Jacquie Im seeing

Jacquie Im seeing microhepatica and increased portal markings. With those values hepatic failrue is of concern. I would run a bile acid profile, rule out a shunt (see ECVIM 2010 study) and do a left intercostal core bx . If you havent performed a bx from intercostal just stay 11-13 left side bottom 1/3 of rib cage. The first image shows a nice window for this. This liver would not be typical of hepcutaneous. Look for mucosal striations in the GI for PLE as well and of course check a usa for proetin to be sure no pln. Baseline cortisol or acth stim to rule out addisons too and I would do this first before any sedation. This progression should get you what you need on the dx. Let us know what happens!

Thanks Eric – all very
Thanks Eric – all very helpful.

Here is an update so far. I
Here is an update so far. I am leaning toward a shunt in this pet but finding it hard to explain the skin lesions. This is a very sick patient in poor body condition despite ravenous appetite.
– cbc, biochem, pre and post biles acids, baseline cortisol and clotting factors pending
– bladder sand/uroliths found on u/s – UA confirmed ammonium biurate crystals 2+bilirubin
– adrenals and pancreas normal
– kidneys normal – a little larger – maybe? (wt pet 5kg)
– there was scant abdominal effusion detected near the UB
– the best shot I could get of the PH was the right intercostal transverse view – my colour Doppler was totally uncooperative so disappointing
– the PV does seem smaller than the Ao at the PH to me
– so extrahepatic PSS or really bad liver disease or something else strange?

Body Shot
Body Shot

Skin on legs
Skin on legs

Bladder sand/stones
Bladder sand/stones

Porta Hepatis
Porta Hepatis

Left Kidney
Left Kidney

Can;t tell on the still
Can;t tell on the still there. You need to video clip the c-loop from rk to ao>cvc>pv> pyloric outflow. Most shunts will be seen in this video clip taken at different angles.

The bladder stones are bright
The bladder stones are bright clean and echogenic that would fit with biurates but just an educated guess. can put the skin lesions with the liver but if you do a liver bx then maybe do a skin bx same time.

This is the view you are
This is the view you are looking for, splenocaval shunt in this case.

Hepatocutaneous syndrome on
Hepatocutaneous syndrome on the other hand looks like this.

Skin lesions look more like
Skin lesions look more like superficial pyoderma, immune-mediated disease, drug reaction; rather than hepato-cutaneous lesions. Skin lesions with hepato-cutaneous syndrome tend to be more of a hyperkeratotic appearance. Possible that this dog has two separated issues – liver and skin. As Eric suggested get some skin biopsies.

Attached are two photos of a dog with hepato-cutaneous syndrome.

Second photo
Second photo

Thanks Eric and Remo

Wish
Thanks Eric and Remo

Wish I could get those nice images. Maybe can work on this at IVUSS in the wet lab. I had to go between the ribs to get up into the PH. Blood results came back on this case. Low albumin, low crea, low glucose, low calcium, ALP elevated but only in the 200’s.

Normal pre and post bile acids. Mild regenerative anemia and neutrophilia. ALT and bilirubin normal despite bilirubin in the urine and urate crystals. Cortisol normal.

This was a rescue dog from a puppy mill. Unfortunately the rescue decided to euthanize. He would just lay in a ball and shiver and refused to walk as I think his legs were quite painful from the skin lesions. He was a sick little patient.

But I did do U/S guided liver core biopsies (the left intercostal approach worked quite well) and a post mortem and collected samples from several tissues, including skin. Can’t say that I could identify a shunt in the liver and grossly the liver looked normal, as the rest of the abdomen. Did find several tiny stones in the urinary bladder. Will see if the biopsies give any answers.

With the low albumin consider
With the low albumin consider PLE did you happen to get intestinal bx?? corn oil test would have been great on this and intestinal dysbiosis can cause the bile acid elevation but not the urate stones that i know of. Great thread everyone thx for the input!!! You all make SonoPath a greater resource every day

Corn oil test?
Yes, did
Corn oil test?
Yes, did biopsy bowel. Hope to get results back soon and try to put some of this together.

