Ann Thorac Surg 2009;87:1595-1597. doi:10.1016/j.athoracsur.2008.09.054
© 2009 The Society of Thoracic Surgeons
Case Reports
Spontaneous Chylopericardium: Delineation of the Underlying Anatomic Pathology by CT Lymphangiography
Maxim Itkin, MDa,*,
Nadar M. Swe, MDb,
Scott E. Shapiro, MDb,
Joseph B. Shrager, MDc
a Hospital of University of Pennsylvania, Philadelphia, Pennsylvania
b Abington Medical Specialists, Abington, Pennsylvania
c Stanford University School of Medicine, Stanford, California
Accepted for publication September 19, 2008.
* Address correspondence to Dr Itkin, University of Pennsylvania, 109 Penarth Rd, Bala Cynwyd, PA 19004 (Email: itkinmax{at}uphs.upenn.edu).
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Abstract
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Primary isolated chylopericardium is a rare condition with little known cause. This is the case of a 22-year-old woman in whom idiopathic chylopericardium developed. A lymphangiogram followed by a computed tomographic scan demonstrated occlusion of the thoracic duct and multiple lymphatic collaterals abutting the pericardial sac. Thoracic duct ligation resulted in the complete cure of the patient's condition. We theorized that the development of the pathologic lymphatic ducts in close proximity to the pericardium resulted in the development of the slowly accumulating chylopericardium.
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Introduction
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Primary or idiopathic isolated chylopericardium is accumulation of chylous fluid in the pericardial space in the absence of any known precipitating factors [1], such as a history of major systemic disease, mediastinal tumors, trauma, body weight loss, mediastinal radiation, lymphoma, thoracic surgery, filariasis, subclavian vein thrombosis, or congenital lymphangiectasia [2]. We present a case of primary isolated chylopericardium discovered incidentally during an abdominal ultrasonogram. Occlusion of the upper part of the thoracic duct with reflux of the lymphatic fluid into the peribronchial lymphatics was demonstrated by lymphangiogram and computed tomography (CT), and the problem was successfully treated by surgical thoracic duct ligation.
A 21-year-old woman presented with a 3-month history of intermittent epigastric and right upper quadrant pain unrelated to meals and two syncopal episodes. There was no significant past medical or surgical history. The ultrasound examination of the abdomen demonstrated a large pericardial effusion. A subsequent chest roentgenogram showed enlargement of the cardiac silhouette. The patient was admitted to the hospital for further evaluation.
On admission, a transthoracic echocardiogram demonstrated a large pericardial effusion, with right ventricular collapse consistent with tamponade physiology (Fig 1).
Therapeutic pericardiocentesis was performed and 1 L of milky fluid was removed, and a drain was left it place. A fluid analysis demonstrated triglycerides of 675, a white blood cell count of 598/uL (72% lymphocytes), total proteins of 4.5 g/dL, glucose of 90 mg/dL. Cytology stains and cultures were all unremarkable. Blood tests for rheumatologic, endocrinologic, and autoimmune disorders were conducted, as well as tests for vasculitides, infectious processes of bacterial, fungal, myobacterial, and viral sources including HIV, Epstein-Barr virus, syphilis, and Lyme's disease were negative.
A repeat echocardiogram on the second and third day post-drainage showed no re-accumulation of the fluid. The drainage tube was removed after a total of 300 cc fluid drained for 3 days. During the second week after discharge, the patient had a near-syncopal episode, and on the third week she felt sharp, stabbing substernal chest pain.
Echocardiogram demonstrated the pericardial effusion. Repeat pericardiocentesis yielded 800 cc of chylous fluid. Lymphoscintigraphy established no definite activity in the pericardium. The patient transferred for a thoracic surgical evaluation to another institution. Ultrasound of the left subclavian vein was performed to exclude thrombosis as a cause for poor thoracic duct drainage, and this showed a patent vein.
Bilateral pedal lymphangiogram was performed, which revealed occlusion of the upper part of the thoracic duct with fine branches from the duct overlaying the cardiac silhouette (Fig 2). To better visualize the lymphatic vessels, a nonenhanced CT chest scan was performed. The peribronchial lymphatic vessels abutting the pericardium were highlighted and correlated with the lymphatic vessels overlaying the heart on fluoroscopy images (Fig 3). Attempted percutaneous embolization of the thoracic duct failed, due to minimal opacification of the cysternae chyle.
