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Chylothorax as the first manifestation of constrictive pericarditis

      To the Editor:
      Chylothorax is defined as an accumulation of chyle in the pleural space due to disruption of the thoracic duct or one of its major divisions (
      • Merrigan B.A.
      • Winter D.C.
      • O’Sullivan G.C.
      Chylothorax.
      ). We describe a 35-year-old white man with a 4-month history of bilateral chylothorax who presented with recent-onset ascites following thoracic duct ligation.
      The patient’s past history included aortoplasty for aortic coarctation at the age of 5 years. At admission, he appeared pale and ill. The physical examination revealed jugular venous distention at the angle of the jaw (8 cm above the sternal angle) while he was sitting. Heart sounds were normal. There was massive intra-abdominal free fluid. There was slight hepatomegaly and pitting edema of the legs. Blood chemical levels were normal except for a slight elevation in alkaline phosphatase and γ-glutamyl transpeptidase levels. Tuberculin skin test was negative. Paracentesis yielded a milky fluid with the following biochemical composition: triglycerides, 1080 mg/dL; cholesterol, 89 mg/dL; total protein, 3.8 g/dL; and lactate dehydrogenase, 108 U/L. Cytological and microbiologic examinations of peritoneal fluid were unremarkable. A chest radiograph showed minimal cardiac silhouette enlargement without pericardial calcification and moderate left pleural effusion. Abdominal echography disclosed a dilated inferior vena cava, which did not change in diameter during respiration. Computed tomographic (CT) scan of the thorax revealed a 1-cm thick pericardium (Figure) with some pericardial effusion and left pleural effusion. Right and left heart catheterization displayed an early diastolic dip followed by a mid-through-late diastolic plateau of the pressures in both ventricles. Cardiac index was reduced to 1.2 L/min/m2. Pericardiectomy showed a thick and encasing pericardium. Microscopic examination revealed nonspecific chronic pericarditis. Ascites progressively resolved, and the patient had an uneventful recovery and was asymptomatic 24 months later.
      Figure thumbnail GR1
      FigureComputed tomographic scan (axial view) of the chest showing left pleural effusion and marked thickening of the pericardium (arrows).
      Pleural effusion may be present in constrictive pericarditis (
      • Metha A.
      • Mehta M.
      • Jain A.C.
      Constrictive pericarditis.
      ). In our patient, errors in diagnosis and the physical examination led to delayed treatment. After a preliminary CT scan ruled out masses as an intra-thoracic cause of lymphatic obstruction, the past cardiovascular procedure became suspect. This assumption led to surgical ligation of the thoracic duct, which resulted in massive chyloperitoneum. Previous cardiovascular surgery is associated with pleural chylous effusion in less than 0.5% of cases (
      • Cerfolio R.J.
      • Allen M.S.
      • Deschamps C.
      • Trastek V.F.
      • Pairolero P.C.
      Postoperative chylothorax.
      ). It is most common after resection of a coarcted aorta (
      • de Beer H.G.
      • Mol M.J.
      • Janssen J.P.
      Chylothorax.
      ). However, in our patient there was an interval of 30 years between heart surgery and chylothorax onset, making this diagnosis very unlikely even if anatomical venolymphatic junction modification following surgical correction may have played a role in the generation of chylous effusion after high pressure development in the venous system. Experimentally induced constrictive pericarditis is followed by thoracic duct hypertension and dilation (
      • Savage M.P.
      • Munoz S.J.
      • Herman W.M.
      • Kusiak V.M.
      Chylous ascites caused by constrictive pericarditis.
      ). The diameter of the duct may increase to up to four times its normal size and lymph flow may increase up to 12 times the normal rate in heart failure, as has been described in previous reports of peritoneal chylous effusion in heart disease and constrictive pericarditis (
      • Hurley M.K.
      • Emiliani V.J.
      • Comer G.M.
      • Patel A.
      • Navarro C.
      • Maiki C.O.
      Dilated cardiomyopathy associated with chylous ascites.
      ). In our patient, free peritoneal fluid, which was ruled out at the time of pleural effusion onset, resulted because of the need for alternative drainage following thoracic duct ligation.
      Although the etiology of constrictive pericarditis has not been established, constrictive pericarditis should be considered in the differential diagnosis of chylothorax.

      References

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