IN PRACTICE

 

Unilateral superior vena cava syndrome: An uncommon complication of a commonly used procedure

 

 

N Goolam^I; M Bester^II; D Schietekat^III

^IMB ChB, Dip HIV Man; Department of Internal Medicine, Tygerberg Academic Hospital, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South Africa
^IIMB ChB; Department of Internal Medicine, Khayelitsha District Hospital, Western Cape Department of Health, Cape Town, South Africa
^IIIMB ChB, MMed (Int Med); Department of Internal Medicine, Tygerberg Academic Hospital, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South Africa

Correspondence

 

 

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ABSTRACT

Superior vena cava (SVC) syndrome constellates symptoms of plethora and facial and upper thoracic swelling with distension of neck veins, and the consequences of this condition range from mild discomfort to upper airway obstruction. The aetiology has a strong linkage with malignancy, and is always to be excluded. SVC syndrome is commonly a bilateral presentation, and unilateral SVC syndrome is rare, with very few non-malignant causes reported. Here, we demonstrate a unilateral occurrence with an uncommon cause.

Keywords: SVC syndrome, central venous catheter, superior vena cava

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The superior vena cava (SVC), formed by the right and left brachiocephalic veins, is responsible for venous drainage of the upper head and neck, and obstruction of the venous return by various mechanisms creates a congestive syndrome.^[1,2] Classically, symptoms of SVC syndrome are bilateral neck swelling, which can lead to life-threatening upper airway compromise and subclavian vein compression with upper torso oedema.^[3] Current evidence attributes >70% of cases to mediastinal malignancies, although the rarer benign aetiology has a better prognosis.^[4] Therefore, unilateral SVC syndromes, although uncommon, may still be due to a malignancy, and hence this should always be investigated for.^[5] Device-associated SVC syndrome can be caused by pacemakers and central venous catheters, with resultant stenosis and thrombi and, as in our clinical case, may result in acute SVC syndrome with obstruction and painful unilateral symptoms.^[6] In our clinical setting, with a high burden of comorbid disease, patients often present late and acutely ill, requiring interventions such as central venous catheters, and these need to be meticulously and carefully placed to avoid complications.^[7] Sinister causes and even vascular anomalies need to be excluded post this rare unilateral syndrome occurrence.^[8] Treatment modalities of SVC syndrome depend on the aetiology as well as the severity of the syndrome and its complications.^[8] Outcomes of any treatment employed depend on the aetiology and the progression or severity of the clinical state that the patient presented with.^[9] Therefore, it is important to note that treatment outcomes may range from relief of acute symptoms to resolution of the syndrome, depending solely on the underlying disease process.^[10] Our patient described here depicts a rare unilateral SVC syndrome caused by a thrombus from a central venous catheter placed in a recent hospitalisation.

 

Case presentation

A 38-year-old female known to be HIV infected on antiretroviral therapy (ART) with suppressed viral level was diagnosed with urogenital tuberculosis (TB) and left renal abscess at a tertiary hospital. The urogenital TB was confirmed on a positive TB culture and Gene Xpert. A nephrostomy was required to manage subsequent hydroureter and hydronephrosis. During that admission she required intravenous fluids through a right internal jugular central venous catheter to resolve her haemodynamic instability after nephrostomy insertion, and was discharged when she was stable.

She presented to our district level hospital 2 weeks later for acute medical admission with a 2-week history of swelling of the right side of her upper body, which was localised to her face, neck, breast and bilateral lower limbs. She reported a 1-week history of exertional dyspnoea with reduced effort tolerance and orthopnoea.

On clinical examination, this patient appeared acutely unwell and tachypnoeic, with a respiratory rate of 30 breaths per minute (bpm), saturating at 98% in room air, while the other vitals were within normal ranges. She had unilateral and tender soft-tissue swelling of the right side of her upper body that involved her face, neck, breast, arm and the right side of her abdomen. Visibly distended right-sided external jugular vein was also present. There were no signs of airway compromise and on auscultation, no bibasal crackles. The cardiovascular findings included bipedal oedema, loud and palpable second heart sound and a raised jugular venous pressure waveform, pronounced on her right side (Fig. 1).

 

 

Special investigations performed

Chest radiography performed at the emergency department upon her arrival showed features of an enlarged cardiothoracic ratio, as well as bilateral small pleural effusions without evidence for pulmonary oedema.

An echocardiogram of the heart was performed that revealed dilated cardiomyopathy with an ejection fraction of 30%, with secondary mitral regurgitation as well as tricuspid regurgitation. No regional wall motion abnormalities were detected (Table 1).

