Congenital extensive central venous thrombosis with chylous ascites and chylothoraces

We describe a case of congenital extensive central venous thrombosis presenting as polyhydramnios and massive ascites, requiring amnioreduction prenatally and refractory chylous ascites and chylothoraces postnatally. Echocardiography, computed tomography angiogram (CTA), and magnetic resonance venogram (MRV) were helpful in defining the nature and extent of the lesion. The patient underwent staged procedures of repeated abdominal paracentesis, chest drain insertion, and right internal jugular vein exploration initially. Subsequently, open thromboembolectomy from the upper venous system veins and pericardial patch angioplasty of the right internal jugular and right innominate veins were required and managed by catheter-directed thrombolysis for the residual thrombosis with successful recovery.