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Vessel embolization can be a valuable adjunct procedure in transjugular intrahepatic portosystemic shunt (TIPS). During the creation of a TIPS, embolization of portal vein collaterals supplying esophageal varices may lower the risk of secondary rebleeding. And after creation of a TIPS, closure of the TIPS itself may be indicated if the resulting hepatic encephalopathy severely impairs mental functioning. The Amplatzer Vascular Plug (AVP; AGA Medical, Golden Valley, MN) is well suited for embolization of large-diameter vessels and has been employed in a variety of vascular lesions including congenital arteriovenous shunts. Here we describe the use of the AVP in the context of TIPS to embolize portal vein collaterals (n = 8) or to occlude the TIPS (n = 2).

We present a case with hepatic myelopathy (HM) due to a surgical splenorenal shunt that was successfully treated by endovascular interventional techniques. A 39-year-old man presented with progressive spastic paraparesis of his lower limbs 14 mo after a splenorenal shunt. A portal venogram identified a widened patent splenorenal shunt. We used an occlusion balloon catheter initially to occlude the shunt. Further monitoring of the patient revealed a decrease in his serum ammonia level and an improvement in leg strength. We then used an Amplatzer vascular plug (AVP) to enable closure of the shunt. During the follow up period of 7 mo, the patient experienced significant clinical improvement and normalization of blood ammonia, without any complications. Occlusion of a surgically created splenorenal shunt with AVP represents an alternative therapy to surgery or coil embolization that can help to relieve shunt-induced HM symptoms.

A congenital intrahepatic portosystemic shunt is a rare anomaly; but, the number of diagnosed cases has increased with advanced imaging tools. Symptomatic portosystemic shunts, especially those that include hyperammonemia, should be treated; and various endovascular treatment methods other than surgery have been reported. Hepatic masses with either an intra- or extrahepatic shunt also have been reported, and the mass is another reason for treatment. Authors report a case of a congenital intrahepatic portosystemic shunt with a hepatic mass that was successfully treated using a percutaneous endovascular approach with vascular plugs. By the time the first short-term follow-up was conducted, the hepatic mass had disappeared.

Budd-Chiari syndrome is a spectrum of manifestations which develops as a result of hepatic venous outflow obstruction. Transjugular intrahepatic portosystemic shunt (TIPS) is a minimally invasive vascular and interventional radiological procedure indicated in the management of refractory ascites in such patients. Conventional TIPS requires the presence of a patent hepatic vein and reasonable accessibility to the portal vein, and in patients with totally occluded hepatic veins, this procedure is technically challenging. Direct intrahepatic portosystemic shunt (DIPS) or so called “percutaneous TIPS” involves ultrasound-guided percutaneous simultaneous puncture of the portal vein and inferior vena cava followed by introduction of a guidewire through the portal vein into the inferior vena cava, as a deviation from conventional TIPS. Described here is our experience with DIPS. Three patients with BCS who had refractory ascites but were unsuitable for conventional TIPS due to occlusion of the hepatic veins were chosen to undergo the DIPS procedure. Our technical success was 100%. The shunts placed in two patients remain patent to date, while the shunt in a third patient with underlying antiphospholipid syndrome was occluded a month after the procedure. The percutaneous TIPS procedure seems to be technically feasible and effective in the management of refractory ascites as a result of BCS, particularly in the setting of occluded hepatic veins.

From being a mere (though important) diagnostic tool, radiology has evolved to become an integral part of therapy in portal hypertension today. Various procedures are currently available, the choice depending on the etiology and location of disease, the pathoanatomy, and the symptomatology. The main aim of any procedure is to reduce the portal pressure by either direct or indirect methods. This can be achieved with transjugular intrahepatic portosystemic shunt (TIPS), recanalization of the hepatic vein outflow, recanalization of the portal vein and its tributaries, recanalization of dysfunctional portosystemic shunts, partial splenic embolization, and embolization of arterioportal shunts. When any of these procedures cannot be performed due to anatomical or physiological reasons, the symptoms can often be controlled effectively with embolization of varices or balloon-occluded retrograde transvenous obliteration of varices (BRTO). This article briefly describes the procedures, their results, and their current status in the treatment of portal hypertension.

