Transhepatic catheter-directed thrombolysis for portal vein thrombosis after partial splenic embolization in combination with balloon-occluded retrograde transvenous obliteration of splenorenal shunt

A 66-year-old woman underwent partial splenic embolization (PSE) for hypersplenism with idiopathic portal hypertension (IPH). One week later, contrast-enhanced CT revealed extensive portal vein thrombosis (PVT) and dilated portosystemic shunts. The PVT was not dissolved by the intravenous administration of urokinase. The right portal vein was canulated via the percutaneous transhepatic route under ultrasonic guidance and a 4 Fr. straight catheter was advanced into the portal vein through the thrombus. Transhepatic catheter-directed thrombolysis was performed to dissolve the PVT and a splenorenal shunt was concurrently occluded to increase portal blood flow, using balloon-occluded retrograde transvenous obliteration (BRTO) technique. Subsequent contrast-enhanced CT showed good patency of the portal vein and thrombosed splenorenal shunt. Transhepatic catheter-directed thrombolysis combined with BRTO is feasible and effective for PVT with portosystemic shunts.     

Surgical treatment for portosystemic encephalopathy in patients with liver cirrhosis: Occlusion of portosystemic shunt in combination with splenectomy

Aim: Operative ligation of the portosystemic shunt may control hepatic encephalopathy effectively, but the subsequent increase in portal vein pressure (PVP) leads to high mortality. Splenectomy can decrease inflow into the portal system, resulting in decreased portal pressure. Methods: We retrospectively examined the effect of splenectomy in combination with shunt closure on portosystemic encephalopathy. Results: Clinical symptoms of encephalopathy disappeared in all six patients who underwent splenectomy in combination with portosystemic shunt ligation, with the exception of one patient who had relapsing encephalopathy after 6 months. Follow‐up computed tomography showed complete obliteration of the portosystemic shunts, except in the one patient with relapsing encephalopathy who underwent balloon‐occluded retrograde transvenous obliteration for the remaining splenorenal shunt 8 months after surgery. PVP significantly decreased after splenectomy. PVP did not increase to the baseline PVP value after ligation of the shunts, except in two patients who had elevated PVP after surgery: PVP increased from 18 to 19 mmHg after ligation in one patient and from 18 to 23 mmHg in one patient. Conclusion: Splenectomy followed by surgical ligation of the portosystemic shunt may be feasible and safe for cirrhotic patients with portosystemic shunts.     

The incidence of portal vein thrombosis at liver transplantation.

The incidence of portal vein thrombosis was examined in 885 patients who received orthotopic liver transplantations for various end-stage liver diseases between 1989 and 1990. The thrombosis was classified into four grades. Grade 1 was thrombosis of intrahepatic portal vein branches, grade 2 was thrombosis of the right or left portal branch or at the bifurcation, grade 3 was partial obstruction of the portal vein trunk, and grade 4 was complete obstruction of the portal vein trunk. Among the 849 patients without previous portosystemic shunt, 14 patients (1.6%) had grade 1, 27 patients (3.2%) had grade 2, 27 patients (3.2%) had grade 3 and 49 patients (5.8%) had grade 4 portal vein thrombosis. The incidence of portal vein thrombosis was highest (34.8%) in the patients with hepatic malignancy in the cirrhotic liver, followed by those with Budd-Chiari syndrome (22.2%) and postnecrotic cirrhosis of various causes (15.7%). The patients with encephalopathy, ascites, variceal bleeding, previous splenectomy and small liver had significantly higher incidences of portal vein thrombosis than the others. The total incidence of portal vein thrombosis among the 36 patients with previous portosystemic shunt was 38.9%, which was significantly higher than that (13.8%) of those without shunt.     

