Extrahepatic Portosystemic Shunt Research Articles

A large spontaneous extrahepatic portosystemic shunt in a cirrhotic patient: from main portal vein to right renal vein

In this paper, we illustrated a very rare type of spontaneous portosystemic shunt originating from main portal vein to right renal vein in a cirrhotic patient. The diagnosis of such a large spontaneous portosystemic shunt was established according to the axial contrast-enhanced computed tomography scans. The cause and outcome were also discussed.

Surgical strategy according to the anatomical types of congenital portosystemic shunts in children

BackgroundCongenital portosystemic shunts (CPSS) with intrahepatic portal vein (IHPV) hypoplasia or absence cause encephalopathy or pulmonary hypertension (PH). Acute shunt closure may result in postoperative portal hypertension. The aim of this study was to propose a surgical strategy according to the anatomical types of CPSS and IHPV. MethodsTwenty-three CPSS patients were diagnosed from1990 to 2015. All patients were evaluated by computed tomography, angiography, and PV pressure monitoring under a shunt occlusion test. CPSS were categorized into 5 types according to the anatomical shunt location. ResultsThe median age at diagnosis was 34months. Three of 23 total patients, who had an extrahepatic portosystemic shunt with a hypoplastic IHPV, died before treatment initiation because of severe PH. Fourteen cases received surgical or interventional treatment at the median age of 5years. A total of 6 cases received surgical therapy, including liver transplants for 2 absent IHPV cases. The remaining 8 cases received interventional coiling. All shunt ligations were successfully accomplished in 1-stage ligation without any complications. After the treatment, the hypoplastic IHPV gradually enlarged with an efficient portal inflow. ConclusionA precise pretreatment anatomical evaluation of CPSS and IHPV types is mandatory for the selection of surgical treatment. Level of evidenceDiagnostic study – level II and treatment study – level III.

Imaging and Clinical Outcomes in 20 Dogs Treated with Thin Film Banding for Extrahepatic Portosystemic Shunts.

To prospectively evaluate the efficacy of thin film band attenuation of congenital extrahepatic portosystemic shunts (CEPSS) in dogs using clinical, biochemical, and imaging-related outcome measures. Prospective case series. Dogs with CEPSS (n=20). Client-owned dogs with CEPSS were enrolled and thin film banding of the shunting vessel was performed. Before and at least 6 months after surgery, serum bile acids and computed tomography (CT) angiography were performed and owners completed a health questionnaire regarding the dog's clinical signs. Postoperative CT images were assessed for the effectiveness of band closure, change in portal vein/aorta ratio, change in liver volume/weight ratio, and whether the band was placed in the appropriate location. Preoperative and postoperative health questionnaire data and serum bile acids were compared. The band resulted in complete closure of the vessel around which it was placed in 13 dogs. In the remaining 7 dogs, the vessel lumen was narrowed but not completely closed. In 8 dogs the band location was suboptimal, allowing systemic drainage of visceral blood or secondary shunting branches to persist. Liver volume/body weight ratios and portal vein/aorta diameter ratios increased in most dogs. Serum bile acids decreased in all but 1 dog and owners reported improved health in 19 dogs. Thin film banding resulted in complete occlusion of many, but not all vessels around which it was placed. Even in dogs with inappropriate band location or with incomplete closure, clinical improvement can be expected based on our results.

The Abernethy malformation-myriad imaging manifestations of a single entity.

Abernethy malformation, also known as congenital extrahepatic portosystemic shunts (CEPS) is a rare clinical entity and manifests with different clinical symptoms. CEPS are abnormalities of vascular development where there is shunting of portal blood into the systemic venous system. Multidetector computed tomography (MDCT) is a fast and effective modality for evaluation of CEPS. CT displays all the information desired by the surgeon as well as the clinician including the anatomy of the splenic and superior mesenteric veins, size and site of the shunt, presence or absence of the portal vein radicles, and helps to plan the therapy and even the follow-up of these patients. Contrast-enhanced magnetic resonance imaging (MRI) has also emerged as a promising tool for the evaluation of liver lesions associated with the malformation. The Radiologist should be aware of the various imaging appearances of this entity including its complications. In this article, we describe the imaging appearances of CEPS, their complications, and their imaging appearances on CT and MRI. We have also described various associated anomalies.