Although endovascular transcatheter embolization of arteriovenous fistulas is minimally invasive, the torrential flow prevailing within a fistula implies the risk of migration of the deployed embolization devices into the downstream venous and pulmonary circulation. We present the endovascular treatment of a giant postnephrectomy arteriovenous fistula between the right renal pedicle and the residual renal vein in a 63-year-old man.

The purpose of this case report is to demonstrate that the Amplatzer vascular plug (AVP) can be safely positioned to embolize even relatively large arteriovenous fistulas (AVFs). Secondly, we illustrate that this occluder can even be introduced to the fistula via a transvenous catheter in cases where it is initially not possible to advance the deployment-catheter through a tortuous feeder artery. Migration of the vascular plug was ruled out at follow-up 4 months subsequently to the intervention.

Thus, the Amplatzer vascular plug and the arteriovenous through-and-through guide wire access with subsequent transvenous deployment should be considered in similar cases.

Background

Hepatic vein thrombosis (Budd-Chiari Syndrome) is a rare disorder resulting from an obstruction to the outflow of blood from the liver. Early decompression is needed to prevent liver dysfunction and death. Radiological intervention includes angioplasty of stenosis and webs and the placement of transjugular intrahepatic portosystemic shunts (TIPPS). Side-to-side portacaval shunt (SSPCS) remains the gold standard for achieving good long-term results.

Case presentation

A 37-year old lady underwent side-to-side portacaval shunt for Budd Chiari syndrome. She had early shunt blockage and this was successfully treated with the placement of a metallic stent across the shunt.

Conclusion

At five years, she remains asymptomatic, with normal liver functions, no ascites, and normal flow through the stent on Colour Doppler examination.

Congenital disorders of the hepatic portal vasculature are rare in man but occur frequently in certain dog breeds. In dogs, there are two main subtypes: intrahepatic portosystemic shunts, which are considered to stem from defective closure of the embryonic ductus venosus, and extrahepatic shunts, which connect the splanchnic vascular system with the vena cava or vena azygos. Both subtypes result in nearly complete bypass of the liver by the portal blood flow. In both subtypes the development of the smaller branches of the portal vein tree in the liver is impaired and terminal branches delivering portal blood to the liver lobules are often lacking. The clinical signs are due to poor liver growth, development, and function. Patency of the ductus venosus seems to be a digenic trait in Irish wolfhounds, whereas Cairn terriers with extrahepatic portosystemic shunts display a more complex inheritance. The genes involved in these disorders cannot be identified with the sporadic human cases, but in dogs, the genome-wide study of the extrahepatic form is at an advanced stage. The canine disease may lead to the identification of novel genes and pathways cooperating in growth and development of the hepatic portal vein tree. The same pathways likely regulate the development of the vascular system of regenerating livers during liver diseases such as hepatitis and cirrhosis. Therefore, the identification of these molecular pathways may provide a basis for future proregenerative intervention.

This report presents the case of a 78-year-old female with hepatic encephalopathy due to an inferior mesenteric venous-inferior vena cava shunt. She developed hepatocellular carcinoma affected by hepatitis C virus-related cirrhosis and underwent posterior sectionectomy. Portal vein thrombosis developed and the portal trunk was narrowed after hepatectomy. Portal vein thrombosis resulted in high portal pressure and increased blood flow in an inferior mesenteric venous-inferior vena cava shunt, and hepatic encephalopathy with hyperammonemia was aggravated. The hepatic encephalopathy aggravated by portal vein thrombosis was successfully treated by balloon-occluded retrograde transvenous obliteration via a right transjugular venous approach without the development of other collateral vessels.

