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.     

Per rectal portal scintigraphy in the diagnosis and management of feline congenital portosystemic shunts

Five cats with a congenital portosystemic shunt (CPSS) were examined using transcolonic portal scintigraphy before and after surgical ligation of the shunting vessel. The mean shunt index before surgery was 52 per cent (range 45 to 61 per cent). Repeat portal scintigraphy, six to eight weeks after surgery, indicated a significant reduction in shunt index (mean 13 per cent, range 5 to 25 per cent) in four cats. In one of these cats a marked reduction in the shunt index, as determined by scintigraphy, preceded normal fasting blood ammonia. In the fifth cat there was no significant change in the shunt index, fasting serum bile acids and blood ammonia six months after surgery, although its clinical signs of hepatic encephalopathy had improved. Portal scintigraphy is useful in the diagnosis of CPSS and enables a quantitative assessment of the effects of surgery and may be a more accurate indicator of the degree of shunting after surgery than blood ammonia and serum bile acids.     

Neurological dysfunction in dogs following attenuation of congenital extrahepatic portosystemic shunts

Eleven of 89 dogs (12 per cent) developed neurological signs within six days of surgical attenuation of a congenital extrahepatic portosystemic shunt. Neurological signs were not associated with hepatic encephalopathy or hypoglycaemia. Signs varied in severity from non-progressive ataxia (three dogs) to generalised motor seizures (four dogs), progressing to status epilepticus (three dogs). In a further four cases, ataxia and disorientation were treated vigorously with anticonvulsant medication, presumably preventing the development of seizures. Two dogs that developed status epilepticus died or were eventually euthanased. All other animals survived, although some had persistent neurological deficits. Postligation neurological complications were not prevented by gradual shunt attenuation. Prophylactic treatment with phenobarbitone (5 to 10 mg/kg preoperatively, followed by 3 to 5 mg/kg every 12 hours for three weeks) did not significantly reduce the incidence of neurological sequelae (2/31 [6 per cent] dogs with phenobarbitone vs 9/58 [16 per cent] without phenobarbitone; P = 0.2). However, no animal receiving phenobarbitone experienced generalised motor seizures or status epilepticus. In conclusion, these observations suggest that postligation neurological syndrome comprises a spectrum of neurological signs of variable severity. Perioperative treatment with phenobarbitone may not reduce the risk of neurological sequelae, but may reduce their severity.     

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.     

Neurological dysfunction in three dogs and one cat following attenuation of intrahepatic portosystemic shunts

Neurological dysfunction is an uncommon complication following extrahepatic portosystemic shunt ligation. Three dogs and one cat are described that developed neurological signs within 21 to 42 hours of attenuation of intrahepatic portosystemic shunts. None of these cases had biochemical evidence of hepatic encephalopathy postoperatively. Two dogs died during management of status epilepticus following aspiration of food. One dog died six months postoperatively. The cat had persistent neurological dysfunction at discharge, but was alive and had recovered most of its neurological function at the time of writing, 37 months after surgery. This report demonstrates the potential for animals with intrahepatic portosystemic shunts to develop postoperative neurological signs and highlights the difficulty of managing such cases. Two dogs had both intrahepatic and extrahepatic portosystemic shunts. Large intestinal malrotation (partial situs inversus) may have been linked to the development of a portosystemic shunt in the remaining dog.     

Portovenogram findings in cases of elevated bile acid concentrations following correction of portosystemic shunts.

The case records of 36 cats and dogs undergoing surgical correction of a single extrahepatic portosystemic shunt were reviewed. In 12 animals, the shunt was fully ligated during the first surgical procedure, while, in the remaining 24, the shunting vessel could only be partially ligated. Assessment of serum bile acid concentrations demonstrated complete shunt occlusion in 15 of these latter 24 animals (63 per cent) between one and six months postoperatively. Ten animals (28 per cent) had persistently high serum bile acid concentrations postoperatively. Portovenogram findings in these individuals revealed six that demonstrated shunting solely through the original vessel; In five of these, full shunt attenuation was achieved at second surgery. Further shunt manipulation was not possible in the sixth case due to extensive adhesion formation. In the remaining four animals with raised bile acid concentrations, the portovenogram demonstrated shunting through the original vessel as well as the development of multiple acquired shunts.     

