29 April 2009


I saw something recently which I have seen only once before, as a medical student.  It's really quite rare in this day and age, but the presentation was so classic that I knew it practically as soon as I laid eyes on the patient.  I was very satisfied to make the clinical diagnosis in advance and be proven correct by the work-up.

The Chief Complaint was "shortness of breath;" the patient complained of trouble breathing when he laid down, and some extremity swelling, so I kind of assumed that he had CHF.  He was about 60, and had no significant medical history, and the vitals looked fine.  The only odd thing I noticed that that he complained that his face was swelling, too.

The interview wasn't much more revealing than that.  He had a dry cough and diminished exercise tolerance, but not a lot of other symptoms.  No fevers or chest pain.  He was losing weight, which I thought was odd in a potential CHFer, who usually gain weight as they retain fluid.  He was a long-term smoker.  Also, he added, there was a sense of fullness in his neck that he thought was really uncomfortable, and that's what made it hard to breathe when supine, because he felt like he was choking.

The exam was noteworthy for facial swelling and edema, with a distinct dusky flushing in the skin color.  The neck was diffusely swollen, but I could not make out any lymph nodes or cords or other masses.  The neck veins were very prominent (which is defined as "the ER doctor noticed them").   Strikingly, there were varicose veins in the shoulders, chest, and upper extremities, all of which were distended, and indeed, the upper extremities were swollen and shared the same dusky color as the face.  The skin below the chest was normal in color, and there was no swelling or abnormality in the lower extremities.

I'll skip the x-ray and go straight to the CT (click images to embiggen):



Uploaded with plasq's Skitch!

What we have here are images of the chest with IV contrast.  The contrast shows up as bright white, mostly inside blood vessels. The contrast was injected through an IV in the right arm. There is a mass adjacent to the right hilum, probably an invasive bronchogenic carcinoma.  This mass is compressing the superior vena cava (SVC), which returns blood from the head and arms to the heart.  It's the main vein in the chest, thin-walled and usually a couple centimeters in diameter.   The SVC is almost completely effaced in the top picture, and reduced to a millimeter in size in the middle one.

The obstruction of the vena cava causes increased venous pressure in the arms, head and neck, which is responsible for the dusky color and swelling.  The fullness in the neck is probably from massive distention of the jugular vessels.   The increased venous pressure causes blood to find alternate pathways back to the heart, in this case through venous collaterals, mostly subcutaneous.  You can seem them in the right armpit (on the left of the picture) and under the skin in the front of the chest, all bright white.

This is called SVC Syndrome, and given the early detection of lung cancers these days is pretty uncommon (also, syphilitic aortic aneurysms and tuberculosis are less common than in the past).  This patient had all the classic symptoms and clinical findings.   The tumor has to get pretty big to compress the Vena cava.  This patient did note that his cough had been going on quite a while, but his policy of "seeing the doctor ever ten years, whether I need it or not" turned out to not be the best one.  His 60+ pack-year history of smoking didn't help, either.  This is a pretty terrible diagnosis for the patient.  It's too early to say, but this looks like a non-survivable lesion.

My six-year old asked me the other day: if smoking is bad for you, why do they let you do it?  We had a little conversation about freedoms which was quite frankly over his head.  Little kids are too used to being told what to do to understand the concept and value of liberty.  He came away firmly convinced that it should be a rule that you're not allowed to smoke.  After I had a very difficult "you have cancer" conversation with this gentleman, his wife and three daughters, it was hard not to agree with my six-year old.

This is also one of the weird contradictions of being an ER doc.  I was, I admit, pretty juiced about this case.  It was rare and I was very satisfied about making the diagnosis.  But it was also awful for the patient.   I remember something one of my teachers taught me in residency: "Never forget that your best day is by necessity someone else's worst day."


  1. Great case Shadowfax. And I agree that it means really bad news for the patient.

    Forget an ER doc, even I, a radiologist could come up with a working diagnosis of "SVC syndrome" from your description before I saw the images.

    I’m sure your radiologist must have given you more reconstructed images that show all the associated findings like anterior and lateral chest wall venous collaterals better.

    Two other notable findings that I find in these images are a significant right pleural effusion and a dilated Azygos vein.

  2. Saw this at least three times as a Hospice nurse, and it is one of the very few hospice "emergencies" requiring immediate treatment. Once you have seen the varicosities and edema along with dyspenia, you never forget it. My last case was lung CA with mets doing the compression. After treatment, the 37 year old father lived another 6 weeks, long enough to say goodbye to his kids.

    Pattie, RN

  3. I've diagnosed this once in twenty+ years. I recall the young woman stating "my face swells up when I lye down." It was an adeno of unknown origin.

  4. You can also get SVC syndrome with Hodgkins and non-Hodgkins lymphoma. I was in the early stages of it when dx'd, so I recognized the symptoms you were describing here within the first few sentences or so. :)

  5. It's a RIBBFOMP, a term I have unsuccessfully been trying to get my entire residency to use. really interesting but bad for my patient.

  6. What is a RIBBFOMP, Graham? A Google search didn't give me an answer, but led me to this case from WhiteCoat.

  7. Excuse my stupidity Graham & Shadowfax. Can't believe I didn't notice that.
    My excuse - I did this early in the morning without having my coffee ;)

  8. Did this guy have a positive Pemberton's sign?

    Not that you need a Pemberton's sign when the rest of the clinical findings are so suggestive.

  9. As we say in Radiology, you never want to be a "great case".


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