Overlapping TIPS stents in Budd-Chiari with distorted anatomy: case one

Dr Homoyoon Mehrzad presents a complex case of Budd-Chiari syndrome (BCS) recurrence in a 42-year-old woman. Facing hepatic stenosis relapse, Dr. Mehrzad employed a TIPS procedure as a long-term solution. Initial treatment involved venoplasty and strategic placement of two uncovered metal stents. However, two years later, stent occlusion necessitated urgent intervention. Dr Mehrzad navigated challenging portal vein access, placing additional TIPS stents to restore patency. This case highlights the importance of novel techniques and comprehensive follow-up in managing complex vascular conditions.

Dr Homoyoon Mehzrad (00:15):
Hello, I’m Dr. Homoyoon Mehzrad, Consultant and Interventional Radiologist at the Queen Elizabeth Hospital of Birmingham, United Kingdom. Today’s topic is Overlapping TIPS stents in Budd-Chiari patients with distorted anatomy, and it’s part of the TIPS Complex Case Educational series brought to you by Cook Medical. So let’s begin with today’s first talk.

(00:37):
I want to discuss a case of a 42-year-old lady who had Budd-Chiari syndrome and presented to us with intermittent abdominal pain and ascites in 2010. She had venography and venoplasty at that time. The venography demonstrated single patent right hepatic vein with a stenosis at its origin. She went on and had the venoplasty as mentioned and this improved her symptoms. She was doing well for the first few years with annual surveillance but in 2015, she had a recurrence of the symptoms and came back to the hospital where we did the repeat venography demonstrating recurrence of the original stenosis.

(01:17):
So rather than repeating the venoplasty at this time, we decided to place two hepatic vein stents as a longer-term solution to open up the stenosis and there was a good outcome of this. Let me show you some pictures. So this is the CT scan of the patient that shows a large, distorted liver. You can see it’s quite irregular. There is a different enhancement throughout the liver. It’s slightly abnormal. As you can see, the spleen is enlarged. One thing to note, she’s got some ascites but the other thing to keep in mind is the portal vein, which I will highlight here, is on really to the lateral side of the liver. So rather than being centrally positioned, it is to the… You can see the portal vein here basically to the side of the liver and that’s a key thing to keep in mind. Here, again, we can see that the anatomy is distorted. There is central atrophy of the liver and it’s quite abnormal.

(02:28):
Let me show you some pictures of the venography we undertook in 2015. You can see that we’ve gone beyond the stenosis here. We’ve injected contrast. There is back pressure. The contrast is backfilling into the veins and there is a stenosis at the origin of the vein. You can see along here there is a stenosis between the hepatic vein and the right atrium. We undertook a balloon plasty. You can see the wasting on the balloon there where the stenosis is.

(02:59):
After the venoplasty, there was still holdup of contrast without much improvement in the stenosis. So this is when we undertook placing two uncovered metal stents to open up the stenosis and this is the final position of the two stents. When we now repeat the venography, there is good flow through the stents, improvement in the stenosis, and flow back to the right atrium. This patient went on to have annual venography which showed that there was still patent stents. She was obviously anti-coagulated. There was a little bit of narrowing at the distal aspect of the stents, but we found we could control that with some balloon plasty. Here, you can see it being ballooned with a seven-millimeter balloon. Again, you can see some new intramural hyperplasia going on inside the uncovered stents, but they were patent, and she was symptom free.

(03:57):
Unfortunately, in 2017 she represented quite unwell. She came back with abdominal pain, features of acute liver congestion, and she was admitted onto our intensive care unit. She was placed on the super urgent liver transplant list and the clinicians came to us to arrange an urgent CT scan and also asked us whether there was any intervention we could do to improve her symptoms and prevent her having a liver transplant at that stage. So we said, “We may be able to do something.”

(04:33):
So this is the CT scan, we repeated it in January 2017. You can see that the hepatic vein stents are there, but they’re occluded. There’s the stent. The liver is obviously still abnormal. There is fluid and the liver looks quite congested now. You can see a patchy enhancement of the liver, ascites, and thrombus within the stent itself basically. So we undertook a venogram at this time and we found that the hepatic vein stents had occluded.

(05:06):
You can see that we then decided with the team to undertake a TIPS stent to try and relieve the congestion of the liver and the only way to do this was to go through the side struts of the uncovered stents. I’m using Cook Rösch-Uchida TIPS set. We undertook a TIPS procedure. Now, key things to keep in mind here on these images are that the hepatic vein stents are occluded. We’ve got our long sheath down through here. We have gone through the side strut of the hepatic vein here and we are trying to puncture the portal vein which we did after a couple attempts and the anatomy is quite distorted.

(05:49):
So you can see looking at the sheath alone, how torturous it is and eventually, we go into the portal vein which you can see here. Rather than being in a central position, it is quite distorted and going around lateral aspect of the liver, okay? So this was a very distorted anatomy. We used the combination of ultrasound and fluoroscopy to get into this but really, it was quite taxing and one of the most difficult TIPS procedures I’ve done.

(06:17):
Once we got into the portal vein, we secured our position with the wire and we started to place balloon plasty in the side struts of the hepatic vein and we placed our TIPS stents, which you can see there are two here, through the occluded hepatic vein stents into the portal vein. There, you can see we’re doing the plasty with the balloon.

(06:45):
This is the final picture at that first stage. We can see we’ve got an occluded hepatic vein stents. We’ve got the TIPS stents in the sutured… We’ve placed them through the hepatic veins into the portal vein. When we inject dye, there is good flow of contrast back into the right atrium, so this alleviated the congestion and pressure. However, two days later we found that she initially improved but there was a deterioration in symptoms. We brought her back for a second venogram where we found that the TIPS stents had partially occluded. You can see that there is thrombus within the TIPS stents as well as the hepatic vein stents so there isn’t much flow and it slowed significantly.

(07:30):
So we said we need to do a thrombectomy to try and remove some of the thrombus and we may have to extend the TIPS stents even further. So there are already two TIPS stents in there and two hepatic vein stents. Once we got through, we used a thrombectomy device to get rid of the clot burden. We ballooned the stents as well. And then, we found that the problem was really at the distal end. The distal end was impeded. It was in a side part of the portal vein and not the main portal vein here. So we decided to extend the TIPS stents distally. We placed another TIPS stent, this would be the third stent, around corner into the main portal vein which was, again, very distorted. But this time, we found that there was very good flow through the portal vein through the three TIPS stents back up to the right atrium.

(08:23):
This is a reconstructed picture. You can see in the two images; this is the stents within the patient, and this is what they look like. So you can see the hepatic vein stents here and through that, we’ve taken the TIPS stents through into the portal vein, but this just shows you the distortion of the anatomy we were dealing with.

(08:46):
That patient has since been well. She comes for annual checkups. The TIPS stents are working very well. There hasn’t been any more thrombus, no more issues, and she remains anti-coagulated. That wraps up this complex case. We hope you found it useful. If you have any comments of case ideas you’d like to see in future episodes, please send your feedback to TIPScases@cookmedical.com and be sure to keep an eye out for the next complex case. Thank you.

Dr Homoyoon Mehrzad was a paid consultant of Cook Medical at the time of recording.