Coagulation profiles in dogs with congenital portosystemic shunts before and after surgical attenuation

BACKGROUND: Serious postoperative hemorrhage has been reported in dogs after closure of congenital portosystemic shunts (CPS). HYPOTHESIS: In dogs with portosystemic shunting, low coagulation factor activity is responsible for coagulopathy, which can cause complications after surgery. ANIMALS: Thirty-four dogs with CPS and 39 healthy dogs. METHODS: In a prospective study, coagulation times, platelet count, and the activity of 8 coagulation factors were measured in dogs before and after surgical shunt attenuation and in 31 healthy dogs. The effect of abdominal surgery on hemostasis was determined at ovariectomy in 8 healthy dogs. RESULTS: Dogs with CPS had lower platelet counts, lower activity of factors II, V, VII, and X, and increased factor VIII and activated partial thromboplastin time (APTT) compared to healthy dogs. After surgical attenuation, dogs with CPS had decreased platelet counts and activity of factors I, II, V, VII, IX, X, and XI and a prolonged prothrombin time (PT). Ovariectomy resulted in decreased activity of factors VII and X. Six weeks after surgery, portosystemic shunting persisted in 9 of 30 dogs, with no improvement of hemostatic values. CPS dogs without shunting had improved coagulation times and increased activity of factors II, V, VII, and X. CONCLUSIONS AND CLINICAL IMPORTANCE: Dogs with CPS have lower activity of clotting factors compared to healthy dogs, resulting in a prolonged APTT. Surgical attenuation of the shunt results in increased abnormalities in coagulation times and factors immediately after surgery. Hemostasis is normalized after complete recovery of shunting after attenuation, in contrast to dogs with persistent shunting.     

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.     

Gauged attenuation of congenital portosystemic shunts: results in 160 dogs and 15 cats

Portosystemic shunts were ligated over a gauged stainless steel rod in 160 dogs and 15 cats, using a midline celiotomy. The diameter of the rod varied with the size of the shunt and the diameter of the portal vein cranial to the shunt. Shunts were narrowed to the smallest diameter that did not cause signs of portal hypertension such as cyanosis of the stomach, pancreas, and small intestine. A slight discoloration was accepted only if the heart rate, end-expiratory CO2%, or arterial blood pressure (if available) did not deviate more than 15% from the values that were recorded at the beginning of the surgical procedure. The perioperative mortality (0-30 days) was 29%. The most common cause of death was euthanasia because of hypoplasia of the portal vein cranial to the shunt. Animals with intrahepatic shunts had a significantly lower probability of survival than animals with extrahepatic portocaval or portoazygos shunts. In dogs, large breed and a high body weight were also significant risk factors for non-survival. Age had a significant effect on risk of non-survival, with an increased risk for older dogs, irrespective of the breed of the dog (large breed vs. small breed). The probability of survival without recurrence of hepatoencephalopathy (HE) after 1 and 4 years was 61.3% and 55.7%, respectively. The only variable that was significantly associated with non-recurrence of HE was the breed of the dog, there being a lower probability for large breeds. Among the animals that survived surgery for more than 30 days, there was a significant higher probability of recurrence of HE in cats than in dogs.     

Treatment of intrahepatic congenital portosystemic shunts in dogs: a systematic review

The aim of this study was to establish the evidence base for the treatment of intrahepatic congenital portosystemic shunts in dogs through a systematic review of the pertinent literature. Studies were filtered for evidence to answer the question “Which of the treatment options for intrahepatic CPSS in dogs offers the best short‐ and long‐term outcome?” Studies were assigned a level of evidence based on a system published by the Oxford Centre for Evidence‐Based Medicine. Thirty‐two studies were included in the review. Twenty‐six provided level 4 evidence and six provided level 5 evidence. There were no level 1, 2 or 3 studies. One study compared surgical treatment with medical management and one study compared suture ligation with ameroid constrictor placement. The remaining studies were case series describing the outcome for one treatment method alone. Methods and timings of assessments of short‐ and long‐term outcomes were highly varied, making direct comparisons challenging. The evidence regarding the treatment of intrahepatic congenital portosystemic shunts in dogs is weak, with only two studies directly comparing treatments. There is a lack of evidence regarding short‐ and long‐term outcomes on which to base clinical decisions.     

