Massive Primary Chylopericardium: By Catherine
A. Musemeche,
Fernando A. Riveron, Carl L. Backer, Vincent Chicago,
6 A large pericardial effusion was discovered in an asymptomatic 12-year-old boy admitted for an elective orthopedic procedure. On physical examination, heart rate was 96 and blood pressure was 130/70 without paradox. The neck veins were not distended, but heart tones were distant. Chest roentgenogram (CXR) showed an enlarged cardiac silhouette. Echocardiogram showed a massive pericardial effusion compressing the right atrium, with depressed ventricular contractility. Pericardiocentesis yielded 466 mL of chylous fluid. A percutaneous pericardial drain was placed and drained another 466 mL of chyle. Pericardial fluid reaccumulated even though the patient was on a low-fat diet, and 1 week after admission left thoracotomy was performed with partial pericardiectomy and pericardial window. There was 1 L of chyle in the pericardial sac; frozen section of the pericardium showed lymphangiectasia. Chest tube drainage diminished rapidly and the patient was discharged. Follow-up CXR at 1 week showed fluid in both pleural spaces requiring bilateral tube thoracostomies again draining chyle. Even with total parenteral nutrition (TPN). 666 mL/d of chyle drained from the pleural tubes. Right thoracotomy with ligation of the thoracic duct was performed after 1 week of TPN. Pleural drainage abruptly dropped, and there has been no reaccumulation in either the pleural spaces or pericardium at 6-month followup. This case dramatically supports early thoracic duct ligation and partial pericardiectomy as the treatment of choice for primary massive chylopericardium. o 1990 by W. B. Saunders Company. INDEX WORDS: thoracic duct.
Chylopericardium;
pericardial
effusion;
T
HE ACCUMULATION of chyle in the pericardial sac can occur as a primary event or secondary to operative procedures and malignancy. Since the first report of primary chylopericardium in 1954 by Groves and Effler’ there have been only 32 case reports. The presentation may be subtle, as in this patient, or dramatic with cardiac tamponade and respiratory distress. In this report a case of primary chylopericardium in a 12-year-old boy is presented in which two surgical
From the Division Cardiovascular-Thoracic Surgery, Children’s Memorial Hospital, and the Department of Surgery, Northwestern University Medical School, Chicago, IL. Presented at the 38th Annual Meeting of the Surgical Section of the American Academy of Pediatrics. Chicago, Illinois. October 21-23.1989. Address reprint requests to Carl L. Backer, MD, Division of Cardiovascular-Thoracic Surgery, Box 22, Children’s Memorial Hospital, 2300 Children’s Plaza, Chicago, IL 60614. Q 1990 by W.B. Satmders Company. 0022-3468/90/2508-0002$03.00/0
040
A Case Report R. Zales, and Farouk S. ldriss
Illinois
treatments were used with quite different results. The diagnostic workup and surgical approaches for chylopericardium are discussed. CASE
REPORT
A pericardial effusion was discovered in a 12-year-old white boy during a screening ultrasound of the kidneys. The patient had undergone uneventful left femoral epiphysiodesis for leg length discrepancy. No jugular or subclavian vein cannulizations had been performed. There was no history of heart disease or other medical problems. Physical examination showed a well-developed white boy with a casted left leg. The heart rate was 96 and the blood pressure was 130/70 without paradox. The neck veins were not distended and the chest was clear to auscultation. The heart sounds were distant and there were no murmurs or clicks. The chest roentgenogram showed an enlarged cardiac silhouette (Fig 1). There were no previous chest roentgenograms available for comparison. The blood counts were: hemoglobin 12.9 g%; white blood cell count 6,20O/mm” with 64% neutrophils, 21% lymphocytes, 7% monocytes, and 6% bands. An echocardiogram was performed, which showed a massive pericardial effusion compressing the right atrium. Ventricular contractility was depressed. A pericardiocentesis yielded 435 mL of chylous fluid. The cell count included 6,060 red blood cells and 3,600 white blood cells (100% monocytes). Triglyceride level was 461 mg% and the protein was 5.3 g%. Gram stain and cultures were negative. Follow-up echocardiogram 1 day after pericardiocentesis showed a reaccumulation of pericardial fluid. A pericardial drain was placed percutaneously and an additional 360 mL of creamy fluid was withdrawn. The pericardial fluid continued to reaccumulate. One week after admission the patient underwent left thoracotomy with partial pericardiectomy and pericardial window. At