Medical Image of the Month: Superior Vena Cava Syndrome

Figure 1. Large mediastinal lymph nodes (red arrow) causing compression of the superior vena cava (blue arrow). Numerous enlarged lymph nodes can also be seen in the axillary, cervical, and upper abdominal regions (green arrows).

History: A 74-year- old man with a history of diastolic heart failure, chronic kidney disease (CKD), and chronic lymphocytic leukemia (CLL) presented with a complaint of dyspnea. He has had several hospitalizations in the last year for heart failure exacerbation and his home bumetanide was recently increased from twice to three times daily due to persistently increasing weight. His CLL was diagnosed two years prior and treatment was stopped three months ago due to side effects. In the emergency department he reported three weeks of worsening dyspnea especially when lying flat, as well as increased swelling in his legs, abdomen, arms, and face. His weight was up to 277lbs from 238lbs the month before. His diuretics were transitioned to IV, but over the next few days he remained clinically volume overloaded. A noncontrast chest CT was obtained to help evaluate his ongoing respiratory distress (Figure 1). It demonstrated innumerable lymph nodes involving the cervical, axillary, mediastinal, and upper abdominal regions, which had significantly increased in size and number from prior exam several months before. The CT also showed several particularly bulky lymph nodes which appeared to be compressing the superior vena cava.

Discussion: The superior vena cava (SVC) is responsible for about one-third of the venous return to the heart. Because of its thin walls relative to arterial vasculature, it is susceptible to compression from adjacent structures which may subsequently impair venous return to the heart, a process known as SVC syndrome. Intrathoracic malignancy is responsible for 60-85% of cases of SVC syndrome, and common symptoms include facial or neck swelling, swelling of the arms, and dyspnea (1). In this case, the patient’s apparent resistance to diuresis was felt to be partially secondary to SVC syndrome. In stable patients, contrast-enhanced CT is the preferred imaging modality if SVC syndrome is suspected, which can define the extent of SVC blockage. Duplex ultrasound may be used first to exclude thrombus. In this patient with acute kidney injury on CKD it was decided to forgo the contrast study to avoid further kidney damage. Management of SVC syndrome depends on severity, with emergent treatment focused on maintaining the airway and endovenous recanalization. Definitive treatment is directed at the underlying cause (2).

After about a week of aggressive IV diuresis, the patient’s breathing and volume status improved and he was transitioned back to oral diuretics. He was discharged home with plans for hospice.

Matthew R. Borchart MD, Daniel Yu MD, and Indrajit Nandi MD

University of Arizona College of Medicine, Phoenix

Phoenix, AZ USA

References

1. Rice TW, Rodriguez RM, Light RW. The superior vena cava syndrome: clinical characteristics and evolving etiology. Medicine (Baltimore). 2006 Jan;85(1):37-42. [CrossRef] [PubMed]
2. Wilson LD, Detterbeck FC, Yahalom J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med. 2007 May 3;356(18):1862-9. [CrossRef] [PubMed]

Cite as: Borchart MR, Yu D, Nandi I. Medical Image of the Month: Superior Vena Cava Syndrome. Southwest J Pulm Crit Care. 2020;21(6):136-7. doi: https://doi.org/10.13175/swjpcc060-20 PDF

Medical Image of the Month: Superior Vena Cava Syndrome

Figure 1. Coronal CT thorax with contrast showing a large apical mass with near complete atelectasis of the right upper lobe, mediastinal extension and effacement of the superior vena cava (arrow).

Figure 2. Caval-superficial-umbilical-portal pathway.  EMV = external mammary vein, EV = epigastric vein, IEV = inferior epigastric vein, IMV = internal mammary vein, SEV= superior epigastric vein (2).

Figure 3. Axial CT thorax with contrast showing avid arterial enhancement of hepatic segment IV (arrow, hot quadrate sign), consistent with superior vena cava syndrome.

Although superior vena cava syndrome (SVCS) may result from internal or external occlusion of the superior vena cava, 60-90% of cases are caused by external compression from malignant tumors, predominately lung cancer and lymphoma (1). Additional causes of SVCS via external occlusion include fibrosing mediastinitis, while internal occlusion may result from pacemaker lead or indwelling central venous catheter thrombosis (1). Symptoms of SVCS, such as facial and neck swelling, dyspnea and cough, typically develop over 2-4 weeks prior to diagnosis, during which collateral vessels develop (2). More severe symptoms of disease include laryngeal edema, cerebral edema, orthostatic syncope secondary to decreased venous return and altered mental status (3). In the presence of SVCS, cavoportal collaterals that may develop include caval-superficial-umbilical-portal pathways and caval-mammary-phrenic-hepatic capsule-portal pathways (3). Figure 2 demonstrates the anastomosis of inferior and superficial epigastric veins with internal and external mammary veins, allowing for recanalization of the paraumbilical vein and drainage into left portal vein. The presence of a caval-superficial-umbilical-portal pathway may be detected as a wedge-shaped area of increased enhancement in segment IV of the liver on CT or MRI, a radiographic finding known as the hot quadrate sign (Figure 3). Following diagnosis of SVCS in the setting of malignancy, goals of management may be palliative or curative and should take into account life expectancy. Endovascular stenting can provide near immediate symptomatic relief of SVCS, but requires the addition of chemotherapy, radiotherapy or combined-therapy if the goals of treatment are curative (1). Although the median life expectancy of a patient with SVCS due to underlying malignancy is often reported as 6 months, the prognosis is dependent on tumor type and the presence or absence of poor prognostic factors, including age >50 years old, history of tobacco use and treatment with corticosteroids (3).

Elliot Breshears MS IV, Lev Korovin MD, and Veronica Arteaga MD.

Department of Medical Imaging

The University of Arizona

Tucson, AZ, USA

References

1. Wan JF, Bezjak A. Superior vena cava syndrome. Hematol Oncol Clin North Am. 2010;24(3):501-13. [CrossRef] [PubMed]
2. Kapur S, Paik E, Rezaei A, Vu DN. Where there is blood, there is a way: unusual collateral vessels in superior and inferior vena cava obstruction. RadioGraphics. 2010;30(1):67-78. [CrossRef] [PubMed]
3. Manthey DE, Ellis LR. Superior vena cava syndrome (SVCS). In: Todd KH, Thomas CR Jr. Oncologic Emergency Medicine: Principles and Practice. Switzerland: Springer; 2016:211-222. Available at: https://books.google.com/books?id=_qQqDAAAQBAJ&pg=PA211&lpg=PA211&dq=Manthey+DE,+Ellis+LR.&source=bl&ots=MWH6bcbHSf&sig=L7Ul5sfS1sSGBTF5cnK7MvKF9eA&hl=en&sa=X&ved=2ahUKEwjGkoTC9LrdAhUEEHwKHbV2CF4Q6AEwAHoECAEQAQ#v=onepage&q=Manthey%20DE%2C%20Ellis%20LR.&f=false (accessed 9/14/18).

Cite as: Breshears E, Korovin L, Arteaga V. Medical image of the month: superior vena cava syndrome. Southwest J Pulm Crit Care. 2018;17(4):114-5. doi: https://doi.org/10.13175/swjpcc103-18 PDF