Outcomes after extrahepatic portosystemic shunt ligation in 49 dogs

OBJECTIVE: To evaluate outcomes after attenuation of extrahepatic portosystemic shunts in dogs using surgical silk. DESIGN: Retrospective study. PROCEDURE: Case records were reviewed for degree of surgical attenuation, experience of the primary surgeon, perioperative mortality and problems related to persistent portosystemic shunting or shunt ligation. Presence of portosystemic shunting after surgery was evaluated by ammonia tolerance testing, measurement of postprandial serum bile acid, plasma urea and cholesterol concentrations and liver enzyme activity. The influence of age, postocclusion portal pressure, primary surgeon, degree of attenuation and postoperative biochemical findings on the occurrence of postoperative problems was assessed. RESULTS: The mortality rate was 2.1%. Shunt attenuation was complete in 34% and partial in 66% of dogs. Portal hypertension necessitating ligature removal was encountered in only one dog. Five dogs experienced neurological abnormalities (seizures or ataxia), possibly as a manifestation of 'postligation seizure syndrome'. Postoperative liver function was normal in 78% of dogs, including 70% with partial shunt attenuation. Experience of the surgeon was related positively to outcome after partial attenuation (P = 0.002). Postoperative biochemical evidence of abnormal liver function was the most sensitive predictor of recurrence of clinical signs referable to persistent portosystemic shunting. CONCLUSIONS: In the hands of an experienced surgeon, surgical attenuation of single extrahepatic shunts was safe and effective, even in animals with partial attenuation. Most dogs with biochemical evidence of persistent shunting suffer relapse of clinical signs within 18 months of surgery. Postligation neurological syndromes of variable intensity may be more common than previously thought.     

Outcome of ameroid constrictor occlusion of single congenital extrahepatic portosystemic shunts in cats: 12 cases (1993-2000)

OBJECTIVE: To determine postoperative (< or = 6 days), short-term (< or = 90 days), and long-term (> or = 6 months) outcomes of cats undergoing ameroid constrictor occlusion of single congenital extrahepatic portosystemic shunts (PSS) and identify factors associated with outcome. DESIGN: Retrospective study. ANIMALS: 12 cats. PROCEDURE: Cats with single congenital PSS that underwent surgical placement of ameroid constrictors were identified. Follow-up information was obtained through telephone interviews and facsimile correspondence with referring veterinarians and owners. Results-All cats survived the surgery and were discharged from the hospital. One cat had seizures during the postoperative period. Five cats were clinically normal during follow-up evaluations within 90 days after the surgery. Long-term follow-up information was available for 9 cats. Three were clinically normal, 4 had been euthanatized because of progressive neurologic disease, and 2 had neurologic abnormalities that could not be controlled with medication. Four of 7 cats with continued or recurrent neurologic abnormalities 1 or more months after surgery had normal scintigraphic or hepatic function test results 2 to 6 months after surgery. CONCLUSIONS AND CLINICAL RELEVANCE: Results suggest that the long-term outcome of ameroid constrictor occlusion of PSS in cats is poor. Owners of older cats and cats with preexisting neurologic signs should be made aware of the potential for a poor outcome when considering surgical correction of this disease.     

Congenital portosystemic shunts in toy and miniature poodles

Three male Poodles (two Toy, one Miniature) were presented to their veterinarians for evaluation of urolithiasis and varying degrees of hepatic encephalopathy. All three dogs were diagnosed as having intrahepatic shunts and referred for surgical correction. In each case, shunts arose from the right branch of the portal vein and were amenable to perivascular dissection caudal to where the vessel entered the hepatic parenchyma and to placement of perivascular cellophane bands to achieve shunt attenuation. During the same period, a female Miniature Poodle also presented for treatment of a congenital portosystemic shunt discovered during evaluation for generalised motor seizures. This animal had an extrahepatic portoazygous shunt that was completely ligated. Congenital portosystemic shunts have not previously been identified in Toy and Miniature Poodles at the University Veterinary Centre, Sydney and the anatomical types of shunt seen in this breed have not previously been reported in a consecutive series of cases. The three male dogs are noteworthy for a number of reasons: all had intrahepatic shunts, despite being small breed dogs; all three presented in a similar fashion, and all had shunts of an anatomical type amenable to placement of cellophane bands. One male dog died within 12 hours of surgery, the remaining three dogs survived and their liver function was normal at follow-up between 2 and 3 months after surgery. Use of cellophane bands for successful attenuation of intrahepatic shunts has not been previously reported.     

