Direct Intrahepatic Portosystemic Shunt Research Articles

Successful direct intrahepatic portosystemic shunt (DIPS) creation following transmesenteric porta hepatis access in a young patient with recurrent variceal bleeding

BackgroundTransmesenteric access for portal vein reconstruction and transjugular intrahepatic portosystemic shunt allows for intervention in patients with unfavorable anatomy and can be performed via multiple methods but may be difficult to obtain in patients with complex anatomy.Case presentationWe present a case report describing a method of obtaining transmesenteric access in the porta hepatis to facilitate direct intrahepatic portosystemic shunt creation in a young patient with recurrent variceal bleeding. This patient anatomy was unfavorable, and initially he was thought to be a poor candidate for any intervention, but this technique allowed for successful decompression of the varices safely and effectively.ConclusionsThis is a technique to consider in similar complex cases and expands treatment for those who previously would not have been considered for intrahepatic shunt formation.Graphical

Comparison of Balloon-Occluded Thrombolysis with Catheter-Directed Thrombolysis in Patients of Budd-Chiari Syndrome with Occluded Direct Intrahepatic Portosystemic Shunt.

Objectives Direct intrahepatic portosystemic shunt (DIPS) stent placement is an effective treatment for patients with Budd-Chiari syndrome (BCS); however, thrombotic occlusion of DIPS stent remains a cause of concern. The purpose of this study is to describe a novel technique of balloon-occluded-thrombolysis (BOT) for occluded DIPS stent, and compare it with the conventional catheter-directed-thrombolysis (CDT). Methods In this retrospective study, the hospital database was searched for BCS patients who underwent DIPS revision for thrombotic stent occlusion between January 2015 and February 2021. Patients were divided into CDT group and BOT group. The groups were compared for technical success, total dose of thrombolytic agent administered, duration of hospital stay, and primary assisted stent patency rates at 1- and 6-month follow-up. Results CDT was performed in 12 patients, whereas 21 patients underwent BOT. Complete recanalization was achieved in 66.7% (8 of 12) patients of CDT group as compared to 81% (17 of 21) patients of BOT group (nonsignificant difference, p = 0.420). BOT group had a short hospital stay (1.8 ± 0.7 vs. 3.5 ± 1.0 days) and required less dose of thrombolytic agent ([2.2 ± 0.4]x10 5 IU versus [8.3 ± 2.9]x10 5 IU of urokinase) as compared to the CDT group and both differences were statistically significant ( p < 0.001). Further, 6-month patency rate was higher in BOT group as compared to CDT group ( p = 0.024). Conclusion The novel BOT technique of DIPS revision allows longer contact time of thrombolytic agent with the thrombi within the occluded stent. This helps in achieving fast recanalization of thrombosed DIPS stent with a significantly less dose of thrombolytic agent required, thus reducing the risk of systemic complications associated with thrombolytic administration.

Endovascular Alternatives to Transhepatic Shunt Creation.

Portomesenteric decompression is often necessary to treat patients with refractory symptoms of portal hypertension. When transjugular or direct intrahepatic portosystemic shunt creation is not feasible or is inadequate, surgical portosystemic shunt creation is considered, which carries significant morbidity and mortality in these high-risk patients. Surgery is further complicated in patients with portomesenteric thrombosis who require concurrent thrombectomy and long-term anticoagulation. In this article, we outline the technique for performing advanced endovascular alternatives to intrahepatic portosystemic shunt creation including mesocaval and splenorenal shunting. We will also discuss some of the clinical considerations for treating these patients with symptomatic portal hypertension and portomesenteric thrombosis.

Accessory Hepatic Vein Stenting in the Management of Budd-Chiari Syndrome: A Case Report

The Budd-Chiari Syndrome (BCS) is associated with hepatic venous outflow obstruction. A 22-year-old female patient presented with abdominal pain and backache for 12 days, along with generalised weakness. Physical examination revealed pitting pedal oedema and abdominal distension. An abdominal ultrasound with Doppler Ultrasonography (USG) was performed, revealing liver parenchymal disease with gross ascites and occlusion of all three Hepatic Veins (HV), suggestive of BCS. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) of the liver, as well as a right hepatic venogram using Digital Subtraction Angiography (DSA), showed chronic total occlusion at the ostium. An Accessory Hepatic Vein (AHV) was identified, joining the Right Hepatic Vein (RHV) to the Inferior Vena Cava (IVC). The accessory RHV exhibited high-grade stenosis (approximately 90-95%) at its junction with the IVC. Recanalisation of the AHV (balloon dilatation/stent insertion) was performed. Post-procedure accessory hepatic venogram showed a patent stented segment with a significant reduction in adjacent venous collaterals. No procedure-related complications were observed. The present case highlights the importance of AHV stenting in BCS patients, as it helps maintain normal physiology, in contrast to Direct Intrahepatic Porto-systemic Shunt (DIPS), which alters normal physiology by allowing portal venous blood to mix directly into the systemic circulation, bypassing the liver parenchyma.