Time-resolved 3D cine phase-contrast magnetic resonance imaging (4D-flow MRI) can quantitatively assess portosystemic shunt severity and confirm normalization of portal flow after embolization of large portosystemic shunts

Diagnosis and severity assessments of portosystemic shunts (PSSs) are important because the pathology sometimes results in severe hepatic encephalopathy, which can be treated almost completely by shunt embolization. At present, morphological assessment of PSS is performed mainly by computed tomography, and ultrasound is used for blood flow assessment. In two cases of PSS-related hepatic encephalopathy, we used time-resolved 3D cine phase-contrast (4D-flow) magnetic resonance imaging (MRI) to assess blood flow before and after shunt embolization. Before the intervention, blood flow in the main trunk of the superior mesenteric vein was mostly hepatofugal. However, post-interventional 4D-flow MRI revealed hepatopetal superior mesenteric vein flow with significantly increased portal vein blood flow. 4D-flow MRI is an ideal adjunct to Doppler ultrasonography, allowing for objective and visual assessment of morphology and blood flow of the portal venous system, including PSSs, and is useful in determining the indications for, and outcome of, PSS embolization.     

Intrahepatic Portosystemic Venous Shunt: Diagnosis by Color Doppler Imaging

Intrahepatic portosystemic venous shunt is a rare clinical entity; only 33 such cases have been reported. It may be congenital, or secondary to portal hypertension. Five patients with this disorder are presented, each of whom was diagnosed by color Doppler imaging, including waveform spectral analysis. One patient with clinical evidence of cirrhosis and portal hypertension had episodes of hepatic encephalopathy and elevated blood levels of ammonia. This patient had a large tubular shunt between the posterior branch of the portal vein and the inferior vena cava. Shunts of this type are considered to be collateral pathways which develop in the hepatic parenchyma as a result of portal hypertension. The other four patients had no evidence of liver disease, and all four evidenced an ancurysmal portohepatic venous shunt within the liver parenchyma. Shunts of this type are considered congenital. The diagnosis of intrahepatic portosystemic venous shunts was established by color Doppler imaging, which demonstrated a direct communication of color flow signals between the portal vein and hepatic vein, in addition to the characterization of the Doppler spectrum at each sampling point from a continuous waveform signal (portal vein) to a turbulent signal (aneurysmal cavity), and finally, to a biphasic waveform signal (hepatic vein). As demonstrated by the five patients, color Doppler imaging is useful in the diagnosis of an intrahepatic portosystemic hepatic venous shunt, and the measurement of shunt ratio may be useful in the follow-up and determining the therapeutic option.     

经皮经肝曲张血管栓塞术治疗门体分流性脑病疗效分析

目的探讨经皮经肝TH胶栓塞治疗分流性脑病的临床疗效。方法经皮经肝门静脉穿刺插管,治疗12例反复发作的肝性脑病患者,将TH胶注射到门、体分流道内,使其永久闭塞。术后随访观察疗效。结果成功率100%（12/12）。术后门体压力梯度由（21.9±1.8）mm Hg升至（26.3±3.2）mm Hg,差异有统计学意义（P=0.00）。发热和腹痛是常见的并发症,发生率分别为50.0%（6/12）和41.7%（5/12）;16.7%（2/12）患者出现可控制的腹水,未出现肝脏穿刺道出血、异位栓塞等严重并发症,无围手术期死亡病例。患者随访6～37个月,平均随访时间（22.7±9.8）个月。所有患者肝性脑病症状都明显好转。在随访期间有25.0%（3/12）患者肝性脑病复发,16.7%（2/12）患者出现上消化道出血,原因分别为食管静脉曲张破裂出血和门静脉高压性胃病出血,死亡2人（16.7%）,死亡原因分别为肝功能衰竭和肝癌。结论经皮经肝曲张血管栓塞术在复发性分流性脑病的治疗中显示出了可靠的疗效和良好的安全性,值得推广研究。     

Spontaneous porto-systemic shunts in liver cirrhosis: Clinical and therapeutical aspects