that time the pericardial sac was found to be tilled with nearly 1 L of chylous fluid. Chyle was draining from the lymphatics adjacent to the left pulmonary artery. This area was cauterized and oversewn. A frozen section of the pericardium in this area showed lymphangectasia. Postoperatively, the patient was placed on a no-fat diet. The chest tube drainage decreased to zero over a 4-day period. The tubes were removed, and he was discharged on postoperative day 6. One week later, a follow-up chest x-ray showed large bilateral pleural effusions. Bilateral chest tubes were placed draining nearly 500 mL from each pleural space. The patient was placed on parenteral nutrition and made NPG. Left chest tube output continued to average 400 mL/d of chyle. Six days after admission he underwent ligation of the thoracic duct near the diaphragm through a right lateral thoracotomy. Following this operation the left chest tube drainage rapidly decreased and it was removed on postoperative day 7. The patient was discharged on postoperative day 9. Follow-up clinical examination, chest roentgenograms, and echocardiograms have shown no reaccumulation of pericardial fluid at 12 months postoperatively (Fig 2). DISCUSSION
Chylous pericardium is not an infrequent occurrence in association with thoracic operations, chest trauma, or mediastinal tumors. In contrast, there have been Journal of Pediafric Surgery, Vol 25, No 8 (August), 1990: pp 840-842
MASSIVE PRIMARY CHYLOPERICARDIUM
Fig 1. Preoperative cheat roentgenogram of 12-year-old boy with massive primary chylopericardium. Note severely enlarged cardiac silhouette.
very few patients described with primary or idiopathic chylopericardium. It has been reported in patients from the newborn period up to age 62 years. Most investigators now agree that the cause is some abnormality in the lymphatic connections between the thoracic duct and the pericardium. The diagnosis of chylopericardium can be made by careful analysis of aspirated pericardial fluid. The fluid is milky in appearance and microscopically fat droplets are seen. The protein, cholesterol, and triglyceride content are similar to the patient’s blood serum or may be elevated and depend on dietary intake. An echocardiogram aids in pericardiocentesis by localizing the fluid collection around the heart. It can demonstrate right atria1 compression, and further delineate myocardial function.’ Other diagnostic modalities include lymphangiography, which has been used by Gallant et al3 to demonstrate the specific morphology of lymphatic lesions in chylopericardium and their extent throughout the body. Lymphangiectasias have been demonstrated in up to 50% of patients who underwent this study. Daniel and Bressie4 described using ‘311-triolein radionuclide scanning for noninvasive diagnosis of chylopericardium. This entails feeding the patient a radionuclide oil, triolein, and then scanning the pericardium for radioactivity. If there is pooling of the nuclide in the mediastinum the thoracic duct could be obstructed; however, no anatomical detail is obtained.
841
The success of thoracic duct ligation and partial pericardiectomy has been substantiated throughout the literature.3*5 In a review in 1975, Dunn’ found that of 16 patients who had undergone this procedure none had a reaccumulation of pericardial fluid. Other therapeutic options have been exercised with variable success. The use of a pericardial window alone has been advocated by other authors. Savran et al6 and others advocate subxiphoid pericardial biopsy with closed tube pericardial drainage and have reported successful outcomes with this approach. Chylopericardium that occurs following cardiac surgery has been treated by pericardial window and oral fat restriction or by exploratory thoracotomy with direct ligation of the injured lymphatic.7 A conventional approach to chylothorax that has not been discussed in the management of chylopericardium is the use of medium-chain triglycerides or total parenteral nutrition.’ Kosloske et al9 in 1967 reported the management of a chylous effusion in the pleural space with repeated thoracentesis and medium-chain triglycerides. Medium-chain fatty acids do not undergo resynthesis to triglycerides and do not contribute to lymph synthesis. They pass directly into portal venous blood and are transported as free-fatty acids. Therefore, the amount of chylous effusion should be diminished if the patient is restricted to medium-chain triglyceride fat intake or has no oral intake. We found no reports of a similar management approach for chylopericardium in the literature, although it is wellknown for patients with chylothorax.
