Interesting Case of Facial Swelling.

55 yo F w/ PMHx of HTN, DM, and ESRD on HD presented to the ED w/ c/o facial redness and swelling progressively worsening for 3 days.

Patient  discharged 7 days prior from outside hospital but unable to attain records or d/c summary.  Last H/D 1 day prior. No fever, no CP, no new prescriptions, no Hx of malignancy, no travel.

On exam

T 98.6  HR 92  R 18  BP 179/89  Sat 95% on RA

Morbidly obese female w/ facial swelling and erythema

+JVD, throat clear w/ no swelling

lungs CTA and no wheezing

belly soft

bilateral extremities swollen R>L but difficult exam due to habitus, AV fistula on R arm w/ +thrill, distal pulses intact, no leg swelling or erythema, no peripheral catheters in place, no rash

Superior vena cava (SVC) syndrome results from any condition that leads to obstruction of blood flow through the SVC. Obstruction can be caused by external compression or direct invasion of the SVC by an adjacent pathologic process (usually malignancy) involving the right lung, lymph nodes, and other mediastinal structures, or by thrombosis of blood within the SVC. In some cases, both external compression and thrombosis coexist.

In the pre-antibiotic era, syphilitic thoracic aortic aneurysms, fibrosing mediastinitis, and other complications of untreated infection were frequent causes of the SVC syndrome. In the post-antibiotic era, malignancy became the most common cause, accounting for 90% of cases. More recently, the incidence of SVC syndrome due to thrombosis has risen, largely because of increased use of intravascular devices such as central vein catheters and pacemaker wires. In the present era,  20-40% of cases of SVC syndrome are seen in patients w/out malignancy.

The introduction indwelling catheters into the central veins has been shown to lead to the development of central venous stenosis.  Patients on chronic HD w/ multiple central catherizations are at increased risk.

In one study in which 133 hemodialysis patients underwent venography for access-related concerns over a 14-month period, 41% had evidence of significant central vein stenosis.  Risk factors included length of time patient had been on HD and number of catherizations.

In another study of 69 consecutive patients undergoing insertion of RIJ catheters, venography was performed prior to insertion of a guide-wire. Evidence of unexpected stenosis was found in 42% of patients with those who had previously received catherization having more than double the incidence of such abnormalities than those who had not.

In another study that used serial venographic studies to evaluate the long-term effects of subclavian vein dialysis catheters in 42 patients. At the time of catheter removal, 45% of patients had stenoses and 7% had total thrombosis of the subclavian vein.

In a retrospective study of 279 central venous infusion catheters in 238 patients, catheter-related venous thrombosis occurred in 13% of patients with subclavian vein catheters vs. 3% of patients with internal jugular vein catheters.  The mean time to thrombosis was 36 days for subclavian catheters and 142 days for internal jugular vein catheters.

In conclusion:

SVC syndrome is related to Malignancy and Indwelling Catheters

In the case of malignancy, consider Lung CA, Lymphoma, and metastatic disease

In the case of indwelling catheters, remember HD patients are at increased risk w/ the lenth of time on HD as well as number of catherizations and location being independent risk factors.

 

This patient was actually found to have an SVC stent which had thrombosed, patient was unaware of having the stent or the reason for its placement.

Special thanks to Dr. Slovis for his morning report presentation and interesting case which inspired this pearl.

 

References:

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