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Extra-hepatic PSS? Portal Vein Hypoplasia?!

Hi everyone,

Here it goes:

German Shepherd, 6yo, MC, with history of ascites, peripheral edema, hypoalbuminemia (as low as 1,1-1,9 g/dL), low creatinine and urea, mild elevation of liver enzymes, intermitent diarrhea, for the last 2-3 months. Bile acids are extremely increased (165 fasting [Normal 0-0,8 umol/L], 170 postprandial [Normal 0-30 umol/L]).

My differentials before U/S were severe diffuse hepatic disease (with possible acquire PSShunts), colestasis and congenital portal vasculature anomalies (PSS, PVHypoplasia/microvascular dysplasia).

What I see on U/S:

– No urinary stones, normal kidneys, liver apparently normal to subnormal in size (parenchyma seems OK), mild proeminence of GI mucosa

– No colestasis seen, d-pap OK

– Ascites

– Small PV and big CVC (ratio of 0,47) – consistent with EHPSS

– No turbulence seen in the CVC (excludes portocaval shunt?), no double aorta sign (can’t really eliminate portoazygos shunt, right?), no intrahepatic vascular anomalies seen

– Portal flow was 17cm/s while awakened, while sedated I couldn’t get a good measure (too low, do you think it was affected by the sedation? Maybe portal hypertension here?)

 

First clip shows the doppler on the CVC, demonstrating absence of turbulence

Second clip shows Ao entering the diaphragm, with no double ao sign seen

On the third clip you can compare the width of the PV, CVC and Ao

Fourth clip – d-pap, without evidenc of obstruction

 

What do you think? It’s not colestasis and severe liver disease seems unlikely. EHPSS? PVHypoplasia?

 

Help is much appreciated, thank you!

 

jobrag

3 responses to “Extra-hepatic PSS? Portal Vein Hypoplasia?!”

  2. So im ruling out primary ehpss because essentially never occur with ascites because the primary shunt adjusts for the pressures according got the liver experts I have spoke to or heard lecture. I would have to agree with maybe one exception in my career where a primary ehpss was present with ascites form diffuse liver disease but that was a zebra and I never confirmed completely. Ascites presenc e would be secondary from diffuse liver disease or portal vein hypoplasia  caiusing portal hypertension which is likely the case here and maybe secondary shunting.

    Also if you base the probability of  primary shunting on our research you have a number of negative predictive factors in this case for primary ehpss such as lack of bladder stones and small liver, renal stones and so forth.

    Check that out here:

    ECVIM 2010

    https://sonopath.com/resources/research-publications

    The cvc is bigger than the aorta and pv here because of sedation likely… especially if dex domitopr or similar was used and this will lower the pv flow velocity.

    In the last clip that dilated vessel to the right of the pylorus is the pancreatoduodenal vein that gets congested and hence pancreatic congestion when portal hypertension is present

    If you want to get a ton of rapid experience in shunt hunting from ultrasound and CT just do the basic search for “shunt” and we have it all in the archive… the results of our shunt hunting over the years:

    https://sonopath.com/members/case-studies/search?text=shunt&species=All

    Nice post

  3. I agree with you, there aren’t any positive predictive factors, but couldn’t the ascites appear just because the severe hypoalbuminemia? (sometimes it was as low as 1,1!)

    This dog was indeed sedated with dexmedetomidine, which could cause the false portal hypertension and maybe the pancreaticoduodenal v. congestion?

     

    I recommended CT scan and if negative maybe hepatic biopsy. Do you agree?

     

    Thank you so much EL,

     

    Joao

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