Fig 2. Chest roentgenogram following thoratic duct ligation. Cardiac silhouette has returned to normal size and there are no pleural effusions.
842
MUSEMECHE ET AL
In the case of this patient, a significant amount of chest tube drainage persisted after partial pericardiectomy despite eliminating his dietary intake of fat and placing him on total parenteral nutrition. It was only after thoracic duct ligation that chyle stopped accumulating in the pericardium.
In summary, the diagnostic evaluation of a primary pericardial effusion should include an echocardiogram with aspiration and analysis of the pericardial fluid. For massive primary chylopericardium low thoracic duct ligation and partial pericardiectomy is the treatment of choice.
REFERENCES 1. Groves LK, EfTer DB: Primary ChyIopericardium. N Engl J Med 250:520-523,1954 2. Ross P, Joseph S, Walker D: A case of isolated primary chylopericardium. Br Heart J 4 1:508-5 1 I ( 1979 3. Gallant TE, Hunziker RJ, Gibson TC: Primary chylopericardium. The role of lymphangiography. AJR 129:1043-1045, 1977 4. Daniel TG, Bressie JL: Primary chylopericardium. J Thorac Cardiovasc Surg 57:408-411, 1969 5. Dunn RP: Primary chylopericardium. A review of the literature and an illustrated case. Am Heart J 89:369-377, 1975 6. Savran SV, Ratshin RA, Shirley JH, et al: Idiopathic chyloperi-
cardium. ‘3’I-trioIein scan for noninvasive diagnosis. Ann Intern Med 82:663-665,1975 7. Bakay C, Wijers TS: Treatment of cardiac tamponade due to isolated chylopericardium following open heart surgery. J Cardiovast Surg 25:249-25 1, 1984 8. Jalili F: Medium-chain triglycerides and total parenteral nutrition in the management of infants with congenital chylothorax. South Med J 80:1290-1293,1987 9. Kosloske AM, Martin LW, Shubert WK: Management of chylothorax in children by thoracentesis and medium-chain triglyceride feedings. J Pediatr Surg 9:365-371, 1974
A. Musemeche,
Fernando A. Riveron, Carl L. Backer, Vincent Chicago,
6 A large pericardial effusion was discovered in an asymptomatic 12-year-old boy admitted for an elective orthopedic procedure. On physical examination, heart rate was 96 and blood pressure was 130/70 without paradox. The neck veins were not distended, but heart tones were distant. Chest roentgenogram (CXR) showed an enlarged cardiac silhouette. Echocardiogram showed a massive pericardial effusion compressing the right atrium, with depressed ventricular contractility. Pericardiocentesis yielded 466 mL of chylous fluid. A percutaneous pericardial drain was placed and drained another 466 mL of chyle. Pericardial fluid reaccumulated even though the patient was on a low-fat diet, and 1 week after admission left thoracotomy was performed with partial pericardiectomy and pericardial window. There was 1 L of chyle in the pericardial sac; frozen section of the pericardium showed lymphangiectasia. Chest tube drainage diminished rapidly and the patient was discharged. Follow-up CXR at 1 week showed fluid in both pleural spaces requiring bilateral tube thoracostomies again draining chyle. Even with total parenteral nutrition (TPN). 666 mL/d of chyle drained from the pleural tubes. Right thoracotomy with ligation of the thoracic duct was performed after 1 week of TPN. Pleural drainage abruptly dropped, and there has been no reaccumulation in either the pleural spaces or pericardium at 6-month followup. This case dramatically supports early thoracic duct ligation and partial pericardiectomy as the treatment of choice for primary massive chylopericardium. o 1990 by W. B. Saunders Company. INDEX WORDS: thoracic duct.