We describe two cases of post liver transplant encephalopathy caused by persistent portosystemic shunts despite good graft function. Such recurrence of encephalopathy due to persistent shunting has not been reported in the deceased donor liver transplant literature. Our patients had episodic hepatic encephalopathy concordant with elevated serum ammonia levels due to well documented persistent portosystemic shunts. In one of our cases, the shunt was obliterated via coil embolization. This patient's encephalopathy resolved completely and has not recurred over seven months of follow up. The second patient has declined an intervention, but has remained symptom free on maintenance lactulose and rifaximin.

Variceal bleeding from enterostomy site is an unusual complication of portal hypertension. The bleeding, however, is often recurrent and may be fatal. The hemorrhage can be managed with local measures in most patients, but when these fail, surgical interventions or portosystemic shunt may be required. Herein, we report a case in which recurrent bleeding from stomal varices, developed after a colectomy for rectal cancer, was successfully treated by placement of transjugular intrahepatic portosystemic shunt (TIPS) with coil embolization. Although several treatment options are available for this entity, we consider that TIPS with coil embolization offers minimally invasive and definitive treatment.

ABSTRACT

Hepatic encephalopathy (HE) is a common complication encountered by patients after transjugular intrahepatic portosystemic shunt (TIPS) creation. Although most patients respond well to conservative medical therapy, up to 7% of patients do not and require more invasive therapeutic approaches. One option is emergent liver transplantation; however, most patients are not suitable candidates. During the past decade, various percutaneous techniques have been described that alter the hemodynamics through the TIPS by occluding it with coils or balloons or by reducing its diameter using constrained stents or stent-grafts. These endovascular techniques have produced symptomatic improvement in many patients with refractory HE, with either complete resolution or substantial reduction of HE symptoms that can be controlled with additional medical therapy. Unfortunately, despite all attempts, some patients remain incapacitated and ultimately die. Further research is necessary to improve our understanding of HE after TIPS creation so that less invasive and safer procedures can be developed to treat this difficult clinical problem.

ABSTRACT

The liver depends on a dual blood supply from the hepatic artery and the portal vein. The normal liver receives 70% portal flow and 30% hepatic arterial flow, with most arterial blood feeding the biliary tree. As cirrhosis robs the liver of its regenerative capacity, the portal flow decreases and intrahepatic portosystemic shunting increases with a variable increase in arterial flow across arterioportal shunts. This compensation mechanism attempts to reperfuse remaining sinusoids. Transjugular intrahepatic portosystemic shunts (TIPS) or surgical portosystemic shunts may acutely diminish portal perfusion further, leading to hepatic failure. Small-diameter TIPS or surgical shunts reduce the incidence of complications by preserving nutritive portal flow. Although the inverse relationship of arterial and portal flow is physiologically valid, there is individual variation in the ability to substitute one blood supply for another. This variability may result from anatomic or functional factors influencing the flow across arterioportal shunts. Hepatic perfusion curves derived from enhanced imaging studies can subtype cirrhotic patients into favorable versus unfavorable perfusion patterns. Patients with high arterial flow to the liver or patients with retained portal-type flow curves have better survival and morbidity compared with those patients with unfavorable flow manifest by diminished arterial-type curves on hepatic perfusion analysis.

Background

Portal hypertension leads to the formation of portosystemic collateral veins in liver cirrhosis. The resulting shunting is responsible for the development of portosystemic encephalopathy. Although ammonia plays a certain role in determining portosystemic encephalopathy, the venous ammonia level has not been found to correlate with the presence or severity of this entity. So, it has become partially obsolete. Realizing the need for non-invasive markers mirroring the presence of esophageal varices in order to reduce the number of endoscopy screening, we came back to determine whether there was a correlation between blood ammonia concentrations and the detection of portosystemic collateral veins, also evaluating splenomegaly, hypersplenism (thrombocytopenia) and the severity of liver cirrhosis.

Methods

One hundred and fifty three consecutive patients with hepatic cirrhosis of various etiologies were recruited to participate in endoscopic and ultrasonography screening for the presence of portosystemic collaterals mostly esophageal varices, but also portal hypertensive gastropathy and large spontaneous shunts.

