Congenital Extrahepatic Portosystemic Shunt Research Articles

Comparison of hematologic variables between dogs with congenital intrahepatic and extrahepatic portosystemic shunts.

Dogs with congenital intrahepatic portosystemic shunt (IHPSS) are predisposed to gastrointestinal inflammation, ulceration, and bleeding, unlike dogs with congenital extrahepatic portosystemic shunt (EHPSS). Limited information is available about hematologic differences between dogs with IHPSS and dogs with EHPSS. Compare hemogram variables between dogs with IHPSS and EHPSS. We hypothesized that hematologic variables would differ between the 2 populations, with a higher frequency and severity of anemia and microcytosis in dogs with IHPSS. Twenty-six client-owned dogs with IHPSS and 35 client-owned dogs with EHPSS. Retrospective cross-sectional study. Dogs were included if a CBC was performed before shunt attenuation. Contingency analysis was performed to determine if the frequency of clinical signs and of hematologic variables below the reference range differed between groups. Hematologic and selected biochemical variables were compared between groups using an analysis of covariance with age as a covariate. Gastrointestinal clinical signs (IHPSS, 81% vs EHPSS, 34%; P = .01), anemia (31% vs 6%; P = .01), microcytosis (77% vs 29%; P = .002), and hypochromia (77% vs 49%; P = .03) were more common in dogs with IHPSS than in dogs with EHPSS. Dogs with IHPSS had lower packed cell volume (34% vs 41%, P = .04), hemoglobin concentration (11.5 g/dL vs 13.7 g/dL, P = .03), mean corpuscular volume (57 fL vs 65 fL; P = .001), and mean corpuscular hemoglobin concentration (32 g/dL vs 33 g/dL; P = .04) than dogs with EHPSS. Dogs with IHPSS had a higher frequency of anemia, microcytosis, and hypochromia and exhibited more gastrointestinal clinical signs.

Anatomical classification of feline congenital extrahepatic portosystemic shunts based on CT angiography: A SVSTS and VIRIES multi-institutional study in 231 cats.

The prevalence of anatomical-based subtypes of feline congenital extrahepatic portosystemic shunts (EHPSS) has not been completely elucidated. The goal of this study was to use CT angiography to create an anatomical-based nomenclature system for feline congenital EHPSS. Additionally, subjective portal perfusion scores were generated to determine if intrinsic portal vein development was associated with different shunt conformations or patient age at the time of CT. The SVSTS and VIRIES list services were used to recruit cases. Data collected included patient DOB, gender, breed, weight, CT date, and reported diagnosis. Shunts were classified based upon (1) the shunt portal vessel(s) of origin, (2) the shunt systemic vessel(s) of insertion, and (3) any substantial portal vessels contributing to the shunt. Additionally, hepatic portal perfusion was subjectively scored between 1 (poor/none) and 5 (good/normal) based on the caliber of the intrahepatic PVs. A total of 264 CT scans were submitted from 29 institutions. Due to exclusion criteria, 33 (13%) were removed, leaving 231 CT scans to be included. Twenty-five different EHPSS anatomies were identified with five classifications accounting for 78% of all shunts (LGP [53%], LGC-post [11%], LCG [7%], LGC-pre [4%], and PC [4%]). Shunt origin involved the left gastric vein in 75% of the described classifications. Significant differences were identified among the five most common shunt types with respect to age at the time of CT scan (P=.002), breed (P<.001), and subjective portal perfusion score (P<.001). This refined anatomical classification system for feline EHPSS may enable improved understanding, treatment comparisons, and outcome prediction for cats with these anomalies.

Laparoscopic treatment of congenital portosystemic shunts with portal pressure measurement and portal angiography in 36 dogs.

