Synopsis
Prof. Bernhard Gebauer reviews a complex case involving a 23-year-old female with hepatomegaly, persistent abdominal pain, Factor V Leiden mutation, focal bleeding, and refractory ascites. CT scans revealed BCS with an enlarged liver compressing the inferior vena cava and collateral veins. Following Baveno VII recommendations and Prof. Dr Ziv Haskal’s literature, a gun-sight approach was used to create a direct intrahepatic portosystemic shunt (DIPS). The procedure, involving advanced materials and meticulous technique, significantly reduced the portal pressure gradient from 46 to 25 mmHg. The patient remained symptom-free for nine years, highlighting the efficacy of these novel techniques for managing complex vascular conditions.
Transcript
(00:15):
Hello, my name is Bernhard Gebauer. Today’s topic is DIPS with gun-sight technique in Budd-Chiari syndrome and it’s part of TIPS Complex Case Educational Series brought to you by Cook Medical.
(00:29):
So the case I would like to present you is a 23-year-old female. She had ascites, hepatomegaly, and abdominal pain for more than three years. She has a heterozygote factor V Leiden mutation and she had recurrent oesophageal bleeding and ascites. It was a referral from another hospital that is 600 kilometres away for evaluation of a liver transplant. And if you look at the image, there is a suspicion of Budd-Chiari syndrome in that patient.
(01:03):
You see, for Budd-Chiari syndrome, the typical sign of an inhomogeneous perfusion of the liver, it’s impossible to really see the hepatic veins, of course. And what is also very, very typical is the very, very enlarged Segment One in these patients because usually the Segment One has its own liver veins draining into the IVC. So usually from the Budd-Chiari syndrome, the Segment One is not that much affected. So it could enlarge, and then that patient, that Segment One, was really much enlarged.
(01:50):
So if you go through the images, you see a lot of collaterals in that patient. You see this arterial inhomogeneous contrastation, and then the venouses face, additionally, a lot of collateral veins that come from a compression of the IVC. So see that the IVC is much compressed here in the hepatic segment due to the enlargement of the Segment One, and that’s the reason why there are so many paravertebral collaterals in that patient.
(02:24):
So if you go to the Baveno recommendations again, then you see that then, obviously, Budd-Chiari syndrome is a good indication for TIPS insertion. And that patient liver transplant, because the liver function was still in good condition, so liver transplantation was foreseen in the future, but not at the moment. And so that is all we were requested, if it would be possible to place a TIPS in that patient.
(02:59):
She also had therapy-refractory ascites and recurrent bleedings, and you see the challenge was, if you see the CT, here’s the IVC, and you see due to the enlarged Segment One, here is the portal vein. So the distance is very, very long. I’ll show you a reconstruction of that image as well. Here you see the compression of the IVC due to the enlarged Segment One, and here you see the distance. Here is the IVC, where the liver veins are draining, and here is the portal vein, so a very, very long distance to go. I measured that and it’s something like, I think 12 centimeters, something like that. So very, very difficult to do it with a conventional TIPS access.
(03:58):
So if you go to the literature, then Ziv Haskal described what he calls the gun-sight approach. Meaning that you put a snare into the IVC, you put transhepatically a snare into the portal vein, and then you turn the angiography as long as both snares are over each other, and then with a needle, you puncture through both of the snares and you establish a through and through wire. And with that, you place the TIPS in the patient.
(04:36):
Here is another drawing illustrating that, how you could do that, and here you see one snare, and what’s not drawn here is the second snare.
(04:47):
So how did we do the DIPS or TIPS in that complex case? First of all, percutaneously we punctured the portal vein. You see the small needle for the puncture of the portal vein, and through that we put in a Goose Neck snare, and then we directly percutaneously punctured in the direction of the IVC. In the IVC, as I told you before, was a snare. You see here the snare marking the dimension of the IVC, and under fluoroscopy we punctured into that snare. So we were able to snare the wire, then we had a through and through wire, and with that we advanced the 10 French sheath just in front of the portal vein, and then we redirected the wire into the portal vein. And with that, we were able then to have access to the portal system transhepatically. We’ve withdrawn here the, can you see the, another sheath that was in there percutaneously? We’ve withdrawn that and glued that with a fibrin glue, and then we put in here one VIATORR® stent graft and two extensions. For that we used two Luminex™ stents to establish the TIPS tract in that patient.
(06:17):
The material we used was a Cook Micropuncture® access set for the puncturing of the portal vein. We had obviously two snares, a large one, 25 millimeters for the IVC, and the smaller one, 10 millimeters for the portal vein system. We used one VIATORR stent graft and an extension with two uncovered, BARD Luminexx stents.
(06:42):
The outcome, if you see that the pressure gradient dropped. The pressure gradient was 46 mercury where we started and it dropped to 25. Patient is now nine years post DIPS procedure with gun-sight technique, without ascites and bleedings, and even without liver transplantation at the moment.
(07:03):
There are possible modifications. The original technique is that you put a snare here and the second snare in the portal venous system, and then you puncture with a needle through both of the snares. There is a modification you can use, if you put a snare here and you put a balloon there, sometimes that is even more easy, especially if the IVC is relatively small at that position. And the other option is to put a snare here and to take a transsplenic access, especially if the portal venous system is very small and it’s very difficult to access it transhepatically percutaneously, then a transsplenic access is much easier to do that.
(07:55):
So the resume would be percutaneous access is very helpful in difficult and long TIPS procedure tracts. The gun-sight technique is, as we used, is a very nice way to do that. There’s also the opportunity to do it with a balloon, so balloon-assisted techniques. You can use additional transsplenic access, and also in the literature it is described to use CT- guided access. Actually we are more in favor of an angiography access together with ultrasound.
(08:27):
That wraps up this complex case. We hope you find it useful. If you have any comments or case ideas you’d like to see in future episodes, send your feedback to TIPScases@cookmedical.com and be sure to keep an eye out for the next complex case.
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Prof. Bernhard Gebauer was a paid consultant of Cook Medical at the time of recording.
Goose Neck is a registered trademark of Covidien LP.
VIATORR is a registered trademark of W. L. Gore & Associates, Inc.
Luminexx is a registered trademark of C.R. BARD, INC.