Glomerulopathy in dogs with congenital portosystemic shunts

Kidney specimens from 12 dogs with congenital portosystemic shunts were examined histologically. Glomerulopathy of variable severity was present in the kidney sections of all 12. Marked irregular thickening of the glomerular capillary wall was the most prominent pathological change, the renal interstitium being largely unaffected. The severity of lesions was not correlated with the age of dogs at the time of necropsy. An immunoperoxidase technique failed to demonstrate significant IgA or IgG deposition in affected glomeruli. Proteinuria was generally mild or absent despite significant glomerular lesions, except in dogs with concurrent urinary tract infection.     

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.     

Progressive remission of portosystemic shunting in 23 dogs after partial closure of congenital portosystemic shunts

The congenital portosystemic shunts in 23 dogs were closed partially in 18 and completely in five with a single silk ligature. The clinical results were studied and the degree of portosystemic shunting was measured by a scintigraphic method, the results being expressed as the shunt index (SI). In 17 of the dogs, the mean (sd) SI decreased from 0.92 (0.16) before surgery to 0.34 (0.25) during surgery after the attenuation of the shunt, and then to 0.10 (0.12) one month later. The dogs' venous ammonia concentration decreased from 203 (122) microM before surgery to 36 (18) one month after surgery. At the same time the clinical scores improved significantly. There were positive correlations between the SI and the general evaluation of the dogs' well-being by their owners (rs = 0.60), the ammonia concentration (rs = 0.86), and the diameter of the shunt (rs = 0.86). In the other six dogs, the intraoperative and/or postoperative SI was high. In two of them the shunt was further attenuated during a second operation, which resulted in lower SI values; in two a second small shunt was responsible for the high SI; in one multiple portosystemic shunts were found postmortem; and one dog was lost to follow-up.     

Congenital portosystemic shunts in five mature dogs with neurological signs

Congenital portosystemic shunts are a common cause of hepatic encephalopathy and are typically first identified when dogs are <2 years of age. This case series describes five dogs with congenital portosystemic shunts; the dogs were presented for severe encephalopathic signs during middle or old age. Three dogs had portoazygos shunts, and four dogs had multifocal and lateralizing neurological abnormalities, including severe gait abnormalities and vestibular signs. All five dogs responded to medical or surgical treatment, demonstrating that older animals can respond to treatment even after exhibiting severe neurological signs.     

Hemostatic profiles in 39 dogs with congenital portosystemic shunts

OBJECTIVES: To determine if there were significant changes in prothrombin time (PT), partial thromboplastin time (PTT), and fibrinogen levels in dogs with naturally occurring congenital portosystemic shunts (CPSS) and to determine if there was any association between these values, serum albumin concentration, and the ability to attenuate the shunt vessel. STUDY DESIGN: Retrospective clinical study. ANIMALS: Thirty-nine client-owned dogs. METHODS: Medical records of 60 dogs with confirmed CPSS were retrospectively evaluated. Hemostatic profiles had been performed before surgery in 39 cases. RESULTS: Dogs with CPSS had significantly higher values for PTT (P < .001) when compared with normal dogs. Of the total number of dogs, 64.1% had a PTT greater than 16 seconds (25/39). PTT was prolonged by 25% or more in 51.3% of dogs (20/39). PT tended to be higher in dogs with CPSS (P = .036), although only 7.7% (3/39) of dogs had a PT greater than 12 seconds (the maximum reference value). Dogs with CPSS had significantly lower values for albumin and fibrinogen (P < .001). Platelet numbers were within the normal range in 87.2% of cases (34/39). Of the 5 dogs with platelet numbers outside the normal range, 3 were mildly thrombocytopenic. Fibrin degradation product concentrations were not elevated in any dogs tested (N = 22). There was no significant difference in any of the measured variables between dogs with extrahepatic shunts and those with intrahepatic shunts (P > .1). For PT, PTT, albumin, and fibrinogen, there was no significant difference between dogs that underwent total, partial, or no attenuation (P > .3). CONCLUSIONS: Dogs with CPSS have a tendency to have a prolonged PTT. There was no significant difference in hemostatic profile results between dogs with intrahepatic shunts versus extrahepatic shunts. Preoperative hemostatic profile abnormalities were not useful as predictors of ability to attenuate CPSS. CLINICAL RELEVANCE: Prolonged PTT was not associated with bleeding tendencies in any of the dogs. Assays of individual clotting factors may help to further characterize the abnormalities present in animals with CPSS and may identify specific factor deficiencies. This might enable identification of a noninvasive diagnostic or prognostic indicator.     

