A 13-month-old female child presented with a history of excessive cry and irritability for 7 days. On examination, she was afebrile, tachypneic, and tachycardic with intermittent nasal flaring, intercostal retractions, and muffled heart sounds. CXR showed cardiomegaly. Echocardiography revealed a structurally normal heart with a massive PE with features of early cardiac tamponade. There was no history of trauma, surgery, or family history of a similar condition.
She underwent pericardiocentesis, and 210 ml of milky white fluid was drained. Pericardial fluid analysis revealed glucose -145 mg/dl (blood glucose -160 mg/dl), protein -6 g/dl, cholesterol -11 mg/dl, triglyceride -2303 mg/dl, and cell count -3500 cells/mm3 with 90% lymphocytes and was suggestive of chylopericardium. Gram stain and acid-fast bacillus stain were negative. GeneXpert from the fluid was negative for tuberculosis. Fluid culture did not detect any organism. Sepsis markers were negative. Serum cholesterol was 237 mg/dl (normal <200 mg/dl), LDL -153 mg/dl, and triglyceride -140 mg/dl. Her liver and renal function was normal. High resolution computerised tomography (HRCT) thorax revealed PE [Figure 1c].
The child was initially managed conservatively with fluids and parenteral nutrition. Enteral nutrition with MCT and low-fat diet was commenced after 72 h. She remained hemodynamically stable. Octreotide was commenced at 1 mcg/kg/h from day 3. Over the course of the next 10 days, she had 100-120 ml of chylous fluid aspirated per day despite being on octreotide infusion. As MR lymphangiogram was not feasible in a small child in our setup, we could not rule out lymphangiomatosis.
Pericardiectomy with creation of adequate pericardioperitoneal window was done. Thoracic duct ligation was not done in this second child. Pleural and pericardial chest drains were removed on the 4th postoperative day as the drain output was almost negligible. The child was discharged on the 6th postoperative day with MCT, fat-soluble vitamins, and subcutaneous octreotide injection.
She was followed up regularly with monthly echocardiography and chest X-ray [Figure 1d]. Octreotide was gradually weaned off over the next 3 months. At 12-month follow-up, she is growing well (25th centile for height and weight) and achieving milestones as per age.