Search Results (22)

Congenital portosystemic shunts (CPSSs) are often associated with life-threatening systemic complications, which may be detected by identifying hypergalactosemia in newborn screening (NBS). However, diagnosing CPSS at an early stage is not easy. The purpose of this study was to predict CPSS early using screening values and general blood tests. The medical records of 153 patients with hypergalactosemia who underwent NBS in Saitama Prefecture between 1 December 1997 and 31 October 2023 were retrospectively analyzed. We provided the final diagnosis of the analyzed patients. Of the 153 patients, 44 (29%) were in the CPSS group and 83 (54%) were in the transient galactosemia group. Using the initial screening items and the six blood test items, we attempted to extract a CPSS group from the transient galactosemia group. Finally, a model for CPSS prediction was established. From multiple logistic regression analysis, filtered blood galactose-1 phosphate/galactose, serum total bile acid, and ammonia were adopted as explanatory variables for the prediction model. If the cut-off value for predicted disease probability value (P) was >0.357, CPSS was identified with 86.4% sensitivity (95%CI 72.6–94.8%) and 81.9% specificity (95%CI 72.0–89.5%). This predictive model might allow prediction of CPSS and early intervention. Full article

Hepatocellular carcinoma (HCC) is a frequent complication of various liver diseases, occurring with or without underlying cirrhosis. While cirrhosis and chronic liver inflammation are well-established major drivers of hepatocarcinogenesis, HCC can also develop in patients with vascular liver diseases (VLDs), highlighting an alternative pathway of disease progression. Alterations in liver perfusion appear to underlie the process of carcinogenesis. However, the specific molecular mechanisms involved in this process as well as the clinical presentation and imaging features of HCC in the most common VLDs are still a matter of debate. This review aims to evaluate the available literature on the topic to provide a deeper comprehension and analysis of current knowledge about the relation between VLDs and HCC. Specifically, we investigate how HCC affects VLDs such as Budd–Chiari syndrome, Fontan-associated liver disease, congenital portosystemic shunts, cavernous transformation of the portal vein, and porto-sinusoidal vascular disorder. Exploring the pathogenetic mechanisms and diagnostic challenges in HCC related to VLDs may have important therapeutic implications, helping to define targeted treatments for this poorly understood medical entity. Full article

The portosystemic shunt is characterized by the presence of an anomalous vessel, whether congenital or acquired, that connects the portal circulation to the systemic circulation. Surgical treatment is indicated in congenital cases and involves the progressive occlusion of the anomalous vessel. The objective of this study is to report the use of an innovative and fully laparoscopic technique for the attenuation of a gastrophrenic shunt. The procedure was performed on a two-year-old female German Spitz dog with a history of frequent vomiting and diarrhea. The diagnosis was established through computed tomography. For the procedure, three portals were placed in the left lateral region, employing an ameroid constrictor. This device featured a specific perforation for the passage of a pre-mounted suture, which was subsequently occluded intracorporeally, facilitating the closure of the ring defect. The absence of flow in the anomalous vessel was confirmed five weeks after the procedure. It is concluded that the reported implantation technique represents a new modality for the treatment of this type of shunt using a minimally invasive approach. Full article

Background/Objectives: A functional liver is vital for normal protein metabolism. Alterations of circulating amino acid (AA) concentrations have previously been reported in dogs with hepatocellular carcinoma, chronic hepatitis, and hepatocutaneous syndrome. The purpose of this study was to compare serum AA profiles between dogs with a congenital portosystemic shunt (CPSS) and healthy control dogs. Methods: Serum samples were collected from 50 dogs with an extrahepatic congenital portosystemic shunt (eCPSS) and 10 dogs with an intrahepatic congenital portosystemic shunt (iCPSS) at time of surgical intervention and from 21 healthy control dogs. Serum AA and other nitrogenous compounds were measured with a dedicated amino acid analyzer. The concentration of each AA was compared between groups using a Kruskal–Wallis test followed by Dunn’s multiple comparisons tests, as appropriate. The Benjamini–Hochberg procedure was used to control for false discovery. Significance was set at q < 0.05. Results: Compared to healthy controls, dogs with a CPSS had significantly increased serum concentrations of ammonia, asparagine, glutamic acid, histidine, phenylalanine, serine, and tyrosine and had significantly decreased concentrations of isoleucine, leucine, threonine, urea, and valine. There were no significant differences in serum AA concentrations between dogs with an eCPSS and dogs with an iCPSS. Conclusions: Dogs with a CPSS had altered serum AA concentrations compared to healthy control dogs, including decreased branched-chain amino acids (BCAAs) and increased aromatic amino acids (AAAs). In summary, serum AA profiles can differentiate dogs with a CPSS from healthy dogs but not dogs with an eCPSS from dogs with an iCPSS. Full article