Chylopericardium;
pericardial
effusion;
T
HE ACCUMULATION of chyle in the pericardial sac can occur as a primary event or secondary to operative procedures and malignancy. Since the first report of primary chylopericardium in 1954 by Groves and Effler’ there have been only 32 case reports. The presentation may be subtle, as in this patient, or dramatic with cardiac tamponade and respiratory distress. In this report a case of primary chylopericardium in a 12-year-old boy is presented in which two surgical
From the Division Cardiovascular-Thoracic Surgery, Children’s Memorial Hospital, and the Department of Surgery, Northwestern University Medical School, Chicago, IL. Presented at the 38th Annual Meeting of the Surgical Section of the American Academy of Pediatrics. Chicago, Illinois. October 21-23.1989. Address reprint requests to Carl L. Backer, MD, Division of Cardiovascular-Thoracic Surgery, Box 22, Children’s Memorial Hospital, 2300 Children’s Plaza, Chicago, IL 60614. Q 1990 by W.B. Satmders Company. 0022-3468/90/2508-0002$03.00/0
040
A Case Report R. Zales, and Farouk S. ldriss
Illinois
treatments were used with quite different results. The diagnostic workup and surgical approaches for chylopericardium are discussed. CASE
REPORT
A pericardial effusion was discovered in a 12-year-old white boy during a screening ultrasound of the kidneys. The patient had undergone uneventful left femoral epiphysiodesis for leg length discrepancy. No jugular or subclavian vein cannulizations had been performed. There was no history of heart disease or other medical problems. Physical examination showed a well-developed white boy with a casted left leg. The heart rate was 96 and the blood pressure was 130/70 without paradox. The neck veins were not distended and the chest was clear to auscultation. The heart sounds were distant and there were no murmurs or clicks. The chest roentgenogram showed an enlarged cardiac silhouette (Fig 1). There were no previous chest roentgenograms available for comparison. The blood counts were: hemoglobin 12.9 g%; white blood cell count 6,20O/mm” with 64% neutrophils, 21% lymphocytes, 7% monocytes, and 6% bands. An echocardiogram was performed, which showed a massive pericardial effusion compressing the right atrium. Ventricular contractility was depressed. A pericardiocentesis yielded 435 mL of chylous fluid. The cell count included 6,060 red blood cells and 3,600 white blood cells (100% monocytes). Triglyceride level was 461 mg% and the protein was 5.3 g%. Gram stain and cultures were negative. Follow-up echocardiogram 1 day after pericardiocentesis showed a reaccumulation of pericardial fluid. A pericardial drain was placed percutaneously and an additional 360 mL of creamy fluid was withdrawn. The pericardial fluid continued to reaccumulate. One week after admission the patient underwent left thoracotomy with partial pericardiectomy and pericardial window. At that time the pericardial sac was found to be tilled with nearly 1 L of chylous fluid. Chyle was draining from the lymphatics adjacent to the left pulmonary artery. This area was cauterized and oversewn. A frozen section of the pericardium in this area showed lymphangectasia. Postoperatively, the patient was placed on a no-fat diet. The chest tube drainage decreased to zero over a 4-day period. The tubes were removed, and he was discharged on postoperative day 6. One week later, a follow-up chest x-ray showed large bilateral pleural effusions. Bilateral chest tubes were placed draining nearly 500 mL from each pleural space. The patient was placed on parenteral nutrition and made NPG. Left chest tube output continued to average 400 mL/d of chyle. Six days after admission he underwent ligation of the thoracic duct near the diaphragm through a right lateral thoracotomy. Following this operation the left chest tube drainage rapidly decreased and it was removed on postoperative day 7. The patient was discharged on postoperative day 9. Follow-up clinical examination, chest roentgenograms, and echocardiograms have shown no reaccumulation of pericardial fluid at 12 months postoperatively (Fig 2). DISCUSSION
Chylous pericardium is not an infrequent occurrence in association with thoracic operations, chest trauma, or mediastinal tumors. In contrast, there have been Journal of Pediafric Surgery, Vol 25, No 8 (August), 1990: pp 840-842
MASSIVE PRIMARY CHYLOPERICARDIUM
Fig 1. Preoperative cheat roentgenogram of 12-year-old boy with massive primary chylopericardium. Note severely enlarged cardiac silhouette.
