Imaging diagnosis--Complex intrahepatic portosystemic shunt in a dog.

An unusual form of congenital intrahepatic portosystemic shunt was identified in a 3 1/2-month-old female Labrador Retriever with neurologic signs. Ultrasonography and contrast-enhanced computed tomography were used to characterize the shunt morphology. An unusual, looping right-divisional shunt connected back to the portal vein that formed an ampula in the right-central portion of the liver. An irregularly shaped window-like opening connected the combined right-divisional loop and aneurysmal portal vein, and the caudal vena cava, while this vascular pool gradually fused more cranially. Imaging features of this complex vascular anomaly, which has not been previously reported, are presented.     

An unusual portosystemic shunt in a dog

An unusual portosystemic shunt was diagnosed in a 4-month-old male Miniature Poodle examined because of stunted growth and episodes of dementia characterized by hysteria, ataxia, and staggering. Operative splenoportography revealed a portosystemic shunt. Exploratory surgery to identify and correct the shunt was attempted. Evidence of any portal circulation was not detected, and the dog was euthanatized. The possible embryologic basis for this vascular anomaly is discussed.     

Surgical Management of Multiple Congenital Intrahepatic Shunts in Two Dogs

Objective —To present details of an unusual type of portosystemic shunt and its surgical management in two dogs.
Animals —Two young dogs that had a tentative diagnosis of a portosystemic shunt on the basis of clinical signs and serum biochemical abnormalities. Abdominal ultrasonography and contrast portography demonstrated multiple intrahepatic shunts. In both cases, the multiple shunts arose from a single branch of the portal vein.
Outcome—It was possible to locate and attenuate flow through the shunts via a transportal venotomy under conditions of hepatic vascular occlusion. Clinical and biochemical abnormalities resolved after surgery in both dogs. Postoperative sonography revealed complete obliteration of the shunt plexus in one of the dogs.     

Unusual congenital portosystemic communication resulting from persistence of the extrahepatic umbilical vein

An unusual congenital portosystemic shunt was identified in one dog and two cats with clinical signs and laboratory evidence of hepatic dysfunction. In all the animals, the abnormal vessel arose from the portal system between the left medial and quadrate liver lobes and travelled within the falciform fat, exiting the abdomen through the caudal ventral left diaphragm. The intrathoracic course of these vessels was not established. The anatomical location of this anomalous vessel may have hindered attempts at ultrasonographic identification since it was not visualised before surgery in any of the animals. In addition, while the anatomical location of the vessel may facilitate rapid identification and surgical attenuation, it could predispose the vessel to trauma during the coeliotomy approach. It is hypothesised that this form of portosystemic communication results from failure of a portion of the left umbilical vein to degenerate during embryogenesis. This is in contrast to other forms of congenital extrahepatic portosystemic shunt that are presumed to be developmental errors resulting in an abnormal communication between the embryonic vitelline and cardinal venous systems. The prognosis for animals with the vascular anomaly reported here is probably similar to that for animals with other forms of congenital portosystemic shunt.     

Effect of breed on anatomy of portosystemic shunts resulting from congenital diseases in dogs and cats: a review of 242 cases

OBJECTIVE: To evaluate the effect of species and breed on the anatomy of portosystemic vascular anomalies in dogs and cats. DESIGN: Retrospective study of 233 dogs and nine cats presenting to the University Veterinary Centre, Sydney. METHODS: Case records were evaluated for breed, sex, age, anatomical and histological diagnosis. Cases were included when a portosystemic vascular anomaly resulted from a congenital or developmental abnormality of the liver or portal venous system. RESULTS: Disease conditions included single congenital portosystemic shunt with patent portal vasculature (214 dogs, nine cats), portal vein aplasia (nine dogs), multiple acquired shunts resulting from portal vein hypoplasia (seven dogs), biliary atresia (one dog) and microvascular dysplasia (one dog). One Maltese had a single, congenital shunt and multiple acquired shunts resulting from hepatic cirrhosis. Breeds that were significantly over-represented included the Maltese, Silky Terrier, Australian Cattle Dog, Bichon Frise, Shih Tzu, Miniature Schnauzer, Border Collie, Jack Russell Terrier, Irish Wolfhound and Himalayan cat. Bichon Frise with shunts were significantly more likely to be female than male (12:2, P < 0.001). Two hundred and fourteen dogs (91.4%), and all cats, had shunts that were amenable to attenuation. Inoperable shunts occurred in 19 dogs (8.2%). Fifty six of 61 (92%) operable shunts in large breed dogs were intrahepatic, versus 10/153 (7%) in small breeds (P < 0.0001). Breeds that were not predisposed to portosystemic shunts were significantly more likely to have unusual or inoperable shunts than dogs from predisposed breeds (29% versus 7.6%, P < 0.0001). No significant relationship between breed and shunt type could be determined in cats. CONCLUSION: Breed has a significant influence on shunt anatomy in dogs. Animals presenting with signs of portosystemic shunting may suffer from a wide range of operable or inoperable conditions. Veterinarians should be aware that unusual or inoperable shunts are much more likely to occur in breeds that are not predisposed to congenital portosystemic shunts.     