Generalized motor seizures after portosystemic shunt ligation in dogs: five cases (1981-1988)

Severe generalized motor seizures developed in 5 dogs between 13 hours and 3 days after surgical attenuation of a single extrahepatic portosystemic shunt. Three dogs died, and a fourth was euthanatized because of poor prognosis for recovery. The fifth dog, although improved, was still not clinically normal 15 months after surgery. An intra-or extracranial cause for the seizures could not be identified in any of these dogs. Postmortem examination results in 3 dogs were similar to findings previously reported in dogs with portosystemic shunts, but which did not undergo surgical shunt ligation. Of the 5 dogs, 4 were greater than 18 months old, which may indicate that older dogs are more likely to develop seizures after shunt ligation than are younger dogs.     

Outcomes of cellophane banding for congenital portosystemic shunts in 106 dogs and 5 cats

OBJECTIVE : To report outcomes after cellophane banding of single congenital portosystemic shunts in dogs and cats. STUDY DESIGN : Retrospective study of sequential cases. ANIMALS : One hundred and six dogs and five cats. METHODS : Medical records were reviewed for breed, sex, age at surgery, shunt anatomy, results of pre- and postoperative biochemical analysis, development of postligation neurologic dysfunction, portal hypertension or other serious complications, and the owners' perception of their animal's response to surgery. RESULTS : Ninety-five dogs and all 5 cats had extrahepatic shunts. Eleven dogs had intrahepatic shunts. Six dogs (5.5%) died as a result of surgery from portal hypertension (2 dogs), postligation neurologic dysfunction (2), splenic hemorrhage (1) and suspected narcotic overdose (1). Serious complications were more common in dogs with intrahepatic shunts than those with extrahepatic shunts (P=.002). Postligation neurologic dysfunction necessitated treatment in 10 dogs and 1 cat; 8 dogs and the cat survived. Clinical signs attributed to portosystemic shunting resolved or were substantially attenuated in all survivors. Postoperative serum bile acid concentrations or results of ammonia tolerance testing were available for 88 animals; 74 (84%) were normal and 14 (16%) were abnormal. Multiple acquired shunts were documented in two animals. CONCLUSIONS : Cellophane banding is a safe and effective alternative to other methods of attenuation. CLINICAL RELEVANCE : Slow occlusion of portosystemic shunts using a variety of methods is being evaluated world wide. Cellophane banding is a relatively simple procedure with comparable safety and efficacy to previously reported techniques.     

Mass screening of Irish wolfhound puppies for portosystemic shunts by the dynamic bile acid test.

Five hundred and sixty-six Irish wolfhound puppies aged six to 15 weeks were tested for congenital portosystemic shunts by the dynamic bile acid method. Plasma ammonia concentration was also measured in 165 of the puppies both fasting and postprandially. Nineteen puppies (3.4 per cent), nine males and 10 females, had portosystemic shunts. Smaller litters appeared to be more likely to contain affected puppies. The postprandial bile acid concentration was a reliable predictor of the presence of a shunt, with the highest concentration in a normal puppy being 38 mumol/litre and the lowest in an affected puppy being 43 mumol/litre. In contrast, fasting bile acid concentrations were normal in the majority of the affected puppies. There was considerable overlap in fasting plasma ammonia concentrations between normal and affected puppies (26 puppies, 15.8 per cent of those tested). Postprandial ammonia concentration appeared to give better separation between the two groups, apart from two individuals which produced bizarre results. It was concluded that the postprandial or dynamic bile acid test is an appropriate test for the mass screening of Irish wolfhound puppies for portosystemic shunts, and guidelines are proposed for the interpretation and follow-up of the test.     

