A case of inherited thrombophilia with superior vena cava syndrome: pharmacological thrombolysis

Abstract

Venous thrombosis involving vena cava is an uncommon phenomenon seen in children, even in those with inherited thrombophilia syndromes. We present a case of protein C and S deficiency with superior vena cava (SVC) thrombosis along with pyo-pericardium, treated successfully with intravenous (IV) tissue plasminogen activator (TPA) and unfractionated heparin (UFH). A 4-year, male patient presented with massive pericardial effusion (causing cardiac tamponade). Fluoroscopic and echo-guided pericardiocentesis was done. Pericardiocentesis continued for the next 2 days, after which the catheter was removed as fluid volume came down. However, the child soon showed features of SVC syndrome. Ultrasonography and CT angiogram of neck vessels showed occlusive thrombus. Systemic thrombolysis with IV alteplase was performed, followed by continuous UFH IV infusion. Blood investigations showed protein S deficiency and low protein C level. Gradually, symptoms and cardiac function improved. We discharged the patient on warfarin, aspirin and clopidogrel. On follow up, he is doing well. The development of SVC syndrome due to inherited thrombophilia syndromes like protein C and protein S deficiency is rare in children. With that, thrombolytic therapy in children has not been studied much in the past, with extrapolation of data from adult studies being made, putting into the question of its safety and efficacy. However, we successfully managed to restore blood flow in thrombus occluded areas by TPA therapy with no complication from the therapy. SVC syndrome is attached to many causes, one being protein S and C deficiency. However, it can be successfully managed with thrombolysis using TPA.