Laparoscopic surgery is used for canine congenital extrahepatic portosystemic shunts (CEHPSS). However, outcomes of laparoscopic surgery involving simultaneous portal vein angiography and portal pressure measurement to attenuate or completely occlude the shunt vessel in canines remain unclear. This study aimed to evaluate outcomes and complications of laparoscopic portosystemic shunt occlusion (LAPSSO) for CEHPSS. Between June 2014 and March 2021, data on dogs undergoing cellophane banding (CB) and complete occlusion of laparoscopically treated congenital extrahepatic port shunts were collected from hospital records. Cases in which complete occlusion was laparoscopically performed, or a CB was used for gradual occlusion were included. A total of 36 dogs (14 males; median age 32.5 months [range, 5-99] with median body weight, 4.2 kg [range, 1.5-7.9]) that underwent LAPSSO for CEHPSS were included. All the dogs underwent computed tomographic angiography (CTA), and data on blood and radiological examinations were collected. Shunt vessel morphology was categorized using CTA findings. Portal pressure measurements and portal angiography were performed by accessing mesenteric and splenic veins in 30 and 6 cases, respectively. The most common shunt types were spleno-phrenic shunts 16/36 (44.4%), followed by spleno-azygos 9/36 (25.0%), spleno-caval 4/36 (11.1%), right gastric-caval 6/36 (16.6%), and right gastric-caval with caudal loop shunts 1/36 (2.7%). The median portal pressure after complete occlusion was 11.5 mmHg (range, 4-16); portal pressures in the two dogs undergoing CB attenuation were 22 and 24 mmHg. The median operating time in the dogs with right (n = 25) and left (n = 11) recumbent positioning was 55 min (range, 28-120) and 54 min (range, 28-88), respectively. One dog had pneumothorax due to injury to the diaphragm. Another dog developed postoperative hypernatremia and succumbed 5 h post-procedure. Nevertheless, no other dogs exhibited signs of portal hypertension within 72 h. Blood tests and abdominal ultrasounds performed 1-2 months postoperatively revealed no residual shunts. LAPSSO, coupled with portal pressure measurement and portal angiography, was shown as safe and effective approach that facilitated successful occlusion of CEHPSS. Further large-scale prospective studies and analyses of perioperative complications are needed.

Enlargement of the hepatic artery is present in dogs with a congenital extrahepatic portosystemic shunt and is independent of shunt insertion into the systemic circulation.

The accurate diagnosis of portovascular anomalies has been facilitated by improvements in diagnostic imaging technology. In humans, hepatic arterial blood flow changes in response to the reduction in portal blood flow. The hepatic arterial buffer response characterizes an intrinsic regulatory mechanism in response to reduced portal venous blood flow, which results in hepatic arterial enlargement. At the authors' institution, enlargement of the hepatic artery has been anecdotally observed in a population of dogs with extrahepatic portosystemic shunting, consistent with previous literature that documents variability in hepatic arterial size. In this retrospective, blinded, analytical study, a hepatic artery:aorta (Ha:Ao) ratio was assessed on CT studies from 112 dogs, with (n=43) and without (n=69) an extrahepatic congenital portosystemic shunt in order to compare the hepatic artery size independent of body weight between the two populations. A significant increase in the Ha:Ao ratio was documented in dogs with an extrahepatic portosystemic shunt (EHPSS) compared with those dogs with no EHPSS independent of the location of shunt insertion into the systemic circulation (P<.001). Three cases had repeat CT after surgery, and all had Ha:Ao ratio reductions following treatment. The authors propose that this may be an additional imaging feature observed in dogs with an EHPSS.

Clinical outcomes for 20 cats with congenital extrahepatic portosystemic shunts treated with ameroid constrictor ring attenuation (2002-2020).