The aim of this study was to develop three-dimensional anatomical models of dogs with congenital extrahepatic portosystemic shunts (CEPSs) using 3D printing, as well as to detail their development process and compare the final models to volume rendering (VR) derived from computed tomography (CT) scans. CT scans in the Digital Imaging and Communications in Medicine (DICOM) format of two canine patients were used—one with splenocaval deviation and the other with right gastrocaval deviation. The images were segmented using 3DSlicer software, generating 3D files in Standard Tessellation Language (STL) format, which were then subjected to refinement and mesh adjustment using Blender software. The models were printed on a J750™ Digital Anatomy™ printer, followed by post-processing in a 2% sodium hydroxide solution for 72 h, with subsequent rinsing to remove support resin residues. The printed models showed colored anatomical structures, including the liver; spleen; kidneys; part of the arterial, venous, and portal circulations; and CEPSs. For comparison purposes, VR of the scans was recreated in the RadiAnt DICOM Viewer software. Despite some limitations of the segmentation software, the 3D-printed models effectively represented the anatomy of the patients and the CEPSs, demonstrating good equivalence to the VR. Full article

Background and Clinical Significance: When lactate production surpasses the body’s clearance capacity, hyperlactatemia (lactate ≥ 2 mmol/L) or lactic acidosis (lactate ≥ 4 mmol/L) can develop. Lactic acidosis is classified into type A, which arises from regional or global tissue hypoperfusion, and type B, resulting from metabolic disturbances without tissue hypoxia. Type A lactic acidosis, often associated with conditions like sepsis or shock, is a critical marker of life-threatening conditions, whereas type B lactic acidosis is less frequently recognized in clinical practice. Case Presentation: A 95-year-old man presents with an asthma exacerbation and is treated with an albuterol inhaler. However, he is found to have persistently high lactate levels. Further investigation reveals a congenital intrahepatic portosystemic shunt on imaging. This, in conjunction with the ongoing use of beta-adrenergic receptor agonists, contributes to the development of type B lactic acidosis. Conclusions: The impact of lactic acidosis depends on its severity and clinical context. While beta agonists are a recognized cause of type B lactic acidosis, a potential role for structural liver abnormalities in reduced lactate clearance must be examined further. Full article

Hepatic encephalopathy (HE) in dogs is a metabolic disorder of the central nervous system that occurs secondarily to liver dysfunctions, whether due to acquired or congenital causes. A portosystemic shunt is the presence of abnormal communications between the hepatic vessels (portal and suprahepatic veins). As a result of this, the blood brought from the digestive tract through the portal vein bypasses the liver, and the unmetabolized components of the portal bloodstream enter directly into systemic circulation, causing clinical symptoms of metabolic encephalopathy (HE). A 3-month-old Bichon canine patient with a history of seizures secondarily to a portosystemic shunt (PS), confirmed through color Doppler ultrasound exam and computed tomography, was presented for evaluation. The typical electroencephalographic (EEG) traces recorded were characterized by the presence of bilateral symmetrical triphasic waves, resembling non-convulsive status epilepticus. The presence of this EEG pattern is useful in choosing the best therapeutic option in order to not accentuate the HE sings and, consequently, to decrease the mortality risk due to a prolonged status epilepticus. Full article

CTHV is a non-invasive and accurate method for assessing liver volume in dogs. CTHV has not been studied in each type of extrahepatic PSS in dogs. This study aimed to use CTHV to compare liver volumes in dogs with different types of PSSs that had been confirmed by computed tomography angiography. Dogs with PSSs were retrospectively included and categorized into IH, EHPC, EHPA, or EHPP shunt groups. Manual CTHV was performed, and the normalized liver volume (nLV) and the difference in nLV from the estimated liver volume calculated based on body weight (LV%diff) was calculated. The study included 57 dogs: 20 IH, 21 EHPC, 9 EHPA, and 7 EHPP. The median nLV (cm³/kg) and LV%diff (%) for each group were as follows: IH 17.3 (−40.4%); EHPC 16.9 (−60.3%); EHPA 15.1 (−56.7%); and EHPP 17.2 (−59.2%), respectively. There were no significant differences in nLV among the PSS types. However, LV%diff was significantly more pronounced in the EHPC and EHPA groups compared with the IH group. Additionally, smaller dogs exhibited more severe microhepatia, with a significant positive correlation between LV%diff and body weight (p < 0.01). These findings suggest that microhepatia severity varies by shunt type and is more severe in smaller dogs, highlighting the need for further research to understand the underlying mechanisms. Full article

