Case Report

A 29-month-old, previously healthy girl visited an external referring hospital because of fever, cough, and sputum production for 2 weeks prior to referral. Cardiomegaly was observed on chest X-ray. Echocardiography revealed a large pericardial effusion. Pericardiocentesis was performed and 200 ml of pericardial effusion was drained daily via percutaneous catheter drainage. The patient was transferred to our hospital because the massive pericardial effusion persisted for >2 weeks.

Her chest X-ray showed cardiomegaly with diffuse interstitial thickening in both lungs at the time of the visit to our hospital. Echocardiography showed a massive pericardial effusion.

Her effusion was serosanguinous and exudative: albumin, 2.8 g/dl (serum albumin, 3.4 g/dl). On analysis of pericardial fluid, the cell profile revealed a lymphocyte-dominant (96%) cell count, negative fluid cultures, and no malignant cells. During her usual diet without any specific restrictions, the triglyceride (100–115 mg/dl) and total cholesterol levels (93–133 mg/dl) were mildly elevated, with cholesterol/triglyceride ratio of 0.9:1.2.

The pericardial effusion resolved spontaneously 5 weeks after the initial detection of the effusion and the patient was discharged. However, massive pericardial effusion recurred repeatedly, 3 months and 9 months after the first episode.

Although the effusion did not have prominent chylous features (triglyceride, 106 mg/dl; total cholesterol, 132 mg/dl in the pericardial effusion and serum triglyceride, 58 mg/dl; total serum cholesterol, 154 mg/dl), lymphoscintigraphy using Technetium-99m antimony trisulfide colloid was performed due to chronic and recurrent pericardial effusion and pulmonary lymphangiectasia, which was suspected based on the findings of a previous CT. As lymphoscintigraphy revealed mild radioactivity around the heart, contrast enhanced magnetic resonance lymphangiography was performed by injecting contrast medium (Dotarem/normal saline 1:1 mixture 10 ml) through right inguinal lymph nodes.

The magnetic resonance lymphangiography showed a tortuously dilated thoracic duct in the mediastinum with suspected chylous reflux to the pericardial space at the level of the aortic arch (Fig 1a). We presumed that the pericardial effusion was caused by chylous reflux. On invasive, percutaneous fluoroscopic contrast enhanced lymphangiography using the retrograde catheter through the femoral vein, superior vena cava, innominate vein, lymphovenous junction, and abdominal thoracic duct, abnormal communication of prominent lymphatic collaterals to the pericardial cavity was more prominently visualised (Fig 1b), because this catheter unexpectedly blocked lymphatic drainage into the innominate vein at the lymphovenous junction, which led to the abrupt aggravation of pericardial effusion. From this finding, we could confirmed that chylous reflux from thoracic duct caused massive chronic pericardial effusion. Thoracic duct embolisation was performed using multiple microcoils and 1:2 glue/lipiodol mixture at the T11 level (Fig 1c). The patient could have been discharged after resolution of pericardial effusion. However, a large amount of pericardial effusion recurred after 5 months. The second magnetic resonance lymphangiography using undiluted Dotarem solution 10 ml through bilateral inguinal lymph nodes showed enhancement of the periaortic structures, suggestive of persistent lymphatic fluid inflow to the pericardium through the remnant upper part of the thoracic duct (Fig 2a). A second lymphatic embolisation was performed to completely occlude the remaining upper part of the thoracic duct and abnormal lymphatic vessels alleged to distribute over not only the pericardium but also bilateral lung hila. Because the conventional antegrade access to the thoracic duct through cisterna chyli was not possible due to prior thoracic duct embolisation, two microcatheters were retrogradely inserted from the common femoral venous access via the junction between the left subclavian vein and thoracic duct at the venous angle. First, the outflow of the thoracic duct was occluded using multiple microcoils to prevent pulmonary embolism of the embolic agent. Then, the 1:5 volume mixture of n-BCA Histoacryl-tissue adhesive (B. Braun Melsungen AG W. Germany) and Lipiodol (Lipiodol Ultrafluide, Laboratoire Guerbet, Aulnay-Sous-Bois, France) was injected through the other microcatheter to occlude abnormal lymphatic vessels in the mediastinum as well as the remaining part of the thoracic duct (Fig 2b). After the second embolisation, the patient has been doing well without recurrence of pericardial effusion for 12 months until now.

Figure 1. (a) Dynamic T1-weighted magnetic resonance lymphangiography obtained after intranodal injection of gadolinium contrast medium to the inguinal lymph node shows the tortuously dilated thoracic duct connected to the pericardium through an anomalous mediastinal lymphatic vessel (arrow). (b) Direct catheter lymphangiography with the tip in the central conducting duct at the abdomen confirmed a reflux from the thoracic duct into dysplastic lymphatic channels in the mediastinum and left pericardial space (arrow). Note the pigtail catheter in the pericardial space. (c) Chest radiograph obtained after thoracic duct embolisation shows multiple microcoils (arrow) and glue cast at the lower thoracic spine level.

Figure 2. (a) The major collateral vessel around the occluded thoracic duct was observed on magnetic resonance lymphangiography. (b) Thoracic duct embolisation was performed at the cervical–thoracic level with 1:3 glue after coil embolisation (arrow) to prevent venous reflux of glue.