Given the atypical right-sided presentation of oedema, further diagnostic evaluation was commenced. Contrast-enhanced computed tomography angiography scan of the neck revealed a unilateral right-sided thrombus extending from the proximal/mid-internal jugular vein to the SVC inlet (Fig. 2A and B).

The patient was started on anticoagulation. However, considering the extent of the thrombus as well as the painful unilateral SVC syndrome present, this patient was discussed with vascular surgery at a tertiary hospital. It was concluded that the likely aetiology of the thrombus waslinked to a prolonged right internal jugular central venous catheterisation. The discussion regarding a thrombectomy was considered. However, it was deferred on the basis of limited data available on prognosis and outcomes post surgery. During the patient's hospital stay, the bipedal oedema cleared up within 3 days on antifailure treatment, yet the right-sided oedema of the face, neck and upper torso persisted, and ultimately resolved with anticoagulation therapy within 3 weeks.

 

Discussion

Our patient's oedematous state was clearly a combination of unilateral SVC syndrome and dilated cardiomyopathy. The overwhelming right-sided upper-body painful oedema was atypical for dilated cardiomyopathy, which led us to investigate further. The aetiology here was not common nor of malignant origin; however, the syndrome can still manifest with severe consequences. Adequate history taking and seeking common causes through imaging remain important. Unilateral SVC syndrome has a variable outcome that is dependent on the cause of the syndrome. In our case, the cause of internal jugular vein thrombosis was benign, and the proposed mechanisms for thrombus formation around the catheter site may be attributed to venous stasis and possibly inflammatory cytokine adhesion molecules. Early surgical involvement remains essential to prevent life-threatening consequences. Our literature search did not find much information on the duration of anticoagulation for internal jugular vein thrombosis, and it was decided by both the medical and surgical teams to treat with life-long anticoagulation, and to continue antifailure therapy for the dilated cardiomyopathy. Central venous catheterisation is a much-needed lifesaving intervention, and care should always be taken to ensure correct insertion and frequent monitoring of complications post insertion.

The authors declare that appropriate written informed consent was obtained for the publication of this article and accompanying images.

Declaration. None.

Acknowledgements.

Author contributions. All authors were involved in the clinical management of the patient. NG and MB wrote the manuscript, which was critically reviewed by all co-authors.

Funding. None.

Conflicts of interest. None.

 

References

1. Cohen R, Mena D, Carbajal-Mendoza R, et al. Superior vena cava syndrome: A medical emergency? Int J Angiol 2008;17(1):43-46. https://doi.org/10.1055/s-0031-1278280        [ Links ]

2. Lepper PM, Ott SR, Hoppe H, et al. Superior vena cava syndrome in thoracic malignancies. Respir Care 2011;56(5):653-666. https://doi.org/10.4187/respcare.00947        [ Links ]

3. Seligson MT, Surowiec SM. Superior Vena Cava Syndrome. StatPearls, 26 September 2022. https://www.ncbi.nlm.nih.gov/books/NBK441981/ (accessed 12 September 2024).         [ Links ]

4. Azizi AH, Shafi I, Shah N, et al Superior vena cava syndrome. JACC Cardiovasc Interv 2020;13(24):2896-2910. https://doi.org/10.1016/j.jcin.2020.08.038        [ Links ]

5. Khera SY. Superior vena cava syndrome as unilateral right breast enlargement. J Vasc Surg Cases Innov Tech 2022;8(3):477-479. https://doi.org/10.1016/j.jvscit.2022.04.018        [ Links ]

6. Morriello F, Brown A, Granton J, et al. Catheter-associated superior vena cava syndrome. CMAJ 2023;195(2):E72-E75. https://doi.org/10.1503/cmaj.220669        [ Links ]

7. Edginton S, Fundytus A. Hyperacute superior vena cava syndrome associated with central venous catheter insertion. Indian J Crit Care Med 2019;23(3):152-154. https://doi.org/10.5005/jp-journals-10071-23140        [ Links ]

8. Desai UD, Joshi JM. Unilateral superior vena cava syndrome. J Postgrad Med 2014;60(4):390-393. https://doi.org/10.4103/0022-3859.143966        [ Links ]

9. Forman J, Unger K. Unilateral superior vena caval syndrome. Thorax 1980;35:314-315. https://doi.org/10.1136/thx.35.4.314        [ Links ]

10. Rice TW, Rodriguez RM, Light RW. The superior vena cava syndrome: Clinical characteristics and evolving etiology. Medicine 2006;85(1):37-42. https://doi.org/10.1097/01.md.0000198474.99876.f0        [ Links ]

 

 

Correspondence:
N Goolam
nadirahgoolam@gmail.com

Received 30 June 2024
Accepted 1 September 2024