If this ends up being PLE
If this ends up being PLE here is a sample chapter on PLE from our upcoming textbook The Curbside guide. Its in final editing and should have it available in 6-8 weeks hard copy and digital download:

PLE: Protein Losing Enteropathy
Description: Protein losing enteropathies (PLE) are syndromes where different diseases cause loss of protein through the gastrointestinal mucosa. Clinical signs can be seen when the albumin drops below 20g/l (2 g/dl), at which point a loss of oncotic pressure leads to formation of ascites, thoracic effusion and peripheral edema. Causes for PLE can include inflammatory changes of the mucosa, ulcerations, erosions, neoplasia and lymphangiectasia.
Clinical Signs: Anorexia, weight loss, vomiting, diarrhea are often associated with protein losing enteropathy mainly in canine patients. In light of these clinical signs combined with subnormal albumin levels, the clinician should consider ultrasound to evaluate the big 4 (GI, Renal, Liver, Adrenals) to identify the potential sources of albumin loss (GI/Renal) or lack of production (Liver) or linked to hypoadrenocorticism (Adrenal) that may also be associated with this clinical profile (isoechoic flattened adrenals < 0.4 cm on ultrasound). Diagnosis: Typical diagnostics for protein losing enteropathy (PLE) include the clinical profile of hypoalbuminemia (but may also be within normal serum levels), a sonogram with suggestive intestinal wall prominence and mucosal striations noted, and clinical signs of vomiting, weight loss, diarrhea. Be warned some cases can have a lack of GI signs altogether and no weight loss. PLE is preferably definitively diagnosed by full thickness or endoscopy guided biopsies after feeding a fatty meal the night before in order to dilate lacteals to adequately diagnose lymphangiectasia. PLE can be associated with: 1. Inflammatory bowel disease (IBD) 2. Granulomatous disease 3. Neoplasia 4. Immunoproliferative enteropathy Often GI blood loss may occur potentially caused by lymphosarcoma, ulcerative disease, or intussusception. A dynamic sonogram testing procedure feeding corn oil (0.5-1 cc/kg) 45 minutes prior to the sonogram of an NPO patient will enhance the presence of mucosal striations in the small intestine during the sonogram. This is a reliable test founded by Marks et al. at UC Davis (NAVC 2011). Endoscopy both anterior and posterior approaches are ideal in order to access the stomach/duodenum in the former case and colon/ileum in the latter in order to maximize the biopsy information. However, this will not detect transmural disease such as lymphoma affecting the muscularis and submucosa that are not typically obtained readily via endoscopy. Ultrasound evaluation of the GI tract can help decide whether the pathology is luminal, and available for sampling through endoscopy, or mural and necessitating US-guided FNA or core biopsy or surgical biopsy ideally guided by intra-operative ultrasound. More information regarding intra-operative ultrasound may be found in “resources” at http://www.sonopath.com.
B12, Folate, Ionized calcium, ionized magnesium and antithrombin levels should all be measured in the clinical PLE patient and levels should be corrected with Calcium gluconate (50-150 mg/kg Iv over 12-24 hours) or 1 tablet extra strength Tums (300 mg calcium) PO SID to TID as needed to maintain normal calcium levels over 3-4 weeks. Mg sulphate (1mEq/kg/day IV) or MgOxide 10-20 mg/kg PO BID (Milk of magnesia) may be utilized for magnesium supplementation but milk of magnesia may cause diarrhea.
Yorkshire Terriers are 10X more likely to develop IBD and 9X more likely to suffer hypocalcemia and hypomagnesemia with IBD. Therefore magnesium and calcium supplementation may be in order.

Other differentials for Hypoalbuminemia include:
1. PLE
2. Liver failure
3. Glomerulonephritis/amyloidosis/protein losing nephropathy (PLN) (typically with elevated serum globulins)
4. Vasculitis
5. Exocrine pancreatic insufficiency (EPI)
6. Addison’s disease (typical or atypical).
Hypocalcemia may be present owing to albumen loss (carrier proteins). If depressed cholesterol is also present with hypoalbuminemia then either liver failure or PLE should be suspected. Hypocalcemia may also be an issue with PLE and general causes of Hypoalbuminemia.
Hypocalcemia differentials include:
1. Hypoalbuminemia
2. Hypovitaminosis D
3. Hypomagnesemia
4. Hypoparathyroidism
5. Pancreatitis
6. Spurious/idiopathic
Treatment: Given that a significant fraction of PLE cases are caused by a food allergy causing IBD, then lymphangiectasia and leading to PLE, Purina HA diet should be fed. This diet is low (not restricted in fat) in fat; low enough to use for lymphangiectasia cases. Plus, it is hydrolyzed, meaning there is nothing in which the body can respond antigenically. No other hydrolyzed or novel protein diets are as low in fat. (Z/D, RC, HP, and IVD diets are moderately high in fat).
It is a good idea to rule out parasitism and/or utilizing empirical treatment with Fenbendazole 50 mg/kg SID for 5 days & repeat in 2 weeks combined with Metronidazole 15mg/kg BID for 10 days. Also ruling out neoplasia with FNA or biopsy, if a target is available or full thickness may be performed. Occasionally GI lymphoma or mast cell disease may be emerging in these cases.
Feeding normal levels of fat with lymphangiectasia patients causes further dilation of the lacteals with rupture, exacerbating protein losses. Many recommend a low fat diet with lymphangiectasia, however, if the primary cause was a food allergy, this recommendation could be counterproductive.
Feed Purina HA for a few weeks and monitor total protein and albumin. If the levels are still low, I would transition to a novel protein diet that is ultra restricted in fat (ostrich, kangaroo, crab). Since none exist commercially, the diet would have to be homemade, which can be expensive for the owner to make. This largest challenge is to make the diet balanced. Homemade diet formulations created through a nutritional consultation is recommended.
Low dose lasix (1-2 mg/kg BID) and/or spironolactone (2-4 mg/kg BID) may be utilized for ascites until oncotic pressures are restored with a colloid or plasma therapy. Abdominocentesis should be utilized only to keep the patient comfortable owing to excessive abdominal distention. Excessive drainage will cause further depletion of the protein supply that we are trying to restore.
B12 supplementation of 250-1,000 ug (cat/small dog lower end, Large breed higher end dose or 1/4cc-1cc) SC weekly x 6 weeks would be recommended owing to loss of B12 in the ileum regardless of serum levels.
Aspirin therapy is suggested (1 mg/kg sid) to assist in potential thromboembolic episodes that are the often the source of sudden death in these cases owing to antithrombin III loss. This dose will not cause side effects in GI tract or elsewhere.
Azothiaprine or cyclosporine may also be considered for refractory cases.
In a last ditch effort if this doesn’t work and biopsies are still not possible then a prednisone trial would be in order. I always prefer utilizing cortisones in these patients based on biopsy results but realize that this is not feasible in many cases.

