What Do I Do Next?: Facial Swelling

Mike Weinstock, MD and Mizuho Spangler, MD

Pearls

SVC syndrome occurs 15,000 times per year in the US. 60% of SVC syndromes are malignant in origin and 35% are catheter-associated from permanent indwelling lines for chemo or antibiotic administration. 82% of SVC syndromes present with facial edema and/or erythema.





CASE: 35 yo woman presenting with the chief complaint of facial swelling.

HPI:

The patient reports 2 days of painful swallowing and throat swelling. No history of rash, anaphylaxis or allergic reaction and denies new foods and medications. She is allergic to latex but denies any recent exposure. She tried to take ibuprofen, but reports the pills got stuck and she could not swallow them. She reports some perioral numbness and tingling. No difficulty handling secretions, no fevers, blurred vision, bruising or rash.

Physical exam:

HR 125 RR 20 BP 143/87 O2 Sat 100%.

The patient is breathing normally without stridor, drooling or respiratory distress. Posterior oropharynx is erythematous and symmetrically swollen with the uvula midline and minimally edematous. Airway is widely patent without evidence of abscess. Voice is normal in tone and volume. Her face is symmetric, obese, and difficult to determine if there is swelling. There is some anterior cervical lymphadenopathy. Rapid strep test is sent and negative. There is no anasarca, lower extremity edema or clinical concern for CHF.

What Do I Do Next?

Red flags in this case: elevated heart rate, particularly in the abscess of fever is concerning. It is also concerning that she was unable to swallow pills.

Differential Diagnosis:

Peritonsillar abscess—less likely with symmetrical swelling and no uvular deviation.



Ludwig’s Angina—patients are usually ill-appearing with a brawny appearance to their chin.



Anaphylaxis or angioedema—but there is no concerning history or medications.

CASE CONTINUES:

The patient was treated for an allergic reaction with PO steroids and a hydrocodone/paracetamol (Lortab) elixir. Her chart notes that the provider expected that she would improve in the next 6 hours as the steroids started to take effect. Before discharge, her heart rate was rechecked twice and came down to 100 and 104.

Shortly thereafter, the patient bounces back to a different clinic where the documentation reveals that she had previously been to a dentist and received an anesthetic. They feel she is having an allergic reaction and prescribe prednisone and discharge the patient home again.

Nine days later, she presents to a different ED with the chief complaint of worsening facial and neck swelling and redness over 2 weeks. The ED notes that her voice is hoarse and that the patient reports sharp and stabbing chest pain and shortness of breath. No tongue swelling. She also has right-hand numbness, leg swelling and tightness.





A chest x-ray is performed and reveals an anterior mediastinal mass that measures 10cm x 12cm x 9cm and extends into the lower neck causing superior vena cava (SVC) syndrome. Collateral vessels are present.





During her ED course, she develops respiratory distress that requires intubation. Her mass is biopsied and she is diagnosed with Burkitt’s Lymphoma.

SVC Syndrome: An uncommon diagnosis.

Occurs 15,000 times per year in the US.

The SVC carries 1/3 of the venous return from the heart.

The etiology has evolved with time. In the 1950s, 50% of SVC syndromes were infectious in origin either from syphilitic aneurysmal dilation of the aorta or from tuberculosis. During the 1990s, 90% of SVC syndromes were caused by malignancy, primarily from small cell lung cancer or lymphoma. As recently as the 2010s, only 60% are malignant in origin and 35% of SVC syndromes are catheter-associated from permanent indwelling lines for chemo or antibiotic administration.

Most common presenting symptoms.

Facial edema and/or flushing or erythema - 82%

Upper extremity edema - 16%

Pemberton Sign: raising the patient’s hands above their head results in increased facial flushing and sometimes shortness of breath as the blood in the arm tries to drain but gets diverted to the face and neck vessels.

Dyspnea, coughing

Dilated superficial chest veins

Dizziness

Orthopnea

Chest pain

Syncope

Clinical Course.

Patients generally do not die from the SVC syndrome. It is rare to progress to the point that it causes cerebral or laryngeal edema or respiratory decompensation.

Patients with SVC syndrome usually die from the underlying process.

With chemotherapy, 80% of patient experience complete relief of symptoms if the SVC syndrome is caused by Non-Hodgkin's lymphoma, but less than half have complete relief of symptoms if the SVC syndrome is caused by small cell lung cancer.

CASE CONCLUSION:

The patient had surgery and was able to be extubated. She then underwent treatment for Burkitt’s Lymphoma.