Primary Chylopericardium

In a 23-year-old man with massive chylous pericardial effusion, oral administration of Sudan III and radioactive 131iodine-labelled triolein revealed an abnormal communication between the lymphatic system and the pericardial sac. Despite partial pericardiectomy and resection of the thoracic duct, the fluid reaccumulated, and a postoperative lymphangiogram showed an obstructed left thoracic duct. Resection of this left thoracic duct resulted in full recovery. This is the first reported case of this disease with a duplicate thoracic duct. Careful attention must be paid to the possibility of this variation before and during surgery. In a 23-year-old man with massive chylous pericardial effusion, oral administration of Sudan III and radioactive 131iodine-labelled triolein revealed an abnormal communication between the lymphatic system and the pericardial sac. Despite partial pericardiectomy and resection of the thoracic duct, the fluid reaccumulated, and a postoperative lymphangiogram showed an obstructed left thoracic duct. Resection of this left thoracic duct resulted in full recovery. This is the first reported case of this disease with a duplicate thoracic duct. Careful attention must be paid to the possibility of this variation before and during surgery. Primary isolated chylopericardium unrelated to trauma is rare, and we could find only 31 case reports since 1954.1Groves LK Effler DB Primary chylopericardium.N Engl J Med. 1954; 250: 520-523Crossref PubMed Scopus (60) Google Scholar, 2Lee CY Di Loreto PC Kim S Isolated primary chylopericardium in pregnancy.Obstet Gynecol. 1974; 43: 586-591PubMed Google Scholar, 3Patney NL Primary massive chylopericardium.J Indian Med Assoc. 1974; 62: 318-321PubMed Google Scholar, 4Dunn RP Primary chylopericardium: a review of the literature and an illustrated case.Am Heart J. 1975; 89: 369-377Abstract Full Text PDF PubMed Scopus (48) Google Scholar, 5Jenner RE Oo HLA Isolated chylopericardium due to mediastinal lymphangiomatous hamartoma.Thorax. 1975; 30: 113-117Crossref PubMed Scopus (11) Google Scholar, 6Sarvan SV Ratshin RA Shirley JH et al.Idiopathic chylopericardium: 131I-triolein scan for noninvasive diagnosis.Ann Intern Med. 1975; 82: 663-665Crossref PubMed Scopus (13) Google Scholar, 7Charnilas Y Levo Y Weiss A et al.Isolated idiopathic chylopericardium.J Thorac Cardiovasc Surg. 1977; 73: 719-721PubMed Google Scholar, 8Gallant TE Hunziker RJ Gibson TC Primary chylopericardium: the role of lymphangiography.AJR. 1977; 129: 1043-1045Crossref PubMed Scopus (24) Google Scholar, 9Ross P Joseph S Walker D A case of isolated primary chylopericardium.Br Heart J. 1979; 41: 508-511Crossref PubMed Scopus (8) Google Scholar The prognosis is poor without treatment, but once diagnosed, thoracic duct ligation and a partial pericardiectomy result in complete cure. We present an unusual case which required resection of the thoracic ducts bilaterally. A 23-year-old Japanese man was admitted to our hospital on June 23, 1978, because of exertional dyspnea. He had been fairly well except for frequent attacks of bronchial asthma of 20 years’ duration. In December 1975, the patient had experienced high fever, productive cough, and dyspnea; and since then, he had had exertional dyspnea and lost 12 kg (26 Ib) in weight. There was no past history of tuberculosis or thoracic trauma. Physical examination disclosed a slender man in slight respiratory distress. His blood pressure was 124/90 mm Hg, and the pulse rate was 96 beats per minute and regular. There was no pulsus paradoxus. The veins of the neck were normal. Auscultation of the lung disclosed prolonged expiration and dry rales. Cardiac dullness was enlarged on both sides, but no apex beat was palpable. Auscultation of the heart revealed third and fourth heart sounds. The findings from the remainder of the physical examination were not remarkable. Laboratory findings included the following: hemoglobin level, 18.1 g/100 ml; and uric acid level, 8.2 mg/100 ml. Arterial blood gas levels with the patient breathing room air were an arterial oxygen pressure (PaO2) of 62 mm Hg and pH of 7.39. An electrocardiogram showed a normal sinus rhythm, QRS axis at 105°, p-dextrocardiale, and low voltage. A chest x-ray film taken in April 1976, at the time of a routine annual health examination, showed marked cardiac enlargement and increased pulmonary markings. In September 1975, the cardiac silhouette had been almost normal. By the time of admission, the cardiac size had become much larger (Fig 1). An echocardiogram and thoracic isotope blood pool scanning showed a large pericardial effusion. Pericardiocentesis yielded 1,000 ml of yellowish milky fluid. It had a specific gravity of 1.047 and contained 6.4 g of protein, 610 mg of triglyceride, and 20 mg of cholesterol per 100 ml. The sediment contained numerous red blood cells and some white blood cells. The fluid was sterile on culture. Since the fluid proved to be chyle, lymphangiographic study was performed at the Department of Radiology (Fig 2). It showed a dilated thoracic duct which was not visualized beyond the level of the carina. Small peribronchial lymphatic vessels as well as ectatic lymphatic vessels in the posterior mediastinum were seen. These findings suggested obstruction of the thoracic duct, but no pericardial pooling of contrast material was seen. The patient was then fed 50 mg of Sudan III in corn oil and the dye was demonstrated in the pericardial fluid later. Furthermore, precordial