Results

Based on Child-Pugh classification, the median level of blood ammonia was 45 mcM/L in 64 patients belonging to class A, 66 mcM/L in 66 patients of class B and 108 mcM/L in 23 patients of class C respectively (p < 0.001).

The grade of esophageal varices was concordant with venous ammonia levels (rho 0.43, p < 0.001). The best area under the curve was given by ammonia concentrations, i, e., 0.78, when comparing areas of ammonia levels, platelet count and spleen longitudinal diameter at ultrasonography. Ammonia levels predicted hepatic decompensation and ascites presence (Odds Ratio 1.018, p < 0.001).

Conclusion

Identifying cirrhotic patients with high blood ammonia concentrations could be clinically useful, as high levels would lead to suspicion of being in presence of collaterals, in clinical practice of esophageal varices, and pinpoint those patients requiring closer follow-up and endoscopic screening.

Objective

To determine the feasibility of transcaval transjugular intrahepatic portosystemic shunt (TIPS) in patients with occluded previous TIPS.

Materials and Methods

Between February 1996 and December 2000 we performed five transcaval TIPS procedures in four patients with recurrent gastric cardiac variceal bleeding. All four had occluded TIPS, which was between the hepatic and portal vein. The interval between initial TIPS placement and revisional procedures with transcaval TIPS varied between three and 31 months; one patient underwent transcaval TIPS twice, with a 31-month interval. After revision of the occluded shunt failed, direct cavoportal puncture at the retrohepatic segment of the IVC was attempted.

Results

Transcaval TIPS placement was technically successful in all cases. In three, tractography revealed slight leakage of contrast materials into hepatic subcapsular or subdiaphragmatic pericaval space. There was no evidence of propagation of extravasated contrast materials through the retroperitoneal space or spillage into the peritoneal space. After the tract was dilated by a bare stent, no patient experienced trans-stent bleeding and no serious procedure-related complications occurred. After successful shunt creation, variceal bleeding ceased in all patients.

Conclusion

Transcaval TIPS placement is an effective and safe alternative treatment in patients with occluded previous TIPS and no hepatic veins suitable for new TIPS.

Intracranial pial arteriovenous fistulas (AVFs) are rare vascular lesions of the brain. These lesions consist of one or more arterial connection to a single venous channel without true intervening nidus. A 24-year-old woman visited to our hospital because of headache, vomiting, dizziness and memory disturbance that persisted for three days. She complained several times of drop attack because of sudden weakness on both leg. Cerebral angiograms demonstrated a giant venous aneurysm on right frontal lobe beyond the genu of corpus callosum, multiple varices on both frontal lobes fed by azygos anterior cerebral artery, and markedly dilated draining vein into superior sagittal sinus, suggesting single channel pial AVF with multiple varices. Transarterial coil embolization of giant aneurysm and fistulous portion resulted in complete disappearance of pial AVF without complication.

Purpose

To evaluate the effect of hepatic encephalopathy (HE), hepatic failure, and portosystemic shunt (PS) on the brain volume alteration in cirrhotic patients with MRI voxel-based morphometry (VBM).

Methods

Sixty cirrhotic patients (overt HE [OHE], n = 11; minimal HE [MHE], n = 19; non HE [nHE], n = 30) including 12 with pre- and post-transjugular intrahepatic portosystemic shunt (TIPS) scanning and 40 healthy controls were recruited. Neuropsychological and laboratory tests were performed in all patients. VBM was analyzed with ANOVA test among 4 groups, and t-tests for patients with different hepatic function, PS scores, and TIPS. Multiple linear regression was performed to investigate the effect of venous blood ammonia levels, Child-Pugh scores, and PS on the brain volumes in all patients.

Results

Cirrhotic patients exhibited decreased volume in many areas of gray matter (GM), increased volume in thalamus, and increased whiter matter (WM) volume, with the extent of affected brain volume greater in HE patients than nHE patients. Hepatic failure also resulted in decreased GM volume. Patients with high PS scores and TIPS displayed decreased GM and increased WM volume in some regions. Post-TIPS patients displayed increased GM volume in the thalamus. Multiple covariate regression results suggested that Child-Pugh score was a major factor to affect GM volume, while PS mainly affected WM volume.

