Severe Refractory Hepatic Encephalopathy


Experts discuss management of challenging patients with repeat recurrence of hepatic encephalopathy despite being on maximal medical therapy.

Arun B. Jesudian, MD: Elliot, despite maybe perfect management of a patient with HE, we’ve all had those patients who have the really refractory type of hepatic encephalopathy. And what are some of your thoughts when you encounter a patient like that who’s on maximal medical therapy and still having frequent episodes?

Elliot B. Tapper, MD: These patients are particularly challenging and in many ways heartbreaking. I think [there are a few] key things to think about that don’t often come to the front of your mind when you’re meeting somebody with repeated episodes, despite adherence to lactulose and rifaximin. One is that, how much of their blood is getting around the liver? And it turns out that many patients may not have a TIPS [transjugular intrahepatic portosystemic shunt], but they could, and that TIPS might be too big for its own good, and you might narrow it.

And two, many patients have spontaneous splenorenal shunts. So they basically have their own portosystemic shunt that their body made. And these can be closed down by our colleagues in interventional radiology. And although there’s not much evidence for this per se, remember, we were talking about how many patients—particularly those with recurrent encephalopathy—tend to be muscle wasted.

And in these patients we are doing everything that we can to improve their nutritional status. And the guidelines recommend that patients receive 1.25 grams [of protein] per kilogram [of] ideal body weight. That basically comes down to 1 gram per kilogram of the weight that you get in clinic.

And the way that we give it to these patients is also important. Sometimes, if they’re not hungry, we may have to arrange [enteric] feeding or supplemental feeding with things like Ensure. But the key takeaway, one pearl if I may, is that they need a nighttime snack. The nighttime snack is 1 of the most effective ways to prevent further loss of muscle in patients with cirrhosis. They just don’t have the hepatic reserves to tolerate 6 to 8 hours of fasting the way you and I can with glycogen in our healthy livers.

So when they’re sleeping, they’re fasting almost [as if] you and I hadn’t eaten for 3 days. So the best thing that we can do is make sure that they have something nutritious, like an Ensure, some toast with peanut butter on it, something; whatever they would be willing to tolerate that’s loaded with calories, and hopefully protein, at night.

Arun B. Jesudian, MD: And let me ask you a question, because I continue to see it sometimes. Old recommendations about a low-protein diet as a management strategy for hepatic encephalopathy. What are your thoughts on that?

Elliot B. Tapper, MD: This is one of the things that it continues to persist within the community. And I’ll say two things. One, if you eat a steak, your ammonia is going to [go] up. But that does not mean that it’s going to cause hepatic encephalopathy, because there’s more to that clinical syndrome than just the ammonia level. But over time that ammonia will continue to erode [your] muscle stores. So starving somebody of protein is only going to worsen their sarcopenia, and paradoxically increase their overall burden of ammonia.

Now if you’re really worried about that, and really worried about protein, vegetable protein is your go-to. What do you think about that?

Steven L. Flamm, MD: I absolutely agree. This whole ammonia, the whole protein-restriction thing is, it’s another one of those things in medicine that was taught to people many years ago and just can’t go away, and it needs to go away.

Now people shouldn’t necessarily be eating 5 grams per kilogram of body-weight protein. That, of course, would be bad. But they are supposed to have a certain protein intake, and nutrition services, dietitians, know this. And if you have patients who are very sick like this, who’ve had encephalopathy, who have decompensated liver disease, it’s not a bad idea to have them see a nutritionist actually, because they can help you manage the patients and help the patients.

And I do want to amplify one thing Elliot said: this refractory or recurrent encephalopathy thing. Two things. Look first for precipitating factors that were missed. Again, diuretics [are] the most common in my experience. Patients [who] are on diuretics because they have edema, because they have ascites, they’re complaining to doctors, maybe not you, and diuretics are started. Diuretics are increased, and that has to be dealt with in patients [who] are having recurrent encephalopathy.

And the second thing is to get a good imaging procedure to look for these large, spontaneous portosystemic shunts. And you need good imaging. That’s not an ultrasound. It’s usually not even a CT [computed tomography scan]; it’s often an MRI [magnetic resonance imaging]. MRIs will pick up these spontaneous splenorenal shunts. They may be periumbilical. They can be different places in the abdomen. Everybody doesn’t get them for some reason. Certain patients do. Everybody had collaterals with HE. But often they’re so small [that] you can’t really see them. Those you can’t do anything about. But patients [who] have a giant one for some reason, they are the ones in the setting of refractory encephalopathy that you have to identify. And if you can’t treat them medically, these patients, because they’re breaking through, those are patients you would consider referring to interventional radiology to embolize or close off that shunt.

Arun B. Jesudian, MD: Great point. And I found that if I suspect [a potential] shunt, it’s helpful to give the radiologist a heads-up, because it’s not something they’re always used to reading or looking for.

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