Application of transmesenteric vein extrahepatic portosystemic shunt in treatment of symptomatic portal hypertension with cavernous transformation of portal vein.

Purpose: To evaluate the feasibility and efficacy of a transmesenteric vein extrahepatic portosystemic shunt (TmEPS) for the treatment of cavernous transformation of the portal vein (CTPV). Materials and methods: The clinical data of 20 patients with CTPV who underwent TmEPS between December 2020 and January 2022 â€‹at Henan Provincial People's Hospital were retrospectively collected. The superior mesenteric vein (SMV) trunk was patent or partially occluded in these patients. An extrahepatic portosystemic shunt between the inferior vena cava and the SMV was established using a stent graft through an infraumbilical median longitudinal mini-laparotomy. The technical success, efficacy, and complication rates were evaluated, and the pre- and postoperative SMV pressures were compared. Patients' clinical outcomes and shunt patency were assessed. Results: TmEPS was successfully performed in 20 patients. The initial puncture success rate of the balloon-assisted puncture technique is 95%. The mean SMV pressure decreased from 29.1 â€‹± â€‹2.9 â€‹mmHg to 15.6 â€‹± â€‹3.3 â€‹mmHg (p â€‹< â€‹0.001). All symptoms of portal hypertension resolved. No fatal procedural complications occurred. During the follow-up period, hepatic encephalopathy occurred in two patients. The remaining patients remained asymptomatic. All shunts were patent. Conclusions: TmEPS is a feasible, safe, and effective treatment option for patients with CTPV.

Congenital extrahepatic Portosystemic shunt with hypoplasia of the intrahepatic inferior vena cava: A rare case report.

Congenital portosystemic shunt (CPS) is a rare vascular anomaly resulting in diversion of splanchnic or portal blood into the systemic circulation. Other vascular malformations associated with this entity are uncommon. A 4-year-old female child with a diagnosis of acute viral hepatitis had an incidental finding of extrahepatic CPS on a Doppler abdominal ultrasound. Contrast-enhanced computed tomography showed a dilated portal vein having H-type side-to-side communication with a hypoplastic intrahepatic portion of the inferior vena cava and a prominent dilated azygos vein. There was retroaortic left renal vein drained into the IVC which was visualized in its entirety. Echocardiography findings were normal and the patient was discharged after symptomatic treatment that achieved improvement. With the expansion of abdominal imaging, incidental cases of CPS are increasingly being diagnosed in children. Although vascular malformations associated with CPS are rare, early diagnosis of cases helps avoid complications during shunt closure.

Percutaneous sequential closure of an Abernethy malformation: A case report.

Abernethy malformation (congenital extrahepatic portosystemic shunt [CEPS]) is rare and is characterized by an aberrant connection between the portal and systemic veins, bypassing the liver. It can have varying presentations and can lead to severe complications if left untreated. It is usually diagnosed incidentally on abdominal imaging. Occlusion venography and measurement of portal pressures (pre- and postocclusion) is an important step in management. Complete occlusion of the malformation in cases where the portal veins in the liver are very small and the gradient is more than 10 mm Hg, can potentially lead to acute portal hypertensive complications, such as porto-mesenteric thrombosis. We report a case of Abernethy malformation diagnosed on an abdominal computed tomography scan that presented with neurological symptoms and was successfully managed by interventional radiology via endovascular closure through placement and sequential occlusion of 2 metal stents.

Diameter of the shunt vessel in dogs with incidental extrahepatic portosystemic shunts.

Clinical signs of extrahepatic portosystemic shunts (EHPSS) depend on the amount of blood shunted. In this study, dogs with EHPSS without noticeable clinical signs including 34 left gastro-phrenic, 3 left gastro-azygos and 2 left spleno-gonadal shunts were evaluated. In dogs with EHPSS without noticeable clinical signs, the median maximum diameter of the shunt vessel was significantly smaller compared to PV (p < 0.005). Whenever the diameter of the EHPSS is small in relation to the diameter of the PV, it seems likely that no obvious clinical signs of EHPSS are observed by the owners.

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.

