the specific treatment options available in IR specifically, starting with sclerotherapy.
Sclerotherapy closes off a malformation by injecting sclerosant into the sacs filled with the venous blood or lymph until the sacs collapse and so this will be direct puncture of the malformation. Sclerosant irritates the vessel wall resulting in clot formation.
The clot stops blood flow through the vessel and the inflammation of the inner wall will destroy the vessel, which the body will replace with scar tissue. Patients often do require repeat treatments to block all of the abnormal vessels.
We do expect that the area will swell and the patient will have pain once the treatment is completed which is good to know, you know, when we go to discharge them. We expect this to swell, often it'll become a lot larger than it was before, which is completely normal.
Swelling typically lasts about three to five days post treatment and then often times it's treated and the patient may go several periods of time without needing to come back. So I'm going to talk a little bit about the sclerosants that we use at UNC for treatment,
starting with venous malformation sclerosants, we use STS, Bleomycin and Ethanol. And the mechanism of action generally speaking of all sclerosants, they damage endothelial cells leading to inflammation and fibrosis. Effect depends on the concentration of sclerosant used
and contact with the endothelial cells, they are generally bound to blood proteins rendering them ineffective. The process to treat venous and lymphatic malformations is very similar and there is overlap in the sclerosant that may be used. So STS is three percent sodium tetradecyl sulfate.
Mechanism of action, it causes direct endothelial cytotoxicity, it disrupts the cell membrane causing thrombosis, leading to occlusion of the injected vessel. The benefits of using STS, less swelling and neuropathy and no cardiac toxicity. It's minimally invasive and generally safe and effective,
but we have to talk about possible complications. And I will preface this by saying that I have not seen any of the complications that I will show you, but, you know, if they extravasate or, you know, get into surrounding tissues it can cause skin ulceration.
If it's, if you're doing arterial access it could cause necrosis, DIC and swelling. Swelling we are going to, you know, obviously be particularly careful with our patients that are having like the tongue sclerotherapy, so. All right, the next sclerosant is ethanol,
we use 100 percent ethanol which, again, causes direct endothelial cytotoxicity, disrupts cell membranes, denatures proteins, causes a thrombosis, it's pretty effective and inexpensive. Possible complications, a lot of the same kind of stuff, ulceration, especially with
arterial access, microemboli, DIC, swelling. Bleomycin is another agent that we use, Bleomycin is an antineoplastic and an antibiotic agent. It inhibits DNA, RNA and protein synthesis, it's pretty inexpensive and it's got well known effectiveness, minimal
and controllable side effects. Because it is an antineoplastic we obtain this from our Chemotherapy Pharmacy and you need to use, you know, precautions because it is a chemotherapeutic agent. So when you're handling it and delivering it
you just have to use those precautions. Possible complications, fibrosis, alopecia and you can see there is flagellated hyperpigmentation. This is an actual patient that we treated, this is the lip venous malformation. She was treated with Bleomycin and this is
a picture of her five days status post the sclerotherapy with the Bleo. Mom was concerned about the swelling which we looked at the pictures and we said this is completely normal for it to swell like this after treatment.
You don't want it to continue past, you know, a week and a half or so. Lymphatic malformations use many of the same sclerosants as in venous. Macrocystic we use Doxycycline, STS and Ethanol, for our microcystic we'll use Bleomycin,
I've already discussed STS, Ethanol and Bleo. So Doxycycline is an antibiotic that inhibits protein synthesis, causes direct endothelial cytotoxicity, mitochondrial level cell death and it's generally safe and effective. And we have a patient here that was treated,
he was the lymphatic malformation that I showed you earlier that had the giant neck malformation. And he was sclerosed with Doxycycline and you can almost not see that it was even there. Possible complications with Doxy, skin necrosis, neurotoxicity and neutropenia.
And just generally speaking with our sclerosants, any one of them can cause skin necrosis because, especially when we're dealing with superficial malformations, if the sclerosant diffuses, causes extravasation, if you have arterial penetration or direct injection into the artery or use of a tourniquet.
So basically the physician just has to be very careful when they're injecting the sclerosant.
This is another example. This is a complex aneurysm. This is an aneurysm that's actually involved the common trunk of the SMA and the splenic artery. So we can't embolize the splenic artery. That's not a good thing.
This shows you a lot of teaching points, a lot of technical teaching points about the management of these complex aneurysms. So this is a complicated... This is a complicated aneurysm. It involves the common trunk of the SMA and the celiac axis.
So the plan is, we need, going down low, is actually the SMA, so what we need to do is to embolize the splenic artery, proximal as possible, okay, to allow as much collateral as possible to go to the spleen. Do a stint graft from the aorta into the SMA to exclude the aneurysm and then thrombose it.
The embolizing material in this case, plugs, it could be coils as well, would do the same principle of preventing back bleeding into the aneurysm. So you're kinda seeing all these principles being put together in this complex procedure.
So the first thing, we went up to the splenic artery and we embolized it with plugs, okay and that's kind of embolized, and then we went into the SMA and put balloon expandable stints and excluded the aneurysm. Here you see the aneurysm with the eggshell calcification,
see this anatomy perfectly, almost on the dead lateral. We put the stint grafts. On our follow up CT, the aneurysm had shrunk partially thrombosed, but still there is still more flow in the aneurysm. So this most likely kind of like a type 1 endo leak
into the aneurysm. The patient was on Plavix and on Aspirin. So technically, what you can do is to put another stint proximally and close that kind of type 1 endo leak. What we chose to do is actually stop the Plavix and stop the Aspirin, just stop it,
'cause they're anticoagulants, stop it for a month and follow up CT. That helps actually thrombose it. Okay so instead of subjecting a patient to another procedure, you just stop the Aspirin and Plavix for a month. Maybe that's enough to help it thrombose.
Then resume the Aspirin and Plavix, and that's all we needed to do is just stop the Aspirin and Plavix. Kind of several technical and medical teaching points
And the etiology of this is numerous, it's diagnosis of exclusion, it's diagnosed in less than 1.5% of liver transplants in the United States and Japan. For some reason it is diagnosed at 25% in Germany, so somebody's overcalling it and somebody's undercalling it.
Over the years, I can tell you, 10 years ago, most transplant surgeons in the United States did not believe in it. Now they're believing it more and more, they're calling it more and more. It probably is happening in 1-2% of transplants
in the United States, not 25%. This is a perfect example where the hepatic artery is open, the hepatic artery is so slow that the actual splenic vein catches up with it. The splenic vein actually catches up with the flow in the hepatic artery.
That's how slow and stagnant that hepatic arterial flow is. Your differential diagnosis is a fistula. It's that there's a fistula between the hepatic artery, that's kind of differential diagnosis. However, this is truly a splenic phase. You see the spleen.
Contrast has gone right through the spleen up, down the splenic vein and caught up with the hepatic artery, so this is a true, slow flow in the hepatic artery in a transplant. Initially, we thought that the best way to deal with this is to actually embolize the spleen and there are two types.
There's a GDA spleen, and a splenic artery steal. So there's a GDA steal, which is even rarer, and a splenic artery steal. So initially we thought that the best way to deal with this is just to embolize the spleen, because this is splenic steal.
The spleen is stealing flow from the hepatic bed. Simple, simple thought process. Embolize the spleen, or embolize the GDA in case if it's a GDA. But it's actually a lot more complicated than that.