Spontaneous porto-systemic shunts(SPSS) are frequent in liver cirrhosis and their prevalence increases as liver function deteriorates, probably as a consequence of worsening portal hypertension, but without achieving an effective protection against cirrhosis' complications. Several types of SPSS have been described in the literature, each one associated with different clinical manifestations. In particular,recurrent or persistent hepatic encephalopathy is more frequent in patients with splenorenal shunt, while the presence of gastric varices and consequently the incidence of variceal bleeding is more common in gastrorenal shunt. In the advanced stage, the presence of large SPSS can lead to the so called"portosystemic shunt syndrome", characterized by a progressive deterioration of hepatic function, hepatic encephalopathy and, sometimes, portal vein thrombosis. The detection of SPSS in patients with liver cirrhosis is recommended in order to prevent or treat recurrent hepatic encephalopathy or variceal bleeding.     

Transjugular Intrahepatic Portacaval Stent Shunt as a Rescue Treatment for Life-Threatening Variceal Bleeding in a Cirrhotic Patient with Severe Liver Failure

Variceal bleeding in cirrhotic patients with severe liver failure that is not controllable by endoscopic sclerotherapy is a life-threatening situation. We report the case of a patient with decompensated cirrhosis (Pugh class C) who bled repeatedly from gastric varices despite multiple sessions of sclerotherapy. The portal vein was catheterized by a transjugular approach. A tract between a hepatic vein and the portal vein was created after balloon dilatation, and this opening was stented with an expandable stainless steel Palmaz stent. The portal vein pressure decreased from 35 mm Hg to 19 mm Hg after shunting. Gastric varices also were embolized. Two months later, bleeding had not recurred; the shunt remained opened and the marked decrease in portal pressure still persisted. Endoscopy showed the disappearance of gastric varices. This procedures could become a life-saving therapeutic option for such critically ill cirrhotic patients.     

Treatment of refractory hepatic encephalopathy induced by spontaneous portosystemic shunt: Selective splenic vein embolization versus shunt embolization

Background and aimsSpontaneous portosystemic shunt (SPSS) can cause refractory hepatic encephalopathy (HE) in cirrhotic patients. The embolization of the shunt (ES) can resolve the HE, while the selective embolization of the splenic vein (SESV) can treat splenorenal shunts related HE. The aim of this study was to compare the clinical outcomes of ES and SESV when applied for the treatment of SPSS-induced refractory HE in cirrhotic patients.MethodsPatients with refractory HE who were treated with ES or SESV were retrospectively identified. The clinical outcomes were compared and analyzed.ResultsThe 6-month mortality after the ES procedure was significantly higher than that after the SESV procedure. During the 6-month follow-up, both the white blood cell and the platelet counts were significantly lower after the ES procedure than after the SESV procedure. There was a significant increase in aspartate aminotransferase levels after ES. However, the albumin levels as well as the Child–Pugh score and grade were found to be significantly improved at 6 months after the undertaking of an SESV (as compared with baseline).ConclusionThe 6-month mortality was improved after SESV (as compared with ES) in the treatment of SPSS-induced refractory HE. A prospective multicenter study for validation is warranted.     

Atypical liver hemangioma with shunt: long-term follow-up

With a cavernous hemangioma the vascular space does not form an anastomosis, and the blood flow is slow. In contrast, several atypical hemangiomas, including those with shunt formation, have been recently recognized. We report here two extreme cases of atypical hemangioma with severe clinical symptoms due to shunts. In the first case, hemangiomatosis of the entire liver with arteriovenous shunt was treated by embolization of the hepatic artery with as many as 82 steel coils to control high‐output heart failure. Although the effect of the treatment was late, the patient is free from heart failure despite a remnant arteriovenous shunt 12 years after treatment. In the second case, an atypical hemangioma in the posterior segment with arterioportal and portosystemic shunt was treated by ligation of the right hepatic artery and the portal vein branch. The patient died of massive bleeding from a deep duodenal ulcer that penetrated the hepatic artery 6 years after treatment. The second case should have been curatively treated only by liver transplantation. It should be noted that there are atypical hemangiomas with shunt that are difficult to treat and cause fatal clinical symptoms and complications.     