Congenital portosystemic shunts (CPSS) are vascular anomalies resulting in liver hypoplasia and hepatic insufficiency. Cats with CPSS typically show signs of hepatic encephalopathy associated with increased ammonia, inflammatory cytokines, and oxidative stress. Surgical attenuation of the CPSS results in improved liver function, resolution of clinical signs, and increased portal blood flow. Hepatic gene expression has not previously been investigated in cats with CPSS. Here, we compared the hepatic expression of genes involved in the urea cycle (CPS1, NAGS), angiogenesis (VEGFR2, NPPA, NPR1, NPPC, NPR2, HIF1a), liver regeneration (SERPINB1, HGF, TGFβ), and metabolism (FGF21) from a small series of cats (n = 18) with CPSS to that of control cats (n = 10). The expression of TGFβ, VEGFR2, HGF, FGF21, and CPS1 was significantly elevated in liver biopsies from cats with CPSS. Cats that could only tolerate partial closure of their CPSS had increased hepatic expression of SERPINB1, HIF1a, and NPR2 compared with those that could tolerate complete ligation. Furthermore, there were no significant correlations between gene expression and pre-operative plasma ammonia concentrations in cats with CPSS. The changes in hepatic gene expression in cats with CPSS are in direct contrast to those seen in dogs with CPSS, suggesting alternative mechanisms may be involved in mediating hepatic changes in cats with CPSS. Full article

The neutrophil-to-lymphocyte ratio (NLR) can help in assessing inflammatory diseases, sepsis, and chronic hepatic conditions in humans. Dogs with congenital portosystemic shunts (PSSs) have signs of generalized inflammation, and the clinical signs can overlap with other conditions, including hypoadrenocorticism (HOC). Thus, the potential diagnostic and prognostic value of leukocyte ratios as surrogate markers was assessed in a retrospective case–control study including 106 dogs diagnosed with PSSs. The disease control groups were dogs with parenchymal hepatopathy (PH; n = 22) or HOC (n = 31). In the PSS dogs, the blood NLRs were associated with the severity of systemic inflammation but not with the shunt type, hepatoencephalopathy, systemic infection, or hypoglycemia. The baseline NLRs did not differ between the three disease groups, between medically and surgically treated PSS dogs, or between those with successful PSS ligation and dogs experiencing peri-/post-surgical complications. However, dogs requiring two consecutive surgical interventions had significantly higher NLRs, and an NLR of <2.53 distinguished dogs with successful shunt ligation in one surgery from those requiring two consecutive surgeries for PSS closure. The blood NLR might be a useful clinicopathologic variable in PSS, but its value in helping differentiate PSS from HOC cases appears low. Integrating the NLR into a diagnostic algorithm may allow for a prediction of the number of surgical interventions required. Full article

The main objective was to conduct a prospective study reporting the outcome for dogs with an extrahepatic congenital portosystemic shunt (CPSS) treated with a ‘complete ligation where possible’ philosophy. The second aim was to compare the outcomes following complete (C) polypropylene suture ligation versus partial thin film band (TFB) attenuation of a CPSS in dogs. Dogs that could not tolerate acute complete shunt ligation at surgery received partial shunt attenuation with TFB. Peri-operative complications, mortality, follow-up imaging findings, pre- and post-operative bile acid stimulation test results and details of any revision surgery performed were recorded. A follow-up health-related quality of life questionnaire enabled the calculation of a postoperative clinical shunt score, a quality of life score, and determined if any dogs were still on a hepatic diet and/or other medical management at a minimum of 6 months after surgery. Of the 110 dogs enrolled, 57 received complete ligation and 53 received partial TFB attenuation. Peri-operative mortality, the occurrence of post-attenuation neurological complications, the occurrence of multiple acquired shunts, the postoperative clinical shunt score and quality of life score were not significantly different between the two groups. Dogs in the C group were older, heavier and demonstrated a greater number of shunt classifications where the entry into the systemic circulation was the phrenic vein or azygous vein. Dogs in the TFB group had a greater number of unchanged bile acid concentrations after surgery, were more likely to remain on the hepatic diet and/or medical management after surgery and underwent a greater number of revision surgeries. There was variability in the precision of both ultrasound and computed tomographic angiography follow-up imaging compared to intra-operative mesenteric portovenography findings at revision surgery. Overall, dogs with an extrahepatic portosystemic shunt receiving either complete acute shunt ligation or partial TFB shunt attenuation are expected to have an excellent long-term clinical outcome and there is no reason to suggest that a dog able to tolerate complete acute shunt closure should be denied the benefit of this. Full article

