Pericardial fenestration and thoracic duct ligation for treatment of chylopericardium as first symptom of underlying generalized lymphatic anomaly: a case report

INTRODUCTION: Chylopericardium represents a rare condition of chyle accumulation within the pericardial sac, caused by abnormal thoracic duct anatomy or prolonged increased pressure. Nothing by mouth (NPO) policy and total parenteral nutrition (TPN), even in combination with pericardial drainage, render only a temporary solution. Surgical intervention with thoracic duct ligation and creation of a pericardial window is believed to be the most effective treatment. CASE PRESENTATION: An 18-year-old male was referred with a persisting idiopathic chylopericardium for five months. Various drainages and conservative treatment failed. To more permanently treat the chylopericardium, we created a pericardial window and ligated the thoracic duct by right-sided uniportal video-assisted thoracoscopic surgery. Postoperative NPO and TPN for 1 week were initiated, followed by medium-chain-triglycerides diet for 1 week. Clinical improvement occurred and the chest drain was removed two weeks after surgery. Magnetic resonance imaging of the whole body showed multiple cystic lesions in spleen and skeleton compatible with generalized lymphatic anomaly (GLA), proven by the anatomopathological examination of the thoracic duct and lung biopsy. Sirolimus was initiated for further treatment. CONCLUSION: In this case of idiopathic chylopericardium, we successfully performed thoracoscopic thoracic duct ligation and creation of a pericardial window, diagnosing a GLA.