Arterio-portal shunt in liver rescued by hepatectomy after arterial embolization

Arterio-portal shunts are generally treated with transcatheter arterial embolization, as a therapeutic measure for bleeding of esophageal varices. However, transcatheter arterial embolization is frequently associated with reestablishment of arterio-portal shunts. We now report our experience with partial hepatectomy to remove the arterio-portal shunt associated with esophageal varices, which recurred after transcatheter arterial embolization. The patient was a 60-year-old female, who had massive hematemesis caused by rupture of esophageal varices. Doppler sonography and arteriography demonstrated an arterio-portal shunt in the right anterior superior segment of the liver. Temporary hemostasis was achieved with transcatheter arterial embolization, however, hemorrhage recurred one month later. The second transcatheter arterial embolization failed to manage the shunt and varices. The patient developed hepatic coma. After recovery from coma, she was referred to our hospital. We carried out partial hepatectomy, which provided remarkable hemodynamic improvement; the portal vein flow changed from hepatofugal to hepatopetal. Esophageal varices and hepatic coma have totally disappeared. This patient has had no complaint and has remained free of esophageal varices, for 3 years postoperatively. She is having a normal life. The partial hepatectomy to remove the arterio-portal shunt induced complete resolution of the arterio-portal shunt, as well as dramatic improvement in portal flow and hepatic coma. Our experience in the present case suggests that partial hepatectomy should be considered as a radical therapy for arterio-portal shunt, without insistence on transcatheter arterial embolization.     

Is there an Alternative to TIPS? Ultrasound-guided Direct Intrahepatic Portosystemic Shunt Placement in Budd-Chiari Syndrome

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.     

Treatment of post-shunt portal systemic encephalopathy by embolization of the shunt

Operative ligation of portosystemic shunts is effective in controlling chronic portosystemic encephalopathy (CPSE) but is associated with significant mortality. Review of the records of five patients with CPSE treated with radiologic occlusion procedures showed that these are suitable alternatives to surgery. Three patients had alcoholic cirrhosis, one had hepatic fibrosis from schistosomiasis, and one had post-necrotic cirrhosis. All had CPSE with progressive, severe cerebral impairment refractory to clinical treatment. Four patients had a spontaneous splenorenal shunt, and one had a surgically created mesocaval shunt (MCS). Partial splenic embolization was performed in two patients, direct shunt embolization was performed via percutaneous transhepatic portography in two other patients, and an MCS embolization was performed in one patient through the inferior vena cava. In four patients embolization controlled the CPSE. In the remaining patient it could not be evaluated because of his premature death from intraabdominal bleeding, a late complication of the procedure. Interventional radiologic procedures are effective in the control of CPSE in selected patients.     

Treatment of Budd-Chiari syndrome by transjugular intrahepatic portosystemic shunt

Patients with Budd-Chiari syndrome may require therapy with portal decompressive surgery. Transjugular intrahepatic portosystemic shunt has recently emerged as a new treatment alternative to surgical shunting, but reported literature is scarce and its long-term outcome is to be evaluated. The aim of our study was to present two patients with Budd-Chiari syndrome treated with transjugular intrahepatic portosystemic shunt. Two female patients with acute and subacute Budd-Chiari syndrome respectively, uncontrolled by medical therapy, were referred for transjugular intrahepatic portosystemic shunting. Both patients had refractory ascites, while one had hepatic insufficiency. Hepatic vein thrombosis was without definitive etiology in one patient and due to essential thrombocytosis in the other. Transjugular intrahepatic portosystemic shunt was successfully created in both patients; in one a transcaval approach was performed. Bare stents were used in one, while in the other a polytetrafluoroethylene-covered stent-graft. Technical success was 100% with no complications. Ascites resolved in both patients and liver function improved. The first patient remains asymptomatic with good shunt patency 6 months after intervention. The second patient is symptom-free two years after the shunt creation, despite stent occlusion. She remains asymptomatic due to hepatic vein collateral development. In patients with acute or subacute Budd-Chiari syndrome uncontrolled by medical therapy, transjugular intrahepatic portosystemic shunt is highly effective with no complications and an associated mid-term clinical success.     