Serum hyaluronic acid in dogs with congenital portosystemic shunts

Objectives : To compare the serum level of hyaluronic acid in dogs with congenital portosystemic shunt with that in healthy dogs and to investigate the perioperative change in serum hyaluronic acid following shunt attenuation. Methods : Blood samples were obtained from 29 congenital portosystemic shunt dogs before the operation, and 2 and 4 weeks after the operation from 17 and 7 dogs, respectively. The serum hyaluronic acid level of these dogs was measured and compared with that of 10 healthy beagles. Results : The median preoperative hyaluronic acid level in dogs with congenital portosystemic shunt was significantly elevated compared with that in healthy dogs. Furthermore, the median postoperative hyaluronic acid level significantly decreased compared with the median preoperative levels in congenital portosystemic shunt dogs. Clinical Significance : In the case of dogs with congenital portosystemic shunt, the reduction of intrahepatic portal blood flow might lower the clearance rate of hyaluronic acid in hepatic sinusoidal endothelial cells, so hyaluronic acid clearance could be improved by attenuation of a shunt vessel. Hence, serum hyaluronic acid levels might be useful to evaluate liver function and also have the potential to evaluate successful attenuation of a shunt vessel in dogs with congenital portosystemic shunt. Further investigations are required to clarify whether serum hyaluronic acid offers significant benefits over existing markers such as serum bile acid or ammonia concentrations.     

Ultrasonography in the diagnosis of congenital portosystemic shunts in dogs

Summary

The value of ultrasonographic examination in the diagnosis of congenital portosytemic shunts was assessed in 36 dogs, using the right lateral approach. The sensitivity, specificity, and accuracy were 0.74, 1.0, and 0.86 respectively. The conclusion is that ultrasonography is highly specific and reasonably sensitive in diagnosing congenital portosystemic shunts in dogs     

Adrenal response to adrenocorticotropic hormone in dogs before and after surgical attenuation of a single congenital portosystemic shunt

BACKGROUND: Dogs with single congenital portosystemic shunts (CPSS) often develop postoperative hypoglycemia and prolonged anesthetic recovery. These abnormalities could be attributable to inadequate adrenal response. However, adequacy of adrenal response after CPSS surgery is unexplored. HYPOTHESIS: Dogs with CPSS have inadequate postoperative adrenal response. ANIMALS: Eight nonoperated, 8 ovariohysterectomy (OHE), and 16 CPSS dogs. METHODS: Consecutive day ACTH stimulation tests were performed on nonoperated healthy dogs, healthy dogs before and after OHE, and CPSS dogs before and after surgery. Adequate response was defined as >50% or >30 ng/mL increase in cortisol after ACTH administration. Blood glucose (BG) was monitored before and after surgery. Prolonged anesthetic recovery and refractory hypoglycemia episodes were recorded. RESULTS: Results of consecutive day ACTH stimulation tests did not vary in normal dogs. Results of preoperative ACTH stimulation tests of CPSS and OHE dogs were not significantly different. Dogs with CPSS had higher postoperative baseline cortisol concentrations (median, 329 ng/mL) than OHE dogs (median, 153 ng/mL). Postoperative cortisol increase after ACTH in CPSS was < or =50% in 10/16 and < or =30 ng/mL in 6/16. After surgery, BG was < or =60 mg/dL in 7/16 CPSS dogs. Cortisol concentrations were not correlated with BG. Two CPSS dogs had refractory hypoglycemia and 4 had delayed recovery; all improved with dexamethasone administration (0.1-0.2 mg/kg/IV). CONCLUSIONS AND CLINICAL IMPORTANCE: Contrary to previous reports, baseline cortisol concentrations in CPSS and healthy dogs are similar. Many CPSS dogs have postoperative hypercortisolemia. Response to ACTH does not correlate with postoperative hypoglycemia or prolonged anesthetic recovery.     

