Recognition and Management of Portosystemic Shunts in Dogs What are portosystemic shunts?

Portosystemic shunts are abnormal vascular connections between the hepatic porta l vein (the blood vessel that connects the gastrointestinal tract with the liver ) and the systemic circulation (Figure 1). Such anomalies cause blood in the gas trointestinal 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 de fenses 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 th e result of another disease process later in life. Congenital shunts are more co mmon, representing approximately 75% of all canine cases, and generally result f rom anatomic abnormalities of the portal vasculature or persistence of fetal ves sels. One or occasionally two vessels are involved, and the shunts are classifie d according to their location as either outside of (extrahepatic) or within (int rahepatic) the liver. CONGENITAL SHUNTS Congenital shunts occur more commonly in purebred dogs than in mixed breeds; min iature schnauzers, Yorkshire Terriers, and Irish Wolfhounds appear to be at incr eased 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 shu nts, and are typically seen in small breeds of dogs, such as the Miniature Schna uzer 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 v ena cava remaining open; in most dogs this connection closes 3 days after birth but, for unknown reasons, remains open in dogs with intrahepatic congenital shun ts. Hepatic microvascular dysplasia is an unusual form of intrahepatic portosystemic shunting in which no gross vascular abnormality can be identified. This rare co ndition 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 sus tained pressure at some point within the portal vein causes embryonic, nonfuncti onal vascular communications to open. These are generally seen in older dogs wit h cirrhosis, hepatitis, or neoplasia of the liver. In contrast to congenital por tosystemic 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 failu re of the liver to eliminate various toxic matter, drugs, and bacteria absorbed from the gastrointestinal tract. Certain materials particularly those produced f rom dietary protein, are potential neurotoxins (e.g., ammonia, gamma-aminobutyri c acid, natural benzodiazepines, and mercaptans), which affect the function of t

. Owners may complain that the animals fail to thrive or grow and that skin and coat condition are poor. as a result the live r is underdeveloped and it's metabolic. Nevertheless. storage. and occasionally coma. Other clinical signs arise from the liver being deprived of portal blood flow.(Table 1).. temporary blindness..he central nervous system and produce a syndrome called hepatic encephalopathy. The latter two criteria are important when considering whether the shunt is amen able to surgical correction and the likely outcome of such surgery. excess sal ivation. These signs tend to wax and wane. disorientation. and urinary tract. and l ethargy . the lower the fraction of shunting. affected animals are often in poor bo dy condition and of small body stature. and may be vague and subtle such as anorexia. Generally. head pressing and circling. especially when compared to their litter mates. SIGNALMENT AND HISTORY Dogs with congenital portosystemic shunts are typically purebred dogs less than 1 year old. Other clinical signs tend to be intermittent or pe riodic in nature and relate to the central nervous system. occurring in over three-quarte rs of all cases. the milder and later in o nset are the clinical signs. More specific signs include episodes of hyperactivity. DIAGNOSIS Although signalment and clinical signs may strongly suggest the presence of a p ortosystemic shunt. a series of investigative steps (Table 2) must be taken to: Confirm the diagnosis Identify the anatomic location and severity of the shunt Indicate whether the shunt is congenital or acquired. A significant number of dogs (up to 25% of cases of portosystemic shunts) may de velop signs later in life. which are formed because of excessive ex cretion of ammonia and uric acid in urine. Other less common signs of portosystemic shunting include recurrent fevers and ascites. CLINICAL SIGNS Central nervous system signs are the most common. Some dogs. seizures. and excretory functions are fur ther impaired. weakness. Evidence of l ower urinary tract disease is present in approximately one-half of cases and is usually due to ammonium urate crystals. have polydipsia and polyuria (excessive drinking and urination). 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. although the latter is generally seen only in dogs with acqu ired shunts. w hich is essential for the normal development of the liver. There may also be a history of an adverse response to seda tion or anesthesia. gastrointestinal trac t. and their onset may be connected with the recent ingestion of a protein-rich meal th at resulted in increased production of neurotoxins within the large intestine. 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. Gastrointestinal signs Vomiting and/or diarrhea are present in about two-thirds of cases. particularly those that de velop signs later in life. although the previous history may have been uneventful until the appearan ce of weight loss and associated clinical signs. depression. dogs with acquired shunts have signs similar to those with congenital di sease.

