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Dear Validig Colleagues
I would like to ask your opinion on the case of splenic thrombosis and gastric varices bleeding
We are following a 42-year-old female patient with previuous diagnosis of PH in liver cirrhosis and patent TIPSS, inserted 2 years earlier after recurrent variceal bleeding and ascites requiring paracentesis.
She came to our department for recurrent hemorrhage from gastric varices, endoscopically treated with cyanoacrylate.
The CT scan showed huge collateral peri-splenic and peri-gastric circles, thrombosis of the intrahepatic portal vein and of the ascending branch of the splenic vein up to the splenic hilum, patency of TIPSS, patency of mesenteric vein, non-occlusive thrombosis of trunk portal vein; splenomegaly.
The patient was previously followed-up in another Center, and received immunosuppressive therapy because of a suspected autoimmune etiology of liver disease (high ANA titer, but normal SMA and IgG). At admission, we have reconsidered liver histology, ruling out the classical features of autoimmune disease. Moreover, there was not a classical picture of cirrhosis at prior biopsy. Considering the procoagulant diathesis (heterozygotic mutation of factor V (H1299R), of MTHFR (C677T) and of factor XIII (Val34Leu), we have hypothesized a EHPVO/NCPH.
The patient has an optimal performance status, no episodes of encephalopathy, and a well-preserved liver function (Child-Pugh score B7 due to mild ascites and hypoalbuminemia, MELD score 11).
Which treatment do you think I can carry out having the patient still gastric varices at high risk of further bleeding? Do you believe that a stent in the splenic vein could be useful?
Thanks for your help
Antonio Colecchia MD
Borgo Trento University Hospital
Verona,Italy -
Please provide the images of pancreas to further identify the presence of pancreatic disease related portal hypertension.
There is some evidence regarding anticoagulation for splenic vein thrombosis, but controversy remains.
Additionally, as mentioned by you, there is some case series evidence regarding stent for splenic vein thrombosis.
Endoscopic treatment with cyanoacrylate should be cautious. Occlusion of gastric varices will further increase the pressure of local left portal hypertension secondary to splenic vein thrombosis. Risk of recurrence of gastric variceal bleeding is high. -
In this case probably we can consider a combined treatment with balloon-occluded retrograde transvenous obliteration and thrombolysis associated. In addition a TIPS revision have to be considered to rule out a shunt dysfunction as cause of the thrombosis.
Federica Cerini MD
Hepatology Unit
San Giuseppe Hospital – Milan -
Dear Antonio and colleagues,
What about the current anticoagulation treatment of this patient? Recanalisation of the splenic vein seems very important (with or without stent) to decrease portal pressure in the left/splenic sector.
Since the portal circulation is patent, as well as the TIPS, in case of failure of recanalisation of the splenic vein, splenectomy should be discussed.
Looking forward to the opinion of others.
Kind regards
Andrea -
Dear Antonio, dear friends,
Happy new year to the VALDIG group.
I agree that revising TIPS, including pressure measurement in the portal trunk, would be very useful. This thrombosis of portal trunk is surprising and desserves investigations.
Anticoagulation will likely be useful to avoid extension of thrombosis, particularly if thrombosis of the portal trunk and of splenic vein are recent.
I would be very cautious with splenectomy given the bad results the VALDIG group reported in Hepatology: PMID: 30924941
I would really favor -as mentioned above- splenic vein recanalization since it would likely be -if feasible- a good option.
Best regards
Pierre-Emmanuel -
Dear Antonio and dear VALDIG friends,
Happy New Year!
I would treat the patient with a combination therapy of BRTO (in order to avoid any further bleeding due to gastric varices) plus anticoagulation (the latter due to a procoagulant diathesis, a suspected PSVD and the presence of thrombosis likely causing the bleeding).
The presence of TIPS, with a non-occlusive thrombosis of the portal vein trunk should mitigate the effects of a further increase in portal hypertension due to BRTO.
However, it would be useful, as already mentioned, to revise the TIPS in order to exclude any disfunction and to assess the degree of portal hypertension. I would suggest to perform TIPS revision at the same time of BRTO (measuring portal pressure before BRTO, then temporary occluding the gastric varix with the balloon and rechecking portal pressure before definitively occluding the varix). In case of important increase of portal pressure, and without any disfunction in TIPS, I would proceed by performing BRTO and adding NSBB to decrease portal pressure as recently suggested.
Moreover, despite this patient still has a good liver function as demonstrated by her MELD, taking into account the multiple episodes of decompensation in a young patient, I would start screening her for liver transplantation in order to be prepared in case of a further rapid deterioration of her liver function.
Best regards,
Laura Turco -
Dear All,
Interesting the debate raised by your patient. Unfortunately we can not review the images of the CT-Scan. May I suggest to discuss the case through the ERN CPMS system? This will allor to uplaod the CT-scan and even (I think this part is not still working) the liver biopsy. It would be interesting to see the degree of stenosis of the splenic vein. I would suggest to do TIPSreview with an accurate measuremets of pressures of all the system pre and post splenic vein stenosis to assess the impact of the stenosis in the pressure gradient. If pressure in the portal vein and splenic vein before the stenois is OK (meaning that TIPS is adequately decompressing the system) and the pressure is increased proximally to the stenois, then stenting/angioplasty of the splenic stenosis would be my first choice. If despite that, still is feeding of the gastric varices I will suggest to embolize (through the splenic vein). Not BRTO.All my best
JUan Carlos Garcia-Pagan
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Dear Valdig friends,
I have much appreciated your precious and interesting comments.
According to your suggestion we will perform vascular assessment with pressure measurement and TIPS revision; furthermore we will perform also a liver biopsy. Subsequently, we will discuss the case with our interventional radiologists and surgeons of our transplant center.
We will inform you on the results of the patient’s exams and next management steps
All the best,
Antonio
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