Bleeding portal-hypertensive gastropathy managed successfully by partial splenic embolization

The use of partial splenic embolization to decrease portal pressure and reduce gastric bleeding from portal-hypertensive gastropathy, a complication of liver cirrhosis, is described. A 62-year-old man with hepatic cirrhosis secondary to hepatitis C and documented portal hypertension was admitted with hypersplenism and bleeding esophageal varices. Endoscopic ligation successfully controlled acute bleeding, but blood loss continued over the next 45 days. Bleeding secondary to portal-hypertensive gastropathy was diagnosed endoscopically. The patient's poor surgical status precluded a portosystemic shunt procedure, so partial splenic embolization was performed radiologically by the injection of Gelfoam squares. Splenic volume decreased 50% following partial embolization. Over 3 weeks, the hemoglobin concentration increased from 8.5 g/dL to 9.8 g/dL, and the platelet count increased from 41,000 to 90,000/microL. Repeat endoscopy found no gastric bleeding 18 days post-procedure. Partial splenic embolization is a radiologic procedure which can be performed safely in patients too ill to undergo portosystemic shunt. This report documents its successful use to manage hypersplenism and reduce portal pressure in a cirrhotic patient with portal-hypertensive gastropathy and hypersplenism.     

Acute Budd-Chiari syndrome with liver failure: the experience of a policy of initial interventional radiological treatment using transjugular intrahepatic portosystemic shunt

BACKGROUND AND AIMS: The optimal management of acute Budd-Chiari syndrome (BCS) with liver failure is controversial. Options include anticoagulation, portal systemic shunting or liver transplantation. In recent years, transjugular intrahepatic portosystemic shunts (TIPS) have been tried in place of shunt surgery. We report our experience with a policy of initial interventional radiological treatment (TIPS) in this patient group. METHODS: A consecutive, non-selected series of five patients with acute BCS with liver failure presenting to our liver unit over a 36-month period from October 2000 were treated with a policy of initial attempt at transjugular shunt. RESULTS: Stents were successfully inserted in four patients. In one patient the hepatic vein remnant could not be cannulated. One of the four stented patients had a hepatic venous web. Rapid recurrent portal and hepatic vein thrombosis occurred in one patient despite anticoagulation and trans-shunt thrombectomy. This patient and the patient with failed stenting underwent successful liver transplantation. One successfully stented patient died unexpectedly at home 2 weeks after TIPS insertion. Four of five patients are alive (80%) at a mean follow-up time of 30 months. CONCLUSION: Initial TIPS, with liver transplantation for treatment failures, may be a reasonable, cost-effective and surgery-sparing treatment for acute Budd-Chiari presenting with liver failure.     

Symptomatic hepatic arterioportal shunt resolved through conservative treatment

Arterioportal shunt in the liver is a rare vascular disorder that may be due to congenital vascular malformation (hereditary hemorrhagic telangiectasia), trauma, iatrogenic causes (after a hepatic biopsy) or neoplasm. Initial treatment consists of transcatheter arterial embolization with different kinds of materials. We present the case of a 64-year-old woman with signs of portal hypertension and severe diarrhea. Doppler ultrasonography, computed tomography and angiography revealed arterioportal fistulae between the hepatic artery and right portal vein. Transcatheter arterial embolization with n-butyl-2-cyanoacrylate surgical glue (Glubran) was successfully performed. After 2 years of follow-up, the patient remains asymptomatic. Transcatheter arterial embolization with Glubran should be considered as a therapeutic option in arterioportal shunts and could be a definitive therapy.     

