Call Me Dr. House

Let me preface this by saying that I don’t regularly watch the show, but this week I had my first “Dr. House” moment — and by that I don’t mean that popped a Vicodin and got belligerent with my colleagues. Rather, I had my first light-bulb moment, where all the seemingly disjointed aspects of my patient’s signs, symptoms and hospital course came together to form a text-book presentation of a rare disease.

Here’s how the magic happened (disclaimer: certain aspects will be changed to ensure the privacy of the patient, and no identifiers will be used). He was a 20-something year-old with no past medical problems, who, though completely healthy just several weeks ago, came into the ER complaining of flu-like symptoms (malaise, aches, and fever) for the week prior, with severe right-sided chest pain which began only 2 days before. In the ER he was found to be febrile, with findings on chest X-ray suggestive of pneumonia with a small amount of fluid (known as an effusion) surrounding the lungs. Thinking it was a run-of-the-mill community-acquired pneumonia, he was started on Levaquin, a commonly-used and appropriate antibiotic for such a case, and was admitted to the general medicine floor for monitoring.

Up on the medicine ward he began to show signs of significant respiratory compromise, with his O2 saturation (the metric used for determining how much oxygen someone is actually getting into their blood — and subsequently their organs) dropping below safe levels. In response to his worsening condition he was given supplemental oxygen and taken for a stat CT scan of his chest. In this type of instance, a CT offers a fast, reliable way to view the lungs with a level of detail that a plain-film chest X-ray lacks. With regards to this patient, what they saw on the scan was worse than they had anticipated from the X-ray.

Scrolling through the results it was obvious his lungs were in bad shape. There were, indeed, infiltrates (a radiologic term basically meaning junk that shouldn’t be there) within the lung tissue proper, as suggested by the chest X-ray. But what jumped out most was the extent to which the fluid first seen on the X-ray had progressed. This buildup of fluid within what’s called the pleural space (the space between the lining of the chest wall and the lining of the lung itself), isn’t all that unusual in hospitalized patients, but in this case was complicated by infection. From the looks of the CT, it was clear that the patient had what is called an empyema, which is a build-up of pus and all kinds of nastiness within that pleural space. But unlike your every-day effusion, in the case of an empyema, the body tends to react aggressively and wall it off, making it harder for antibiotics to penetrate and work their magic on the infection.

So even though the presence of the empyema itself wasn’t good news, at the very least, they had a partial diagnosis: the chest pain and the trouble breathing were being caused primarily by this infectious buildup of fluid that was pressing on his lung and making it hard for him to take a breath. Now the only problem was that the patient was becoming hemodynamically unstable, a sign that the infection had progressed to such a point that the body was beginning to lose control. His heart was racing, his blood pressure dropping, and he was spiking a high fever, making a secondary diagnosis of sepsis a very real possibility. Of the various causes associated with this condition, the admitting doctors suspected that the culprits were bacteria in his blood, and drew several samples for culture (along with a good number of other samples for a slew of different tests and analyses). Meanwhile, they knew they needed to tap the fluid that was building up, both to relieve the pressure and get a sample of the fluid itself for analysis and culture. But hospital bureaucracy being the way it is, this took about a day and a half to execute, during which time, though hemodynamically stable, the patient continued to spike fevers and labor with each breath.

And as if all this weren’t enough, while convalescing in the ICU (Intensive Care Unit) waiting for his tap, the patient began to have some transient swelling localized only to the right side of his face and right upper extremity. Now, localized swelling can mean many things, but the way it presented in this patient suggested the formation of a clot in the veins around his neck or upper extremities, which was confirmed with a venous ultrasound which was able to visualize an active clot in his right internal jugular vein. Now, in the average 60+ y/o hospital patient, venous clotting is not unusual, and is something that is actively addressed due to the serious complications it can cause down the line. But even in those patients for whom it’s more common, it is much more likely to occur in the lower extremities. Needless to say, in a young, previously healthy individual such as our patient, it was highly unusual. So the hematologist chalked it up to local trauma to that area and ordered close monitoring for any progression of the symptoms or signs that the clot was expanding.

When they did finally get the interventional radiology team to sample the fluid, for the most part it was what they’d expected: a turbulent (signifying a build up of pus), bloody mix, although the amount of fluid that they were able to drain was less than anticipated. This was because the empyema had walled itself off into various little sections, termed loculations, which, in itself, is never a good sign. It was clear that the patient would need a surgical procedure called a thoracotomy and decortication to enter into the infected, scarred pleural space and tear down all the loculations, such that the full extent of the fluid buildup could be manually drained.

Enter: the cardiothoracic surgery team, who, after hours in the operating room, managed to successfully rid our patient of all the infectious build-up that had set up shop around his lung. The surgeon noted that the pus had extended all the way from the patient’s armpit down to the base of the lung, and was the most extensive empyema he’d ever dealt with. And so, with 2 chest tubes in place to drain any subsequent build-up (and prevent another empyema from establishing itself), the patient was sent to the ICU for close monitoring to ensure that his breathing was stable in the first few days following the surgery.

When he was finally deemed stable enough to breathe on his own, he was transferred to my team on the general medicine floor; his chart — which at this point contained countless progress notes from the various specialists, ICU doctors, and residents who had been taking care of him up to that point — came along with him. It was my job to make sense of this mess of information and present it to my senior resident, so that we would be able to pick up his care where everyone else had left off.