Conclusion

Brain structure abnormalities appeared bilaterally symmetrical in cirrhotic patients, and the impairment was more extensive in HE patients than those without HE. Increased thalamus volume was not associated with HE progression. Hepatic failure and PS altered cirrhotic patients’ brain structure.

An extrahepatic arterioportal fistula (APF) involving the gastroduodenal artery and superior mesenteric vein is rare and mostly results from iatrogenic injuries. The clinical symptoms associated with APFs may include abdominal pain, gastrointestinal bleeding, ascites, nausea, vomiting, diarrhea, or even congestive heart failure. We present the case of a 70-year-old man who presented with chronic abdominal pain and gastrointestinal bleeding secondary to APF and portal vein thrombosis. The endovascular embolization of APF was accomplished successfully, and symptoms of portal hypertension resolved immediately after intervention. Unfortunately, the patient did not respond well to anticoagulation therapy with warfarin. Therefore, the patient underwent implantation of a transjugular intrahepatic portosystemic shunt, and the complications of portal hypertension resolved. In conclusion, the embolization of APF is technically feasible and effective and can be considered the first-choice therapy in selected patients.

ABSTRACT

Budd-Chiari syndrome (BCS) denotes a heterogeneous group of diseases characterized by hepatic venous outflow obstruction at the level of the hepatic veins or inferior vena cava resulting in portal hypertension. Traditional approach to treatment of BCS involves systemic thrombolysis and surgical portosystemic shunt or transjugular intrahepatic portosystemic shunt in progressive cases of BCS or as a bridge to transplantation. Recently, an increasing number of successful reports of BCS therapy have involved endovascular techniques, including angioplasty and stent placement. The present report illustrates successful percutaneous recanalization of complete hepatic vein occlusion by angioplasty and stent implantation in a patient with membranous obstruction.

Dr. Takao Hiraki is a scientist carrying out interventional radiology research in the Department of Radiology at Okayama University Medical School, Japan. He has conducted animal and human clinical studies on interventional radiology for various conditions. For example, he clarified the hepatic hemodynamic changes caused by hepatic venous occlusion. He also developed new devices, such as hydrogel coils for the occlusion of the aneurismal sac after an endovascular stent-graft of an aortic aneurysm to prevent endoleakage and small intestinal submucosa-covered stents for transjugular intrahepatic portosystemic shunts. Further, he performed a number of studies on the radiofrequency ablation of lung cancer, mediastinal lymph node metastasis, and computed tomography-fluoroscopy-guided lung biopsies. He intends to continue to dedicate his academic career to expand the role of interventional radiology in clinical medicine.

We report a case of 3-year-old girl who had persistence of large residual flow following transcatheter closure of a 6 mm ‘Type A’ patent ductus arteriosus using a 12 × 10 mm duct occluder. Angiography revealed a large left-to-right shunt coursing through and exiting around the implanted device. Near total abolition of the residual shunt was achieved by initial implantation of an embolization coil within the duct occluder and subsequently an Amplatzer duct occluder (ADO II) adjacent to the duct occluder. This challenging case describes an additional technique of abolishing a large residual flow in and around a Nitinol duct occluder device.

Patent foramen ovale and atrial septal defect are risk factors for paradoxical embolism and subsequent cerebral ischemic events. The transseptal passage of emboli from the right to the left cardiac chambers appears to play an important role. The therapeutic options are medical therapy (anti-aggregation or anticoagulation), surgical closure, or transcatheter closure. Transcatheter closure of atrial septal defects affords the advantage of closing an atrial defect without the associated morbidity of open-heart surgery and the bleeding sequelae of oral anticoagulation. After closure, however, the presence of a residual shunt is independently associated with an increased risk of recurrent ischemic events. Newer devices, such as the AMPLATZER Septal Occluder, have decreased the risk of residual shunting and thromboembolic events. In addition, they have a very low risk of device dislodgement, migration, and embolization.