Evaluation of outcome from endovascular therapy for Budd-Chiari syndrome: a systematic review and meta-analysis

This study was performed to evaluate the outcome of endovascular intervention therapy for Budd-Chiari syndrome (BCS) and compare recanalization, transjugular intrahepatic portosystemic shunt (TIPS)/direct intrahepatic portosystemic shunt (DIPS), and combined procedure treatment. For the meta-analysis, 71 studies were identified by searching four databases. The individual studies’ samples were used to calculate a confidence interval (CI 95%), and data were pooled using a fixed-effect model and random effect model. The pooled measure and an equal-weighted average rate were calculated in all participant studies. Heterogeneity between the studies was assessed with I2, and T2 tests, and publication bias was estimated using Egger’s regression test. A total of 4,407 BCS patients had undergone an endovascular intervention procedure. The pooled results were 98.9% (95% CI 97.8‒98.9%) for a technical success operation, and 96.9% (95% CI 94.9‒98.9%) for a clinical success operation. The re-intervention rate after the initial intervention procedure was 18.9% (95% CI 14.7‒22.9%), and the survival rates at 1 and 5 years after the initial intervention procedure were 98.9% (95% CI 96.8‒98.9%) and 94.9% (95% CI 92.9‒96.9%), respectively. Patients receiving recanalization treatment (98%) had a better prognosis than those with a combined procedure (95.6%) and TIPS/DIPS treatment (94.5%). The systematic review and meta-analysis further solidify the role of endovascular intervention treatment in BCS as safe and effective. It maintains high technical and clinical success and long-term survival rates. The recanalization treatment had a better prognosis and outcome than the combined procedures and TIPS/DIPS treatment.

Budd chiari syndrome: A case report on a rare disorder

Budd Chiari syndrome (BCS) is a rare disease that threatens life due to a hepatic vein blood flow obstruction. As per the literature, thrombosis is a significant factor for hepatic venous obstruction. It can treat BCS based on the severity of the patient’s condition by three methods like hepatic vein angioplasty, interventional radiology stenting with direct intrahepatic portosystemic shunt, or liver transplantation. We describe the case of a 34-years-old female patient who presented with abdominal distension and generalized weakness with a history of BCS. A cooperative collaboration of hepatologists and interventional radiologists helps in effectively treating the syndrome.

S2198 Are Migrated Variceal Coils Amenable to Endoscopic Treatment?

Introduction: Embolization coil migration into the gastrointestinal tract is an extremely rare complication. Several case reports have described coil migration into the gastrointestinal tract after treatment of gastric varices, splenic artery pseudoaneurysm, and post-liver transplant. There is no standard of care for migrated coils, and treatment has ranged from conservative management to surgical/endoscopic removal. We report a rare case of coil migration into the stomach after direct intrahepatic portosystemic shunt (DIPS) and variceal coiling of gastric varices. Case Description/Methods: A 61-year-old man admitted with 3-week onset of epigastric pain. He has history of NASH cirrhosis complicated by bleeding esophageal and gastric varices status post coiling and embolization with DIPS in 2016. Pain was exacerbated by certain positions, and improved in the left lateral decubitus position. Pain was not related to food intake. Vitals were stable and labs were unremarkable at presentation.CT scan of abdomen and pelvis with IV contrast showed possible foreign body in the gastric lumen (Figure 1A). EGD showed migration of previous variceal coils into the gastric lumen, particularly around in the fundus and cardia (Figures 1B-C). Venogram was obtained to asses anatomy and coil location. This demonstrated complete occlusion of the previously embolized gastric varices. After the venogram, EGD was completed with successful removal of visibly migrated coils using small biopsy forceps (Figure 1D). No bleeding or complications occurred after the procedure. Although subsequent imaging showed remaining coils, there were significantly less compared to admission. The patient had intermittent resolution of his abdominal pain, but was readmitted several times afterwards for EGD removal of remaining coils. Discussion: Although etiology is unclear, the mechanism of variceal coil migration is likely related to fistula formation and inflammation within the gastric wall. In this case we attempted treatment endoscopically with removal of variceal coils with biopsy forceps. Successful removal of visualized coils alleviated abdominal pain, though it was temporary. An alternative treatment would be surgical intervention, however given risk of decompensation of cirrhosis and complications, it would be as a last resort. In the setting of successfully embolized vessels, we believe it is safe for endoscopic retrieval of the gastric coils in symptomatic patients.Figure 1.: (A) CT abdomen and pelvis showing metallic foreign body (red arrow) - coil migration from previous embolization. (B-D) Endoscopic images showing coils (yellow arrow) from previous IR intervention found in stomach fundus and cardia status post removal using small biopsy forceps.

