Prehospital Care

Prehospital caregivers are aware of the superior vena cava syndrome (SVCS) diagnosis only on occasion.

The usual attention to airway, breathing, and circulation (ABCs) is required.

Superior vena cava syndrome only rarely manifests as a life-threatening entity; therefore, other causes for the symptomatology must be sought.

Emergency Department Care

Superior vena cava syndrome only rarely manifests as an acute emergency. Typically, the syndrome develops over weeks to months and is recognized when the typical signs of facial and upper extremity edema occur. Confusion related to associated cerebral edema or stridor from laryngeal edema and impending airway obstruction represent acute emergencies.

Attention to the ABCs is essential.

If patients are allowed to sit upright, they may experience some relief of the usual dyspnea.^[10]

Stabilize the airway, as needed, and consider steroids.

If cerebral/airway edema is present, consider diuretics; however, diuretics have not shown consistent benefit in the emergency department (ED).

Endovascular shunts are increasingly used, as are thrombolytics if a thrombotic cause is present.

Cui and colleagues reported on the development of acute superior vena cava (SVC) syndrome in a 28-year-old woman with end-stage renal disease who was implanted with a left-side hemodialysis reliable outflow graft and a right-side double lumen hemodialysis catheter via internal jugular veins. The patient’s symptoms were not alleviated after catheter removal and systemic anticoagulation therapy. Eventually, she was successfully treated with catheter-directed thrombolysis, demonstrating that this method can be safely used to treat refractory catheter-induced acute SVC syndrome in end-stage renal disease patients.^[11]

After a tissue diagnosis, radiation and chemotherapy may be initiated.

Consultations

Over the last 20 years, considerable experience with endovascular stenting of superior vena cava syndrome has been achieved.^{[12, 13, 14, 15, 16]}At many centers, endoprostheses have become the initial choice for palliative treatment of superior vena cava syndrome.

In a retrospective study that evaluated the self-expanding nitinol Sinus-XL stent for the treatment of superior vena cava (SVC) obstruction caused by non-small cell lung cancer (NSCLC), investigators studied 23 patients with NSCLC and acute SVC obstruction who were scheduled for urgent stent implantation. The primary study endpoints were technical success, residual stenosis < 30%, and clinical efficacy. Results provided evidence that in this palliative setting, implantation of the self-expanding Sinus-XL stent for treatment of SVC obstruction caused by NSCLC is a safe and effective urgent treatment.^[17]

Emergent consultation with radiation therapy may be necessary, depending upon the acuteness of the presentation.

Because most causes of superior vena cava syndrome are related to lung cancer, a pulmonary or oncology consultation may be obtained.

Generally, considering the diagnosis in the ED is important. If the diagnosis is made de novo in the ED, only rarely is emergent consultation necessary. Exceptions include sudden airway compromise or acute superior vena cava thrombosis, which may occur from an indwelling catheter.

Medication

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Janssen M, Logtenberg S. Vena cava superior syndrome six years after central venous catheter removal in a patient on hemodialysis. Case Rep Nephrol Dial. 2022 Aug 29. 12(2):132-7. [QxMD MEDLINE Link].
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Sun M, Chen X, Li H, et al. Clinical analysis of 48 cases of malignant superior vena cava syndrome. World J Surg Oncol. 2021 Jun 23. 19(1):185. [QxMD MEDLINE Link]. [Full Text].
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Dhaliwal RS, Das D, Luthra S, et al. Management of superior vena cava syndrome by internal jugular to femoral vein bypass. Ann Thorac Surg. 2006 Jul. 82(1):310-2. [QxMD MEDLINE Link].
[Guideline] Kvale PA, Selecky PA, Prakash UB. Palliative care in lung cancer: ACCP evidence-based clinical practice guidelines (2nd edition). Chest. 2007 Sep. 132(3 Suppl):368S-403S. [QxMD MEDLINE Link].
Andersen PE, Duvnjak S. Palliative treatment of superior vena cava syndrome with nitinol stents. Int J Angiol. 2014 Dec. 23(4):255-62. [QxMD MEDLINE Link]. [Full Text].
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Cui J, Kawai T, Irani Z. Catheter-directed thrombolysis in acute superior vena cava syndrome caused by central venous catheters. Semin Dial. 2015 Sep-Oct. 28(5):548-51. [QxMD MEDLINE Link].
Lanciego C, Pangua C, Chacon JI, et al. Endovascular stenting as the first step in the overall management of malignant superior vena cava syndrome. AJR Am J Roentgenol. 2009 Aug. 193(2):549-58. [QxMD MEDLINE Link].
Sato Y, Inaba Y, Yamaura H, Takaki H, Arai Y. Malignant inferior vena cava syndrome and congestive hepatic failure treated by venous stent placement. J Vasc Interv Radiol. 2012 Oct. 23(10):1377-80. [QxMD MEDLINE Link].
Courtheoux P, Alkofer B, Al Refai M, et al. Stent placement in superior vena cava syndrome. Ann Thorac Surg. 2003 Jan. 75(1):158-61. [QxMD MEDLINE Link].
Quencer KB. Superior vena cava syndrome: etiologies, manifestations, and treatments. Semin Intervent Radiol. 2022 Jun. 39(3):292-303. [QxMD MEDLINE Link].
Hamzik J, Chudej J, Dzian A, Sokol J, Kubisz P. Endovascular stenting in malignant obstruction of superior vena cava. Int J Surg Case Rep. 2015. 13:84-7. [QxMD MEDLINE Link]. [Full Text].
Mokry T, Bellemann N, Sommer CM, et al. Retrospective study in 23 patients of the self-expanding sinus-XL stent for treatment of malignant superior vena cava obstruction caused by non-small cell lung cancer. J Vasc Interv Radiol. 2015 Mar. 26(3):357-65. [QxMD MEDLINE Link].
Armstrong BA, Perez CA, Simpson JR, Hederman MA. Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol Biol Phys. 1987 Apr. 13(4):531-9. [QxMD MEDLINE Link].
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Media Gallery

Superior Vena Cava Syndrome in Emergency Medicine. Patient with a 4-week history of increasing facial edema and known lung cancer.
Superior Vena Cava Syndrome in Emergency Medicine. Chest radiograph of a patient with known superior vena cava syndrome (SVCS) and bronchogenic carcinoma (CA).
Superior Vena Cava Syndrome in Emergency Medicine. Chest radiograph of a 50-year-old woman with complaint of shortness of breath and facial swelling. No previous history of cancer but 30 pack-year history.
Superior Vena Cava Syndrome in Emergency Medicine. CT scan of the same 50-year-old woman in the previous image with an initial presentation of shortness of breath and facial swelling. This shows a large tumor mass in the right mediastinum nearly surrounding the right main stem bronchus and partially occluding the superior vena cava.