scanning after oral administration of radioactive 131iodine-labelled triolein showed strong radioactivity at the level of obstruction of the thoracic duct at seven hours and accumulation of radioactivity over the precordium at 24 hours. These findings indicated that there was a communication between the thoracic duct and the pericardial sac, and a diagnosis of primary chylopericardium was made. Despite a diet low in fat and the addition of medium-chain triglyceride, the fluid reaccumulated rapidly, so that a right thoracotomy was performed on Sept 29 at the Second Division, Department of Surgery. The pericardium appeared slightly thickened, and 1,000 ml of yellowish milky fluid was removed. No pleural effusion was seen. The thoracic duct was dilated, measuring 7 mm in diameter. No tumor was found. A section of the thoracic duct 7 cm in length was removed near the diaphragm, and a partial pericardiectomy was done. After surgery, a milky white discharge continued from the pericardial drain, and a postoperative lymphangiogram (Fig 3) showed a left thoracic duct with stasis of the contrast medium at the level of the seventh to eighth thoracic vertebrae. Also, reflux of the contrast material along the peribronchial sheaths was seen. Because these findings suggested obstruction of the left thoracic duct, a left thoracotomy was done on Oct 25. The left thoracic duct was resected from the level of the diaphragm to the aortic arch; and, again, a pericardial window was made. The postoperative course was uneventful, except for transient serous pleural effusion; and this time, lymphographic study showed no obvious lymphatic channels in the chest The cardiac silhouette remains normal 20 months after surgery. Primary chylopericardium is diagnosed by the demonstration of chylous effusion and a communication between the lymphatic system and the pericardial sac. The recommended therapy is thoracic duct ligation and partial pericardiectomy; all of the 23 patients in the literature were cured after both procedures, but three of the five treated with pericardiectomy alone showed reaccumulation of chyle. In our case the first operation failed, despite resection of the thoracic duct beyond the level of the entrance of dye injected into the pericardium. Resection of the left thoracic duct, which was found by postoperative lymphographic study, resulted in full recovery. In none of the previous case reports was there a duplicate thoracic duct, although five patients had segmental plexuses or multiple channels. ill of these findings are seen not infrequently as anatomic variations; Cha and Sirijintakam10Cha EM Sirijintakarn P Anatomic variation of the thoracic duct and visualization of mediastinal lymph nodes: a lymphographic study.Radiology. 1976; 119: 45-48Crossref PubMed Scopus (39) Google Scholar reported duplication in 19 of 243 patients who had lymphangiograms, the complete type in six of them. A segmental plexus was found in 33 without obstruction. Although preoperative recognition of dual pathways is essential to avoid reoperation, pedal lymphographic study has some limitations in that the visualization of the lymphatic system is largely influenced by the positioning of the patient and the timing of radiographic study.10Cha EM Sirijintakarn P Anatomic variation of the thoracic duct and visualization of mediastinal lymph nodes: a lymphographic study.Radiology. 1976; 119: 45-48Crossref PubMed Scopus (39) Google Scholar Multiple exposures during and after the injection10Cha EM Sirijintakarn P Anatomic variation of the thoracic duct and visualization of mediastinal lymph nodes: a lymphographic study.Radiology. 1976; 119: 45-48Crossref PubMed Scopus (39) Google Scholar may be useful in patients with chylous effusion; however, in our case, postoperative visualization of the left thoracic duct might have been due to increased flow in it after resection of the right thoracic duct. Stranahan et al11Stranahan A Alley RD Kausel HW et al.Operative thoracic ductography.J Thorac Surg. 1956; 31: 183-198PubMed Google Scholar reported two cases of chylothorax with malignant lymphoma in which surgical thoracic ductographic study was performed and showed dual or multiple channels. The technique was that the thoracic duct was transected above the diaphragm, both ends were cannulated, contrast medium was injected both proximally and distally, and an x-ray film was taken. In their case 1, retrograde filling of the cisterna chyli by injection into the distal catheter could have visualized the accessory duct arising in the cisterna chyli. Although their cases improved only partially after the ligation of these channels, it was probably because of malignancy. Intraoperative recognition of variations of the lymphatic system by this method would be of great value in preventing reoperation. Various causes have been reported, including hamar-tomatous lymphangiomatosis, cystic hygroma, and thoracic surgery and trauma; but in about one-half of the cases, there was no obvious cause. Our patient had none of these conditions, but obstruction of both thoracic ducts associated with insufficient collateral vessels might have caused reflux of chyle. The enlarged cardiac silhouette was first noted after an episode of high fever in 1975, and this suggests destruction of the lymphatic system by upper mediastinitis or severe cough. Furthermore, elevated intrathoracic pressure caused by bronchial asthma might have disturbed the drainage of lymph into the venous system.