Diagnosis Of Congenital Portosystemic Shunt Research Articles

Congenital Portosystemic Shunts in Dogs and Cats: Classification, Pathophysiology, Clinical Presentation and Diagnosis.

Congenital portosystemic shunts (CPSS) are abnormal vascular communications between the portal and the systemic circulation, bypassing the hepatic parenchyma and resulting in liver hypoplasia and hepatic insufficiency. Such connections develop in utero and persist postnatally. CPSS are among the two most common congenital vascular anomalies of the liver in small animals, along with primary hypoplasia of the portal vein without portal hypertension (PHPV without PH). CPSS can be extrahepatic (ECPSS), most commonly diagnosed in small and toy breed dogs and cats, or intrahepatic (ICPSS), most commonly seen in large breed dogs. Single ECPSS is the most common type encountered in both dogs and cats. Clinical signs of CPSS are non-specific and may wax and wane, while laboratory findings can raise clinical suspicion for CPSS, but they are also not specific. Definitive diagnosis will be established by evaluation of liver function tests, such as determination of fasting plasma ammonia (FA) levels, and pre- and postprandial serum bile acids concentrations, and diagnostic imaging. The purpose of this article is to review the definition, classification, pathogenesis, clinical presentation, and diagnosis of CPSS in dogs and cats, highlighted by the authors' clinical experience.

Congenital portosystemic shunts: surgical treatment experience

Congenital porto-caval shunts are rare and may have a different morphological structure (intra- and extrahepatic shunts, with or without portal blood flow). The main method of treating patients with this pathology is endovascular shunt occlusion. However, in some cases, this method is ineffective.
 The article contains a description of six clinical examples of surgical treatment of congenital porto-systemic shunts in children. In the diagnosis of congenital portosystemic shunts, the leading role belongs to Doppler ultrasound, multislice computed tomography, and angiography. The indication for surgical treatment was the anatomical features of the shunt, which makes endovascular occlusion technically impossible. In one observation a wide Arantian duct was diagnosed, its open ligation was performed. In another case, the portal vein emptied directly into an aneurysmal dilatation, performed reconstructive plastic surgery on the vessels of the portal vein. In the next observation, a pronounced retrograde blood flow was determined along the dilated inferior mesenteric vein, blood was discharged through the sacral plexus into the internal iliac vein. The left internal iliac vein was isolated and ligated, the dysplastic inferior mesenteric vein was ligated and partially removed. In 2 patients, the portal vein flowed directly into the inferior vena cava in the area of aneurysmal expansion; an operation was performed open ligation of the shunt. In one observation, a deep hypoplasia of the intrahepatic branches of the portal vein was diagnosed, and therefore the restoration of portal blood flow after the closure of the shunt is impossible. The child was sent to decide on a liver transplant.
 Each case of congenital porto-caval shunts is unique. The surgeon determines the tactics directly during the operation, depending on the morphological structure of the organs, since the preoperative examination does not always give an unambiguous idea.

A rare and curable cause of cyanosis – Congenital portosystemic shunts

Congenital portosystemic shunts (CPSSs) are rare developmental vascular anomalies which are classified into extrahepatic and intrahepatic malformations. Here, we report a 7-year-old girl who presented with a history of progressive dyspnea, pandigital cyanosis, and clubbing. On evaluation, she was found to have a rare combination of both type II extrahepatic and intrahepatic portosystemic shunts which were closed endovascularly in the same setting. Diagnosis of CPSS needs a very high index of clinical suspicion. Timely diagnosis with specific imaging modalities will help in deciding the treatment strategy and may prevent long-term complications.

Prenatal diagnosis of congenital portosystemic shunt: A single-center study.