BACKGROUND: In dogs with a congenital extrahepatic portosystemic shunt (EHPSS), outcome after surgical attenuation is difficult to predict. OBJECTIVES: Develop a minimally invasive test to predict outcome after surgical EHPSS attenuation and establish risk factors for postattenuation seizures (PAS). ANIMALS: Eighty-five client-owned dogs referred for surgical attenuation of a single EHPSS. METHODS: 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. RESULTS: 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. CONCLUSIONS AND CLINICAL IMPORTANCE: 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.

Laparoscopic attenuation of a congenital extrahepatic portosystemic shunt in a dog-a thin-film banding for splenophrenic shunt: A case report.

A 6-year-old castrated male Shih-Tzu dog weighing 6. 5 kg presented with chief complaints of pollakiuria and urine dribbling. He had a history of urolithiasis for 3 years, which was confirmed by the presence of ammonium urate in the urinary stone analysis, performed 2 years prior to the presentation. Blood examination showed high values of fasting ammonia, post-prandial bile acid, and low blood urea nitrogen. Microhepatica and urolithiasis were identified on plain radiography and ultrasonography. A computed tomography angiography demonstrated a shunting vessel, diameter up to 9.6 mm, originated from the splenic vein, and linked with the phrenic vein. A surgical attenuation with a thin-film banding was performed under laparoscopic visualization. Left triangular ligament was incised, and one stay suture was placed to the stomach to expose the vessel. The shunting vessel was dissected before it entered the diaphragm, and a thin-film band was applied around the vessel. The patient recovered uneventfully without post-attenuation neurologic signs. Portal vein diameter increased with time, and complete closure of the shunting vessel was identified on computed tomography angiography performed at 14 months after attenuation. The patient was doing well for 31 months after surgery without protein restriction. This is a report of laparoscopic attenuation for splenophrenic type of canine congenital extrahepatic portosystemic shunt with a favorable outcome using thin-film banding.

A rare presentation of type II Abernethy malformation and nephrotic syndrome: Case report and review.

Type II Abernethy malformation is an extremely reported congenital extrahepatic portosystemic shunt in complication with nephrotic syndrome. We present the case of an 8-year-old boy who presented with symptoms of type II Abernethy malformation and nephrotic syndrome. This diagnosis of this type II Abernethy malformation was based on physical examination, blood tests, urinalysis, nephrotic and hepatic function tests, routine clinical lipid measurements, abdominal ultrasonography, and computed tomographic angiography. A kidney biopsy revealed the pathological features of nephrotic syndrome. This is the second reported patient diagnosed with type II Abernethy malformation and nephrotic syndrome. Captopril treatment was effective in improving the symptoms of this case. A patient with type II Abernethy malformation related to immune complex-mediated glomerular injury was effectively improved with medication. Type II Abernethy malformation is a causative factor of immune complex-mediated glomerular injury in nephrotic syndrome. Captopril treatment significantly improved the symptoms in this case.

A rare Abernethy Ib malformation was initially misdiagnosed as chronic portal vein thrombosis in a 27-year-old female.

Abernethy malformation or congenital portosystemic shunt is a rare congenital vascular malformation and anomaly of the splanchnic venous system defined by diverting portal blood away from the liver. It is commonly associated with multiple congenital anomalies. Imaging modalities such as computed tomography or magnetic resonance have a crucial role in prompting diagnosis and determining the prognosis based on the type of malformation and associated anomalies. Misdiagnosis could be harmful and may lead to inappropriate treatment. We present a case of Abernethy malformation with a complete end-to-side shunt of portal venous flow into the systemic venous flow and complete bypass of the liver, which was initially misdiagnosed with portal venous thrombosis.

Intercostal thoracotomy for surgical attenuation of portoazygos extrahepatic portosystemic shunts in three dogs: surgical technique and short-term outcomes.