Per Rectal Portal Scintigraphy Using 99mTechnetium Pertechnetate to Diagnose Portosystemic Shunts in Dogs and Cats

Per rectal portal scintigraphy using 99mTechnetium pertechnetate (99mTcO4-) was used to diagnose portosystemic shunts (PSS) before surgical confirmation in seven dogs and two cats. Shunt fractions, representing the percent of portal blood that bypasses the liver, were determined by computer analysis of the scintigraphic images. Animals with portosystemic shunts had a mean preoperative shunt fraction of 84.02% (n = 9). The mean postoperative shunt fraction in four animals was 58.22%. The mean shunt fraction in ten control dogs was 5.00%. Per rectal portal scintigraphy is an innovative, easily performed, inexpensive method to diagnose congenital portosystemic shunts in dogs and cats.     

Cellophane banding of congenital intrahepatic portosystemic shunts in two Irish wolfhounds

Two three-month-old, male Irish wolfhound siblings were diagnosed with breed-typical left divisional congenital intrahepatic portosystemic shunts consistent with patent ductus venosus. The shunts were amenable to surgical dissection at a posthepatic location. Both dogs had cellophane banding for shunt attenuation. One dog was euthanased after developing post-ligation neurological dysfunction, which was refractory to treatment. The other dog survived and demonstrated shunt attenuation. Successful surgical management using cellophane banding of a patent ductus venosus has not been previously described in a large-breed dog.     

Use of transcolonic portal scintigraphy to evaluate efficacy of cellophane banding of congenital extrahepatic portosystemic shunts in 16 dogs

OBJECTIVE: To evaluate the efficacy of cellophane banding of single congenital extrahepatic portosystemic shunts in dogs using transcolonic portal scintigraphy. To investigate the portal circulation of those dogs with elevated postoperative shunt fractions to determine the cause of the persistent shunting. Further, to evaluate whether presenting signs, clinical pathology findings and liver histopathology are predictive of outcome. DESIGN: Prospective study of 16 dogs presenting with single congenital extrahepatic portosystemic shunts. PROCEDURE: Dogs with single extrahepatic portosystemic shunts attenuated by cellophane banding underwent portal scintigraphy and bile acids tolerance testing pre- and post-operatively. Dogs identified with elevated shunt fractions at 10 weeks post-operatively underwent mesenteric portovenography. Qualitative hepatic histopathology from all dogs was reviewed by a veterinary pathologist and assigned a semi-quantitative score to identify any abnormalities that may predict surgical outcome. RESULTS: At 10 weeks post cellophane banding, 10 of 16 cases (63%) had normal shunt fractions, whilst six dogs (37%) had increased shunt fractions and seven dogs (44%) had increased serum bile acids. Of these dogs, mesenteric portovenography revealed incomplete closure of the shunt in three dogs (18.6%) and multiple acquired shunts in three dogs (18.6%). Liver histopathology findings were similar for all dogs, regardless of outcome. CONCLUSIONS: Cellophane banding is an efficacious method for complete gradual occlusion of single extrahepatic shunts when the shunt vessel is attenuated to < or = 3 mm. Transcolonic portal scintigraphy is a reliable method for assessment of shunt attenuation and, unlike serum bile acids, is not influenced by other causes of liver dysfunction.     

ULTRASONOGRAPHIC DIAGNOSIS OF CONGENITAL PORTOSYSTEMIC SHUNTS IN DOGS: RESULTS OF A PROSPECTIVE STUDY

The aims of this study were to determine if accurate diagnosis of congenital portosystemic shunt was possible using two dimensional, grey-scale ultrasonography, duplex-Doppler, and color-flow Doppler ultrasonography in combination, and to determine if dogs with congenital portosystemic shunts have increased or variable mean portal blood flow velocity. Eighty-two dogs with clinical and/or clinicopathologic signs compatible with portosystemic shunting were examined prospectively. Diagnosis of congenital portosystemic shunt was subsequently confirmed in 38 of these dogs using operative mesenteric portography: 14(37%) dogs had an intrahepatic shunt and 24(63%) had an extrahepatic shunt. Ultrasonography had a sensitivity of 95%, specificity of 98%, and accuracy of 94%. Ultrasonographic signs in dogs with congenital portosystemic shunts included small liver, reduced visibility of intrahepatic portal vessels, and anomalous blood vessel draining into the caudal vena cava. Correct determination of intra - versus extrahepatic shunt was made ultrasonographically in 35/38 (92%) dogs. Increased and/or variable portal blood flow velocity was present in 21/30 (70%) dogs with congenital portosystemic shunts. In one dog with an intrahepatic shunt the ultrasonographic diagnosis was based partly on finding increased mean portal blood flow velocity because the shunting vessel was not visible. Detection of the shunting vessel and placement of duplex-Doppler sample volumes were facilitated by use of color-flow Doppler. Two-dimensional, grey-scale ultrasonography alone is sufficient to detect most intrahepatic and extrahepatic shunts; sensitivity is increased by additional use of duplex-Doppler and color-flow Doppler. Increased and/or variable portal blood flow velocity occurs in the majority of dogs with congenital portosystemic shunts.     

