Portosystemic shunts are abnormal vascular onnections |
between the hepatic portal vein (the blood vessel that |
connects the gastrointestinal tract with the liver) and |
the systemic circulation(Figure 1). Such anomalies |
cause blood in the gastrointestinal track to be diverted |
past the liver, there by limiting the liver's vital functions in |
metabolism and detoxification of compounds and the |
body's defenses against intestinally derived pathogens. |
This effectively exposes the body to toxic by-products of |
digestion (toxins and bacteria) and mimics the effects of |
liver failure. |
. |
Portosystemic shunts can be present at birth (i.e. congenital) |
or acquired as the result of another disease process later in |
life. Congenital shunts are more common, representing |
approximately 75% of all canine cases, and generally result |
from anatomic abnormalities of the portal vasculature or |
persistence of fetal vessels. One or occasionally two vessels |
are involved, and the shunts are classified according to their |
location as either outside of (extrahepatic) or within |
(intrahepatic) the liver. |
, |
CONGENITAL SHUNTS |
. |
Congenital shunts occur more commonly in purebred dogs |
than in mixed breeds; miniature schnauzers, Yorkshire |
Terriers, and Irish Wolfhounds appear to be at increased |
risk. The prevalence of portosystemic shunts in certain breeds |
suggests an inherited predisposition. This has been proven |
only in Irish Wolfhounds, where a number of previously |
unknown genes appear to be involved. |
. |
Extrahepatic shunts are most common, accounting for 61% |
to 94% of congenital shunts, and are typically seen in small |
breeds of dogs, such as the Miniature Schnauzer and |
Yorkshire Terrier. Intrahepatic shunts represent between 6% |
and 40% of congenital shunts and are more common in large |
and giant breeds of dogs such as Irish Wolfhounds and |
Golden Retrievers. The majority of intrahepatic shunts are |
a result of the embryonic connection between the umbilical |
vein and the caudal vena cava remaining open; in most |
dogs this connection closes 3 days after birth but, for |
unknown reasons, remains open in dogs with intrahepatic |
congenital shunts. |
. |
Hepatic microvascular dysplasia is an unusual form of |
intrahepatic portosystemic shunting in which no gross |
vascular abnormality can be identified. This rare condition |
is associated with somewhat milder clinical signs and |
appears to be the consequence of a developmental |
abnormality; it has a higher prevalence in Cairn Terriers, |
suggesting a hereditary basis. |
. |
ACQUIRED SHUNTS |
. |
Acquired shunts arise secondary to diffuse liver disease |
where excessive and sustained pressure at some point within |
the portal vein causes embryonic, nonfunctional vascular |
communications to open. These are generally seen in older |
dogs with cirrhosis, hepatitis, or neoplasia of the liver. In |
contrast to congenital portosystemic shunts, a number of |
vessels are usually affected. |
. |
What are the signs of portosystemic shunts? |
The clinical signs exhibited by dogs with portosystemic |
shunts reflect the failure of the liver to eliminate various |
toxic matter, drugs, and bacteria absorbed from the |
gastrointestinal tract. Certain materials particularly |
those produced from dietary protein, are potential |
neurotoxins (e.g., ammonia, gamma-aminobutyric acid, |
natural benzodiazepines, and mercaptans), which affect the |
function of the central nervous system and produce a |
syndrome called hepatic encephalopathy. Other clinical signs |
arise from the liver being deprived of portal blood flow, |
which is essential for the normal development of the liver; |
as a result the liver is underdeveloped and it's metabolic, |
storage, and excretory functions are further impaired. |
. |
SIGNALMENT AND HISTORY |
. |
Dogs with congenital portosystemic shunts are typically |
purebred dogs less than 1 year old. The severity of clinical |
signs varies and is related to the anatomic position of the |
shunt and the fraction of portal blood that is shunted past |
the liver. Generally, the lower the fraction of shunting, the |
milder and later in onset are the clinical signs. Nevertheless, |
affected animals are often in poor body condition and of |
small body stature, especially when compared to their |
littermates. Owners may complain that the animals fail to |
thrive or grow and that skin and coat condition are poor. |
. |
Other clinical signs tend to be intermittent or periodic in |
nature and relate to the central nervous system, |
gastrointestinal tract, and urinary tract. There may also be |
a history of an adverse response to sedation or anesthesia. |
. |
A significant number of dogs (up to 25% of cases of |
portosystemic shunts) may develop signs later in life; this |
may be due to an acquired rather than congenital shunt or |
because the animal can no longer compensate for a low |
grade congenital shunt. dogs with acquired shunts have |
signs similar to those with congenital disease, although |
the previous history may have been uneventful until the |
appearance of weight loss and associated clinical signs. |
. |
CLINICAL SIGNS |
Central nervous system signs are the most common, occurring |
in over three-quarters of all cases, and may be vague and |
subtle such as anorexia, depression, and lethargy.... More |
specific signs include episodes of hyperactivity, head |
pressing and circling, disorientation, temporary blindness, |
weakness, excess salivation, seizures, and occasionally coma. |
These signs tend to wax and wane, and their onset may be |
connected with the recent ingestion of a protein-rich meal |
that resulted in increased production of neurotoxins within |
the large intestine. |
. |
Gastrointestinal signs (vomiting and/or diarrhea) are present |
in about two-thirds of cases. Evidence of lower urinary tract |
disease is present in approximately one-half of cases and is |