CASE HISTORIES: The medical records of three dogs, presenting to the surgery department at a tertiary private referral hospital (Cork, Republic of Ireland), were retrospectively reviewed. The patients presented for investigation of a 3-day history of progressive lethargy, decreased appetite and hindlimb weakness (Case 1); brachycephalic obstructive airway syndrome (BOAS) and the development of abnormal licking behaviours (Case 2); and a 2-month history of increased thirst with elevated alanine aminotransferase (ALT) activity in serum (Case 3). CLINICAL FINDINGS: Case 1 presented with tachycardia, hindlimb paraparesis and neck pain, while Case 2 presented with facial conformation abnormalities consistent with BOAS. General physical examination and neurological assessment were within normal limits for Case 3. Baseline serum biochemistry measurements, in all three patients, indicated elevated activities of ALT and alkaline phosphatase, as well as elevated concentrations of resting bile acids (BA), suggestive of an extrahepatic portosystemic shunt. Abdominal computed tomography (CT) angiography was performed for each dog, which revealed the presence of a portoazygos shunt (PAS) with final insertion into the caudal azygos vein within the thorax. TREATMENT AND OUTCOME: Abdominal CT angiography images were used for surgical planning and a right-sided intercostal thoracotomy was performed. The location of the thoracotomy was patient-specific and located cranial to the diaphragm, either at the point of PAS insertion into the azygos vein or one rib-space caudal to the insertion, as determined by pre-operative CT images. The intercostal thoracotomy provided good visualisation of the PAS and an appropriate surgical field for placement of a thin film band around the PAS. No surgical complications or post-attenuation seizures were observed. All patients appeared comfortable on oral analgesia and were discharged from the hospital by 48 hours after surgery. All patients demonstrated a clinical improvement when reassessed 6-8 weeks after surgery, and Cases 2 and 3 demonstrated a reduction of resting BA concentrations to within normal limits. The third patient (Case 1) had a considerable reduction in the concentration of resting BA from >140 µmol/L to 20 µmol/L (reference range 0-10 µmol/L) 6-8 weeks after surgery. CLINICAL RELEVANCE: A right-sided intercostal thoracotomy can be considered for surgical management of PAS in dogs. While both intraoperative and short-term results appear promising, further prospective studies are required before this approach can be recommended as the preferred approach for PAS attenuation.Abbreviations: ALP: Alkaline phosphatase; ALT: Alanine aminotransferase; BA: Bile acids; BOAS: Brachycephalic obstructive airway syndrome; CBC: Complete blood count; CT: Computed tomography; EHPSS: Extrahepatic portosystemic shunt; GLDH: Glutamate dehydrogenase; PAS: Portoazygos shunt.

Surgical shunt ligation for a congenital extrahepatic portosystemic shunt with pulmonary hypertension: A case report.

INTRODUCTION AND IMPORTANCE: Congenital extrahepatic portosystemic shunt (CEPS) presents with various symptoms due to abnormal communication between the portal venous system and inferior vena cava. And Klippel-Trenaunay-Weber syndrome is another rare congenital disorder characterized by vascular malformations. CASE PRESENTATION: A 16-year-old male with Klippel-Trenaunay-Weber syndrome was referred to our hospital for surgical treatment of pulmonary hypertension due to CEPS since childhood. Dyspnea had developed about two years before presentation at our hospital and gradually worsened. Right heart catheterization had revealed pulmonary hypertension and a high cardiac output state associated with a portosystemic shunt. Although pulmonary vasodilators improved the dyspnea, pulmonary hypertension remained. The patient was then referred to our hospital for surgical shunt occlusion. The results of the preoperative and intraoperative shunt occlusion tests were within acceptable limits. Therefore, primary shunt ligation was performed. There were no postoperative complications. Continuous intravenous vasodilator was tapered off four months after discharge. No additional or increased doses of medications were required for four years after surgery. CLINICAL DISCUSSION: Surgical shunt ligation for CEPS is effective but can cause acute portal hypertension. Primary shunt ligation could be performed without complications according to the criteria for safe shunt occlusion. CONCLUSION: Surgical portosystemic shunt ligation could be performed safely and ameliorated pulmonary hypertension. Patients with CEPS, especially young patients, should be referred to a tertiary center while asymptomatic, and early therapeutic intervention is necessary.

Updates in Hepatobiliary Surgery: New Data on Portosystemic Shunts and Cholecystectomy in Dogs and Cats.

Advances regarding surgical treatment of hepatobiliary diseases over the past 5 years have resulted in improved outcomes and decreased mortality in veterinary patients. Options for minimally invasive treatment of intrahepatic portosystemic shunts and gallbladder disease have led to decreased morbidity with similar outcomes to open procedures. For extrahepatic portosystemic shunts, studies evaluating long-term outcome between surgical attenuation and medical management, as well as between options for surgical attenuation, are being used to direct evidence-based treatment of this congenital anomaly. In addition, evaluation of timing in surgical intervention for gallbladder mucoceles has led to an opportunity for decreased mortality rates after cholecystectomy.

