Intrahepatic Portosystemic Shunt Research Articles

A rare and curable cause of cyanosis – Congenital portosystemic shunts

Congenital portosystemic shunts (CPSSs) are rare developmental vascular anomalies which are classified into extrahepatic and intrahepatic malformations. Here, we report a 7-year-old girl who presented with a history of progressive dyspnea, pandigital cyanosis, and clubbing. On evaluation, she was found to have a rare combination of both type II extrahepatic and intrahepatic portosystemic shunts which were closed endovascularly in the same setting. Diagnosis of CPSS needs a very high index of clinical suspicion. Timely diagnosis with specific imaging modalities will help in deciding the treatment strategy and may prevent long-term complications.

The Ductus Venosus.

the ductus venosus (DV) is not well known in general radiology, but it plays a role in the daily work of pediatric radiologists. Consequently all general radiologists who also care for a pediatric department should be familiar with the physiological and pathological findings related to the DV. Literature research in Medline, using the keywords "ductus venosus" and "umbilical vein catheter". In the first weeks of life the DV is regularly still patent. It should be recognized as DV and not be mistaken for a pathological portosystemic shunt.The ductus venosus is the lead structure for umbilical vein catheters. Radiologists should be able to assess the correct catheter position. Radiologically important findings of an umbilical vein catheter are mainly malposition and intrahepatic extravasation. Agenesis of the DV can lead to intra- or extrahepatic compensatory portosystemic shunts, in which as well as in the case of persistent patency of the DV, there may be the necessity for radiological-interventional or surgical occlusion. · In the first weeks of life in infants the Ductus venosus is regularly still patent.. · The Ductus venosus should not be mistaken for a pathological portosystemic shunt.. · An umbilcal vein catheter should project onto the Ductus venosus and end caudal to the right atrium.. · Intrahepatic portosystemic shunts in DV agenesis show a high rate of spontaneous closure postnatally.. · Born M. The Ductus Venosus. Fortschr Röntgenstr 2021; 193: 521 - 526.

Prenatal diagnosis of abnormality of the umbilical portal DV complex: difficulty in universal classification due to various alternative routes in hepatic circulation for placental drainage

Objective To review our experience with fetal abnormality of the umbilical-portal-DV complex and to discuss the new classification system for umbilical portal systemic venous shunts (UPSVS) according to our cases. Methods This study was a retrospective analysis of fetuses with a prenatal diagnosis of abnormality of the umbilical-portal-DV complex. The integrity of the fetal umbilical-portal ductus venosus complex and the hepatic venous system were evaluated using two-dimensional color Doppler sonography. The origin of the shunt, the location of the drainage, and the presence or absence of intrahepatic portal venous system and DV were noted. Results 35 cases of abnormality of the umbilical-portal-DV complex were identified. Agenesis of ductus venous was detected in 33 of them. Based on the abnormality of the umbilical-portal-DV complex, we divided the cases into five groups. Group 1, ductus venosus agenesis with normal hepatic venous anatomy (n = 11); Group 2 downward displacement of the umbilical-portal-DV complex (n = 13); Group 3, umbilical-systemic shunt (n = 5); Group 4, intrahepatic portosystemic shunt (n = 4), Group 5, hepatic arteriovenous malformation (n = 2). Three different intrahepatic portosystemic shunt and one different downward displacement of the umbilical-portal-DV complex cases were detected. Conclusions Disruption of the normal anatomy of the umbilical-portal-DV complex causes various alternative pathway of the placental drainage. This illustrates highlights the challenge of creating a universal classification.

Prenatal diagnosis of congenital portosystemic shunt: A single-center study.

