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.

Efficacy and safety of precision-guided transjugular extrahepatic portosystemic shunt (TEPS) in the management of cavernous transformation of the portal vein with portal hypertension: a case series.

Performing a Transjugular intrahepatic portal system shunt (TIPS) in patients with portal vein cavernous transformation (CTPV) poses significant challenges. As an alternative, transjugular extrahepatic portal vein shunt (TEPS) may offer a potential solution for these patients. Nonetheless, the effectiveness and safety of TEPS remain uncertain. This case series study aimed to evaluate the efficacy and safety of TEPS in treating patients with CTPV portal hypertension complications. The study encompassed a cohort of 22 patients diagnosed with CTPV who underwent TEPS procedures. Of these, 13 patients manifested recurrent hemorrhagic episodes subsequent to conventional therapies, 8 patients grappled with recurrent or refractory ascites, and 1 patient experienced acute bleeding but refused endoscopic treatment. Comprehensive postoperative monitoring was conducted for all patients to rigorously evaluate both the technical and clinical efficacy of the intervention, as well as long-term outcomes. The overall procedural success rate among the 22 patients was 95.5% (21/22).During the TEPS procedure, nine patients were guided by percutaneous splenic access, three patients were guided by percutaneous hepatic access, five patients were guided by transmesenteric vein access from the abdomen, and two patients were guided by catheter marking from the hepatic artery. Additionally, guidance for three patients was facilitated by pre-existing TIPS stents. The postoperative portal pressure gradient following TEPS demonstrated a statistically significant decrease compared to preoperative values (24.95 ± 3.19mmHg vs. 11.48 ± 1.74mmHg, p < 0.01).Although three patients encountered perioperative complications, their conditions ameliorated following symptomatic treatment, and no procedure-related fatalities occurred. During a median follow-up period of 14months, spanning a range of 5 to 39months, we observed four fatalities. Specifically, one death was attributed to hepatocellular carcinoma, while the remaining three were ascribed to chronic liver failure. During the follow-up period, no instances of shunt dysfunction were observed. Precision-guided TEPS appears to be a safe and efficacious intervention for the management of CTPV.

Diagnosis and classification of portosystemic shunts: a machine learning retrospective case-control study.

Diagnosis of portosystemic shunts (PSS) in dogs often requires multiple diagnostic tests, and available clinicopathologic tests have limitations in sensitivity and specificity. The objective of this study was to train and validate a machine learning model (MLM) that can accurately predict the presence of a PSS utilizing routinely collected demographic data and clinicopathologic features. Dogs diagnosed with PSS or control dogs tested for PSS but had the condition ruled out (non-PSS) were identified. Dogs were included if a complete blood count and serum chemistry panel were available from PSS diagnostic testing. Dogs with a PSS were subcategorized as having a single intrahepatic PSS, a single extrahepatic PSS, or multiple extrahepatic PSS. An extreme gradient boosting (XGboost) MLM was trained with data from 70% of the cases, and MLM performance was determined on the test set, comprising the remaining 30% of the case data. Two MLMs were created. The first was designed to predict the presence of any PSS (PSS MLM), and the second to predict the PSS subcategory (PSS SubCat MLM). The trained PSS MLM had a sensitivity of 94.3% (95% CI 90.1-96.8%) and specificity of 90.5% (95% CI 85.32-94.0%) for dogs in the test set. The area under the receiver operator characteristic curve (AUC) was 0.976 (95% CI; 0.964-0.989). The mean corpuscular hemoglobin, lymphocyte count, and serum globulin concentration were most important in prediction classification. The PSS SubCat MLM had an accuracy of 85.7% in determining the subtype of PSS of dogs in the test set, with variable sensitivity and specificity depending on PSS subtype. These MLMs have a high accuracy for diagnosing PSS; however, the prediction of PSS subclassification is less accurate. The MLMs can be used as a screening tool to increase or decrease the index of suspicion for PSS before confirmatory diagnostics such as advanced imaging are pursued.

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.

Computed tomographic evaluation of portal vein indices in cats with the extrahepatic portosystemic shunts

Computed tomographic evaluation of portal vein indices in cats with the extrahepatic portosystemic shunts

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.

Neurometabolism and brain morphometry in an adolescent female with an extra-hepatic congenital portosystemic shunt.