To report the clinical perioperative, short-term, and long-term outcomes for cats undergoing ameroid ring constrictor (ARC) attenuation of a congenital extrahepatic portosystemic shunt (EHPSS). Retrospective case series from a single veterinary teaching hospital (2002-2020). Twenty client-owned cats with EHPSS. Data collected from medical records included signalment, history, physical examination, clinicopathologic testing, medications, diagnostic imaging, intraoperative findings, perioperative complications, and postoperative clinical outcomes. Long-term clinical outcome was obtained from a standardized owner interview or medical records. Perioperative complications were reported in five cats out of 20, including blindness (two cats), ascites (one cat), head pressing (one cat), and seizures and death (one cat). Short-term clinical outcome was excellent in 14/18 cats, good in 2/18 cats, and poor in 2/18 cats that were available for follow up, and long term clinical outcome was excellent in 15/18, good in 1/18 cats, and poor in 2/18 cats that were available for follow up. Long-term clinical outcome was good or excellent in 16/18 of cats available for follow up. Perioperative complications were reported in five cats. Surgical attenuation of EHPSS with an ARC can result in resolution of clinical signs and biochemical abnormalities in the majority of cats. The perioperative complication rate for feline patients with EHPSS attenuated with an ARC was lower than reported historically. Seizures may persist in the long term despite normal bile acid stimulation test results, complete blood count, and biochemistry analysis.

Manganese Encephalopathy in Setting of a Congenital Extrahepatic Portosystemic Shunt as Cause of Secondary Parkinsonism

Manganese Encephalopathy in Setting of a Congenital Extrahepatic Portosystemic Shunt as Cause of Secondary Parkinsonism

Type II Abernethy malformation with cystic fibrosis in a 12-year-old girl: A case report

BACKGROUND Abernethy malformation, also known as congenital extrahepatic portosystemic shunt, is an uncommon malformation resulting from aberrant development of the portal venous system. Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations in the CFTR gene. It mainly affects the exocrine glands of the respiratory, digestive and reproductive systems. It is considered extremely rare in the Asian population. We present a clinical case involving a pediatric patient of Asian descent who was diagnosed with Abernethy malformation and CF. CASE SUMMARY A 12-year-old girl presented with a medical history of recurring respiratory infections and hemoptysis, and chest computed tomography (CT) showed bronchiectasis. Whole exome sequencing was performed for the patient, yielding findings that revealed a compound heterozygous variant of the CFTR gene: c.233_c.234insT/p.Trp79fsTer3 (maternal origin); c.2909G&gt;A/p.Gly970Asp (paternal origin). CF was diagnosed. The physician’s attention was drawn to the presence of splenomegaly during disease progression. Abdominal enhanced CT revealed splenomegaly, compression of the left kidney, and multiple tortuous dilated vascular shadows were seen at the splenic hilum, which flowed back into the left renal vein and portal vein, suggesting Abernethy malformation type II. Intraoperatively, the abnormal blood flow was seen to merge into the inferior vena cava through the left renal vein without hepatic processing, and the pathology of liver biopsy showed hypoplastic, dilated or absent portal vein branches, both of which supported the diagnosis of Abernethy malformation type II. This represents the initial documented instance of Abernethy malformation accompanied by a CFTR gene mutation in the existing body of literature. CONCLUSION Coexisting Abernethy malformation and CF are rare. Detailed medical history information, abdominal enhanced CT, venography and genetic testing contribute to diagnosis as well as differential diagnosis.

Regional cerebral blood flow in dogs with congenital extrahepatic portosystemic shunts before surgery and six months after successful closure

Previous studies both in humans and dogs with chronic liver diseases have shown that regional cerebral brain flow (rCBF) is altered. The current study aimed to assess abnormalities in rCBF in dogs with congenital extrahepatic portosystemic shunts (cEHPSS), both at diagnosis and after successful surgical attenuation. Furthermore, the influence of age at diagnosis, severity of hepatic encephalopathy (HE) and type of cEHPSS on rCBF were explored as a base for future research.Single photon emission computed tomography (SPECT) with 99mtechnetium-hexamethylpropylene amine oxime tracer was performed before surgical attenuation and six months postoperatively.Twenty-four dogs with cEHPSS had SPECT at time of diagnosis and 13 dogs with a confirmed closed cEHPSS had a second SPECT six months postoperatively. At diagnosis, dogs with cEHPSS had an altered rCBF distribution compared to healthy dogs. This altered rCBF distribution seemed to be most apparent in dogs ≥ one year and in dogs with overt HE at diagnosis. Six months postoperatively, only the rCBF distribution in the subcortical region decreased compared to pre-operatively.In conclusion, all dogs with cEHPSS had altered rCBF which did not seem to normalize completely six months after successful surgical attenuation. Dogs diagnosed at an older age seemed to have more distinct abnormalities in rCBF compared to younger dogs.