the age of the animal. but samples must be rapidly analyzed soon after collection to detect such an increase. may help in such cases. but the detection of the anomalous vessel is not so easy. and will allow histopathologic confirmation of micro. MANAGEMENT There are two broad management options: surgical ligation of shunts or medical m anagement of the effects of shunting. This generally requires specialized imaging techniques. and the severity of clinical signs. it does provide an extremely accurate estimate of the degree of shunting. The decision as to which is most appropria te needs to be made on a case-by-case basis depending on the type and location o f the shunt. Urinalysis may reveal a low specific gravity and the presence of ammonium blurate crystals. hypogly. noninvasive tool for the detection of portosystemic shunts. Contrast radiography. nonregenerative anemia and microcytosis (undersized red blood ce lls) may also be present in all cases. This ma y reveal hepatocyte atrophy. the abnormal vessel is outlined as blood is diverted into the systemic circulation without a ppearing in the liver (Figure 4).vascular disease. and lateral a nd ventrodorsal radiographs are then taken.Blood chemistry and hematology panels usually show characteristic patterns of mi ld abnormalities that as a group suggest the presence of a portosystemic shunt. Blood ammonia concentrations may also be increased. is administered via the rectum and first accumu lates in the liver in normal animals. Although this does not identify the location of the shunt. an advanced technique. allowi ng the clinician to predict the likely success of management options and to foll ow up the success of surgical management. low blood urea nitrogen and total p lasma protein concentrations. Where there is a shunt. A loop of small intest ine is exteriorized (brought outside of the body) and a tube is placed in a jeju nal vein. A radiochemical. allows ready visualization of t he 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. The next step is to identify whether the shunt is intrahepatic. Surgery . Mild. with small or absent portal vessels. Serum bile acid concentrations taken either after an overnight fast or 2 hours after a meal are usually confirmatory. A water-soluble radiopaque dye is injected via the tube. Intrahepatic shunts are easily visualized: the liver usually appears sm all in size. Portal or transcolonic scintigraphy is an advanced technique whereby the uptake of radiochemicals from the intestinal tract is monitored. Ultrasonography is a useful. There ma y also be significant financial considerations on the part of the owner. Liver biopsy is indicated when there is no obvious shunt or if multiple extrahep atic shunts are identified (as seen in acquired portosystemic shunting).cemia (low blood sugar) and low serum cho lesterol. In dogs with extrahepatic shunts the first two features are usually present. especially where there is a small extrahepatic shunt. and it i s likely that dogs may need to be referred to appropriate specialists. whereby a marker dye is injected into a vein draining the intestine and radiographs are taken immediately. Survey ra diographs of the type normally taken in veterinary practices simply indicate the presence of a small liver (Figure 2). there is reduced visibility of intrahepatic portal vessels. usual ly technetium 99m pertechnetate. or microvascular. These include mildly elevated liver enzymes. and an anomalous blood vessel may be obvious (Figure 3). Application of Doppler ul trasound. In dogs with portosystemic shunts the dist ribution of activity is altered as the radiochemical bypasses the liver and reac hes the heart first. extrahepatic.