Transjugular intrahepatic portosystemic shunt creation may be associated with hyperplastic hepatic nodular lesions in the long term: an analysis of 18 pediatric and young adult patients.

Retrospective studies have demonstrated the efficacy and safety of pediatric and adolescent transjugular intrahepatic portosystemic shunt (TIPS), but long-term outcomes warrant further investigation. To report on the development of hyperplastic hepatic nodular lesion development in children and young adults (<21 years) with TIPS patency >3years. Eighteen children and young adults, including 10 (55.6%) females and 8 (44.4%) males, underwent TIPS creation with >3years' patency and follow-up evaluation at a tertiary children's hospital. The mean age at the time of TIPS creation was 12.5±5.1 years (range: 1.5-20.0years). The mean model for end-stage liver disease (MELD) at the time of TIPS creation was 8.1±1.6 (range: 6-11). Indications for TIPS creation included acute variceal bleeding (8/18, 44.4%), primary (1/18, 5.6%) or secondary (7/18, 38.9%) prevention of varices, portal vein thrombosis (1/18, 5.6%), and splenic sequestration (1/18, 5.6%). Technical successes, intra-procedural parameters, hemodynamic and clinical successes, TIPS patencies, adverse events, imaging evaluations, and follow-ups were recorded. All (100%) TIPS placements were successful; however, a direct intrahepatic portosystemic shunt was created in one (5.6%) patient. Mean reduction of the portosystemic shunt gradient was 9.1±3.3mmHg (range: 4-16 mmHg). Seventeen (94.4%) patients demonstrated clinical success with resolution of their initial clinical indication for TIPS placement. The 3-year TIPS primary, primary-assisted, and secondary patencies were 83.3% (15/18), 94.4% (17/18), and 100% (18/18), respectively. Two (11.1%) patients developed mild, medically controlled hepatic encephalopathy. One (5.6%) patient developed hepatopulmonary syndrome. Nine (50%) patients developed single or multiple hepatic nodules at a mean imaging surveillance time after TIPS of 4.4±3.0years (range: 1.5-10.2years). Six (33.3%) patients developed nodules >1 cm with imaging features most consistent with focal nodular hyperplasia or focal nodular hyperplasia-like nodules. The mean follow-up duration was 5.7±2.9years (range: 3.0-13.1 years). Long-term (>3years) portosystemic shunting via TIPS is associated with the development of hepatic nodular lesions in children. Consequently, children with TIPS may need gray-scale assessment of hepatic parenchyma as part of routine ultrasound exams and extended imaging surveillance until more is understood regarding the natural history of induced nodularity.

Transjugular Intrahepatic Portosystemic Shunt Creation Using a Radiofrequency Wire: Prospective Clinical Safety and Feasibility Trial in Cirrhosis

