lymphatic Malformation

Abstract

Lymphatic malformations (LMs; especially those involving the central conducting lymphatic channels) are characterized by dysplastic and incompetent lymphatic channels in multiple tissues and organs. The major cause of mortality and morbidity in patients with thoracic LM is deterioration of pulmonary function due to chronic chylous effusions and progressive interstitial lung disease. The etiology of these pulmonary processes is unknown, although lymphatic involvement is certain. Understanding of the changes in the lymphatic anatomy in patients with LM has been hindered by difficulty of imaging of the lymphatic system. Recently developed dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) allows dynamic MR imaging of the lymphatic system by injecting gadolinium contrast agent in the groin lymph nodes. Using this technique, pathological lymphatic flow from the central lymphatic system and/or retroperitoneal and mediastinal masses into lung parenchyma ("pulmonary lymphatic perfusion syndrome") has been demonstrated in patients with LM. This abnormal lymphatic perfusion overflows pulmonary parenchyma and results in deterioration of pulmonary function due to interstitial process and/or compression effect of chylous effusions. Percutaneous thoracic duct embolization or lymphatic interstitial embolization of the lymphatic masses results in cessation of the pulmonary lymphatic overflow and significant improvement in pulmonary symptoms in these patients.

Keywords: embolization; interventional radiology; lymphangiography; lymphatic malformations; pulmonary function.

Fig. 1

DCRML of the patient with KL and progressive deterioration of pulmonary function. ( a ) Selected image of the dynamic part of the DCMRL demonstrated dilated and tortuous TD (black arrow) and abnormal pulmonary lymphatic flow that originates from the upper part of the TD (black arrowheads) and from the left retroperitoneal mass (white arrow). ( b ) Delayed, high-resolution part of the DCRML, demonstrating dilated and tortuous TD (black arrow) and lymphatic perfusion of the lungs and mediastinum.

Fig. 2

Heavy T2W imaging of the chest of the patient with GLA and vertebral compression fracture demonstrating lymphatic masses in the vertebrae (white arrows) and small amount of pleural effusion (white arrowhead).

Fig. 3

Heavy T2W imaging of the chest of the patient with GLA and progressive deterioration of pulmonary function, demonstrating dilated TD duct (white arrow) and increased T2 signal of the lung interstitium.

Fig. 4

DCRML imaging of the patient with GLA, and progressive deterioration of pulmonary function and hemoptysis demonstrated dilated TD (white arrow) and abnormal pulmonary lymphatic perfusion that originates in the distal TD toward lung parenchyma (white arrowheads).

Fig. 5

DCRML imaging of the patient with GLA and bilateral pleural effusion demonstrates normal size TD (white arrow) and abnormal pulmonary lymphatic perfusion that originates in the left retroperitoneum and extends into the mediastinum and left pleural cavity (black arrowheads).

Fig. 6

Fluoroscopic image of the injection of the contrast into TD through microcatheter (white arrow) of the patient with KL, and progressive deterioration of pulmonary function and left pleural effusion. ( a ) Image demonstrates abnormal pulmonary lymphatic flow from the TD into the lung parenchyma (white arrowheads). ( b ) Fluoroscopic image of the same patient shows endovascular coils (white arrow) and glue cast (white arrowheads) in the pulmonary lymphatics.

Fig. 7

( a ) Fluoroscopic image of the patient with KL and progressive deterioration of pulmonary function demonstrates opacification of the retroperitoneal lymphatic masses (white arrowheads) opacified through 22-G needle (white arrow). ( b ) Glue cast in the retroperitoneal lymphatic mass (white arrowheads) immediately after glue injection.