Successful Liver Transplantation From a Deceased Donor With Congenital Extrahepatic Portosystemic Shunt: A Case Report

Congenital extrahepatic portosystemic shunt belongs to a family of rare vascular abnormalities. We present a case of congenital extrahepatic portosystemic shunt occurring in a 42-year-old man who died of cerebral hemorrhage and donated his liver. His portal vein angiography revealed that the main portal vein communicated with the left renal vein, suggesting a portosystemic shunt. A liver biopsy showed that the liver tissue structure was normal. His liver was not involved, and the transplantation was carried out smoothly. The recipient recovered smoothly, and the function of the transplanted liver was good.

Repeated specific canine pancreatic lipase measurements do not identify multiple acquired portosystemic shunts in dogs after extrahepatic portosystemic shunt attenuation.

In dogs with portal hypertension (PH), spec cPL is suggested to be increased despite normal pancreatic histology. After attenuation of congenital extrahepatic portosystemic shunts (cEHPSS), multiple acquired portosystemic shunt (MAPSS) can develop as consequence of sustained PH. Presence of MAPSS affects future therapeutic options and prognosis. Evaluate if spec cPL concentrations increase postoperatively in dogs that develop MAPSS and can thus serve as an indicator of PH. Twenty-four dogs with cEHPSS. Dogs classified according to surgical outcome after cEHPSS attenuation (8 with MAPSS [group M], 9 with closed cEHPSS [group C] and 7 with patent blood flow through the original cEHPSS, without evidence of MAPSS [group P]). Spec cPL was measured in preoperative samples (T0), 4 days (T1) and 1 (T2) and 3- to 6-months (T3) after surgery. Spec cPL was within reference interval (<200 μg/L) at all timepoints except at T1. At T1, 2 dogs in group M (321 and >2000 μg/L) and also 1 in group C (688 μg/L) and 1 in group P (839 μg/L) had increased spec cPL concentrations. No differences in spec cPL concentrations between groups or changes over time were identified. Spec cPL is not consistently increased in dogs that develop MAPSS after cEHPSS attenuation and has no potential as a biomarker for the identification of MAPSS after cEHPSS attenuation.

Abernethy malformation: A case report

Congenital portosystemic shunts are rare congenital vascular malformations characterized by a partial or complete portal blood diversion into the systemic circulation. Congenital extrahepatic portosystemic shunts, known as Abernethy malformations, pose a diagnostic challenge due to their low incidence and clinical presentations.&#x0D; A 15-year-old male with a history of chronic epigastric pain and nausea, high arterial blood pressure, recurrent nose bleeds, chest pain, dizziness, dyspnea, low exercise tolerance, hematochezia, and itching was diagnosed with Abernethy malformation type Ib. Imaging studies revealed a dilated portal vein conduit flowing into the inferior vena cava, bypassing the porta hepatis. Multiple liver nodules, heart chamber dilatation, myocardial hypertrophy, and pulmonary hypertension were also discovered. Following multidisciplinary panel meetings, liver transplantation was advised due to the severity of the patients symptoms and shunt anatomy.&#x0D; Furthermore, diagnostic algorithms and other treatment options are discussed.