Contrast‐enhanced ultrasonography is a feasible technique for quantifying hepatic microvascular perfusion in dogs with extrahepatic congenital portosystemic shunts

Extrahepatic‐congenital portosystemic shunt is a vascular anomaly that connects the portal vein to the systemic circulation and leads to a change in hepatic microvascular perfusion. However, an assessment of hepatic microvascular perfusion is limited by conventional diagnostic modalities. The aim of this prospective, exploratory study was to assess hepatic microvascular perfusion in dogs with extrahepatic‐congenital portosystemic shunt using contrast‐enhanced ultrasonography (CEUS) using perfluorobutane (Sonazoid^®). A total of 17 dogs were included, eight healthy dogs and nine with extrahepatic‐congenital portosystemic shunt. The time‐to‐peak (TTP), rising time (RT), and rising rate (RR) in the hepatic artery, portal vein, and hepatic parenchyma, as well as the portal vein‐to‐hepatic parenchyma transit time (ΔHP‐PV) measured from time‐intensity curve on CEUS were compared between healthy and extrahepatic‐congenital portosystemic shunt dogs. The RT of the hepatic artery in extrahepatic‐congenital portosystemic shunt dogs was significantly earlier than in healthy dogs (P = 0.0153). The TTP and RT of the hepatic parenchyma were significantly earlier in extrahepatic‐congenital portosystemic shunt dogs than in healthy dogs (P = 0.0018 and P = 0.0024, respectively). ΔHP–PV was significantly shorter in extrahepatic‐congenital portosystemic shunt dogs than in healthy dogs (P = 0.0018). CEUS effectively revealed changes in hepatic microvascular perfusion including hepatic artery, portal vein, and hepatic parenchyma simultaneously in extrahepatic‐congenital portosystemic shunt dogs. Rapid hepatic artery and hepatic parenchyma enhancements may reflect a compensatory increase in hepatic artery blood flow (arterialization) caused by a decrease in portal vein blood flow and may be used as an additional diagnostic test to distinguish extrahepatic‐congenital portosystemic shunt dogs from healthy dogs.     

Ultrasonographic evaluation of partially attenuated congenital extrahepatic portosystemic shunts in 14 dogs

Doppler ultrasonography was used to evaluate the portal vein in 14 dogs before, immediately after and four weeks after a partial ligation of a congenital extrahepatic portocaval shunt. By four weeks after the operation, the hepatofugal or zero flow in the portal vein segment cranial to the shunt origin had become a hepatopetal flow in 13 of the dogs, which became clinically healthy. The other dog continued to have a hepatofugal flow in the portal vein cranial to the origin of the shunt and continued to show clinical signs of hepatic encephalopathy. The shunt remained functional in six of the dogs, and three of them developed portosystemic collaterals in addition. In the other eight dogs the patent shunt was non-functional, because a hepatopetal flow was detected in the shunt adjacent to the portal vein. This flow was the result of the splenic vein entering the shunt, and the splenic blood dividing; some flowed via the shunt towards the portal vein, preventing the portal blood from shunting, and the rest flowed via the attenuated shunt segment to the caudal vena cava. Shunting of the splenic venous blood was clinically insignificant.     

Ultrasonographic assessment of hemodynamic changes in the portal vein during surgical attenuation of congenital extrahepatic portosystemic shunts in dogs