Multistage closure of a congenital extrahepatic portosystemic shunt.

BACKGROUND: Congenital extrahepatic portosystemic shunts (CEPS) are rare shunts connecting the extrahepatic portal system with the inferior vena cava. Shunt dimensions and the risk of portal hypertension determines the closure strategy. Endovascular treatment is indicated for single stage occlusion of longer length shunts, whereas the remaining shunt types are preferentially surgically occluded. Herein we describe the technical details of a novel endovascular treatment for short length CEPS. CASE PRESENTATION: A 15-years-old male with a short length CEPS complicated with multinodular liver disease was submitted to a multistage closure, as indicated by the high portal pressure values during shunt balloon occlusion venography. Initially a transjugular intrahepatic portosystemic shunt (TIPS) was created and the CEPS occluded with an atrial septal defect occluder. In a second procedure the TIPS was embolized with a flow reductor stent and an amplatzer vascular plug II. At a 1 year follow up the liver nodules size reduced, the patient remains asymptomatic, and the shunt adequately closed. CONCLUSION: This paper outlines the potential use of a TIPS and an atrial septal defect occluder combination in complex CEPS, supporting its usage as an alternative to the standard surgical treatment. LEVEL OF EVIDENCE: Level 4, Case report.

Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II.

This report describes an 8-year-old male who presented with clinical manifestations including systemic edema, heavy proteinuria, hypoproteinemia, and persistent hypocomplementemia. Arachnoid cysts and focal nodular hyperplasia were also detected. Imaging examination and renal biopsy were performed, and Abernethy malformation type II with immune complex-mediated membranoproliferative glomerulonephritis was considered the diagnosis. Due to the persistence of embryonic vessels, Abernethy malformation is a rare congenital vascular malformation of the splanchnic venous system, which can be classified as type I (end-to-side shunt) and type II (side-to-side shunt). Abernethy malformation with glomerulonephritis remains extremely rare. In the patient described, glomerulonephritis mediated by immune complex with "full-house" positive immunohistochemistry was confirmed on renal biopsy. In addition, he was treated with glucocorticoids and tacrolimus. Whether surgical treatment is necessary should be determined according to the state of the disease in the later stages. The present case reflects the association between the congenital portosystemic shunt and the renal region and, to the authors' knowledge, may be the first report to describe arachnoid cysts as a symptom of Abernethy malformation.

An Adult Case of Congenital Extrahepatic Portosystemic Shunt Successfully Treated with Balloon-occluded Retrograde Transvenous Obliteration.

A 42-year-old woman visited our hospital due to syncope. Contrast-enhanced CT revealed portosystemic shunt, portal vein hypoplasia, and multiple liver nodules. The histological examination of a liver biopsy specimen exhibited portal vein hypoplasia and revealed that the liver tumor was positive for glutamine synthetase. The patient was therefore diagnosed with congenital extrahepatic portosystemic shunt type II, and with focal nodular hyperplasia (FNH)-like nodules. She had the complication of severe portopulmonary hypertension and underwent complete shunt closure by balloon-occluded retrograde transvenous obliteration (B-RTO). The intrahepatic portal vein was well developed at 1 year after B-RTO, and multiple liver nodules completely regressed. Her pulmonary hypertension also improved.

Congenital portosystemic shunt occlusion with an Amplatzer PFO occlusion device: a case report.

BACKGROUND: Congenital portosystemic shunts are embryological malformations in which portal venous flow is diverted to the systemic circulation. High morbidity and mortality are seen in patients with concurrent hepatic encephalopathy, hepatopulmonary syndrome, and pulmonary hypertension. Endovascular therapy, in the correct patient population, offers a less invasive method of treatment with rapid relief of symptoms. CASE PRESENTATION: In this report, we discuss the treatment of a two-year-old male with abnormal chorea-like movements, altered mental status, anisocoria and hyperammonemia diagnosed with an intrahepatic congenital portosystemic shunt between the inferior vena cava and right portal vein. Given the patient's amenable anatomy and shunt type, embolization was performed with an 18 mm Amplatzer patent foramen ovale occlusion device. CONCLUSIONS: Portosystemic shunts are a rare congenital abnormality without universal treatment guidelines. An Amplatzer PFO occlusion device can provide a novel method of shunt closure given appropriate shunt type, size and anatomy.

Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature.

Background: Abernethy malformation is an extremely rare anomaly of the splanchnic venous system, and only 2 cases that manifested as syncope had been reported previously. Case Presentation: A 24-year-old male had a 15-year history of jaundice and was in long-term use of hepatoprotective drugs. He was admitted for complaint of syncope. He underwent a series of examinations and cardiac ultrasound showed that his pulmonary artery pressure was elevated. Further imaging revealed the absence of intrahepatic portal veins. His blood ammonia was significantly increased. All signs and symptoms pointed to an Abernethy diagnosis. He was finally diagnosed as having Abernethy type II. He was discharged after 17 days of in-hospital treatment with sildenafil (50 mg/day) and ornithine aspartate (20 g/day). Conclusion: We now report this rare case of syncope that is caused by Abernethy malformation. As a typically pediatric disease, it was not identified in this patient until adulthood due to long-term treatment for jaundice and liver cirrhosis. Furthermore, we present a review of portosystemic shunts previously reported in the literature.

Hepatic encephalopathy due to extrahepatic portosystemic shunt.

Clinicians should recognize extrahepatic portosystemic shunt as a cause of refractory or intermittent hepatic encephalopathy. Treatment strategies should be individualized according to patients' anatomic and hemodynamic status.

Case Report: Hepatic Adenoma in a Child With a Congenital Extrahepatic Portosystemic Shunt.

Congenital extrahepatic portosystemic shunts (CEPS), previously also described as Abernethy malformations, are rare malformations in which the extrahepatic portal system directly communicates with the vena cava inferior, thereby bypassing the liver. A hypoplastic portal vein (PV) exists in most cases. CEPS have been associated with the development of liver nodules, ranging from mostly focal nodular hyperplasia (FNH) to hepatic adenoma (HA) and even hepatocellular carcinoma (HCC). Tumor development in CEPS may be due to changes in perfusion pressures, oxygen supply or endocrine imbalances. It is important to rule out CEPS in children with liver tumors, because resection could impede future shunt occlusion procedures, and benign masses may regress after shunt occlusion. Here, we review the case of a 9-years-old male with CEPS and hepatic nuclear Factor 1-alpha (HNF-1-alpha) inactivated HA to raise awareness of this condition and review histopathological changes in the liver of CEPS.

A basic understanding of congenital extrahepatic portosystemic shunt: incidence, mechanism, complications, diagnosis, and treatment.

Extrahepatic portosystemic shunt belongs to a family of rare vascular abnormalities. The clinical importance and manifestations of this vascular abnormality range from asymptomatic cases to liver or metabolic dysfunctions of various degrees. Congenital extrahepatic portosystemic shunt, also termed as Abernethy malformation, is a very rare congenital vascular malformation in which splenomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial extrahepatic shunt. So far, limited cases of congenital extrahepatic portosystemic shunt have been reported. In this review, incidence, mechanisms, complications, diagnoses and treatments of congenital extrahepatic portosystemic shunt are described.

Living-donor liver transplantation for Abernethy malformation - case report and review of literature.

Abernethy malformation was named for the rare congenital absence of the portal vein (CAPV), also known as congenital extrahepatic portal-systemic shunts (CEPS). This could be classified as complete (type 1) or incomplete shunt (type 2) according to Morgan-Superina classification. Its presentation may show under variable signs and symptoms such as hepatopulmonary syndrome, hyper-ammonia, hepatic masses and liver failure…. This usually combined with other congenital anomalies (cardiac anomaly, trisomy…). This report presented a 10 year-old boy with growth retardation and mild mental recognition, intermittent hyperammonia, elevated liver enzymes, huge inoperable mass in the right liver. MS CT and MRI findings: hyperplasia of liver parenchyma with superior mesenteric vein confluenced with splenic vein before draining directly into the inferior vena cava (Abernethy anomaly type 1b). Living donor liver transplantation underwent using right lobe from his mother. Anatomopathology findings of the native liver showed chronic hepatitis with cirrhosis 4/6 Knodel-Ishak. Postoperatively, the patient still attained optimal liver function and has returned to normal life at 12-month follow-up. Liver transplantation was a reasonable indication for CAPV type 1. Living donor liver transplantation was effective and practical in the scarcity of donor organ.