PurposeTo assess the safety and feasibility of using a radiofrequency (RF) wire for portosystemic shunt creation. Materials and MethodsTen patients undergoing elective creation of a transjugular intrahepatic portosystemic shunt (TIPS) or a direct intrahepatic portosystemic shunt (DIPS) were prospectively enrolled. Primary outcomes were the safety and feasibility of RF wire used for the creation of TIPS and DIPS. Median age was 66.5 ± 6.1 years. Causes of liver disease included alcohol (n = 5), nonalcoholic steatohepatitis (n = 2), hepatitis C virus (n = 1), primary biliary cirrhosis (n = 1), autoimmune hepatitis (n = 1). The median score for model for end-stage liver disease was 11 ± 4.3. The Rosch-Uchida TIPS set was used with intravascular ultrasonography guidance in all cases. A 0.035-inch RF wire was used in lieu of the trocar needle through the 5-F TIPS set catheter to create a track between the hepatic vein and the portal vein. All shunts were created using stent grafts. ResultsTechnical success rate was 100%. In 7 of 10 patients, portal vein access was achieved with a single pass. A DIPS was created in 2 patients based on anatomic favorability. Median fluoroscopy time was 13.3 ± 3.8 min, and median total procedure time was 102 ± 19 min. The wire passed through parenchyma without subjective deflection. There was 1 case of extracapsular puncture with no clinical consequence. The RF wire was too stiff to curve into the main portal vein, requiring wire exchange in all but 1 case. Mean portosystemic gradient decreased from 13.9 ± 3.3 to 5.9 ± 2.1 mm Hg. No immediate complications were encountered. Shunt patency was 100% at 30 days. ConclusionsCreation of TIPS and DIPS using an RF wire was safe and feasible, enabling creation of an intrahepatic track without subjective deflection in cirrhotic patients.

Transsplenic portal vein recanalization and direct intrahepatic portosystemic shunt placement to optimize liver transplantation

BackgroundPercutaneous trans-splenic portal vein recanalization (PVR) has been reported for facilitation of transjugular intrahepatic portosystemic shunts (TIPS), however has not been applied to patients undergoing direct intrahepatic portosystemic shunt (DIPS). We report the utilization of trans-splenic-PVR with DIPS creation in a patient with chronic portal and hepatic vein occlusions undergoing liver transplantation evaluation.Case presentationA 48-year-old male with decompensated alcoholic cirrhosis complicated by refractory ascites, hepatic encephalopathy, and variceal bleeding underwent CT that demonstrated chronic occlusion of the hepatic veins (HV), extrahepatic portal vein (PV), and superior mesenteric vein (SMV). Due to failed attempts at TIPS at outside institutions, interventional radiology was consulted for portal vein recanalization (PVR) with TIPS to treat the portal hypertension and ascites and also facilitate an end-to-end PV anastomosis at transplantation. After an initial hepatic venogram confirmed chronic HV occlusion, a DIPS with trans-splenic PVR was planned. The splenic vein was accessed under sonographic guidance using a micropuncture set and subsequently upsized to a 6 French sheath over a stiff guidewire. A splenic venogram via this access confirmed occlusion of the PV with drainage of the splenic vein (SV) through gastric varices. The thrombosed PV was then recanalized and angioplastied to restore PV flow via the transsplenic approach. A transjugular liver access kit with a modified 21-gauge needle was advanced into the IVC through the internal jugular vein (IJV) sheath and directed towards the target snare in PV. The needle was used to subsequently puncture the PV through the caudate lobe and facilitate placement of a wire into the SV. The initial portosystemic gradient (PSG) was 20 mmHg. The IJV sheath was advanced through the hepatic parenchymal tract into the main-PV and a stent-graft was placed across the main PV and into the IVC. A portal venogram demonstrated brisk blood flow through the DIPS, resolution of varices and a PSG of 8 mmHg. One month after the procedure, the patient had a significant reduction in ascites and MELD-NA score. Patient is currently listed and awaiting transplantation.ConclusionsIn the setting of chronically occluded portal and hepatic veins, trans-splenic PVR DIPS may serve as an effective bridge to liver transplantation by facilitating an end to end portal vein anastomosis.

A Case Report of Intravascular Hemolysis and Heme Pigment-Induced Nephropathy Following AngioJet Thrombectomy for Thrombosed DIPS Shunt.