A prospective study of basal insulin concentrations in dogs with congenital portosystemic shunts

Objectives : Hypoglycaemia is a common cause of morbidity in dogs with congenital portosystemic shunts but the aetiology is unknown. The hypothesis of this study was that dogs with congenital portosystemic shunts would have significantly higher insulin concentrations than dogs without congenital portosystemic shunts. The main objective of the study was to compare peripheral glucose and insulin concentrations between dogs with congenital portosystemic shunts and dogs without congenital portosystemic shunts. Methods : Peripheral serum insulin and plasma glucose concentrations were measured in dogs with congenital portosystemic shunts and without congenital portosystemic shunts and compared both between groups as well as to reference intervals derived from healthy dogs. Results : Congenital portosystemic shunts were diagnosed in 41 dogs. Forty‐eight dogs hospitalised with other conditions acted as controls. Serum insulin concentrations were mildly elevated (Ä40 μU/mL) in seven dogs and were markedly elevated in two dogs with congenital portosystemic shunts, yet mild hypoglycaemia (3·3 mmol/L) was detected in only one of these dogs. Four dogs with congenital portosystemic shunts showed fasting hypoglycaemia, yet insulin concentrations were within or below the reference interval in three. There was no difference between the median insulin concentration of dogs with congenital portosystemic shunts and without congenital portosystemic shunts. Clinical Significance : Hyperinsulinaemia is infrequently observed in dogs with congenital portosystemic shunts. The aetiology of hypoglycaemia in dogs with congenital portosystemic shunts merits further investigation.     

Evaluation of cellophane banding with and without intraoperative attenuation for treatment of congenital extrahepatic portosystemic shunts in dogs

OBJECTIVE: To evaluate the effect of intraoperative attenuation of congenital extrahepatic portosystemic shunts (CEPSSs) during cellophane banding procedures in dogs. STUDY DESIGN: Retrospective case series and prospective study. ANIMALS: 18 cases evaluated retrospectively and 14 dogs evaluated prospectively. PROCEDURES: Gradual occlusion of CEPSSs was performed via cellophane banding. Shunts were occluded to a diameter < 3.0 mm during surgery in dogs prospectively enrolled in the partial attenuation group, whereas the shunt was not attenuated during surgery in dogs prospectively enrolled in the no-attenuation group or in dogs that had previously undergone surgery and were retrospectively evaluated. Postprandial serum bile acids (PPSBA) concentrations were measured before surgery and at various time points after surgery. RESULTS: Mean +/- SD PPSBA concentrations were 26.8 +/- 24.5 micromol/L at < 2.25 months after surgery (n = 16 dogs), 22.1 +/- 14.0 micromol/L from 2.25 to 6 months after surgery (12 dogs), and 34.9 +/- 32.5 micromol/L at > 6 months after surgery (22 dogs). In the prospectively enrolled dogs, mean PPSBA concentrations increased over time in dogs in the partial attenuation group, but not in dogs in the no-attenuation group. CONCLUSIONS AND CLINICAL RELEVANCE: Cellophane banding may be used to occlude larger CEPSSs and may decrease the need for intraoperative monitoring of portal vein blood pressure. The technique may facilitate minimally invasive treatment of CEPSSs in dogs. Intraoperative attenuation of CEPSSs to a diameter < 3.0 mm is not necessary and may result in a less favorable outcome.     

The pathophysiology and laboratory diagnosis of congenital portosystemic shunts in dogs

Congenital portosystemic shunts generally arise as single vascular anomalies that cause the portal blood to bypass the liver and enter the systemic venous circulation directly. The liver is primarily affected, as it is deprived of perfusion by portal hepatotrophic factors such as insulin, glucagon, and amino acids. There is progressive hepatic atrophy, and as a consequence, dysfunction.

Hepatic encephalopathy can result from increased levels of ammonia and gamma-aminobutyric acid within the systemic circulation. Variably toxic amines, captans and short chain fatty acids may act as false neurotransmitters. Hypoglycaemia will exacerbate the effects of these substances. Increased concentrations of ammonia and uric acid in the urine predispose to the precipitation of ammonium biurate crystals and the formation of calculi.

Haematological changes include anaemia, microcytosis, hypoproteinaemia, leucocytosis, and coagulation abnormalities. Gastrointestinal effects are common. They may be displayed as anorexia, vomiting, ptyalism, pica, diarrhoea, or polyphagia. Most dogs are less than 1 year of age at initial presentation.

Diagnosis from a laboratory viewpoint will involve a consideration of the history, clinical findings, haematology, serum biochemistry and urinalysis. If the findings are suggestive of a congenital portosystemic shunt, the demonstration of elevated fasting or, more consistently, post-prandial serum bile acid concentrations, and subsequent histological examination of a liver biopsy will provide a definitive diagnosis.