OBJECTIVE: To determine portal hemodynamic changes associated with surgical shunt ligation and establish ultrasonographic criteria for determining the optimal degree of shunt narrowing and predicting outcome. DESIGN: Case series. ANIMALS: 17 dogs, each with a single congenital extrahepatic portosystemic shunt. PROCEDURE: Pre- and postligation flow velocities and flow directions were determined by Doppler ultrasonography intraoperatively in the shunt and in the portal vein cranial and caudal to the shunt origin. Outcome was evaluated 1 month after surgery by measuring blood ammonia concentration and performing abdominal ultrasonography. RESULTS: Hepatofugal flow was detected in 9 of 17 dogs before shunt attenuation in the portal segment that was between the shunt origin and the entering point of the gastroduodenal vein. If hepatofugal flow became hepatopetal after shunt ligation, hyperammonemia resolved. Hepatofugal portal flow was caused by blood that flowed from the gastroduodenal vein toward the shunt. Shunt attenuation converted hepatofugal flow to hepatopetal in the shunt in 12 of 17 dogs. Chronic portal hypertension developed or perioperative death occurred when the portal congestion index caudal to the shunt origin increased by > 3.6 times. CONCLUSIONS AND CLINICAL RELEVANCE: After hepatopetal flow in the cranial portal vein and the shunt is established, further shunt narrowing is contraindicated. Increase of the portal congestion index caudal to the shunt > 3.5 times should be avoided. Poor outcome because of severe hypoplasia of the portal branches can be expected if the flow direction remains hepatofugal after shunt occlusion cranial to the shunt origin.     

Portal streamlining as a cause of nonuniform hepatic distribution of sodium pertechnetate during per-rectal portal scintigraphy in the dog

The purpose of this study was to evaluate nonuniform patterns of vascular distribution of pertechnetate in the dog during per-rectal portal scintigraphy. Ninety-two studies were reviewed retrospectively to document the patterns of radionuclide distribution. Forty-five studies were classified as normal and 47 were classified as diagnostic for a macrovascular portosystemic shunt. In these dogs, shunt fractions were calculated and compared using a t-test. In dogs with sufficient liver radioactivity for evaluation, the study was classified as having uniform, dorsal, central, or ventral radiopharmaceutical distributions. There were 51 animals (45 normal and six dogs with low-magnitude portosystemic shunts) with sufficient liver activity to assess the radionuclide distribution within the liver. A one-way ANOVA was used to compare shunt fractions between each of the distribution patterns. Two dogs were anesthetized and selective portovenograms were performed. Portovenograms were compared with the scintigraphic images to correlate the vascular distribution of the right, central, and left divisional branches of the portal vein. The shunt fraction in the 45 normal dogs was significantly lower than in the dogs with portosystemic shunts (5.7% +/- 4.8% vs. 78.6% +/- 20.0% (mean +/- SD), P < 0.001). Of the 51 dogs with sufficient liver activity to classify the pattern of distribution, there were 15/51 (31.4%) with uniform radionuclide distribution, 10/51 (19.6%) with focal dorsal distribution, 15/51 (29.4%) with focal ventral distribution, and 10/51 (19.6%) with focal central distribution. There was no significant difference in the shunt fractions between the groups. There were six dogs diagnosed with low-magnitude portosystemic shunt with sufficient liver radioactivity to categorize the vascular distribution of the radionuclide within the liver. Of these six dogs, two had focal dorsal distribution, one had focal central, one had focal ventral and two had uniform distribution. Focal dorsal distribution could result from streamlining of the radionuclide into the right divisional branch of the portal vein. Focal ventral distribution could result from streamlining the radionuclide into the left divisional branch of the portal vein. Focal central distribution could result from streamlining the radionuclide into the central divisional branch of the portal vein.     

IMAGING DIAGNOSIS—PORTAL VEIN APLASIA AND INTERRUPTION OF THE CAUDAL VENA CAVA IN THREE DOGS

Severe portal vascular anomalies have been reported previously accompanying azygos continuation of the caudal vena cava, polysplenia, and situs anomalies in dogs and people. Three dogs with portal vascular anomalies were identified by means of CT angiography as having portal vein aplasia with portal insertion into the caudal vena cava, azygos continuation of the caudal vena cava, and interruption of the pre‐hepatic caudal vena cava. This information confirms that complex embryological defects may occur in patients presenting for congenital portosystemic shunt, and that CT angiography is a non‐invasive method of completely evaluating these potentially non‐surgical portal vascular anomalies.     

A Method for Controlling Portal Pressure After Attenuation of Intrahepatic Portacaval Shunts

Two dogs had right divisional intrahepatic portacaval shunts within the right lateral lobe of the liver. In both dogs, an extrahepatic portacaval vascular anastomosis was created, using an autologous right external jugular vein graft. The intrahepatic shunts were completely attenuated using a prehepatic intravascular caval approach. The creation of the vascular graft allowed postattenuation rises in portal pressure to be controlled, preventing the development of life threatening portal hypertension. Both dogs recovered from the procedure. One dog is clinically normal and does not require medication (8 months postoperatively); the other dog was euthanatized 5 months after surgery because of renal failure. Scintigraphy studies, performed before surgery, showed significant shunting of portal blood away from the liver (shunt indices 65% and 59%), whereas, similar studies done 4 weeks afterwards showed almost normal portal blood flow (shunt indices 16% and 18%, respectively).     