To review our experience with the prenatal diagnosis of congenital portosystemic shunt (CPSS). This is a retrospective study of CPSS cases examined at an ultrasonographic tertiary referral center from 2013 to 2019. The anatomical origin and drainage of the shunt were assessed. Feto-maternal clinical characteristics and long-term outcomes were investigated via medical files and telephone interviews with the mothers. Eleven cases were reviewed. Based on the anatomical origins, before or after portal vein division, cases were classified into extrahepatic portosystemic shunt (EHPSS, n = 3, 27.3%) and intrahepatic portosystemic shunt (IHPSS, n = 8, 72.7%). Additional abnormalities were also observed in the EHPSS (n = 2, 66.7%) and IHPSS (n = 3, 37.5%) groups. Intrauterine growth restriction was the most common abnormality (n = 4, 80%). The median age of the pregnant women was 31.9 years (range 26 ~ 43 years). Most cases (n = 8, 72.7%) were diagnosed in the third trimester, and the median gestational age was 31+5 weeks (range 24 ~ 36+6 weeks). Three cases underwent karyotype examinations, and one had trisomy 13. The median time after birth was 2 years (range 0.7 ~ 5.7 years). The overall postnatal live-birth rate was 60% (6/10), not including one case with no data on pregnancy outcome. The mothers of the six live births indicated that their children were in excellent health. This study indicates that prenatal CPSS diagnosis is feasible, especially in the third trimester. IHPSS is more common than EHPSS. Complicated cases most often occur with EHPSS. Intrauterine growth restriction is the most common concomitant abnormality. The prognosis of most cases is good.

Outcome of non-surgical dietary treatment with or without lactulose in dogs with congenital portosystemic shunts

Background Congenital portosystemic shunts (CPSS) are vascular anomalies, allowing portal blood to bypass the hepatic parenchyma, thereby accumulating toxic substances such as ammonia in the systemic circulation resulting in hepatic encephalopathy. Aim To evaluate the outcome of non-surgically treated dogs with a CPSS. Methods Case records of 78 dogs with a single congenital CPSS confirmed by ultrasound and/or computed tomography between September 2003 and February 2015 were reviewed. Median age at diagnosis of CPSS in dogs was 10.8 months (range 2–133 months). Non-surgical treatment was started as an adjusted diet (a diet restricted in protein) with or without lactulose. Owners were contacted by telephone to determine survival time and presumed cause of death, if applicable. In addition, a questionnaire was used to retrospectively assess quality of life (QoL) and CPSS scores in 37 dogs before and during non-surgical treatment. Differences between Kaplan–Meier curves were tested by a Log rank test. Results Overall estimated median survival time (EMST) was 38.5 months (range 1 day − 91 months; 78 dogs). No significant differences between EMSTs were found between dogs with extra- (n = 48) or intrahepatic (n = 29) shunts, nor between treatment with only an adjusted diet, or an adjusted diet combined with lactulose. During non-surgical treatment, significant improvement in perceived QoL and CPSS scores were found (P < 0.01). Conclusion Our study demonstrated that an overall median EMST of 3.2 years was reached and that owners retrospectively perceived that non-surgical treatment resulted in an improved QoL and clinical performance, irrespective of intrahepatic or extrahepatic CPSS location.

Pre and postnatal diagnosis of congenital portosystemic shunt: Impact of interventional therapy.

IntroductionCongenital portosystemic shunts (CPSS) are rare vascular malformations that can lead to severe complications. With advanced imaging techniques, diagnosis is becoming more feasible occurring in fetal life. Different approaches have been adopted to manage these cases, with an increased utilization of interventional therapy recently. This cohort aims to describe the course of children diagnosed with CPSS and the impact of interventional therapy on the outcome.MethodsRetrospective chart review was done for all patients who were diagnosed with CPSS in our institution between January 2006 and December 2015.ResultsSix patients were diagnosed with CPSS. During this period, 8,680 mothers carrying 9548 fetuses underwent fetal ultrasound examinations. Three patients were diagnosed antenatally at a median [IQ] gestational age of 33 [26–33] weeks, and three patients were diagnosed postnatally at 0, 2, and 43 months, respectively. At a median follow-up of 87 [74–110] months, 5 patients are alive; 4 of whom had received transcatheter closure for different indications, and one who had spontaneous resolution of her CPSS. One infant died at the age of 6 weeks secondary to sepsis.ConclusionCPSS can result in significant complications in children. Interventional therapy is feasible at any age group, but long-term follow-up is warranted.