Rationale:The AngioJet system is a combined mechanical and pharmacological device used for thrombectomy. As a result of the mechanical disruption of clot, intravascular hemolysis is noted to occur. Rarely, intravascular hemolysis can be severe enough to cause heme pigment–induced acute kidney injury (AKI).Presenting concerns of the patient:We describe a case of a 45-year-old man with Child-Pugh class B cirrhosis, Budd-Chiari syndrome, and antiphospholipid antibody syndrome who required thrombectomy following a thrombosed direct intrahepatic portosystemic shunt (DIPS). He developed evidence of worsening anemia, dark urine, direct antiglobulin test–negative intravascular hemolysis, and severe AKI within 24 hours of the procedure.Diagnosis:Based on his severe AKI in association with elevated hemolytic markers, and the temporal association with the AngioJet procedure, the patient was diagnosed with heme pigment–induced AKI secondary to intravascular hemolysis.Interventions:The patient remained anuric and became volume-overloaded after fluid resuscitation. He was started on dialysis 72 hours after the procedure. Five days after thrombectomy, hemolytic markers returned to normal.Outcome:The patient received hemodialysis for approximately 3 weeks, followed by renal recovery and cessation of dialysis treatments. Five weeks after the AngioJet procedure, his renal function returned to normal.Teaching points:We present a case of heme pigment–induced AKI following an AngioJet procedure that required initiation of dialysis. Although this rare complication has been reported in the literature, it typically occurs when the procedure is used for larger clot burden (ie, venous thromboembolism). To our knowledge, this is the first case of severe hemolysis with associated AKI following the use of the AngioJet for a thrombosed DIPS. Due to the patient’s comorbid conditions, overlapping clinical features, and lack of appreciation of the hemolysis associated with the AngioJet system, the differential diagnosis of the patient’s AKI was quite broad. Nephrologists should be aware of this complication when managing patients with AKI to direct therapy early and avoid unnecessary diagnostic and therapeutic interventions.

1357 Pseudocirrhosis in Subacute/Chronic Budd-Chiari Syndrome With JAK 2 Mutation

INTRODUCTION: Budd Chiari Syndrome (BCS) is a rare disorder often characterized by hepatic venous outflow obstruction. Myeloproliferative disorders especially V617F-JAK2 mutations account for almost 40% of these patients. BCS may present differently depending on location, extent, severity of obstruction and existence of collaterals. The course of disease may be fulminant, acute, subacute or chronic, ranging from incidental findings to fatal acute hepatic failure. CASE DESCRIPTION/METHODS: A 74-year-old female with remote history of alcoholism, presented to our facility with bleeding per rectum for 2 days. Labs on admission revealed WBC 18.4, HGB 16.8, PLT 588, AST 570, ALT 804 and TB 4.6. Ultrasound Abdomen with Doppler revealed ascites and portal vein thrombosis. Follow-up MRI Abdomen confirmed thrombus in main portal vein and non-opacification of major intrahepatic veins suggesting Budd Chiari Syndrome. Hypercoagulability workup showed positive JAK2 mutation. She was started on IV Heparin immediately. Diagnostic paracentesis revealed transudative fluid and liver elastography confirmed cirrhosis. Grade 2 esophageal varices were seen on EGD with a negative colonoscopy. Other possible etiology of congestive hepatopathy/cirrhosis were excluded. Patient was discharged on daily oral Rivaroxaban with intermittent therapeutic paracentesis. Over next 3 months, she had recurrent admissions for decompensated liver disease. During her last visit however, we noted preserved synthetic liver function and inconsistencies in her presentation thus a liver biopsy was obtained which revealed centrilobular hepatocellular necrosis, sinusoidal dilatation and mild portal fibrosis consistent with hepatic venous outflow obstruction. However, there was no evidence of cirrhosis. She was transferred to an advanced facility for portal vein reconstruction to relieve the obstruction from splanchnic venous thrombosis and possibly reverse the “pseudocirrhosis”. DISCUSSION: Pseudocirrhosis is often associated with metastatic cancers in response to chemotherapy induced ischemic injury. Although it radiologically and clinically mimics liver cirrhosis, it can be prevented with early detection. To our knowledge, this is the first reported case of pseudocirrhosis in BCS. Subacute/chronic BCS may present with cirrhotic complications like portal hypertension, ascites and variceal bleeding. Apart from anticoagulation, these patients may require other interventions to bypass the obstruction like Direct Intrahepatic Portosystemic Shunt (DIPS) placement.

Transjugular Intrahepatic Portosystemic Shunt Through the Strut of a Previously Placed Stent: Technical Feasibility and Long-Term Follow-Up Results.