The objective was to conduct a prospective, randomized study to compare mesenteric portovenogram findings following partial polypropylene suture versus thin film band extrahepatic portosystemic shunt attenuation in dogs. Dogs with extrahepatic portosystemic shunts that could not tolerate complete acute shunt closure received a partial attenuation with either a polypropylene suture or synthetic polymer thin film band. At a routine second surgery three months after shunt patency, missed shunt branches and/or development of multiple acquired shunts were assessed using intra-operative mesenteric portovenography. Twenty-four dogs were enrolled, 12 received partial polypropylene suture ligation, and 12 received partial thin film band shunt attenuation. Intra-operative mesenteric portovenography three months later demonstrated that nine dogs (75%) in the thin film band group had achieved complete shunt closure versus two dogs (16.7%) in the polypropylene suture group, which was significantly different (p = 0.004). No dogs in the polypropylene suture group and two dogs (16.7%) in the thin film band group developed multiple acquired shunts. This is the first study directly comparing follow-up intra-operative mesenteric portovenography imaging findings between two methods of partial portosystemic shunt attenuation in dogs. The study provides accurate information on the rates of complete anatomical shunt closure and development of multiple acquired shunts following partial shunt attenuation with either synthetic polymer thin film band or polypropylene suture. Full article

Congenital portosystemic shunts (CPSS) are a common vascular anomaly of the liver in dogs and cats. Clinical signs of CPSS are non-specific and may wax and wane, while laboratory findings can raise the clinical suspicion for CPSS, but they are also not specific. Definitive diagnosis will be established by evaluation of liver function tests and diagnostic imaging. The aim of this article is to review the management, both medical and surgical, complications, and prognosis of CPSS in dogs and cats. Attenuation of the CPSS is the treatment of choice and may be performed by open surgical intervention using ameroid ring constrictors, thin film banding, and partial or complete suture ligation or by percutaneous transvenous coil embolization. There is no strong evidence to recommend one surgical technique over another. Medical treatment strategies include administration of non-absorbable disaccharides (i.e., lactulose), antibiotics, and dietary changes, and are indicated for pre-surgical stabilization or when surgical intervention is not feasible. After CPSS attenuation, short- and long-term post-surgical complications may be seen, such as post-operative seizures and recurrence of clinical signs, respectively. Prognosis after surgical attenuation of CPSS is generally favorable for dogs and fair for cats. Full article

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. Full article

The portal venous system is a network of vessels that carry blood from the capillary beds of the major abdominal organs to the liver. During embryology, the portal venous system can develop aberrantly, leading to vascular connections between the portal and systemic venous circulation known as portosystemic shunts. The purpose of this comparative review with a few short representative case reports was to present the similarities and differences in portosystemic shunts in humans and small animals and their radiologic evaluation. Aberrant vascular connections between the portal and systemic venous circulation enable portal blood to bypass metabolism and detoxification in the liver, leading to significant clinical implications. Portosystemic shunts are very rare in humans, but these connections are much more common in small animals, affecting up to 0.6% of small animals. Portosystemic shunts can be congenital or acquired and are divided into intrahepatic and extrahepatic types. Because of its ability to accurately assess abdominal structures, large vessels, and their flow dynamics without anesthesia, ultrasonography has become the first imaging modality employed for the diagnostic evaluation of portosystemic shunts in both humans and small animals. This is usually followed by contrast-enhanced computed tomographic angiography in order to better define the exact shunt anatomy and to plan treatment. It is important to understand the embryology, anatomy, pathology, and pathophysiology of portosystemic shunts in order to understand the findings of radiologic imaging and to initiate appropriate treatment. Full article