USE OF INTRAOPERATIVE MESENTERIC PORTOVENOGRAPHY IN CONGENITAL PORTOSYSTEMIC SHUNT SURGERY

A retrospective study of the use of intraoperative mesenteric portovenography (IOMP) in the surgical management of congenital portosystemic shunts in 100 dogs and cats was performed. Each portovenogram was scored using a subjective visual analogue scale (VAS) and was assessed for the presence of portal atresia or hypoplasia. VAS scores and portal hypoplasia assessments were obtained for portovenogram images obtained for each animal both before shunt manipulation (preligation) and following the temporary, complete ligation of the vessel (postligation). In each patient, surgical records were reviewed to ascertain the degree of shunt attenuation that was achieved at surgery. Hepatic portal vasculature was significantly different on postligation compared with preligation IOMP. Sixty-two percent of animals had apparent portal hypoplasia or atresia on their preligation IOMP. The majority of these (81%) had an improvement in portal vasculature on postligation IOMP. It was concluded that both preligation and postligation IOMP provided valuable information regarding the morphology of congenital portosystemic shunts. An accurate assessment of an animal's portal vasculature could only be made following the interpretation of a postligation portovenogram, and these findings significantly influenced the surgical management of the patient. Although individuals with high postligation VAS scores were more likely to achieve full shunt attenuation at surgery, there was no quantifiable relationship between IOMP findings and the degree of shunt attenuation achieved.     

Presentation,stabilisation and contrast-enhanced computed tomographic (CT) diagnosis of a left gastrocaval portosystemic shunt in a pony foal

A 2-week-old pony foal was presented for recurrence of neurological dysfunction, obtunded-comatose mentation and collapse. Signalment, history, haematological and clinical chemical measurements and response to treatment prompted a presumptive diagnosis of hepatic encephalopathy secondary to a portosystemic shunt. This case differs from previous reported cases of  congenital portosystemic shunts in horses due to the early onset of clinical signs, which usually occur at 2–6 months of age. In addition, in comparison with previously reported cases, this foal stabilised rapidly with minimal intervention. Contrast-enhanced computed tomography (CT) confirmed a left gastrocaval extrahepatic portosystemic shunt. Surgical intervention was not attempted due to financial constraints, so the foal was subjected to euthanasia, and on post-mortem examination, the anomalous vascular connection could clearly be identified.     

Portosystemic shunts in cats: seven cases (1976-1984)

Congenital portosystemic venous shunt causing signs of hepatic encephalopathy was diagnosed in 7 cats. The left gastric vein served as the shunt in four of these. Increases in blood ammonia and postprandial serum bile acids were the most consistent serum biochemical abnormalities. Excessive variation in red blood cell shape was a common but nonspecific hematologic finding. The jejunal-mesenteric venous injection of contrast material was the preferred method of portography to diagnose portosystemic shunts. Two cats were treated successfully by partial surgical occlusion of their shunts.     

New perspectives on the development of extrahepatic portosystemic shunts

In this essay we use clinical evidence and knowledge of anatomy to examine the relationship between blood flow and formation of congenital extrahepatic portosystemic shunts in dogs and cats. First we report on the clinical findings in a series of 50 dogs and 10 cats and then systematically review peer‐reviewed data on the detailed anatomy of shunts in dogs and cats. In dogs four types of shunt: spleno‐caval, left gastro‐phrenic, left gastro‐azygos and those involving the right gastric vein account for 94% of extrahepatic shunts. Cats also exhibit four types of shunt: spleno‐caval, left gastrophrenic, left gastro‐caval and left gastro‐azygos, and the first three of these account for 92% shunts in this species. Our findings lead us to propose that preferential blood flow influences the subsequent formation of one of a number of defined and consistent congenital extrahepatic portosystemic shunts in dogs and cats.