To review our experience with the prenatal diagnosis of congenital portosystemic shunt (CPSS). This is a retrospective study of CPSS cases examined at an ultrasonographic tertiary referral center from 2013 to 2019. The anatomical origin and drainage of the shunt were assessed. Feto-maternal clinical characteristics and long-term outcomes were investigated via medical files and telephone interviews with the mothers. Eleven cases were reviewed. Based on the anatomical origins, before or after portal vein division, cases were classified into extrahepatic portosystemic shunt (EHPSS, n = 3, 27.3%) and intrahepatic portosystemic shunt (IHPSS, n = 8, 72.7%). Additional abnormalities were also observed in the EHPSS (n = 2, 66.7%) and IHPSS (n = 3, 37.5%) groups. Intrauterine growth restriction was the most common abnormality (n = 4, 80%). The median age of the pregnant women was 31.9 years (range 26 ~ 43 years). Most cases (n = 8, 72.7%) were diagnosed in the third trimester, and the median gestational age was 31+5 weeks (range 24 ~ 36+6 weeks). Three cases underwent karyotype examinations, and one had trisomy 13. The median time after birth was 2 years (range 0.7 ~ 5.7 years). The overall postnatal live-birth rate was 60% (6/10), not including one case with no data on pregnancy outcome. The mothers of the six live births indicated that their children were in excellent health. This study indicates that prenatal CPSS diagnosis is feasible, especially in the third trimester. IHPSS is more common than EHPSS. Complicated cases most often occur with EHPSS. Intrauterine growth restriction is the most common concomitant abnormality. The prognosis of most cases is good.

FIB-4 and APRI-scores predict survival in patients with transjugular intrahepatic portosystemic stent shunts

FIB-4 and APRI-scores predict survival in patients with transjugular intrahepatic portosystemic stent shunts

Canine intrahepatic portosystemic shunt insertion into the systemic circulation is commonly through primary hepatic veins as assessed with CT angiography.

Congenital intrahepatic portosystemic shunts (IHPSS) in dogs are traditionally classified as right, left, or central divisional. There are few descriptive studies regarding the variation of IHPSS within these categories. This multicenter, analytical, cross-sectional study aimed to describe a large series of dogs with CT angiography (CTA) of IHPSS, hypothesizing that there would be variation to the existing classification. Ninety CTA studies were assessed for IHPSS type, insertion, and the relationship of the insertion to the primary hepatic veins. Ninety-two percent of IHPSS inserted into a primary hepatic vein (HV) or phrenic vein, 8% inserted directly into the ventral aspect of the intrahepatic caudal vena cava. The most common IHPSS type was a single right divisional (44%), including those inserting via the right lateral HV or the caudate HV. Left divisional IHPSS (33%) inserted into the left HV or left phrenic vein. Central divisional IHPSS (13%) inserted into the quadrate HV, central HV, dorsal right medial HV, or directly into the ventral aspect of the intrahepatic caudal vena cava. Multiple sites of insertion were seen in 9% of dogs. Within left, central, and right divisional types, further subclassifications can therefore commonly be defined based on the hepatic veins with which the shunting vessel communicates. Relating IHPSS morphology to the receiving primary HV could make IHPSS categorization more consistent and may influence the type and method of IHPSS attenuation recommended.

Liver resection for a congenital intrahepatic portosystemic shunt in a child with hyperammonemia and hypermanganesemia: a case report

BackgroundCongenital portosystemic shunt (CPSS) is a rare malformation that leads to hyperammonemia, hypermanganesemia, and various symptoms. CPSSs are divided into intrahepatic and extrahepatic shunts. In patients with persistent CPSS including an intrahepatic portosystemic shunt (IPSS), early intervention to occlude the shunt reverses the associated complications.Case presentationThe patient was a 1-year-and-7-month-old girl. She presented with hypergalactosemia and elevation of blood ammonia level (75 μg/dL) and total bile acid levels (68.2 μmol/L) during the neonatal period. Two IPSSs were detected using ultrasound and enhanced computerized tomography. Magnetic resonance imaging (MRI) at 1 year and 3 months of age showed abnormally high signal intensity in the pallidum of her brain. Spontaneous closure was not observed. We performed a right hepatectomy at 1 year and 7 months of age. The portal vein pressure was 16 mmHg after temporary occlusion of the right portal vein. Blood ammonia and serum manganese levels decreased immediately after the operation. The abnormal signal on brain MRI disappeared. She had a favorable course with no sign of recurrence of IPSS 5 years postoperatively.ConclusionLiver resection for an IPSS to control the symptoms of a portosystemic shunt is reasonable in a child for whom interventional radiological treatment is not indicated.