Assessment of the attenuation of an intra-abdominal vein by use of a silicone-polyacrylic acid gradual venous occlusion device in dogs and cats.

OBJECTIVE To evaluate the closure rate and completeness of closure for a silicone-polyacrylic acid gradual venous occlusion device placed around an intra-abdominal vein to simulate gradual occlusion of an extrahepatic portosystemic shunt. ANIMALS 3 purpose-bred cats and 2 purpose-bred dogs. PROCEDURES The device was surgically placed around an external (cats) or internal (dogs) iliac vein. Computed tomographic angiography was performed at the time of surgery and 2, 4, and 6 weeks after surgery. Ultrasonographic examinations of blood flow through the vein within the device were performed at the time of surgery and at weekly intervals thereafter. Dogs were euthanized 6 weeks after surgery, and the external iliac veins were harvested for histologic examination. RESULTS The prototype gradual venous occlusion device was successfully placed in all animals, and all animals recovered without complications following the placement procedure. The vessel was completely occluded in 2 cats by 6 weeks after surgery, as determined on the basis of results of CT and ultrasonography; there was incomplete occlusion with a luminal diameter of 1.5 mm in the other cat by 6 weeks after surgery. The vessel was completely occluded in both dogs by 6 weeks after surgery. Histologic examination of the external iliac veins obtained from the dogs revealed minimal inflammation of the vessel wall and no thrombus formation. CONCLUSIONS AND CLINICAL RELEVANCE The prototype device induced gradual attenuation of an intra-abdominal vessel over a 6-week period. This device may provide another option for gradual occlusion of extrahepatic portosystemic shunts.

Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation Type Ib) with Associated Hepatocellular Carcinoma: Case report and Literature Review

Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein (CAPV) is the result of malformation of the splanchnic venous system. Congenital portosystemic shunts are divided into extra-and intrahepatic shunts. Two shunts have been defined: type I is characterized by the complete diversion of portal blood into the vena cava with an associated congenital absence of the portal vein. Type II is defined by an intact but diverted portal vein through a side-to-side, extrahepatic connection to the vena cava. The clinical manifestations of Abernethy malformation are diverse with a typical presentation consisting of hypoxia and hepto-pulmonary syndrome. Histologically, focal nodular hyperplasia, nodular regenerative hyperplasia, liver adenoma, hepatoblastoma, and hepatocellular carcinoma have all been reported. Herein we report a case of Abernethy malformation, type Ib, in a 12 month old male who was found to have a small hepatocellular carcinoma at the time of explant. The immunohistochemical characteristics in relation to the genetic aspects are discussed. To our knowledge, this is the first reported case of hepatocellular carcinoma developing in a patient who is under the age of 5 years old with Abernethy Malformation.

Congenital Extrahepatic Portosystemic Shunt with Multiple Visceral Aneurysms.

Congenital Extrahepatic Portosystemic Shunt with Multiple Visceral Aneurysms.

Surgical management of an extrahepatic portosystemic shunt in a foal: A multidisciplinary problem

SummaryA 5‐week‐old American Miniature filly was referred due to intermittent neurological signs of 4 weeks' duration. Clinicopathological findings showed increased ammonia and bile acids in venous blood samples. Abdominal ultrasonography detailed a portosystemic shunt (PSS). The foal underwent laparotomy and operative mesenteric portovenography, which further defined the PSS as extrahepatic portocaval in configuration. The PSS was occluded and intraoperative portovenography confirmed successful shunt occlusion. The foal made a good recovery from surgery and had a successful showing career until she succumbed to colic just over 3 years after surgery.