Diagnostic and monitoring value of ultrasound, computed tomography angiography, and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts

PurposeTo investigate the diagnostic and monitoring value of ultrasound (US), computed tomography angiography (CTA), and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts (CEPS) in children. MethodsWe retrospectively analysed different imaging examinations of 15 children with CEPS. Development of the portal vein before shunt occlusion, shunt location, portal vein pressure, main symptoms, diameter of the main portal vein, and location of secondary thrombosis after shunt occlusion were recorded. Final classification diagnosis was made by portal venography after shunt occlusion, and consistency with other imaging examinations in diagnosing development of the portal vein was calculated using Cohen’s kappa. ResultsCompared with portal venography after shunt occlusion, US, CTA, and portal venography before shunt occlusion had poor consistency in showing the development of hepatic portal veins (Kappa value 0.091–0.194, P > 0.05). Six cases developed portal hypertension (40–48 cmH2O) during the temporary occlusion test, and US showed that portal veins gradually expanded after shunt ligation. Eight patients with haematochezia had inferior mesenteric vein (IMV)-iliac vein (IV) shunts. After surgery, secondary IMV thrombosis was observed in eight cases and secondary splenic vein thrombosis in four cases. ConclusionPortal venography with occlusion testing is very important to accurately evaluate the development of the portal vein in CEPS. The portal vein needs to expand gradually, and partial shunt ligation surgery is necessary in cases diagnosed as portal vein absence or hypoplasia before occlusion testing to avoid severe portal hypertension. After shunt occlusion, US is effective in monitoring portal vein expansion, and both US and CTA can be used to monitor secondary thrombi. IMV-IV shunts can cause haematochezia and are prone to secondary thrombosis after occlusion.

Outcomes in dogs with congenital extrahepatic portosystemic shunts treated with surgical ligation or medical management.

The objective of this study was to evaluate differences in outcomes in dogs treated for extrahepatic portosystemic shunts (EHPSS) by either complete suture ligation, partial suture ligation or medical management. This wasa retrospective, single institutional study. Dogs (n = 152) with EHPSS treated with suture ligation (n = 62), surgery with no ligation (n = 2), or medical management (n = 88). Medical records were reviewed for data on signalment, treatment variables, complications, and outcome. Kaplan-Meier plots were generated to assess survival across groups. Cox's proportional hazard models were used to assess the relationship between survival times and multiple predictor variables. For outcomes of interest, backwards, stepwise regression was performed (p < 0.05). Complete suture ligation was possible in 46/64 (71.9%) of dogs where surgical attenuation was attempted. One dog was euthanized following partial suture ligation due to suspected portal hypertension. Dogs with complete suture ligation of the EHPSS had a significantly longer median survival time (MST) compared to the medical management group (MST not reached vs. 1730 days [p < 0.001]). Complete resolution of clinical signs (without the need for further medical treatment or dietary changes) was achieved in 16/20 (80.0%) dogs with complete suture ligation and 4/10 (40.0%) dogs with partial suture ligation of their EHPSS. Suture ligation (complete or partial) for the treatment of EHPSS, where clinically possible, yielded the best clinical outcome and increased longevity compared to medical management in this study. While medical management for the treatment of EHPSS in dogs is a valid treatment option, better clinical outcomes are achieved with surgical intervention.

Dogs with congenital extrahepatic portosystemic shunts that have persistent shunting after surgery have a higher prevalence of urolithiasis.