To evaluate technical feasibility, long-term primary patency and clinical outcome of the transjugular intrahepatic portosystemic shunt (TIPS) through the struts of the previously placed stents. Retrospective evaluation of seven consecutive patients (three male and four female, age range 13-65years, median 28) out of a total 95 patients, who underwent TIPS through the strut of the previously placed stents of hepatic vein (HV), inferior vena cava (IVC) or TIPS in a single tertiary care hospital. Six of the patients were diagnosed with Budd-Chiari syndrome(BCS) and one with alcohol-induced chronic liver disease(CLD). Kaplan-Meier test was used to calculate 18- and 60-month primary patency rate of TIPS stent. TIPS through the strut of a previously placed stent was technically successful in all the patients (100%). The TIPS was direct intrahepatic portosystemic shunt (DIPS) in 5/7 cases, due to occluded HV. Mean portosystemic pressure gradient (PPG) reduced from 24mmHg ± 5.9 (range, pre-TIPS 15-31mmHg) to 8.57mmHg ± 4.4 (range, post-TIPS, 3-14mmHg). One patient required three sessions of TIPS revisions. Another patient needed TIPS revision after 5years of TIPS creation. All the patients showed improvement in clinical symptoms and in mean Child-Turcotte-Pugh (CTP) score and modified end-stage liver disease (MELD) score during mean follow-up period 40.57month ± 34.9 (range 3-100month). Primary patency rates of TIPS stent measured with Kaplan-Meier estimate at 18- and 60-month follow-up were 80% (95% CI, 37-97%) and 40% (95% CI, 10-97%), respectively. TIPS through the strut of a previously placed stent is technically feasible with good long-term primary patency and clinical outcome.

Anatomic Recanalization of Hepatic Vein and Inferior Vena Cava versus Direct Intrahepatic Portosystemic Shunt Creation in Budd-Chiari Syndrome: Overall Outcome and Midterm Transplant-Free Survival

PurposeTo assess overall outcome and midterm transplant-free survival of patients with Budd-Chiari syndrome (BCS) undergoing radiologic interventions including anatomic recanalization of the hepatic vein (HV) and inferior vena cava (IVC) and direct intrahepatic portosystemic shunt (DIPS) creation, both as combined and as independent groups. Materials and MethodsFrom November 2010 to October 2014, 136 patients with BCS were treated with HV/IVC recanalization (group 1) or DIPS creation (group 2). Both groups were periodically analyzed for stent patency on Doppler ultrasound, clinical outcome, biochemical parameters, and survival until death, liver transplantation, or last clinical evaluation. ResultsActuarial transplant-free survival for the entire cohort was 94% at 1 year and 5 years with no significant difference in overall survival. There was significant biochemical improvement in group 1 with decrease in mean serum bilirubin level (1.8 mg/dL to 1.4 mg/dL, P < .011), mean serum aspartate aminotransferase (48.6 IU/L to 33.2 IU/L, P < .05), and mean serum alanine aminotransferase (38.7 IU/L to 28.5 IU/L) and increase in mean serum albumin level (3.2 g/dL to 3.45 g/dL, P < .001) after 3 and 24 months. There were 4 deaths in each group at 1-year follow-up; all 4 patients had acute fulminant BCS at presentation. ConclusionsRadiologic interventions for BCS lead to remarkable improvement of liver function and a good overall outcome and midterm transplant-free survival. Patients receiving anatomic recanalization show improved liver synthetic functions compared with patients treated with DIPS.

4:03 PM Abstract No. 289 Physiological recanalization of hepatic veins/inferior vena cava versus direct intrahepatic portosystemic shunt creation in Budd-Chiari syndrome: overall outcome and medium-term transplant-free survival

4:03 PM Abstract No. 289 Physiological recanalization of hepatic veins/inferior vena cava versus direct intrahepatic portosystemic shunt creation in Budd-Chiari syndrome: overall outcome and medium-term transplant-free survival

Ultrasound-guided direct intrahepatic portosystemic shunt in patients with Budd–Chiari syndrome: Short- and long-term results

Background and aims Budd–Chiari syndrome (BCS) is treated with anticoagulation therapy, transjugular intrahepatic portosystemic shunt (TIPS), angioplasty, and liver transplantation. TIPS is not always technically feasible, due to the complete hepatic venous thrombosis. Direct intrahepatic portosystemic shunt (DIPS) is an alternative method for decompression of portal hypertension. This is a retrospective, single-center study aiming to evaluate the outcome of ultrasound (US)-guided DIPS in patients with BCS. Materials and methods Between 2003 and 2016, six patients with BCS were treated with US-guided DIPS. Polytetrafluoroethylene (PTFE)-covered stents were used in two patients and bare-metal stents in four patients. Mean follow-up time was 71.4 months (range: 21–164). Technical/clinical success, technical difficulties, and complications of DIPS were analyzed. Results Technical success without complications was achieved in all patients (100%). In one patient, DIPS was performed through the right femoral vein, without significant amelioration of portal hypertension (clinical success 83.3%). In four out of five patients, ascites and variceal bleeding resolved completely and in the other one ascites was relieved. Six- and 12-month primary patency rates were 100% in PTFE-covered stent group when compared with bare-metal stent group, the rates were 33% and 0%, respectively. Conclusion US-guided DIPS is a safe and effective alternative technique for patients with BCS, with significant clinical improvement.