Diagnostic imaging of congenital porto-systemic shunts in dogs and cats: a review

An overview of clinical, laboratory, and diagnostic imaging features of congenital porto-systemic shunt (PSS) in dogs and cats is presented through the analysis of recent literature, and personal case log. Particular emphasis is given to diagnostic accuracy of ultrasonographic examination of PSS in the evaluation of shunt vessel anatomy, and of ancillary findings such as abnormalities of portal vein flow, portal branches, and liver size. Operative mesenteric portography to obtain information on PSS morphology and position, and quantitative hepatic scintigraphy, which allows the calculation of shunt fractions, are also described. Limitations for each diagnostic imaging technique are given.     

The angiographic anatomy of the portal venous system in the neonatal dog

The angiographic anatomy of the portal venous system in 50 dead, neonatal Labrador/Retriever type puppies is described. Angiography was performed by the injection of radioopaque contrast media through a catheter placed within the umbilical vein. In 49 pups the ductus venosus was a straight vessel arising from the left main portal vein and terminating in an ampulla into which the left hepatic and left phrenic veins entered prior to the ampulla entering the caudal vena cava. The diameter of the ductus venosus was significantly narrower (P<0.001) in pups born alive (n=10) when compared to stillborn individuals (n=39). No discreet narrowing of the ductus venosus indicating a sphincter was found, with closure appearing to be uniform along the vessel's length. A well-developed, patent portal venous system was present in the majority of individuals. One pup showed variation from the others studied having a vascular connection between the portal sinus and the vena cava within the liver. This may represent a normal variant of the ductus venosus, or may be an anatomical abnormality leading to the development of an intrahepatic portosystemic shunt. If this was an intrahepatic shunt, no concurrent ductus venosus was present.     

Hepatic arteriovenous fistulae and portal vein hypoplasia in a Labrador retriever

An 18-month-old male Labrador retriever was referred for investigation of chronic intermittent diarrhoea and vomiting of two months duration. A diagnosis of hepatic arteriovenous fistulae was made. These are extremely rare hepatic vascular anomalies which confer arterial pressure to the portal vein. Liver atrophy, portal vein hypoplasia, portal hypertension and multiple acquired portosystemic collateral vessels are the main complications. Surgical excision is a challenge as resection of large lesions may be associated with significant blood loss. In this dog, persistence of portal vein hypoplasia and extensive collateral pathways following surgery led to a reserved prognosis.     

Complex extrahepatic portocaval shunt with unusual caval features in a cat: computed tomographic characterisation

A two-year-old, neutered male domestic shorthair cat was evaluated for a history of urate calculi, and neurologic signs. Diagnostic imaging revealed an elongated and tortuous single extrahepatic portosystemic shunt which appeared to receive normal tributaries of the caudal vena cava. Surgical correction of the shunt was carried out using cellophane banding. Eight months following surgery, clinical signs had resolved. Computed tomographic angiography allows thorough, rapid imaging of complex vascular anomalies to aid proper surgical correction. Errors in the formation of the portal vein and caudal vena cava can produce complex anomalies of the abdominal vasculature. Persistence of the embryologic left subcardinal vein is proposed to account for the lesion.     

ANATOMY OF EXTRAHEPATIC PORTOSYSTEMIC SHUNTS IN DOGS AS DETERMINED BY COMPUTED TOMOGRAPHY ANGIOGRAPHY

Congenital extrahepatic portosystemic shunts are anomalous vessels joining portal and systemic venous circulation. These shunts are often diagnosed sonographically, but computed tomography (CT) angiography produces high‐resolution images that give a more comprehensive overview of the abnormal portal anatomy. CT angiography was performed on 25 dogs subsequently proven to have an extrahepatic portosystemic shunt. The anatomy of each shunt and portal tributary vessels was assessed. Three‐dimensional images of each shunt type were created to aid understanding of shunt morphology. Maximal diameter of the extrahepatic portosystemic shunt and portal vein cranial and caudal to shunt origin was measured. Six general shunt types were identified: splenocaval, splenoazygos, splenophrenic, right gastric‐caval, right gastric‐caval with a caudal shunt loop, and right gastric‐azygos with a caudal shunt loop. Slight variations of tributary vessels were seen within some shunt classes, but were likely clinically insignificant. Two shunt types had large anastomosing loops whose identification would be important if surgical correction were attempted. A portal vein could not be identified cranial to the shunt origin in two dogs. In conclusion, CT angiography provides an excellent overview of extrahepatic portosystemic shunt anatomy, including small tributary vessels and loops. With minor variations, most canine extrahepatic portosystemic shunts will likely be one of six general morphologies.     