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Fig 2. Fluoroscopic spot image of the lymphangiogram revealing extensive network of the lymphatic vessels overlying the heart shadow.
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Fig 3. (A, B) Computed tomographic image of the chest demonstrating pathologically enlarged lymphatic vessels abutting the pericardial sac (white arrows).
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The diagnosis of the complete occlusion of the upper thoracic duct with reflux of chyle into the peribronchial lymphatic ducts and subsequently into the pericardial space was established. It was recommended to the patient that she undergo surgical thoracic duct ligation and she agreed. Through a low, right mini-thoracotomy, ligation of the thoracic duct, together with all the adjacent soft tissue between the esophagus anteriorly, the azygos posteriorly, and the aorta at the deep margin was performed. A pericardial window was not performed, as the sac was completely flaccid without any evidence of effusion.
The patient recovered uneventfully and follow-up serial chest roentgenograms and echocardiograms up to 6 months demonstrated no recurrence of the pericardial effusion.
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Comment
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We present a case of primary chylopericardium successfully treated by thoracic duct ligation. The lymphangiogram demonstrated obstruction of the upper part of the thoracic duct with reflux of the lymphatic fluid into the peribronchial lymphatics. A subsequent CT scan showed the close relationship of the pathologic lymphatic ducts and the pericardium that seems to have been responsible for the development of chylous pericardial effusion.
Primary chylopericardium is rare and was first reported by Groves and Effler [3] in 1954. The pathophysiology of the disorder was first proposed by Dunn [4] and is believed to include obstruction of the thoracic duct, failure to establish collateral drainage to the right thoracic duct, and reflux of chylous lymph through normal lymphatic channels that drain the pericardium and heart. Abnormally elevated pressure in the thoracic duct, as in lymphangiectasia or damaged thoracic duct valves, or both, has been proposed as an important element [5].
In our case and most other published cases of primary chylopericardium, the accumulation of the chyle is slow, developing over weeks or even months. This time course supports the theory that accumulation of chyle in the pericardium is due to slow transudation of fluid from small, pathologic lymphatic channels rather than through direct communication with a main thoracic duct. Given that the known physiological flow in the thoracic duct is between 2 and 6 L a day, one would expect that a direct communication between the thoracic duct and pericardial cavity would result in much more rapid and symptomatic development of pericardial effusion.
Attempts to demonstrate the anatomical connections between thoracic duct and the pericardial cavity have included lymphangiogram and nuclear scintigraphy [6, 7]. Several authors have reported the observation of the communication between the lymphatic ducts and the pericardial sac using lymphangiogram [6]. However, it is likely that the small amount of the contrast that is spilled into the pericardial space would disperse throughout the entire space and would thus be fairly difficult to visualize using conventional fluoroscopy. In addition, the two-dimensional nature of fluoroscopy makes it difficult to determine the exact location of the contrast, so the observed contrast could be located in the overlapping structures.
The first attempt to better define the connection between the thoracic duct and the pericardium was done by Akamatsu and colleagues [5] using a combination of lymphangiography and CT, as we report here. The authors reported that they observed communications between the thoracic duct and the pericardium during their CT lymphangiogram. However, the time and spatial resolution of the CT technology at that time did not allow direct visualization of the lymphatic ducts along the pericardium due to artifact created by heart motion. The better time and spatial resolution of current CT technology practically eliminates heart motion artifact and allows the detection of lymphatic vessels less than 1 mm diameter. These technologic achievements provide us with a first-time opportunity to observe the pathological lymph vessels and their relationship to the heart and pericardial cavity.
Clearly, the treatment of choice for this condition is a procedure to halt forward (cephalad) flow through the thoracic duct. It was reported initially by our institution that this can often be achieved by percutaneously accessing the cisterna chyli and subsequently embolizing the duct [8]. In our hands this has been successful in approximately 70% of patients, and thus it was the first mode of invasive therapy tried in this patient. Unfortunately, we were unable to cannulate the duct in this patient. Therefore, she underwent surgical thoracic duct ligation. Although this can be achieved thoracoscopically, the patient elected to undergo a mini-thoracotomy, given the extended illness she had suffered and the odds we quoted her for success with thoracoscopic versus "open" approaches.
In summary, we present a rare case of spontaneous chylopericardium in which we used CT lymphangiography for the first time to definitively demonstrate the anatomic pathology underlying this condition. Surgical ligation of the thoracic duct was curative.
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References
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Abstract
Introduction