Chronic hepatic encephalopathy (CHE) has been reported both in patients with congenital porto-systemic shunts (CPSS) and chronic liver disease. CHE is difficult to recognize in children as there is no clear definition and its manifestations are highly variable. CHE is associated with variations in brain volumes and metabolites that have already been demonstrated using 1.5-3T MRI systems. However, the in-depth study of brain metabolism requires the high spectral resolution of high magnetic fields. We analyzed the neurometabolic profile, brain volumes and T1 relaxation times of a child with a CPSS using high field proton magnetic resonance spectroscopy (1H MRS, 7T) combined with MRI and compared it to an age-matched control group. We also evaluated the impact of shunt closure on neurocognitive symptoms using adapted neuropsychological tests. 7T MRS revealed a significant increase in glutamine compared to controls, a decrease in brain osmolytes, and a slight elevation in NAA concentrations. 7T MRI scans showed morphological abnormalities but no changes in the signal intensity of the globus pallidus. Neurocognitive testing revealed attention deficit disorder, language difficulties, and mild intellectual disability. Most of these areas improved after shunt closure. In this paediatric case of type B HE with normal fasting ammonia, neurometabolic profile was compatible with what has been previously shown in chronic liver disease, while also demonstrating an isolated glutamine peak. In addition, neurocognitive function partially improved after shunt closure, arguing strongly for shunt closure in both presymptomatic and symptomatic patients.

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.

Percutaneous transvenous coil embolization (PTCE) for treatment of single extrahepatic portosystemic shunt in dogs

BackgroundThere is limited information regarding percutaneous transvenous coil embolization (PTCE) for single extrahepatic portosystemic shunt (PSS). This study aimed to describe the procedure and outcome of PTCE in dogs with a single extrahepatic PSS. Forty-two privately owned dogs were included in this study. All dogs were diagnosed with extrahepatic PSS by computed tomography (CT). Preoperative CT images were used to evaluate the diameter of the PSS for coil placement. A multipurpose balloon catheter was percutaneously inserted into the PSS via the jugular vein, and transvenous retrograde portography (TRP) and measurement of blood pressure in the PSS (pPSS) were performed during balloon inflation; one or more embolization coils were implanted via the catheter.ResultsIn most cases, preoperative median fasting and postprandial serum total bile acid (TBA) concentrations were high (fasting, 86.5 μmol/L [ 3.7–250.0 μmol/L]; postprandial, 165.5 μmol/L [ 1.5–565.0 μmol/L]). CT revealed that 30 dogs had left gastrophrenic shunt; eight had left gastroazygos shunt; and one each had left gastrocaval, splenocaval, splenophrenic, and left colocaval shunt. TRP revealed that intrahepatic portal vascularity was clearly detectable in all dogs. The median values of pPSS before and during the balloon occlusion were 4.8 mmHg [2.0–13.0 mmHg] and 8.6 mmHg [5.0–18.0 mmHg], respectively. The median number and diameter of coils used were 2 coils [1 – 5 coils] and 8.0 mm [4.0 – 12.0 mm], respectively. The median times of irradiation and PTCE were 9 min [4–26 min] and 40 min [23–75 min], respectively. The median fasting and postprandial TBAs significantly decreased to 8.2 μmol/L [0.3–45.1 μmol/L, n = 38, p = 0.0028] and 19.8 μmol/L [0.3–106.7 μmol/L, n = 38, p = 0.0018], respectively, approximately 1 month after PTCE. The clinical success rate of PTCE without requirement for a second surgery was 95.2% (40/42 dogs). During revision surgery, one dog underwent surgical ligation and, in another dog, an ameroid constrictor was placed.ConclusionsPTCE was clinically effective in treating single extrahepatic PSS in dogs. Preoperative CT and TRP prior to PTCE might be clinically valuable for choosing the size of embolization coils, deciding the appropriate location of coil implantation, and estimating the number of coils to be implanted. PTCE is a promising alternative to conventional surgical procedures for single extrahepatic PSS in 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.

Prospective Study Comparing Outcome following Complete Polypropylene Suture Ligation versus Partial Thin Film Band Attenuation of Congenital Portosystemic Shunts in Dogs.

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.

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.

Intramural esophageal location of a portosystemic shunt in a dog with two congenital portosystemic shunts on CT angiography.

A 5-year-old male neutered pug with hematuria was presented to a referral hospital after identification of an extrahepatic portosystemic shunt (EHPSS) during abdominal ultrasonography. Computed tomographic-angiography revealed two anomalous blood vessels (left gastroazygous and left gastrophrenic). The left gastroazygous vessel followed an atypical path within the dorsolateral esophageal wall before entering the azygous vein. The morphology of this highly unusual vessel has not, based on the authors' review of the literature, been previously reported. In combination with a second anomalous vessel, this resulted in a unique presentation of an EHPSS. Computed tomography-angiography was essential for diagnosis and surgical planning in this case.

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.