Portal Vein Thrombosis in child

[ljw]

My son has portal vein thrombosis, splenomegaly and esophogeal varices. He has a low platelet count as well. Until recently, his liver function has always been normal. Recently, however, he has developed terrible nosebleeds that can only be stopped with packing and carterization at the E.R.

At the E.R., we were told his INR is high at 1.42, his PTT is high at 13.6 sec., his APTT/PTT is high at 36 sec., and his APTT/PTT ratio is high at 1.3. His GI doctor has since put him on Vitamin K to help control the INR.

What is INR, PTT, APTT/PTT, and APTT/PTT ratio? What does all this mean to you?

His G.I. doctor states that he is showing some signs of liver dysfunction, but says he is too healthy at this time to consider for tranplantation. The liver dysfunction is most likely due to an insufficient blood supply getting to the liver.

I have asked if we could correct the 'plumbing' problem with the portal vein. I was told that the blockage occurs close to the liver where the portal vein branches off into many tiny veins and, therefore, it can't be done.

All my son's medical conditions are secondary to this blockage. Can you offer any insight into any potential procedures for correcting the portal vein thrombus? Thank you.

[Kevin Pho, MD]

Hello - thanks for asking your question.

You note portal vein thrombosis, splenomegaly and esophogeal varices.  Increased INR, PTT etc.  

To answer your questions:
1) The INR (international normalized ratio), PTT (prothrombin time), as well as the ratios you mentioned are tests to measure the clotting function of the blood.  Most of the clotting factors are associated with the liver.  Advanced liver disease may manifest in the elevated levels (resulting in a "thinning" of the blood).  Vitamin K is used to lower the INR.  

2) Depending on the stage of portal vein thrombosis, there are several considerations to discuss.  In cases of chronic portal vein thrombosis, non-selective beta blockers may be considered to decrease the risk of varicies.  Depending on the location of the thrombosis, surgical options (i.e. shunting) may be feasible.  Finally, the possibility of chronic anticoagulation has been studied with varying results.  There is no guaranteed benefit, and decision to anticoagulate should be made on an individual basis (i.e. weigh the risk of bleeding vs the risk of recurrent thrombus).

Followup with your personal physician is essential.

This answer is not intended as and does not substitute for medical advice - the information presented is for patient education only. Please see your personal physician for further evaluation of your individual case.

Thanks,
Kevin, M.D.

[pediatric transplant surgeon]

I wanted to add that there is a very successfull operation for portal vein thrombosis in the absence of significant liver disease. Your child sounds like someone who could be helped greatly by diverting blood from the intestines back into the liver instead of through the spleen or gastric varices where they can do harm.
Go to the Children's Memorial website and look under Rex shunt, or do a search with those words on any search engine.