usually due to ammonium urate crystals, which are formed |
because of excessive excretion of ammonia and uric acid in |
urine. Some dogs, particularly those that develop signs later |
in life, have polydipsia and polyuria (excessive drinking and |
urination). |
. |
Other less common signs of portosystemic shunting include |
recurrent fevers and ascites, although the latter is generally |
seen only in dogs with acquired shunts. |
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DIAGNOSIS |
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Although signalment and clinical signs may strongly suggest |
the presence of a portosystemic shunt, a series of investigative |
steps... must be taken to: |
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*Identify the anatomic location and severity of the shunt |
*Indicate whether the shunt is congenital or acquired |
. |
The latter two criteria are important when considering |
whether the shunt is amenable to surgical correction and |
the likely outcome of such surgery. |
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Blood chemistry and hematology panels usually show |
characteristic patterns of mild abnormalities that as a group |
suggest the presence of a portosystemic shunt. These include |
mildly elevated liver enzymes, low blood urea nitrogen and |
total plasma protein concentrations, hypogly-cemia (low |
blood sugar) and low serum cholesterol. Mild, |
nonregenerative anemia and microcytosis (undersized |
red blood cells) may also be present in all cases. Blood |
ammonia concentrations may also be increased, but samples |
must be rapidly analyzed soon after collection to detect such |
an increase. Urinalysis may reveal a low specific gravity and |
the presence of ammonium blurate crystals. Serum bile acid |
concentrations taken either after an overnight fast or 2 hours |
after a meal are usually confirmatory. |
. |
The next step is to identify whether the shunt is intrahepatic, |
extrahepatic, or microvascular. This generally requires |
specialized imaging techniques, and it is likely that dogs |
may need to be referred to appropriate specialists. Survey |
radiographs of the type normally taken in veterinary |
practices simply indicate the presence of a small liver |
. |
(Figure 2). |
. |
Ultrasonography is a useful, noninvasive tool for the |
detection of portosystemic shunts. Intrahepatic shunts are |
easily visualized: the liver usually appears small in size, |
there is reduced visibility of intrahepatic portal vessels, |
and an anomalous blood vessel may be obvious (Figure 3). |
In dogs with extrahepatic shunts the first two features are |
usually present, but the detection of the anomalous vessel |
is not so easy. Application of Doppler ultrasound, an |
advanced technique, may help in such cases, especially |
where there is a small extrahepatic shunt. |
. |
Contrast radiography, whereby a marker dye is injected |
into a vein draining the intestine and radiographs are taken |
immediately, allows ready visualization of the portal vein |
and shunting. This procedure is usually performed in |
combination with surgical correction (so that the dog has |
to be anesthetized only once) and is often referred to as |
operative mesenteric portography. A loop of small intestine |
is exteriorized (brought outside of the body) and a tube is |
placed in a jejunal vein. A water-soluble radiopaque |
dye is injected via the tube, and lateral and ventrodorsal |
radiographs are then taken. Where there is a shunt, the |
abnormal vessel is outlined as blood is diverted into the |
systemic circulation without appearing in the liver |
(Figure 4). |
. |
Portal or transcolonic scintigraphy is an advanced technique |
whereby the uptake of radiochemicals from the intestinal |
tract is monitored. A radiochemical, usually technetium 99m |
pertechnetate, is administered via the rectum and first |
accumulates in the liver in normal animals. In dogs |
with portosystemic shunts the distribution of activity is |
altered as the radiochemical bypasses the liver and reaches |
the heart first. Although this does not identify the location |
of the shunt, it does provide an extremely accurate estimate |
of the degree of shunting, allowing the clinician to |
predict the likely success of management options and to |
follow up the success of surgical management. |
. |
Liver biopsy is indicated when there is no obvious shunt or |
if multiple extrahepatic shunts are identified (as seen in |
acquired portosystemic shunting). This may reveal |
hepatocyte atrophy, with small or absent portal vessels, |
and will allow histopathologic confirmation of micro- |
vascular disease. |
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MANAGEMENT |
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There are two broad management options: surgical ligation |
of shunts or medical management of the effects of shunting. |
The decision as to which is most appropriate needs to be |
made on a case-by-case basis depending on the type and |
location of the shunt, the age of the animal, and the |
severity of clinical signs. There may also be significant |
financial considerations on the part of the owner. Surgery |
wherever feasible, is generally believed to be the treatment |
of choice as it suggests the promise of normal liver function. |
Improvements in dietary and medical management of hepatic |
encephalopathy, however, mean that conservative treatments |
offer a reasonable prognosis for dogs that are not suitable |
for surgery.... |
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SURGICAL MANAGEMENT |
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Ligation of shunt vessels is an advanced surgical technique |
requiring a suitably experienced surgeon, careful selection |
and monitoring of general anesthesia, measurement of blood |
pressure in the portal vein and systemic circulation, and |
appropriate critical care support facilities. Such requirements |
