Chylopericardium

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Chylopericardium is defined as a pericardial effusion which consists of chyle. Chyle comes from the lacteals (the lymphatics draining the small intestine) via the thoracic duct. [1] [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12]. [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] [23]

Pathophysiology

Chylopericardium is most often secondary to injury to the thoracic duct. It may also be primary or idiopathic. As result of the leakage of chyle, there can be nutritional and immunologic sequala. In addition, cardiac complications can occur such cardiac tamponade. Because chyle may be a pericardial irritant, constrictive pericarditis may eventually develop.

Causes

Chylopericardium refers to a communication between the pericardial sac and the thoracic duct, as a result of trauma, congenital anomalies, or as a complication of open-heart surgery, mediastinal lymphangiomas, lymphangiomatous hamartomas, lymphangiectasis, and obstruction or anomalies of the thoracic duct.

Differentiation of Chylopericardium from other Disorders

Natural History, Complications, Prognosis

Infection, tamponade, or constriction may aggravate the prognosis. The pericardial fluid is sterile, odourless, and opalescent with a milky white appearance and the microscopic finding of fat droplets. The chylous nature of the fluid is confirmed by its alkaline reaction, specific gravity between 1010 and 1021, Sudan III stain for fat, the high concentrations of triglycerides (5 to 50 g/L) and protein (22 to 60 g/L).

Diagnosis

Pericardial drainage yields a fluid that is usually milky and opaque in patients with chylopericardium.

Enhanced CT, alone or combined with lymphography, can identify not only the location of the thoracic duct but also its lymphatic connection to the pericardium.

In chylopericardium the cholesterol content can be quite high. However, chylopericardium should not be confused with cholesterol pericarditis. In cholesterol pericarditis the fluid contains cholesterol crystals, foam cells, macrophages and giant cells. The fluid in cholesterol pericarditis is clear, and classically is said to have a glittering "gold paint" appearance.

Treatment

Treatment depends on the aetiology and the amount of chylous accumulation. Chylopericardium after thoracic or cardiac operation without signs of tamponade is preferably treated by pericardiocentesis and diet (medium chain triglycerides). If further production of chylous effusion continues, surgical treatment is mandatory (level of evidence B, class I).

When conservative treatment and pericardiocentesis fail, pericardio-peritoneal shunting by a pericardial window is a reasonable option. Alternatively, when the course of the thoracic duct was precisely identified, its ligation and resection just above the diaphragm is the most effective treatment. In secondary chylopericardium the underlying disease (e.g., mediastinal tumour) should be treated.


References

  1. Spodick, DH. The Pericardium. A comprehensive textbook, Marcel Dekker, New York 1997. p.84.
  2. Valentine, VG, Raffin, TA. The management of chylothorax. Chest 1992; 102: 586.
  3. Wurnig, PN, Hollaus, PN, Ohtsuka, T, et al. Thoracoscopic direct clipping of the thoracic duct for chylopericardium and chylothorax. Ann Thorac Surg 2000; 70:1662.
  4. Brawley, RK, Vasko, JS, Morrow, AG. Cholesterol pericarditis. Considerations of its pathogenesis and treatment. Am J Med 1966; 41: 235.
  5. Mogulkoc, N, Onal, B, Okyay, N, et al. Chylothorax, chylopericardium and lymphoedema--the presenting features of signet-ring cell carcinoma. Eur Respir J 1999; 13: 1489.
  6. Andrade Santiago, J, Robles, L, Casimiro, C, et al. Chylopericardium of neoplastic aetiology. Ann Oncol 1998; 9: 1339.
  7. Arendt, T, Bastian, A, Lins, M, et al. Chylous cardiac tamponade in acute pancreatitis. Dig Dis Sci 1996; 41: 1972.
  8. Tchervenkov, CI, Dobell, ARC. Chylopericardium following cardiac surgery. Can J Surg 1985; 28: 542.
  9. Papaioannou, Y, Vomvoyannis, Andritsakis, G. Combined chylopericardium and chylothorax after total correction of Fallot's tetralogy. Thorac Cardiovasc Surg 1984; 32:115.
  10. Lee, Y, Lee, WK, Doromal, N, et al. Cardiac tamponade resulting from massive chylopericardium after an aorta-coronary bypass operation. J Thorac Cardiovasc Surg 1987; 94:449.
  11. Feteih, W, Rao, S, Whisennand, HH, et al. Chylopericardium: New complication of Blalock-Taussig anastomosis. J Thorac Cardiovasc Surg 1983; 85:791.
  12. Mailander, L, Van Meter, C, Ventura, H, et al. Chylopericardium after orthotopic heart transplantation. J Heart Lung Transplant 1992; 11:587
  13. Nakamura, S, Ohwada, S, Morishita, Y. Isolated chylopericardium following radical esophagectomy: Report of a case. Surg Today 1996; 26: 629.
  14. Yüksel, M, Yildizeli, B, Zonüzi, F, Batirel, HF. Isolated primary chylopericardium. Eur J Cardiothorac Surg 1997; 12: 319.
  15. Svedjeholm, R, Jansson, K, Olin, C. Primary idiopathic chylopericardium-a case report and review of the literature. Eur J Cardiothorac Surg 1997; 11:387.
  16. Mewis, C, Kühlkamp, V, Sokiranski, R, Karsch, KR. Primary chylopericardium due to partial aplasia of the thoracic duct. Eur Heart J 1997; 18:880.
  17. Chan, RMT, Dodek, A. Chylopericardium in a drug addict. Arch Intern Med 1984; 144: 1857.
  18. Bewick, DJ, Johnstone, DE, Landrigan, PL. Primary chylopericardium associated with allergic alveolitis. Can Med Assoc J 1984; 130:1577.
  19. Akamatsu, H, Amano, J, Sakamoto, T, Suzuki, A. Primary chylopericardium. Ann Thorac Surg 1994; 58: 262.
  20. Hamanaka, D, Suzuki, T, Kawanishi, K, et al. Two cases of primary isolated chylopericardium diagnosed by oral administration of 131I-triolein. Radiat Med 1983; 1: 65.
  21. Jensen, GL, Mascioli, EA, Meyer, LP, et al. Dietary modification of chyle composition in chylothorax. Gastroenterology 1989; 97:761.
  22. Nguyen, DM, Shum-Tim, D, Dobell, AR, Tchervenkov, CI. The management of chylothorax/chylopericardium following pediatric cardiac surgery: A 10-year experience. J Card Surg 1995; 10: 302.
  23. Chan, BB, Murphy, MC, Rodgers, BM. Management of chylopericardium. J Pediatr Surg 1990; 25: 1185.



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