very few patients described with primary or idiopathic chylopericardium. It has been reported in patients from the newborn period up to age 62 years. Most investigators now agree that the cause is some abnormality in the lymphatic connections between the thoracic duct and the pericardium. The diagnosis of chylopericardium can be made by careful analysis of aspirated pericardial fluid. The fluid is milky in appearance and microscopically fat droplets are seen. The protein, cholesterol, and triglyceride content are similar to the patient’s blood serum or may be elevated and depend on dietary intake. An echocardiogram aids in pericardiocentesis by localizing the fluid collection around the heart. It can demonstrate right atria1 compression, and further delineate myocardial function.’ Other diagnostic modalities include lymphangiography, which has been used by Gallant et al3 to demonstrate the specific morphology of lymphatic lesions in chylopericardium and their extent throughout the body. Lymphangiectasias have been demonstrated in up to 50% of patients who underwent this study. Daniel and Bressie4 described using ‘311-triolein radionuclide scanning for noninvasive diagnosis of chylopericardium. This entails feeding the patient a radionuclide oil, triolein, and then scanning the pericardium for radioactivity. If there is pooling of the nuclide in the mediastinum the thoracic duct could be obstructed; however, no anatomical detail is obtained.
841
The success of thoracic duct ligation and partial pericardiectomy has been substantiated throughout the literature.3*5 In a review in 1975, Dunn’ found that of 16 patients who had undergone this procedure none had a reaccumulation of pericardial fluid. Other therapeutic options have been exercised with variable success. The use of a pericardial window alone has been advocated by other authors. Savran et al6 and others advocate subxiphoid pericardial biopsy with closed tube pericardial drainage and have reported successful outcomes with this approach. Chylopericardium that occurs following cardiac surgery has been treated by pericardial window and oral fat restriction or by exploratory thoracotomy with direct ligation of the injured lymphatic.7 A conventional approach to chylothorax that has not been discussed in the management of chylopericardium is the use of medium-chain triglycerides or total parenteral nutrition.’ Kosloske et al9 in 1967 reported the management of a chylous effusion in the pleural space with repeated thoracentesis and medium-chain triglycerides. Medium-chain fatty acids do not undergo resynthesis to triglycerides and do not contribute to lymph synthesis. They pass directly into portal venous blood and are transported as free-fatty acids. Therefore, the amount of chylous effusion should be diminished if the patient is restricted to medium-chain triglyceride fat intake or has no oral intake. We found no reports of a similar management approach for chylopericardium in the literature, although it is wellknown for patients with chylothorax.
Fig 2. Chest roentgenogram following thoratic duct ligation. Cardiac silhouette has returned to normal size and there are no pleural effusions.
842
MUSEMECHE ET AL
In the case of this patient, a significant amount of chest tube drainage persisted after partial pericardiectomy despite eliminating his dietary intake of fat and placing him on total parenteral nutrition. It was only after thoracic duct ligation that chyle stopped accumulating in the pericardium.
In summary, the diagnostic evaluation of a primary pericardial effusion should include an echocardiogram with aspiration and analysis of the pericardial fluid. For massive primary chylopericardium low thoracic duct ligation and partial pericardiectomy is the treatment of choice.
REFERENCES 1. Groves LK, EfTer DB: Primary ChyIopericardium. N Engl J Med 250:520-523,1954 2. Ross P, Joseph S, Walker D: A case of isolated primary chylopericardium. Br Heart J 4 1:508-5 1 I ( 1979 3. Gallant TE, Hunziker RJ, Gibson TC: Primary chylopericardium. The role of lymphangiography. AJR 129:1043-1045, 1977 4. Daniel TG, Bressie JL: Primary chylopericardium. J Thorac Cardiovasc Surg 57:408-411, 1969 5. Dunn RP: Primary chylopericardium. A review of the literature and an illustrated case. Am Heart J 89:369-377, 1975 6. Savran SV, Ratshin RA, Shirley JH, et al: Idiopathic chyloperi-
cardium. ‘3’I-trioIein scan for noninvasive diagnosis. Ann Intern Med 82:663-665,1975 7. Bakay C, Wijers TS: Treatment of cardiac tamponade due to isolated chylopericardium following open heart surgery. J Cardiovast Surg 25:249-25 1, 1984 8. Jalili F: Medium-chain triglycerides and total parenteral nutrition in the management of infants with congenital chylothorax. South Med J 80:1290-1293,1987 9. Kosloske AM, Martin LW, Shubert WK: Management of chylothorax in children by thoracentesis and medium-chain triglyceride feedings. J Pediatr Surg 9:365-371, 1974