usually necessitate referral to specialist centers, and this is |
especially true with intrahepatic shunts. |
. |
Single extrahepatic shunts are usually identified as tortuous, |
abnormal vessels. These are ligated close to the vena cava. A |
potential fatal complication is portal hypertension, which |
occurs when intrahepatic vessels are unable to cope with the |
additional volume of blood that is diverted to the liver after |
closure of the shunt vessel. Guarding against this requires |
careful monitoring of portal and systemic blood pressures |
and inspection of the intestines and pancreas for signs of |
cyanosis. Failure to alleviate the hypertension and pain, |
bloody diarrhea, and shock leading to death in 2 to 24 hours |
after surgery. |
. |
A 60% to 80% degree of ligation can usually be achieved |
without complications and is associated with an increase in |
the amount of portal blood that enters the liver and with |
improvements in the patient's clinical status. In some cases, |
the ligation procedure may be repeated on one or more later |
dates to progressively attain complete ligation. This un- |
fortunately necessitates additional costs and, with successive |
surgeries, increases the risk of perioperative, complications. |
On the other hand, repeat surgery may not be necessary as |
some partially ligated shunts appear to spontaneously |
occlude. The prognosis for dogs with partial ligation is |
guarded because approximately 50% show recurrence of |
clinical signs at an average of 3 to 4 years after surgery. |
. |
Ligation of intrahepatic shunts is technically more difficult |
and is associated with higher risks of fatal complications. |
Such cases may be best managed conservatively. |
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MEDICAL AND DIETARY MANAGEMENT |
With better understanding of the pathophysiology of hepatic |
encephalopathy, it has become possible to prescribe specific |
therapies that provide a reasonable prognosis for those dogs |
with portosystemic shunts that are not corrected surgically. |
The primary objective of medical management is to |
eliminate the clinical signs associated with hepatic |
urinary tract disease and reducing the metabolic load on |
the liver. The chief components of medical management |
strategies are dietary modifications and oral antibiotics. |
. |
Medical management is indicated for all dogs with acquired |
shunts and all dogs with microvascular shunts. It should |
also be used for a period in those dogs that are about to |
undergo surgical ligation. This will allow the veterinarian |
and owner to establish the extent to which the condition can |
be managed medically, in case it is not possible to completely |
ligate the shunt at surgery. medical management is also |
indicated in those dogs whose owners are unable to afford |
the cost of referral to a specialist surgical facility or whose |
owners are unwilling to accept the significant risk of |
perioperative mortality. All dogs undergoing surgical |
ligation should continue to receive medical therapy for 2 |
to 4 weeks post-operatively. Finally, some degree of medical |
therapy may be required in dogs with partially ligated |
shunts. |
. |
Dietary manipulations for the control of hepatic |
encephalopathy are designed to limit neurotoxin production, |
which occurs principally in the large intestine, and to reduce |
the subsequent absorption of these toxins into the portal |
vein....The major toxins are all derived from nitrogenous |
materials (protein and urea) and are synthesized by bacteria |
found within the large intestine. The production of these |
toxins is reduced by limiting the amount of protein fed and |
ensuring that the dietary protein is high quality and very |
digestible. These steps reduce the amount of protein that |
reaches the large intestine; further reductions can be |
attained by feeding smaller meals more frequently to |
maximize the digestive capacity of the small intestine. |
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Specific diets with restricted protein contents are available |
from veterinarians. These are ideal because they provide a |
balanced protein-calorie intake, which is important for the |
stable control of hepatic encephalopathy. |
.Including dietary fiber in the daily ration assists in acidi- |
fying the colonic environment and limiting toxin production |
and also acts as a mild laxative to increase the elimination |
of toxic factors in feces. Lactulose, a soluble fiber, is often |
used as a supplement for this purpose and can be readily |
purchased from pharmacists. Supplementation with zinc |
salts also improves the detoxification of ammonia and the |
control of hepatic encephalopathy. A veterinary diet |
specifically designed and tested for the management of liver |
disease and portosystemic shunts is available in Europe; it |
is unique in combining a restricted protein content with in- |
creased zinc and added dietary fiber. Available in America |
as Waltham Veterinary Diets Canine Low and Medium |
Protein, dry and canned. |
. |
Antibiotics are used in most cases to reduce the bacteria |
within the large intestine that are responsible for the |
production of neurotoxins. Orally administered neomycin |
is commonly used for this purpose and is often used in |
combination with lactulose in both the short and long-term |
medical management of portosystemic shunts. |
. |
SUMMARY |
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Portosystemic shunts are serious conditions in dogs and |
require significant efforts to diagnose and treat. Specialized |
surgical techniques, in association with advances in medical |
and dietary management, allow the condition to be effectively |
managed and provide a reasonable quality of life. |
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You can read more here....
http://www.vet.utk.edu/clinical/sacs/shunts_faq.html
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