Clinical Characteristics, Serum Biochemical Changes, and Expression Profile of Serum Cfa-miRNAs in Dogs Confirmed to Have Congenital Portosystemic Shunts Accompanied by Liver Pathologies

Computed tomography angiography (CTA) and biochemical parameters cannot specify liver pathologies in dogs with congenital portosystemic shunts (CPSS) that are easily determined by invasive histopathology. This study aims to assess the possibility of using circulating serum canine familiaris (cfa) microRNAs (miRNAs) as novel non-invasive serum-based fingerprints for liver injuries associated with various morphologies of extrahepatic and intrahepatic portosystemic shunts (EHPSS and IHPSS). Data were obtained from 12 healthy dogs and 84 dogs confirmed to have EHPSS (splenocaval, splenophrenic, splenoazygos, right gastrocaval (RGC), right gastrocaval with caudal loop (RGC–CL)) and IHPSS (right divisional and left divisional) using CTA. Hepatic pathologies were determined by histopathology. Serum expression of miRNAs was assessed by real-time polymerase chain reaction. Based on the nature of liver injuries in each shunt type, cfa-miR-122 was significantly upregulated in all CPSS groups. Meanwhile, serums cfa-miR-34a and 21 were not significantly expressed in splenophrenic or splenoazygos groups, but they were extensively upregulated in splenocaval, RGC, RGC–CL groups and less frequently in right or left divisional groups. Also, serum cfa-miR126 was significantly upregulated in both IHPSS groups but less significantly expressed in RGC, RGC–CL, and splenocaval groups. Overall, estimated cfa-miRNAs could serve as novel biomarkers to mirror the histopathological and molecular events within the liver in each shunt type.

Protocolo diagnóstico y terapéutico de la hemorragia digestiva alta en urgencias

Upper digestive tract hemorrhage is a common emergency appearing as hematemesis or melena. Peptic ulcer disease and digestive gastrointestinal bleeding secondary to portal hypertension (PHT) are the commonest causes. The main goals of initial management are: to achieve hemodynamic stability, to stage the risk, to determine the most likely etiology and to begin medical treatment according with suspicion. In order to confirm diagnosis and to perform therapeutic maneuvers, early endoscopy has to be carried out (in less than 24hours). If etiology is a peptic ulcer, treatment will be based on high-doses of proton pump inhibitors, and therapeutic endoscopy in presence of injuries at high risk for rebleeding. Vasoactive and antibiotic drugs as well as endoscopic band ligation are mandatory in the cases of esophageal varices caused by PHT; intrahepatic portosystemic shunt will be taken for refractory cases.

Value of virtual touch tissue quantification in evaluating therapeutic effects of transjugular intrahepatic portosystemic stent shunt

Value of virtual touch tissue quantification in evaluating therapeutic effects of transjugular intrahepatic portosystemic stent shunt

2215 An Interesting Case of Intrahepatic Porto-Hepatic Shunt as a Cause of Unexplained Encephalopathy

INTRODUCTION: Congenital portosystemic shunts or Abernethy malformations are rare anatomical abnormalities shunting blood from the portal venous system to the systemic venous system, thereby bypassing passage through the hepatic acinus. The presentation may range from incidental finding on imaging to hepatic encephalopathy and liver failure depending on the degree and type of shunt. Here, we describe an interesting case of unexplained encephalopathy in a patient without liver cirrhosis found to have congenital intrahepatic Type II Abernethy malformation. CASE DESCRIPTION/METHODS: A 73-year-old female with a past medical history of Rheumatoid arthritis and Gilbert syndrome was admitted to hospital with clinical evidence of encephalopathy. Vitals were stable and physical examination was unremarkable except for encephalopathy. Laboratory investigations were unremarkable except for elevated ammonia levels (165 µmol/L). Computed tomography (CT) of the brain was unremarkable. Abdominal ultrasonography revealed the normal size and echotexture of the liver. Abdominal CT angiography showed the presence of dilated portal vein measuring up to 1.8 cm at the porta-hepatis along with dilated superior mesenteric and splenic veins. Multiple dilated vascular channels were seen within the right hepatic lobe. An intrahepatic portosystemic shunt between an enlarged middle hepatic vein and 2 separate branches of the right portal vein was visualized. Pressure gradients were measured and were normal. She received conservative management with lactulose and rifaximin and discharged on day 6 after the resolution of encephalopathy. DISCUSSION: Encephalopathy associated with portal-systemic bypass and no intrinsic hepatocellular disease is often referred to as Type B hepatic encephalopathy as was in our patient. Ammonia which is the culprit for the neuropsychiatric manifestation is usually metabolized by the liver in normal conditions, however in cases of portosystemic shunting, whether congenital or not, ammonia bypasses the liver and enters the systemic circulation and directly reaches the brain. The degree of symptomatology associated with these venous shunts depends on the shunt ratio and patient age. Treatment options include medical management including use of lactulose and non-absorbable antibiotics or endovascular shunt closure. Our patient was managed medically with the plan to refer her for shunt closure if she continues to have recurrent episodes.

