Transabdominal ligation of the thoracic duct with pericardial-peritoneal shunting in a case of primary idiopathic chylous pericardial effusion

Primary chylous pericardial effusion is a rare entity with few cases reported so far. We report a case of idiopathic etiology in a previously healthy 16-year-old boy. The patient presented with intermittent chest pain and dizziness caused by a chronic pericardial effusion. An echocardiogram revealing a pericardial effusion and open pericardiocentesis with a drainage of approximately of 500 ml of chylous fluid established the diagnosis. Patient had no history of trauma, cardiac surgery, central insertion of subclavian catheters or blunt injury. Computed tomography ruled out malignancies in the abdomen and chest. Clinical, laboratory and radiological investigations for the possible underlying cause of the condition were not determined. Management with a dietary regimen consisting of a medium-chain triglyceride-rich diet, octreotide pharmacological treatment and initial subxiphoid resection with pericardial tube drainage was unsuccessful. Surgical approach was required consisting of pericardio-peritoneal window with trans-abdominal ligation (clipping) of the thoracic duct above the diaphragm. Postoperative outcome was uneventful and there was a rapid recovery after surgical management.