EP09.16: Prenatal diagnosis of extrahepatic portosystemic shunt

EP09.16: Prenatal diagnosis of extrahepatic portosystemic shunt

Hepatocellular nodules resulting from congenital extrahepatic portosystemic shunts can differentiate into potentially malignant hepatocellular adenomas.

Hepatocellular nodules caused by congenital extrahepatic portosystemic shunts (CEPS) occur as a result of abnormal portal blood flow, and are mostly cases of benign focal nodular hyperplasia (FNH). However, hepatocellular adenomas (HCA) and hepatocellular carcinomas have been documented in the CEPS patients. HCA can now be immunohistochemically diagnosed; therefore, the concept of hepatocellular nodules resulting from CEPS should be revisited. In this study, we performed a retrospective immunohistochemical investigation of hepatocellular nodules from livers isolated from the CEPS patients undergoing living donor liver transplantation (LDLT). Hepatocellular nodules from livers of five patients with CEPS who underwent LDLT between June 2004 and October 2012 at our institution were immunohistochemically investigated. HCA were classified into four subtypes (HNF1α-inactivated HCA (H-HCA); inflammatory HCA; β-catenin-activated HCA (b-HCA); unclassified HCA). Sixteen hepatocellular nodules were collected from livers of five patients with CEPS who underwent LDLT. Ten hepatocellular nodules were categorized as FNH (62.5%), five were categorized as b-HCA (31.3%), and one was categorized as H-HCA (6.2%). Some of the hepatocellular nodules resulting from CEPS were indicative of HCAs, especially the b-HCA subtype which has the potential for malignant transformation. Surgical or interventional treatments might have to be performed when hepatocellular nodules appear in the CEPS patients.

Congenital portosystemic shunts with and without gastrointestinal bleeding - case series.

The clinical presentation of congenital portosystemic shunt is variable and gastrointestinal bleeding is an uncommon presentation. To describe the imaging features of congenital portosystemic shunt as it presented in 11 children with (n = 6) and without gastrointestinal bleeding (n = 5). We performed a retrospective study on a clinical and imaging dataset of 11 children diagnosed with congenital portosystemic shunt. A total of 11 children with congenital portosystemic shunt were included in this study, 7 with extrahepatic portosystemic shunts and 4 with intrahepatic portosystemic shunts. Six patients with gastrointestinal bleeding had an extrahepatic portosystemic shunt, and the imaging results showed that the shunts originated from the splenomesenteric junction (n = 5) or splenic vein (n = 1) and connected to the internal iliac vein. Among the five cases of congenital portosystemic shunt without gastrointestinal bleeding, one case was an extrahepatic portosystemic shunt and the other four were intrahepatic portosystemic shunts. Most congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was uncommon, but the concomitant rate of gastrointestinal bleeding with this type of shunt was high.

Risk factors for urolithiasis in dogs with congenital extrahepatic portosystemic shunts: 95 cases (1999-2013).

To identify risk factors for urolithiasis in dogs with congenital extrahepatic portosystemic shunts (EHPSSs) and to determine whether portoazygos shunts were associated with increased risk of urolithiasis at the initial evaluation for EHPSS. Retrospective case series. Dogs (n = 95) with EHPSSs confirmed via CT angiography or surgery. Medical records from 1999 to 2013 were reviewed. Variables of interest included signalment, previous medical management, and results of urinalysis, urolith analyses, and diagnostic imaging. Univariable and multivariable logistic regression analyses for assessment of risk factors for urolithiasis at the time of initial EHPSS evaluation were performed. The dogs' median age was 0.9 years (range, 0.2 to 12.6 years). Among the 95 dogs, 27 (28.4%) and 68 (71.6%) had portoazygos and portocaval shunts, respectively. Urinalysis was performed for 79 (83.2%) dogs, 29 (36.7%) of which had crystalluria (mainly ammonium urate and struvite crystals). Uroliths were present in 34 of 95 (35.8%) dogs; 16 of 17 uroliths analyzed were composed of ammonium urate. Portoazygos shunts were not associated with significantly increased odds of urolithiasis at the time of the initial evaluation for EHPSS. However, the odds of urolithiasis was significantly increased for male dogs, older dogs, and dogs that received previous medical treatment. In dogs with EHPSS, shunt morphology was not associated with increased odds of urolithiasis at the initial evaluation. Male dogs, older dogs, and dogs having received medical management for EHPSS prior to initial evaluation should be considered at increased risk for development of urolithiasis.

Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure.

A 54-year-old woman presented with a six-month history of episodic confusion and progressive ataxia. A comprehensive metabolic panel was notable for elevated values of alkaline phosphatase (161 U/L), total bilirubin (1.5 mg/dL), and serum ammonia of 300 umol/L (normal range 9–47). Hepatitis panel, relevant serological tests, tumor markers (CA-19-9, CEA), and urea cycle enzyme studies were unrevealing. Lactulose and rifaximin therapy failed to normalize serum ammonia levels. Imaging revealed a structural vascular abnormality communicating between an enlarged inferior mesenteric vein and the left renal vein, measuring 16 mm in greatest diameter. The diagnosis of congenital extrahepatic portosystemic shunt was made and endovascular shunt closure was performed using a 22 mm Amplatzer II vascular plug. Within a day, serum ammonia levels normalized. Lactulose and rifaximin were discontinued, and confusion and ataxia resolved.

Congenital portosystemic shunt: our experience.

Introduction. Congenital portosystemic venous malformations are rare abnormalities in which the portal blood drains into a systemic vein and which are characterized by extreme clinical variability. Case Presentations. The authors present two case reports of a congenital extrahepatic portosystemic shunt (Type II). In the first patient, apparently nonspecific symptoms, such as headache and fatigue, proved to be secondary to hypoglycemic episodes related to the presence of a portosystemic shunt, later confirmed on imaging. During portal vein angiography, endovascular embolization of the portocaval fistula achieved occlusion of the anomalous venous tract. In the second patient, affected by Down's syndrome, the diagnosis of a portosystemic malformation was made by routine ultrasonography, performed to rule out concurrent congenital anomalies. Because of the absence of symptoms, we chose to observe this patient. Conclusions. These two case reports demonstrate the clinical heterogeneity of this malformation and the need for a multidisciplinary approach. As part of a proper workup, clinical evaluation must always be followed by radiographic diagnosis.

Congenital Extrahepatic Portosystemic Shunts: Spectrum of Findings on Ultrasound, Computed Tomography, and Magnetic Resonance Imaging.

Congenital extrahepatic portosystemic shunt (CEPS) is a rare disorder characterised by partial or complete diversion of portomesenteric blood into systemic veins via congenital shunts. Type I is characterised by complete lack of intrahepatic portal venous blood flow due to an end to side fistula between main portal vein and the inferior vena cava. Type II on the other hand is characterised by partial preservation of portal blood supply to liver and side to side fistula between main portal vein or its branches and mesenteric, splenic, gastric, and systemic veins. The presentation of these patients is variable. Focal liver lesions, most commonly nodular regenerative hyperplasia, are an important clue to the underlying condition. This pictorial essay covers imaging characteristics in abdominopelvic region.

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review.

Abernethy malformation is a rare congenital malformation defined by an extrahepatic portosystemic shunt. The majority of affected patients are young (<18 years of age) and experience various symptoms, including vomiting, jaundice, dyspnea and coma. The current study presents a case of Abernethy malformation in an asymptomatic adult male patient. The patient exhibited congenital absence of the portal vein, congenital heart disease (postoperative ventricular septal defect status), and multiple liver lesions, confirmed to be focal nodular hyperplasia by biopsy. Ultrasonography and magnetic resonance imaging findings revealing the liver lesions, type II congenital absence of the portal vein and the portosystemic shunt are presented. In addition, the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder are investigated by reviewing 101 previously reported cases.