To report the presence of urolithiasis in dogs long-term after gradual attenuation of congenital extrahepatic portosystemic shunts (cEHPSS). 25 client-owned dogs that underwent gradual attenuation of a cEHPSS, of which 19 had a closed cEHPSS and 6 developed multiple acquired portosystemic shunts (MAPSS) following surgery. A retrospective study with prospective follow-up was performed. Dogs that underwent cEHPSS surgery and had their postoperative cEHPSS status determined by transsplenic portal scintigraphy or CT angiography 3 months postoperatively were prospectively contacted and invited for a long-term follow-up visit (a minimum of 6 months postoperatively). Retrospective data were collected, and during the prospective follow-up visit a thorough history, blood tests and urinalysis, and ultrasonography of the urinary tract were performed to assess the presence of urinary signs and urolithiasis. Of 25 included dogs, 1 of 19 (5%) dogs with closed cEHPSS and 4 of 6 (67%) dogs with MAPSS had urolithiasis at long-term follow-up. Three (50%) dogs with MAPSS developed new uroliths. Long-term, dogs with closed cEHPSS that initially presented with and without urolithiasis had significantly less urolithiasis compared to dogs with MAPSS (P = .013 and P = .010, respectively). In the 4 dogs with closed cEHPSS that initially presented with nephrolithiasis, nephroliths became smaller or were no longer visible at the long-term follow-up visit. Dogs that developed MAPSS following cEHPSS surgery are at greater risk of urolithiasis compared to those with closed cEHPSS. Furthermore, ammonium urate uroliths might dissolve if portosystemic shunting ceases to exist.

Portovenography Findings Following Partial Polypropylene Versus Thin Film Band Attenuation of a Single Congenital Extrahepatic Portosystemic Shunt: A Prospective Randomized Study in Dogs.

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.

Opioid‐free anaesthesia for surgical attenuation of a congenital extrahepatic portosystemic shunt in a dog

AbstractAnimals with impaired hepatic metabolism may experience reduced effect from some anaesthetic drugs and are more prone to developing unwanted side effects. Due to reduced blood flow to the liver, it is important to carefully select anaesthetic drugs in patients with extrahepatic shunt. We present the case in which a dog was submitted for surgical attenuation of congenital extrahepatic portosystemic shunt through placement of an ameroid constrictor. A multimodal regimen was designed in order to provide an opioid‐free anaesthesia, relying on analgesia through a locoregional quadratus lumborum block with bupivacaine. Dexmedetomidine was administered intramuscularly as pre‐anaesthetic medication, propofol was used for the induction and anaesthesia was maintained with isoflurane. The present case describes the first case in which an extrahepatic portosystemic shunt was surgically treated using an opioid‐free anaesthesia in a dog.

ABERNETHY MALFORMATION PRESENTING WITH LIVER MASS : A RARE PRESENTATION OF A RARE ENTITY

Abernethy malformation is a very rare congenital extrahepatic porto-systemic shunt (CEPS) that develops between portomesenteric vasculature and systemic veins (IVC / left renal vein / left iliac vein / left hepatic vein) and thus bypassing the liver and resulting in deranged metabolism of various metabolites. They have clinical features varying from that of hypergalactosemia to hyperbilirubinemia to hyperammonemia and pulmonary veins congestion, hepatopulmonary syndrome, hepatic encephalopathy. Another presentation in the spectrum is patient presenting with a hepatic mass lesion, which form as a liver's response to absent/reduced portal ow leading to regenerative nodular hyperplasia. These nodules with time can progress into hepatic tumors like adenoma ,focal nodular hyperplasia(FNH), hepatoblastoma and even hepatocellular carcinoma(HCC). Ultrasound (USG) is a useful tool for screening of congenital anomalies associated with the liver. Diagnosis of abernethy malformation requires a high index of suspicion and can easily be overlooked if not carefully evaluated. In the majority of cases, the complications and other secondary features often lead to diagnosis of abernethy malformation. Also,we should emphasize the search for an underlying abernethy malformation on the background of hepatic mass(as in our case). We report a case of abernethy malformation presenting with liver mass on screening USG and conrmed on contrast enhanced computed tomography (CECT).

Prediction of outcome after ligation or thin film banding of extrahepatic shunts, based on plasma albumin concentration and hematologic expression of 8 target genes in 85 dogs.