CCI clinical practice guidelines: management of TIPS for portal hypertension (2019 edition)

Portal hypertension(PH) is one of the main complications of cirrhosis.Transjugular intrahepatic portosystemic shunt(TIPS) is the percutaneous creation of a conduit from the hepatic vein to the portal vein that is used to manage consequences of PH (i.e., variceal bleeding and refractory ascites) and used as a bridging therapy to liver transplant for decompensated cirrhosis. The following Clinical Practice Guidelines (CPGs) presents profession associational recommendations of the Chinese College of Interventionalists(CCI) on TIPS for PH. The CPGs was written by more than 30 experts in the field of TIPS in China (including interventional radiologists, liver surgeons, hepatologists and gastroenterologist, et al.). The panel of experts, produced these CPGs using evidence from PubMed and Cochrane database searches and combined with relevant expert consensuses and high quality clinical researches in China providing up to date guidance on TIPS for PH with the only purpose of improving clinical practice.

Tu1581 – Outcomes of Trans-Jugular Intrahepatic Portosystemic Stent Shunting in the Presence Or Absence of Portal Vein Thrombosis

Tu1581 – Outcomes of Trans-Jugular Intrahepatic Portosystemic Stent Shunting in the Presence Or Absence of Portal Vein Thrombosis

SAT-022-Transjugular intrahepatic portosystemic shunts is still an effective treatment for refractory variceal haemorrhage in patients with high MELD/Child Pugh scores

SAT-022-Transjugular intrahepatic portosystemic shunts is still an effective treatment for refractory variceal haemorrhage in patients with high MELD/Child Pugh scores

Treatment of aortic thrombosis with retrievable stent filter and thrombolysis: a case report

BackgroundThe retrievable stent filter (RSF) has been previously used for the treatment of vena cava thrombosis. In this study, the RSF was implanted to treat aortic thrombosis and then withdrawn.Case presentationA 47-years-old woman presented with severe abdominal pain and fever. Computed tomography showed massive mural thrombosis in the thoracic and abdominal aorta complicated by portal venous thrombosis. The RSF was implanted, a transjugular intrahepatic portosystemic stent shunt was established and a thrombolytic catheter was inserted for portal vein thrombolysis. The aortic thrombus was successfully compressed and fixed without thrombosis. After 15 days, abdominal pain had ceased, the abdominal aortic thrombus was mostly dissolved and the RSF was retrieved. Catheter angiography confirmed the recovery of portal vein thrombosis.ConclusionsThe RSF was able to compress and fix aortic thrombus without the usual complications of stenting after removal.

Asymptomatic Intrahepatic Portosystemic Venous Shunt: Case Report and Review of Literature

A spontaneous portosystemic shunt is a rare malformation of the liver vessels. The etiology of these shunts is controversial. They can be divided into acquired (most commonly associated with liver cirrhosis) and congenital. Vascular shunts are asymptomatic in the majority of the patients, and when symptomatic were presented by severe complications. The specific way of management can be selected depended on whether the patient was symptomatic or not, and also on the size of the shunt, shunt ratio and whether it was associated with cancer. We will present a clinical case and discuss the importance of the radiological imaging in the screening, diagnosis and follow up of these anomalies.