Bipartite portosystemic shunt with single insertion: a rare congenital vascular anomaly in a dog

A case of a bipartite extra hepatic portosystemic shunt is described in a one-year-old, male entire Maltese Terrier breed dog. On CT angiography, one vessel was visibly a portocaval shunt...

Hypoplasie van de vena portae bij honden

Hypoplasie van de vena portae (PVH) is een aangeboren afwijking waarbij intrahepatische, microscopische shunts aanwezig zijn, waardoor het bloed niet door de leversinusoïden vloeit. De klinische presentatie en laboratoriumbevindingen vertonen sterke gelijkenissen met deze van patiënten met een extrahepatische portosystemische shunt (EHPSS). De differentiatie dient te gebeuren op basis van het al dan niet aanwezig zijn van een macroscopische shunt bevestigd met medische beeldvormingstechnieken. In dit overzichtsartikel worden de belangrijke aspecten van PVH overlopen, inclusief de verschillen met EHPSSs. Verder wordt ingegaan op de uitdaging om beide aandoeningen te diagnosticeren bij honden die zowel PVH als een EHPSS hebben.

Analysis of the relationship of extrahepatic portosystemic shunt morphology with clinical variables in dogs: 53 cases (2009–2012)

To investigate differences in clinical variables among dogs with extrahepatic portosystemic shunts (EHPSSs) of various morphologies. Retrospective case series. 53 dogs with EHPSSs. Medical records of dogs undergoing preoperative CT angiography of an EHPSS over a 3-year period were reviewed. Analysis was performed to investigate relationships of clinical variables with shunt morphology. Morphologies were analyzed individually as well as in several groups. Shunt morphologies included 10 splenocaval, 9 splenophrenic, 11 splenoazygos, 10 right gastric-caval, 12 right gastric-caval with a caudal loop, and 1 right gastric-azygos with a caudal loop. Several biochemical variables associated with EHPSS were lowest in dogs with splenocaval shunts. Preoperative clinical signs were more common in dogs that had shunts with vena caval than right azygos vein insertion (36/41 [88%] vs 7/12 [58%]) and insertion caudal to the liver than diaphragmatic insertion (29/32 [91%] vs 14/21 [67%]). Neurologic signs were more common when shunts inserted into the vena cava caudal to the liver than in other locations (21/32 [66%] vs 6/21 [29%]) and were most frequent with splenocaval shunts. Urinary tract signs were more common when shunts had right gastric vein origin than gastrosplenic vein origin (14/23 [61%] vs 10/30 [33%]). Splenocaval shunts caused more clinical abnormalities than did other shunt morphologies. Results suggested that dogs with shunt insertion in the caudal vena cava, especially caudal to the liver, were most likely to have clinical signs.

Surgical correction of a splenophrenic shunt in a dog: a case report

A 4.3 kg, nine-year-old, spayed female Shih Tzu was presented for a two-month history of seizures, ataxia, and hyper-salivation. A diagnosis of a splenophrenic shunt was made by use of computed tomography angiography with volume-rendered imaging. A cellophane band was placed around the shunt after its isolation from the central tendon of the diaphragm. Clinical signs continued to wax and wane. Preprandial and postprandial bile acids levels were still elevated 10 months after surgery. An ameroid ring constrictor was placed around the shunt vessel before the vessel entered the diaphragm from its caudal aspect. At three months after the second surgery, the dog was near the normal ranges of preprandial and postprandial bile acids. Although a study of the anatomy of different types of extrahepatic portosystemic shunts has been reported in dogs, to the authors&amp;rsquo; knowledge, there is a lack of information on clinical presentation, treatment, and postoperative results in a specific type of extrahepatic portosystemic shunt, such as a splenophrenic shunt. Cellophane banding should be avoided for occlusion of a splenophrenic shunt passing along the central tendon of the diaphragm. &amp;nbsp;