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I would like to add that the data on prophylactic fibrotide (quite expensive in Spain) is mainly in patients without severe choric liver disease. We are refering to a patients with BCS. A previous liver biopsy to stage the disease is very relevant, as well as other potential tools (HVPG, elastography, discarding presence of esophageal varices or oterh signs of PH) that can help to assess severity. In any case, if the hematologist finally accept to do the transplant (in our centers they usually are not prone to accept if we do not demonstarte that the liver problema is controlled and mild) I Will probably probably recommend some type of prophylaxis (although teh data is scarce and null in patients with previous liver disease).
BestDear Dhiraj,
I think that there is no data to support any of these possibilities. We have not personal experience in the issue. Sorry. Interesting to know and maybe collect potential cases.
Best
Juan CarlosDear 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
Dear Friends, As some of you already now we are collecting a large multicenter cohort of patients with IPH whithin the VALDIG network. In this cohort (more than 600 patients there are 5 patients with ICV that received a TIPS. All of them for refractory ascites. All of them died without the possibility to be transplanted because of severe comorbidities (one of these patients is from our center). Approximately one year ago we were requested to do a TIPS in a young female with severe portal hypertension that required a bone marrow transplantation. TIPS procedure was very well tolerated and bone marrow succesfully transplanted. She is still on flllow up.
I hope this may be of help
Best
Juan Carlos Garcia-Pagán
Barcelona Hepatic Hemodynamic lab
Barcelona.On the previously sent link you can find the images of the colonoscopy and some of the images of the Angio CT.
Thanks a lot
Dear Dominique, Federica and Fabio,
thanks for your interest in our patient. We indeed have already done a direct (superior and inferior mesenteric arteriography. No data on arteriovenous fistula was seen).We are considering to do a direct portal punction and measure pressure to discard any prehepatic component of portal hypertension that we have been unable to identify by imaging. I will keep pou all informed aout the evolution. If there are other advices…
Thanks VALDIG consortia
Best and seen you soon in AsconaDear Andrea, If you really think that the traumatism may be the cause of the obstruction and this happens at 6 years of age and the complete thrombophilic study is negative stopping anticoagulation can be a good alternative. However, it looks like the patient had portal hyeprtension with ascites. has the patient varices?. Can you consider that the patients is not responding to medical treatment? is the patient a candidate to go a step further in the treatment?.
Best