This was no easy task; the binder holding all the various records, notes, and test results was bulging, and looked like it was about to explode. But page by page, I went through the chart, deciphering the chicken scratch and trying to fit the pieces together. Why was such a young guy in such bad shape? He had none of the risk factors for having bacteria in the blood (e.g. intravenous drug use) and wasn’t chronically immune suppressed (e.g. in patients with HIV), and moreso, had no history in his family of excessive clotting, which might have explained the clot in his jugular vein. Then I came across the results of the blood cultures they’d taken soon after the patient arrived at the hospital. Turns out they were positive for an organism called Fusobacterium necrophorum, an uncommon pathogen in general, which is even less common in terms of bugs that can enter the blood and cause sepsis, like that seen in this patient.

And yet, that name, Fusobacterium necrophorum, sounded familiar — probably something I’d come across during the massive information binge leading up to my USMLE Step 1 exam — and prompted me to look a little further into the matter. A single database search is all it took: lo and behold, that this little Gram-negative bacterium was the key to making the diagnosis.

It turns out that the patient had a textbook presentation of what’s called Lemierre’s Syndrome, a series of specific signs and symptoms all due to infection with Fusobacterium nerophorum. The French bacteriologist, Andre Lemierre, first described the syndrome in a series of young, previously-healthy, male patients presenting with sore throat, fever and respiratory distress in the early 1900′s. The patients all had the commonality of Fusobacterium in their blood, along with symptoms of recurrent fever, violent chills (termed rigors), respiratory distress (due to lung involvement), and in many cases, infection of other organs as well (including the liver, heart, and even brain).

He realized that the sore throat they were suffering from was due to the Fusobacteria itself, and that once in the tonsils, the organism had free reign to enter the blood stream via the tonsillar veins. This is where it gets interesting. The organisms have several factors on their outer surface that, in certain individuals, can cause blood to clot more easily, leading to an event called supperative thrombophlebitis, in which a clot made up of platelets (normal components of clotting) and large numbers of bacteria, sets up shop in a vessel (in this patient’s case, the right internal jugular vein) and just hangs around, throwing off little bits of clot and bacteria that travel to the rest of the body with every heartbeat. This allows the infection to spread from the initial site in the tonsils to the rest of the body, and also explains why the lungs are almost always involved in patients with Lemierre’s Syndrome — after all, the lungs are one of the first places that oxygen-poor blood from the veins travels as it’s pumped through the heart to be re-oxygenated. In other words, this was the pathophysiologic process underlying our patient’s empyema!

Still, one thing was missing from our patient’s story. He noted having had flu-like symptoms for the week prior to his admission to the hospital, but never mentioned a sore throat, which was one of the tell-tale signs of Lemierre’s Syndrome. This seemed like a set-back, but everything else seemed to fit into place so well. What’s more, I knew that much of the time the notes written when a patient is admitted to the hospital are imperfect and lack bits of useful information (which is why most any attending will tell you to always hear the patient’s story yourself). So I went into his room and took my own history of how exactly everything had played out, only to find that he had, in fact, had a sore throat, with swelling and pain which was worse on the right side (the same side as the clot!), but which had improved shortly before the onset of the flu-like illness and respiratory problems.

And with that, the diagnosis was sealed: bacteremia, supperative thrombophlebitis, pneumonia and parapneumonic empyema due to Fusobacterium necrophorum pharyngitis. In short, textbook Lemierre’s Syndrome.

On an extended course of high-dose, intravenous antibiotics, our patient slowly improved and was able to go home (albeit still on a massive dose of oral antibiotics for several weeks), having come through a rare and serious infection which, back in the days of Lemierre, would have most likely killed him. Lucky for him, it’s 2012, and antibiotics really do work. (Modern medicine for the win!)

But you can throw antibiotics at an infection all day, and there’s a good chance the patient will improve. Nonetheless, there is something incredibly fulfilling in making the diagnosis, and getting to the very bottom of what is going on. As a budding physician, I find myself continually humbled by the herculean processes of diagnostic medicine, but find near-limitless inspiration in the magic I felt in that moment of discovery, when I was finally able to give a name to the previously nameless entity that was afflicting my patient. That “House” moment is what keeps me going, and reaffirms that medicine really is where I belong.

Picutre of author

About Justin Fiala, MD Candidate

Justin is currently in his third year of medical school at UIC's College of Medicine, and is hoping to pursue a career in internal medicine. He has a strong interest in addressing the health needs urban communities and is part of the College of Medicine's Urban Medicine program. Aside from academics, Justin enjoys cooking, listening to public radio, and perusing the New York Times website. He is also a trained pianist and self-professed lover of all kinds of music.

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2 Responses to Call Me Dr. House

  1. AJN says:

    Good Job with the catch/diagnosis of Lemierre's! LS is a crazy syndrome, and I know because I had it myself. I hope to never miss the diagnosis myself (now that I'm a PA student), and now I'm sure you never will too.

  2. Lin yugene says:

    I encountered and treat my first patient Lemierre disease with my colleagues at Taiwan just 10 days ago. I can realize what you experienced .We did more aggressive ( pleural cavity decortication,chest tube drainage ,removal of internal jugular vein ) . Patient was a 25 years old female . She was discharged after about 2 months admission. Mainly due to difficult weaning from ventilator support.
    Thanks for your sharing ….!