Comparison of99mTcO4−Trans‐Splenic Portal Scintigraphy with Per‐Rectal Portal Scintigraphy for Diagnosis of Portosystemic Shunts in Dogs

To evaluate trans-splenic portal scintigraphy (TSPS) and per-rectal portal scintigraphy (PRPS) for diagnosis of congenital portosystemic shunts (CPSS) in dogs, and compare these results with surgical findings. Prospective, randomized cross over clinical trial. Dogs (n=42) with suspected CPSS. Dogs had TSPS and PRPS 48 hours apart; quantity of radionuclide administered was recorded. Three independent, blinded reviewers evaluated each scintigraphic study for study quality, shunt presence, number, and location of shunt termination (caudal vena cava, azygos vein). All dogs had exploratory celiotomy. Negative scintigraphic findings were confirmed with intraoperative mesenteric portography. Ameroid constrictors were placed on all extrahepatic CPSS, and hepatic biopsies were obtained. TSPS was 100% sensitive and specific for diagnosis of CPSS and significantly (P<.05) more likely than PRPS to detect shunt number and termination. Interpretation was consistent between observers, and TSPS required significantly less radionuclide than PRPS. TSPS was as sensitive as PRPS for detection of shunting vessels, and consistently yielded studies of higher quality, allowing detection of shunt number and location with consistent interpretation among radiologists. TSPS provides information about shunt number and location, which allows improved surgical planning. Because it requires significantly less radionuclide, TSPS improves safety, allows for more comprehensive patient care, and earlier surgical intervention.

Portosystemic shunts in dogs and cats: laboratory diagnosis of congenital portosystemic shunts

Portosystemic shunts in dogs and cats: laboratory diagnosis of congenital portosystemic shunts

Association of breed with the diagnosis of congenital portosystemic shunts in dogs: 2,400 cases (1980-2002).

To determine the annual and overall proportion of diagnoses of congenital portosystemic shunts (CPSS) in dogs and identify breeds at increased risk for CPSS. Retrospective study. 2,400 dogs with CPSS from veterinary teaching hospitals that reported to the Veterinary Medical Database (VMDB) from January 1, 1980 to February 28, 2002. The proportion of diagnoses of CPSS was calculated for all dogs and each breed recorded in the VMDB annually and for the 22.2-year period. Odds ratios and adjusted confidence intervals were calculated for breeds with at least 100 accessions by comparing odds of each breed with a diagnosis of CPSS with that of mixed-breed dogs. Congenital portosystemic shunts were reported in 0.18% of all dogs and 0.05% of mixed-breed dogs. The proportion of diagnoses of CPSS increased from 5 in 10,000 dogs in 1980 to 5 in 1,000 dogs in 2001. Yorkshire Terriers had the greatest total number of diagnoses of CPSS. Thirty-three breeds were significantly more likely to have a diagnosis of CPSS, compared with mixed-breed dogs. The greatest proportions of diagnoses were found in Havanese (3.2%), Yorkshire Terriers (2.9%), Maltese (1.6%), Dandie Dinmont Terriers (1.6%), and Pugs (1.3%). Certain breeds appear to be at increased risk for CPSS, compared with mixed-breed dogs. The increased odds ratios among specific breeds support the hypothesis of a genetic predisposition for CPSS. Clients and veterinarians should consider appropriate diagnostic tests for dogs with clinical signs and those used for breeding from breeds with increased risk of CPSS.

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.

Ultrasonographic diagnosis of congenital portosystemic shunt in 14 cats.

Twenty-four cats with clinical and, or, clinicopathological signs compatible with portosystemic shunting were examined prospectively using two-dimensional grey-scale, duplex and colour-flow 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 was present 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 extrahepatic 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.

Ultrasonography in the diagnosis of congenital portosystemic shunts in dogs.

The value of ultrasonographic examination in the diagnosis of congenital portosystemic 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.

The pathophysiology and laboratory diagnosis of congenital portosystemic shunts in dogs

Abstract 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.