Non cirrhotic portal hypertension

[Pvvaladares]

Hi everyone!

I’m looking for a bit of advice on some question I haven’t solved yet. Basically, my whole story started about 2 months ago (july/2020), when my mom started feeling unwell and thought it was due to a umbilical hernia she has. As she was treated for colorectal cancer 14 years ago, she pays a visit to her gastro-oncologist every 6 months. She had gone for an appointment in February/2020, which was all clear. Back to July, she decided to pay him another visit due to her discomfort. He suspected ascites, urging her to take a thorax and whole abdomen Cat scan, a transvaginal ultrasound, an endoscopy and a colonoscopy, along with several blood works, including hepatic functions. All tests came back normal, except for the detection of mild ascites and some esophageal varices in the endoscopy. Her doctor then suggested her to pay a visit to a renowned hepatologist in my city. We met him and he said that It looked like a classical case of cirrhosis, but that he didn’t buy it because all her blood exams and scans were normal. Then he requested for some other blood works, such as albumin, hepatitis and prothrombim time, and a portal system ultrasound with doppler. Then again, all results came back absolutely normal. Then he said he couldn’t give any diagnosis without a further investigation, which would be done through a exploratory laparoscopy. My mom then had the surgery, and nothing abnormal was found, except the surgeon thought the liver looked very slightly fibrotic. He collected several liver samples and ascitic fluid and sent them for biopsy. Results showed normal ascistic fluid and normal liver, without any alteration whatsoever, except for a very very small amount of fat (staging 0). Then the doctor said as cirrhosis was completely ruled out and there was no fibrosis at all, the only possible explanation was non cirrhotic portal hypertension that might have been caused by the chemotherapy she went through 14 years prior. She’s on very small dose of water tablets and low dose propranolol, and is doing great. But I’m just not really satisfied with the diagnosis, since most of scientific articles I’ve read (a whole bunch btw) show that non cirrhotic portal hypertension is almost always followed by an enlarged spleen, low platelets and alterations in the liver blood flow, which could be better seen through biopsy. As none of these things were found in all examinations my mother has had, could it be that we should push matters further with another specialist? I was wondering if the ascites could be caused by something else. The hepatologist agrees that she doesn’t have some classical findings of non cirrhotic portal hypertension, but because she has the varices, he’s set for it. But then again, many articles show that you may as well develop esophageal varices without portal hypertension. It’s just so confusing, and I expected a more curious doctor, who would be willing to look for the cause until there’s no where else to look, which is not the case right now. What are your thoughts on this?

Thank you very much!!!

[Pishi123]

Sometimes they are not able to determine the cause. I have portal hypertension with cirrhosis. I am not a drinker. I used to have varices but then they disappeared much to my delight. My consultant prescribed me with carvedilol nearly five years ago. Am fine on that. My record says crytogenic. I do hope you find the cause for your mum but you may not get one. Best wishes Angela

[Hidden in reply to Pishi123]

I have NCPH which was caused by tablets given to control crohns disease. Mine was picked up as suspected on a CT scan, which also showed enlarged spleen and low platelets. It had to be confirmed with a liver biopsy. I think I would push for that to be done. Best wishes.

[Pvvaladares in reply to Hidden]

Thank you Purplejanee!

Yes, I think liver biopsy is a must whenever it is possible to be performed. My mom had several pieces of het liver removed and sent for histological analysis, which found absolutely nothing wrong which could be accounted for the portal hypertension. That’s mainly what bothers me, cause most works show that for non cirrhotic portal hypertension it is possible to notice some issues on liver specimen when doing a biopsy, and they found nothing with her biopsy. Go figure, human body is just crazy sometimes, right?

[Kristian]

I appreciate how frustrating it can be not to have a formal diagnosis of the cause. I was in the same situation with cirrhosis. We new it was there but couldn't identify the cause. In your mum's case, they know she has some classic signs of portal hypertension, with the varices, but have managed to rule out issues with her liver. So at least they have some protocol to follow to ensure she is getting regular monitoring and treatment to minimise risks associated with that. Eventually that may turn up the cause over time.

As you have indicated, it may well turn out to be just a knock on effect of the treatments she previously had. I discovered I had a rather large clot in my portal vein from the surgeon's notes after transplant. I didnt know that was there beforehand as it was never mentioned in any reports from earlier scans or ultrasounds. Whether any of the very late scans or ultrasounds picked it I dont know, but usually they would say and no one did. Perhaps its worth asking your doc, if her previous treatment could have restricted the blood flow in her portal vein or elsewhere. It may not necessarily be a clot, it may just be a narrowing, perhaps caused by the scar tissue. It may not even necessarily be preventing flow, it could just be a narrowing slowing it down or causing some turbulence. Its a bit of a wild guess to be honest, but there's no harm exploring that possibility with her doctor if you haven't done so already.

Good luck

[Pvvaladares in reply to Kristian]

Thank you for your thorough considerations Kristian!

I’ll ask her hepatologist about some further digging on possible flow obstructions in her hepatic circulation. I had asked him about portal vein thrombosis, but he immediately ruled it out because nothing could be seen on tomography. That’s why I think he went for the chemotherapy side effect diagnosis. We’ll see, her follow up shall be in a month, then we can talk a bit more. But her doctor is pretty much at ease, just told her to keep the water tablets for now along with propranolol e reduce salt intake and drink plenty of water.