Ultrasound-guided direct intrahepatic portosystemic shunt in patients with Budd-Chiari syndrome: Short- and long-term results.

Background and aimsBudd–Chiari syndrome (BCS) is treated with anticoagulation therapy, transjugular intrahepatic portosystemic shunt (TIPS), angioplasty, and liver transplantation. TIPS is not always technically feasible, due to the complete hepatic venous thrombosis. Direct intrahepatic portosystemic shunt (DIPS) is an alternative method for decompression of portal hypertension. This is a retrospective, single-center study aiming to evaluate the outcome of ultrasound (US)-guided DIPS in patients with BCS.Materials and methodsBetween 2003 and 2016, six patients with BCS were treated with US-guided DIPS. Polytetrafluoroethylene (PTFE)-covered stents were used in two patients and bare-metal stents in four patients. Mean follow-up time was 71.4 months (range: 21–164). Technical/clinical success, technical difficulties, and complications of DIPS were analyzed.ResultsTechnical success without complications was achieved in all patients (100%). In one patient, DIPS was performed through the right femoral vein, without significant amelioration of portal hypertension (clinical success 83.3%). In four out of five patients, ascites and variceal bleeding resolved completely and in the other one ascites was relieved. Six- and 12-month primary patency rates were 100% in PTFE-covered stent group when compared with bare-metal stent group, the rates were 33% and 0%, respectively.ConclusionUS-guided DIPS is a safe and effective alternative technique for patients with BCS, with significant clinical improvement.

Budd-Chiari syndrome: outcomes of endovascular intervention-A single-center experience.

Outcomes of endovascular intervention in Budd-Chiari syndrome (BCS) have been reported with varied results. Clinical outcomes of endovascular interventions in BCS and role of various prognostic scores were critically evaluated in this study. This study retrospectively analyzed consecutive patients of BCS who underwent endovascular intervention between January 2007 and May 2016 at our center. Technical, clinical successes and complications were documented. The role of the prognostic scores such as Child-Turcotte-Pugh (CTP), model for end-stage liver disease (MELD), Rotterdam index, and original Clichy score in predicting mortality, clinical success, and need for re-interventions were also assessed. A total of 88 patients were analyzed. The median follow up was 12months (range 1-96months). Thirteen (14.8%) patients had combined inferior vena cava (IVC) and hepatic vein (HV) obstruction; HV obstruction in 33 (37.5%) and inferior vena cava IVC obstruction in 42 (47.7%) patients. The following interventions were done: IVC angioplasty alone (n=11), IVC angioplasty with stenting (n=36), HV angioplasty with stenting (n=26), combined HV and IVC stent (n=2), and direct intrahepatic porto-systemic shunt (DIPS) (n=13). Overall technical success was 87/88 (98.86%), and clinical success was 76/88 (86.36%). Immediate complications were noted in 8 patients (10%). The 1-, 2-, 3-, and 4-year stent patency rates were 90.91%, 81.08%, 74.59%, and 70.45%, respectively. Re-interventions were required in 15 (17%). Overall mortality was 6 (6.8%). Apart from MELD >14, none of the other prognostic score could predict mortality, clinical success, and need for re-interventions. Endovascular interventions play an important role in the management of BCS, in properly selected patients, even if prognostic score is unfavorable.