The primary objective of medical management is to eliminate the clinical sign s associated with hepatic encephalopathy. A potential fatal complication is por tal hypertension. Improvements in dietary and medical manage. repeat surgery may not be necessary as some partially ligated shunts appear to spontaneously occlude. Such requirements usually necessitate referral to specialist centers.ment of hepatic encephalopathy.fortunately necessitates additional costs and. mean that conservative treatme nts offer a reasonable prognosis for dogs that are not suitable for surgery (Tab le 3). and this is especially true with intrahepatic s hunts. The chief com ponents of medical management strategies are dietary modifications and oral anti biotics. A 60% to 80% degree of ligation can usually be achieved without complications an d is associated with an increase in the amount of portal blood that enters the l iver and with improvements in the patient's clinical status. On the other hand. It should also be used for a period in those dogs that are about to undergo surgical ligation. This un. Other goals include minimizing lower u rinary tract disease and reducing the metabolic load on the liver. Finally. me asurement of blood pressure in the portal vein and systemic circulation. . bloody diarrhea. These are ligated close to the vena cava. The prognosis for dogs with partial lig ation is guarded because approximately 50% show recurrence of clinical signs at an average of 3 to 4 years after surgery. abnormal vessels. Such cases may be best managed conservatively. which occurs when intrahepatic vessels are unable to cope with the additional volume of blood that is diverted to the liver after closure of t he shunt vessel. In some cases. it h as become possible to prescribe specific therapies that provide a reasonable pro gnosis for those dogs with portosystemic shunts that are not corrected surgicall y. careful selection and monitoring of general anesthesia. This will allow the veterinarian and ow ner to establish the extent to which the condition can be managed medically. SURGICAL MANAGEMENT Ligation of shunt vessels is an advanced surgical technique requiring a suitably experienced surgeon. some degree of medical therapy may be required in dogs wit h partially ligated shunts. medical manag ement 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. with successive surgeries. increases the risk of perioperative. All dogs undergoing surg ical ligation should continue to receive medical therapy for 2 to 4 weeks postoperatively. Ligation of intrahepatic shunts is tec hnically more difficult and is associated with higher risks of fatal complicatio ns. and shock leading to death in 2 to 24 hours after surgery. in case it is not possible to completely ligate the shunt at surgery.wherever feasible. MEDICAL AND DIETARY MANAGEMENT With better understanding of the pathophysiology of hepatic encephalopathy. complications. is generally believed to be the treatment of choice as it su ggests the promise of normal liver function. Guarding against this requires careful monitoring of portal and systemic blood pressures and inspection of the intestines and pancreas for sign s of cyanosis. however. the ligation procedure may be repeated on one or more later dates to progressively a ttain complete ligation. and app ropriate critical care support facilities. Medical management is indicated for all dogs with acquired shunts and all dogs w ith microvascular shunts. Single extrahepatic shunts are usually identified as tortuous. Failure to alleviate the hypertension and pain.

1998. maltese dogs . in association with advances in medical and dietary management. Specialized surgical techniques. The major toxins are all derived from nitrogenous materials (protein and u rea) and are synthesized by bacteria found within the large intestine. where he currently holds the position of Research Manager. which occurs principally in the large intestine. These steps re duce the amount of protein that reaches the large intestine. This article is reproduced with the permission of Kal Kan Foods. further reductions can be attained by feeding smaller meals more frequently to maximize the digesti ve capacity of the small intestine. Ped igree Breeder Forum magazine. Watson qualified from the Royal (Dick) School of Veterinary Studies at the University of Edinburgh in 1987 and r eceived his PhD from the University of Glasgow in 1992. Specific diets with restricted protein contents are available from veterinarians . The produ ction of these toxins is reduced by limiting the amount of protein fed and ensur ing that the dietary protein is high quality and very digestible. Dr. a soluble fiber. dry and canned. He joined the Waltham Ce nter in 1994.Dietary manipulations for the control of hepatic encephalopathy are designed to limit neurotoxin production. Including dietary fiber in the daily ration assists in acidifying the colonic environment and lim iting toxin production and also acts as a mild laxative to increase the eliminat ion of toxic factors in feces. Vol 7 . Antibiotics are used in most cases to reduce the bacteria within the large intes tine that are responsible for the production of neurotoxins. is often used as a su pplement for this purpose and can be readily purchased from pharmacists. allow the condition to be effectivel y managed and provide a reasonable quality of life. Supplem entation with zinc salts also im-proves the detoxification of ammonia and the co ntrol of hepatic encephalopathy. CA. Vernon. A veterinary diet specifically designed and tes ted for the management of liver disease and portosystemic shunts is available in Europe. which is important for the stable control of hepatic encephalopathy. Lactulose. Orally administered neomycin is commonly used for this purpose and is often used in combination wit h lactulose in both the short and long-term medical management of portosystemic shunts. an d to reduce the subsequent absorption of these toxins into the portal vein (Tabl e 4). These are ideal because they provide a balanced protein-calorie intake. SUMMARY Portosystemic shunts are serious conditions in dogs and require significant effo rts to diagnose and treat. it is unique in combining a restricted protein content with increased z inc and added dietary fiber. Available in America as Waltham Veterinary Diets Ca nine Low and Medium Protein.