In dogs with a congenital extrahepatic portosystemic shunt (EHPSS), outcome after surgical attenuation is difficult to predict. Develop a minimally invasive test to predict outcome after surgical EHPSS attenuation and establish risk factors for postattenuation seizures (PAS). Eighty-five client-owned dogs referred for surgical attenuation of a single EHPSS. mRNA expression of 8 genes was measured in preoperatively collected venous blood samples. Outcome was determined at a median of 92 days (range, 26-208) postoperatively by evaluating clinical performance, blood test results and abdominal ultrasonography. Multivariable logistic regression was used to construct models predicting clinical and complete recovery. The associations between putative predictors and PAS were studied using univariable analyses. Five of 85 dogs developed PAS. Risk factors were age, white blood cell (WBC) count and expression of hepatocyte growth factor activator and LysM and putative peptidoglycan-binding domain-containing protein 2. Clinical recovery was observed in 72 of 85 dogs and complete recovery in 51 of 80 dogs (median follow-up, 92 days). The model predicting clinical recovery included albumin, WBC count, and methionine adenosyltransferase 2 alpha (MAT2α) expression, whereas the model predicting complete recovery included albumin, and connective tissue growth factor precursor and MAT2α expression. The areas under the receiver operating characteristic curves were 0.886 (95% confidence interval [CI]: 0.783, 0.990) and 0.794 (95% CI: 0.686, 0.902), respectively. Two models were constructed for predicting outcome after EHPSS attenuation using venous blood samples. The model predicting clinical recovery showed the best diagnostic properties. Clinical application requires further validation.

Significance of a multidisciplinary approach to congenital extrahepatic portosystemic shunt: A changing paradigm for the treatment.

To review the current institutional practice to treat patients with congenital extrahepatic portosystemic shunt (CEPS) and to determine the optimal strategy. We retrospectively reviewed the records of 55 patients diagnosed with CEPS at our center between December 2008 and March 2022. Among these 55 patients, 44 (80.0%) received treatment for CEPS at a median age of 4.7years. The most common indication for treatment was cardiopulmonary complications (45.5%). Therapeutic intervention included shunt closure by endovascular techniques (50.0%) or surgery (40.9%), and liver transplantation (9.1%). A total of 11 were classified as short shunt types, and surgical ligation was performed in all to preserve the major vascular system and prevent complications (p<0.001). Children who received a surgical ligation were more likely to develop complications after shunt closure (p=0.02). Among seven patients with portopulmonary hypertension (POPH), one patient, who received a shunt ligation at <1year-of-age, was only able to completely discontinue medication. Most other CEPS-related complications were completely resolved. Post-treatment complications, including thrombosis and symptoms of portal hypertension, were seen in 16 patients. After shunt closure, one patient was scheduled to undergo liver transplantation for progressive POPH and large residual hepatocellular adenoma. During follow-up, one patient without any treatment for CEPS developed POPH 16years from the diagnosis. Earlier therapeutic interventions should be strongly considered for patients with POPH related to CEPS. However, in view of the invasiveness and treatment complications, special attention should be paid to the management of patients with short shunt types.

Congenital porto-pulmonary shunt in a dog

Congenital extra-hepatic porto-systemic shunts (CEPS) are a non-rare vascular anomaly observed in dogs, most commonly in small and toy pure breeds. Computed tomographic angiography (CTA) examination is considered the gold standard imaging modality for the diagnosis of anomalous vascular connections. An anomalous congenital porto-pulmonary shunt was incidentally diagnosed in a 5-year-old French Bulldog. The anomalous vessel originated from the ventral aspect of the portal vein and went cranially towards the esophageal hiatus, entering the lobar vein of the caudal left pulmonary lobe. The dog did not show any significant clinical or computed tomography angiography-perceived hepatic abnormalities and no signs of portal hypertension were evidenced. No case of porto-pulmonary shunt in veterinary medicine have ever been reported, while in humans it was rarely described secondarily to portal hypertension, severe hepatopathies or complex cardiac malformations. CTA must be considered the best imaging modality for the diagnosis also of unusual CEPS and in the author's opinion the congenital porto-pulmonary shunt described in the patient was of little or no clinical relevance.