SIMULTANEOUS CONGENITAL AND ACQUIRED EXTRAHEPATIC PORTOSYSTEMIC SHUNTS IN TWO DOGS

Two dogs with simultaneous congenital and acquired portosystemic shunts are reported. The first dog was an eight-month-old, male Golden Retriever with a history of peritoneal effusion, polyuria/polydipsia, and stunted growth. The dog had a microcytic, hypochromic anemia, a mildly elevated AST, and a moderate to severely elevated preprandial and postprandial serum bile acids. Transcolonic portal scintigraphy confirmed the presence of a portosystemic shunt. An intraoperative mesenteric portogram was performed. Two conjoined congenital extrahepatic portosystemic shunts and multiple acquired extrahepatic portosystemic shunts were identified. The second dog was a five-month-old, mixed breed with two week history of peritoneal effusion. Abdominal ultrasound and transcolonic scintigraphy were used to diagnose a portosystemic shunt. A single extrahepatic portosystemic shunt, portal hypertension, and multiple acquired collateral shunts were identified at surgery. The histologic alterations observed in these dogs were consistent with a portosystemic shunt. In these dogs, the presence of congenital and acquired portosystemic shunts and histopathologic findings are considered to represent a combination of congenital portosystemic shunts and noncirrhotic portal hypertension or portal vein hypoplasia.     

Portosystemic shunt in an alpaca cria

A 5-month-old alpaca cria was examined for chronic poor growth and repeated episodes of diarrhea. Examination of feces for parasites yielded negative results. Serum bile acid and blood ammonia concentrations were high. Subsequent examination by ultrasonography, percutaneous splenic portography, and colonic scintigraphy did not reveal evidence of a portosystemic vascular anomaly. Exploratory celiotomy with mesenteric vein portography revealed a colonic vein shunt in the caudal portion of the abdomen from the caudal vena cava to the portal vein. The shunt vessel was ligated without incident. Following surgery, the cria began to gain weight and was more alert. Eighteen months after surgery, the cria was doing well, although it had loose feces and was slightly small for its age. Portosystemic shunts are rare in cattle and horses but should be considered in alpacas with chronic poor growth when parasitism has been ruled out.     

Ultrasonographic diagnosis of congenital portosystemic shunt in 14 cats

Twenty-four cats with clinical and, or, clinico-pathological signs compatible with portosystemic shunting were examined prospectively using two-dimensional grey-scale, duplex and colourflow Doppler ultrasonography. Diagnosis of congenital portosystemic shunt was subsequently confirmed in 14 cats using operative mesenteric portography and surgery. Of the 14 affected cats, nine were purebred; eight were male and six female. The mean age at the time of diagnosis was nine months (range four to 27 months). Ultrasonographic evidence of a small liver was present in seven cats (50 per cent); visibility of intrahepatic portal vessels was reduced in three (21 per cent). An anomalous blood vessel was identified ultrasonographically in each cat; in 10 cats (71 per cent) the vessel was observed to originate from the portal vein and drain into the caudal vena cava. Abnormally variable portal blood flow waspresent in eight of the 10 cats in which it was measured. At surgery, six shunts were intrahepatic and eight extrahepatic; the ultrasonographic diagnosis of intra- versus extra-hepatic shunt was correct in 13 cats (93 per cent). No anomalous blood vessels or abnormalities affecting the portal vein were detected ultrasonographically in any of the 10 cats that did not have congenital portosystemic shunting. Hence, the accuracy of ultrasonography for diagnosis of congenital portosystemic shunting in this series was 100 per cent.     

THREE-DIMENSIONAL MULTISLICE HELICAL COMPUTED TOMOGRAPHY TECHNIQUES FOR CANINE EXTRA-HEPATIC PORTOSYSTEMIC SHUNT ASSESSMENT

The purpose of the present study was to investigate the feasibility and usefulness of three-dimensional (3D) multislice computed tomography (CT) angiography with maximum intensity projection (MIP) and volume rendering (VR) in six dogs with clinical and sonographic findings suggestive of portosystemic shunt. Furthermore, we aimed to estimate the diameter of the portal vein and shunt vessels. MIP and VR reconstructions were performed for each patient and the origin and insertion of all shunt vessels were detected. In addition, 3D reconstructions allowed excellent depiction of vascular morphology and topography. All diagnoses and vessel measurements were confirmed by surgery. 3D multidetector CT angiography is a promising, noninvasive, and accurate method of evaluating dogs with suspected portosystemic shunts.