Congenital intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism: a case report and review of the literature

BackgroundCongenital portosystemic shunts present with various associated complications, such as other congenital malformations, hyperammonemia, or hepatopulmonary syndrome. Few cases of associated hypoglycemia have been reported so far and our case, to the best of our knowledge, describes the most severe extent of hypoglycemia.Case presentationWe describe the case of a newborn Arab boy with two intrahepatic portosystemic shunts, resulting in severe and persistent hypoglycemia, due to which one of the shunts was closed by interventional radiology whereas the other shunt had already closed spontaneously.ConclusionsBecause he showed elevated levels for insulin and prolonged high insulin levels in an oral glucose tolerance test, our case supports the theory that portocaval shunts cause a reduced hepatic insulin reduction due to the high blood volume bypassing the liver. This case provides further insights into glucose regulation mechanisms of the liver and we suggest a consistent screening for hypoglycemia in patients with congenital portosystemic shunts.

The portal vein as a distinct immunological compartment – A comprehensive immune phenotyping study

Advanced liver diseases are associated with impaired intestinal barrier function, which results in bacterial influx via the portal vein to the liver, causing hepatic and systemic inflammation. Little is known about possible concomitant trafficking of immune cells from the intestines to the liver. We therefore performed a comprehensive immunophenotyping study of the portal venous versus peripheral blood compartment in patients with liver cirrhosis who received a transjugular intrahepatic portosystemic stent shunt (TIPS). Our analysis suggests that the portal vein constitutes a distinct immunological compartment resembling that of the intestines, at least in patients with advanced liver cirrhosis. In detail, significantly lower frequencies of naïve CD4+ T cells, monocytes, dendritic cells and Vδ2 T cells were observed in the portal vein, whereas frequencies of activated CD4+ and CD8+ T cells, as well as of mucosa-associated Vδ1 T cells were significantly higher in portal venous compared to peripheral blood. In conclusion, our data raises interesting questions, e.g. whether liver cirrhosis-associated chronic inflammation of the intestines and portal hypertension promote an influx of activated intestinal immune cells like γδ T cells into the liver.

320-slice CT angiography before hepatic intrahepatic portosystemic shunt for evaluating the puncture approach

Objective: This study aims to evaluate the value of 320 slice Computed Tomography Angiography (CTA) in evaluating puncture approaches by simulating Intrahepatic Portosystemic Shunt (IPS) in cirrhotic patients with portal hypertension. Methods: A total of 30 patients with hepatic cirrhosis and portal hypertension, who underwent IPS from November 2014 to January 2017 at Yunnan Province Second People's Hospital, were selected for this study. The CTA data acquired before surgery were retrospectively analysed. The puncture distances and puncture angles from the right hepatic vein, middle hepatic vein and left hepatic vein to the portal vein and its branches were measured and compared with those acquired by Digital Subtraction Angiography (DSA). Then, the differences between the puncture distances and angles acquired by CTA and DSA were evaluated. Results: Differences between puncture distances and angles acquired by CTA and DSA were not statistically significant (P>0.05). Conclusions: Puncture distances and angles acquired by CTA were consistent with those acquired by DSA. This can provide an imaging basis for the preoperative surgical planning of IPS.

Endoscopic Ultrasound-Guided Vascular Interventions: From Diagnosis to Treatment.

The development of endoscopic ultrasound was driven by the need to develop less invasive alternatives to surgical and radiologic interventions for a wide variety of gastrointestinal conditions. During the past decade, there has been a very rapid growth in the clinical role and capabilities of endoscopic ultrasound-guided therapeutic interventions. Endoscopic ultrasound offers both real-time imaging and access to structures within and adjacent to the gastrointestinal tract. The proximity of the gastrointestinal system to vascular structures throughout the abdomen and the mediastinum allows for endoscopic ultrasound-guided vascular access and therapy. The recent development of endoscopic ultrasound-guided vascular interventions has relied both on finding new applications for standard endoscopic accessories and on commandeering tools originally developed for use in interventional radiology. This article provides a review of the literature regarding the current state of endoscopic ultrasound for the management of variceal and nonvariceal bleeding, portal vein angiography and pressure measurements, intrahepatic portosystemic shunts, endoscopic ultrasound-guided fine-needle aspiration for portal vein sampling, drug administration and embolization as well as endoscopic ultrasound-guided cardiac access and treatment.