Case Presentation: Implanted central venous port catheters (ports) are widely used for chemotherapy, total parenteral nutrition and blood monitoring. If placed in the subclavian vein, up to 15% of patients who receive implanted ports have immediate complications, including venous injury, pneumothorax and pneumomediastinum. Long-term ports are associated with delayed complications, including thrombosis, occurring in 1.9-21.5% of patients. Although rare, if thrombosis of the port’s catheter occurs, it can cause significant occlusion of the vein and result in superior vena cava (SVC) syndrome. We report this feared, potentially avoidable complication in two cases of SVC syndrome that developed in patients with previous diffuse large B cell lymphoma (DLBCL) in remission with retained long-term implanted ports.Case 1: A 93-year-old woman with a history of DLBCL status post port placement, treated with R-CHOP and R-CVP with adriamycin chemotherapy, now in remission for 3 years presented with a positional headache for 4 weeks. Associated symptoms included facial flushing, dizziness, dyspnea, chest tightness, and fluctuating left arm swelling over the same period of time. Her exam revealed normal vital signs, but distended neck vessels, facial plethora and a port in the right chest wall. Computed tomography (CT) of the chest with contrast revealed an acute-to-subacute occlusion of the SVC due to thrombosis of the distal tip of the port catheter.Case 2: A 34-year-old man with a history of DLBCL status post port placement, treated with R-CHOP chemotherapy, now in remission for 2 years presented with facial swelling for 2 days. Associated symptoms included a positional headache, dizziness, and bilateral ear fullness. His exam revealed tachycardia (120-130 beats/minute), facial swelling and plethora, maxillary sinus tenderness and a port in the right chest wall. CT venogram of the chest revealed occlusion of SVC due to thrombosis of the port catheter.Both patients had ports placed prior to the initiation of chemotherapy, but these retained ports were not removed following completion of chemotherapy or after positive-emission-tomography-proven remission. In response to their port-associated SVC syndrome, both were started on an unfractionated heparin drip, followed by an oral anticoagulant and surgical removal of port after several months of anticoagulation was recommended. Port removal was deferred by our patient in case 1, thus she elected to continue long-term anticoagulation. However, port removal was pursued by our patient in case 2, with notable improvement in symptoms after removal of the port.

Discussion: SVC syndrome presents as arm and facial swelling, positional headache, retro-orbital pain, blurred vision, stridor, and dyspnea. It has been linked to malignancy in ~80% of the cases, however the incidence of thrombosis as a cause of SVC syndrome has greatly increased with the use of implantable ports and is estimated to be 0.2-3.3%. This risk is further increased in the setting of prothrombotic conditions, like active malignancy.

Conclusions: Our cases highlight the importance of removing the implanted port soon after chemotherapy is completed and remission is confirmed in patients with DLBCL to prevent this important complication and to reduce to burden of treatment associated with anticoagulants.