Multiple Choice Questions

1.Regarding drug induced liver injury (DILI) the following are TRUE EXCEPT:1.Most common cause of drug induced acute liver failure in India is paracetamol.2.Most common cause of drug induced liver injury in India is antitubercular drugs.3.Degree of liver enzyme elevation correlates with severity of liver disease.4.Clinical jaundice is a predictor of mortality.5.Cholestatic pattern of DILI can be prolonged even on stopping the offending drug.2.Regarding surgery in patients with liver disease the following are TRUE:1.Child-Turcotte-Pugh (CTP) and model for end-stage liver disease (MELD) scores can reliably predict operative mortality in chronic liver disease.2.Acute viral hepatitis is not a contraindication for surgery.3.Laparoscopic cholecystectomy can be safely performed in compensated cirrhotics.4.Umbilical hernias have low risk of complications in cirrhotics and elective repair should not be attempted.5.Preferred anaesthetic agents include sevoflurane, desflurane and isoflurane.3.Regarding peri-operative monitoring after liver transplantation the following are TRUE:1.Cardiovascular events are the most common cause of readmission to intensive care and non-graft related deaths after liver transplantation.2.Conventional tests of coagulation like prothrombin time, activated partial thromboplastin time, platelet count and plasma fibrinogen levels are sufficient measure of coagulation intra-operatively.3.Hyperglycemia is an ominous sign of poor graft function.4.Serum potassium levels are independent predictors of death after liver transplantation.5.Commonest acid-base disorder in the immediate post-operative period is metabolic acidosis.4.Regarding tuberculosis (TB) in chronic liver disease the following are TRUE EXCEPT:1.Cirrhotic patients have a higher chance of developing extra-pulmonary tuberculosis than those without cirrhosis.2.Adenosine deaminase for diagnosis of TB in cirrhosis with ascites has high sensitivity.3.Tuberculin skin testing is sensitive for the diagnosis of TB in cirrhotic patients.4.Likelihood of anti-tubercular drug induced hepatotoxicity is higher in pre-existing liver disease patients.5.Isoniazid induced hepatotoxicity is more common in slow acetylators.5.Regarding hepatotoxicity of anti-tubercular therapy (ATT) the following are TRUE:1.Rechallenge with isoniazid is contraindicated if patient has history of isoniazid induced hepatotoxicity.2.Isoniazid induced transaminitis is self limited and asymptomatic in majority of patients.3.Isolated conjugated hyperbilirubinemia is seen with rifampicin.4.Pyrazinamide mainly causes idiosyncratic hepatotoxicity.5.Inactive carriers of hepatitis B do not have increased risk of ATT induced hepatotoxicity.6.Regarding Budd-Chiari syndrome (BCS) the following are TRUE EXCEPT:1.Most common cause of BCS in India is membranous obstruction of inferior vena cava.2.Ascites with high protein and high serum ascitic albumin gradient is suggestive of BCS.3.Initial imaging modality of choice to diagnose BCS is magnetic resonance venogram.4.Failure to visualize the hepatic veins on Doppler is diagnostic of BCS.5.Direct intrahepatic portosystemic shunt can be done even if all hepatic vein ostia are blocked.7.Regarding the role of gut microbiota in hepatic encephalopathy (HE) the following are TRUE:1.Markers of systemic inflammation are better correlated with HE than the severity of liver disease.2.Pathogen associated molecular patterns (PAMPs) are microbial components that initiate inflammatory cascade.3.Gut microbiota of patients with cirrhosis and normal controls is similar.4.Lactulose exerts its actions in HE predominantly by altering the composition of gut microbiota.5.Stool microbiota is an accurate estimate of intestinal microbiota.8.Regarding primary sclerosing cholangitis (PSC) the following are TRUE:1.Majority of patients with PSC have isolated extrahepatic disease.2.Magnetic resonance cholangiography is the diagnostic modality of choice.3.Dominant stricture is a stenosis with a diameter of <1.5 mm in the hepatic duct or of <1 mm in the common bile duct.4.Inflammatory bowel disease is usually diagnosed after diagnosis of PSC.5.Cholecystectomy is recommended in a patient with PSC with a gallbladder mass lesion, irrespective of its size.9.Regarding primary biliary cirrhosis (PBC) the following are TRUE EXCEPT:1.Antimitochondrial antibodies (AMA) are highly sensitive and specific for the diagnosis of PBC.2.AMA negative cases of PBC have less aggressive disease and better prognosis.3.Ursodeoxycholic acid improves symptoms but does not alter natural course of PBC.4.Modafinil may improve fatigue in patients with PBC.5.Sjogren's syndrome occurs more frequently in PBC than matched controls.10.Regarding overlap syndromes the following are TRUE:1.Overlap of autoimmune hepatitis (AIH) and PBC is the most common variant.2.Overlap of PSC and PBC is common.3.Autoimmune sclerosing cholangitis refers to features of PSC in children with AIH.4.The components of the overlap syndrome may develop sequentially with one appearing years after the first.5.UDCA does not have a beneficial role in overlap syndromes.

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.