References:
Rodríguez-Alarcón C, Beristaín-Ruiz D, Pérez-Casio F, et al. Protein-losing enteropathy in a dog with lymphangiectasia, lymphoplasmacytic enteritis and pancreatic exocrine insufficiency. Vet Q 32[3-4] Sep 01, 2012: 193-7.

Valerie J Parker; Lisa M Freeman. Nutritional management of protein-losing nephropathy in dogs. Compend Contin Educ Pract Vet 34[7] Jul 01, 2012: 1-5.
Dossin O, Lavoué R. Protein-losing enteropathies in dogs. Vet Clin North Am Small Anim Pract 41[2] Mar 01, 2011: 399-418.

Eric – looks like a good book
Eric – looks like a good book and I look forward to seeing it. I will keep in mind the corn oil test. It looks like our patient had a liver issue afterall despite normal pre and post bile acids. Talking to internal med at Antech, bile acids can sometimes be normal (but rare) in these cases. Also, possible lab error? Here is the histopath:

Liver wedge biopsy and core biposy: portal arteriolar hyperplasia, multifocal, moderate, chronic with patchy central vein collapse. The vascular changes in this liver are a stereotypic response to insufficient portal blood supply. This may result from congenital shunts, portal vein hypoplasia (microvascular dysplasia), or aquired portal hypertension due to cirrhosis or extrahepatic causes. (dye study recommended)

Jejunum: lymphoplasmacytic enteritis, minimal, chronic. This degreee of inflammtion is mild and is most likely normal for this patient.

Pancreas, Kidneys, Spleen: normal

Skin: dermal fibrosis with follicular hyperkeratosis, mild superficial pleocellular dermatitis, and epidermal hyperplasia – the skin lesions are fairly subtle and I do not see any histological change that could explain some of the severe skin lesions.

So what I take from this, a shunt or severe form of microvascular dysplasia – still can’t explain the skin issues? Sometimes our patients just don’t read the textbook.

Sounds like primary portal
Sounds like primary portal hypoplasia to me which i hate the name because the portal vein prior to the liver is normal size to even bigger as portal hypertension starts but its one or more of the pv branches that is hypoplastic causing hypoxia to the respective lobe(s) and giving a microvascular dysplasia appearance to the parenchyma… or so is the current theory . (Portal vein branch hypoplasia makes more sense)..but in the liver groups they go round and round on this like cardiologists and ace inhibitors so who knows but it makes a nice logical story. Medical case though.Great case, thread and follow-up!!!

The portal hyoplasia would
The portal hyoplasia would explain the urate crystals, hypoalbuminemia, hypoglycaemia, and elevated ALP but not the normal bile acids – as you say some patients do no read the books. However, US was consistent with liver disease. Skin biopsy does not fit with the visible lesions but may have missed the actual lesion, especially if multiple biopsies were not taken.

Yes – this type of liver
Yes – this type of liver disease is quite confusing with what seems to be conflicting information out there ( would be good chapter for your book:)

Plasma ammonium would be
Plasma ammonium would be interesting here …wonder if that would have been elevated. Yeh the whole shunt thing has more chapters to be written for sure. the clinical approach book should help clear some of this up if we can ever push it through the 2 editors to finally get it out there:) Its a beast for sure