We describe the case of a 60-year-old woman with Ebstein anomaly and recurrent ischemic strokes who presented with acute ischemic infarcts and paradoxical embolism 3 years after undergoing transcatheter closure of an atrial septal defect. A right-to-left shunt through a displaced AMPLATZER Septal Occluder was detected. Pulmonary hypertension and resultant right ventricular failure and right atrial dilation could have contributed to the persistent shunting and paradoxical embolism.

To our knowledge, the delayed dysfunction of an AMPLATZER Septal Occluder has not been reported. In addition to describing the patient's case, we review the relevant medical literature.

WEBSITE FEATURE

Objective

To determine the feasibility of transcaval transjugular intrahepatic portosystemic shunt (TIPS) creation in patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins.

Materials and Methods

Transcaval TIPS, performed in six patients, was indicated by active variceal bleeding (n=2), recurrent variceal bleeding (n=2), intractable ascites (n=1), and as a bridge to liver transplantation (n=1). The main reasons for transcaval rather than classic TIPS were the presence of an unusually acute angle between the hepatic veins and the level of the portal bifurcation (n=3), hepatic venous occlusion (n=2), and inadequate small hepatic veins (n=1).

Results

Technical and functional success was achieved in all patients. The entry site into liver parenchyma from the inferior vena cava was within 2 cm of the atriocaval junction. Procedure-related complications included the death of one patient due to hemoperitoneum despite the absence of contrast media spillage at tractography, and another suffered reversible hepatic encephalopathy.

Conclusion

In patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins, transcaval TIPS creation is feasible.

We report a case of duodenal varix bleeding as a long term complication of balloon occluded retrograde transvenous obliteration (BRTO), which was successfully treated with a transjugular intrahepatic portosystemic shunt (TIPS). A 57-year-old man was admitted to the emergency room suffering from melena. He had undergone BRTO to treat gastric varix bleeding 5 mo before admission. Endoscopy and a computed tomography (CT) scan showed complete obliteration of the gastric varix, but the nodular varices in the second portion of the duodenum expanded after BRTO, and spurting blood was seen. TIPS was performed for treatment of duodenal variceal bleeding, because attempts at endoscopic varix ligation were unsuccessful. The postoperative course was uneventful and the patient was discharged without complications. A follow up CT scan obtained 21 mo after TIPS revealed a patent TIPS tract and complete obliteration of duodenal varices, but multinodular hepatocellular carcinoma had developed. He died of hepatic failure 28 mo after TIPS.

Summary

Aneurysmal rupture of the intra-cavernous carotid artery may cause idiopathic carotid-cavernous fistula (CCF), and the treatment choice for occluding shunting fistula in this type of CCF is an endovascular approach using detachable balloons. However, little has been reported on treating such lesions with the intra-aneurysmal embolization using Guglielmi detachable coils (GDCs). To our knowledge, ours is the first reported case of successful treatment by selective intra-fistula and intra-aneurysmal embolization with GDCs. A 74-year-old woman exhibited proptosis and chemosis of her left eye over a period of one month. Symptoms of double vision in conversion and pulsatile murmur in her left eye were also noted. Angiography revealed an intra-cavernous aneurysm of the left internal carotid artery (ICA) with a shunting fistula, which drained into the dilated cavernous sinus, superior orbital vein (SOV), superior petrosal sinus, inferior petrosal sinus, and pterygoid plexus. We thought the fistula would occlude by intra-aneurysmal embolization, but we had no confidence of tight packing of the aneurysm since the aneurismal neck was relatively wide. So, we embolized the venous side of the shunting fistula and then the dome of the aneurysm with GDCs. Immediately after the operation, her symptoms and signs were ameliorated, and complete occlusion of the CCF was observed on long-term follow-up. We suggest selective intrafistula and intra-aneurysmal embolization with GDCs as an alternative method of treatment of idiopathic CCF originating from aneurysmal rupture of the intra-cavernous carotid artery.