Splenic vein embolization as a feasible treatment for patients with hepatic encephalopathy related to large spontaneous splenorenal shunts

Introduction and objectives: Although splenic vein embolization (SVE) has been performed for the management of patients with hepatic encephalopathy (HE) related to large spontaneous splenorenal shunts (SSRS) in recent years, its role remains poorly defined. In this study, we aimed to explore the safety and efficacy of SVE for HE patients with large SSRS.Materials and methods: Data from cirrhotic patients who were confirmed to have recurrent or persistent HE related to large SSRS and underwent SVE from January 2017 to April 2021 were retrospectively collected and analyzed at our center. The primary endpoints were the change of HE severity at 1 week after embolization and the recurrence of HE during the follow-up period. The secondary endpoints were procedure-related complications and changes in laboratory indicators and hepatic function (Child-Pugh score/grade and model for end-stage liver disease score).Results: Of the eight cirrhotic patients included in the study, six were diagnosed with recurrent HE, and the others were diagnosed with persistent HE. Embolization success was achieved for all patients (100%), and no immediate procedure-related complications, de novo occurrence, or aggravation of symptoms related to portal hypertension were observed during the long-term follow-up. HE status was assessed at 1 week after embolization. The results demonstrated that the symptoms were mitigated in three patients and resolved completely in five patients. During the follow-up period, all patients were free of HE within 1 month after embolization, but one patient experienced the recurrence of HE within 6 months and another one experienced the recurrence of HE within 1 year. Compared with the preoperative parameters, the Child-Pugh score and grade were significantly improved at 1 week and 1 month after embolization (all P<0.05), and the serum ammonia level was significantly lower at 1 month after embolization (P<0.05).Conclusions: SVE could be considered as a feasible treatment for patients with HE related to large SSRS, but further validation is required.     

The Portosystemic Shunt Syndrome and Role of Shunt Embolization in the Management of Hepatic Encephalopathy

Purpose of Review

Portosystemic shunting (PSS) is a result of changes in hepatic hemodynamics where portal flow diverts away from the liver due to increased intrahepatic resistance from cirrhosis and is associated with hepatic encephalopathy (HE). Over time, increased PSS may directly lead to worsening liver failure because of severely decreased loss of effective portal inflow towards the liver and result in recurrent or persistent HE. This clinical scenario has been recently defined as “portosystemic shunt syndrome” and has been associated with poor clinical outcomes.

Recent Findings

The presence of PSS is common in patients with cirrhosis and increased PSS size appears to correlate with recurrent or persistent HE. Recent studies have shown that patients with Model for End-Stage Liver Disease (MELD) score <?11 have demonstrated high clinical benefit in recurrent or persistent HE when treated with shunt embolization.

Summary

There is a growing literature that demonstrates potential clinical benefit using PSS embolization for selected patients, particularly with MELD under 11, with recurrent or persistent HE. Further investigation into improved risk stratification in order to determine effective treatments is necessary as well as heightened detection of the portosystemic shunt syndrome in its earlier and possibly reversible stages.

     

Effective balloon-occluded retrograde transvenous obliteration of the superior mesenteric vein–inferior vena cava shunt in a patient with hepatic encephalopathy after living donor liver transplantation

Balloon-occluded retrograde transvenous obliteration (BRTO) has become a common and effective procedure for treating hepatic encephalopathy due to a portosystemic shunt related to cirrhosis of the liver. However, this method of treatment has rarely been reported in patients after liver transplantation. Here, we report the case of a 52-year-old patient who underwent living donor liver transplantation (LDLT) due to hepatitis C virus-infected hepatocellular carcinoma that was complicated with portal vein thrombosis and a large portosystemic shunt between the superior mesenteric vein (SMV) and inferior vena cava (IVC). The SMV–IVC shunt was not obliterated during LDLT because there was sufficient portal flow into the graft after reperfusion. However, the patient was postoperatively complicated with encephalopathy due to the portosystemic shunt. BRTO was performed and was demonstrated to have effectively managed the encephalopathy due to the SMV–IVC shunt, while preserving the hepatic function after LDLT.