Metallic Stents for Hepatic Venous Outflow Obstruction After Living-Donor Liver Transplantation and their Therapeutic Effects.

BACKGROUND: Living-donor liver transplantation (LDLT) is established as a standard therapy for end-stage liver disease; however, vessel reconstruction is more demanding due to the short length and small size of the available structures compared with deceased-donor whole liver transplantation. Interventional radiology (IR) has become the first-line treatment for vascular complications after LDLT. Hepatic venous outflow obstruction (HVOO) is a life-threatening complication after LDLT. The aim of this study of 592 adult-to-adult LDLT cases was to investigate the safety and efficacy of stent implantation for HVOO after LDLT. METHODS: Records of patients who developed HVOO requiring any treatment were collected with special reference to the metallic stent implantation. There were 232 left-side grafts and 360 right-side grafts. Sixteen cases developed HVOO after LDLT with an incidence rate of 2.7%, 5 with a left liver graft (2%), and 11 with a right-side graft (3%). The IR was attempted for 14 cases; among those, 8 cases were treated by stent implantation. RESULTS: The technical success rate of the initial stent implantation was 100%. The pressure gradient at the stenotic site significantly improved from 12.2 (range, 10.9-20.4 cm H2O) to 3.9 cm H2O (range, 1.4-8.2 cm H2O; P = .03). The volume of the congested graft liver decreased significantly from 1448 (range, 788-2170 mL) to 1265 mL (range, 748-1665 mL; P = .01), and the serum albumin level improved significantly from 3.3 (range, 1.7-3.7 g/dL) to 3.7 g/dL (range, 2.9-4.1 g/dL; P = .02). No procedure-related complication was noted, and the long-term stent patency was 100%. CONCLUSION: Metallic stent implantation for stenotic venous anastomosis after LDLT is a safe and effective treatment.

Budd-Chiari syndrome in children: Radiological intervention and role of shear wave elastography in monitoring response.

OBJECTIVES: Radiological intervention (RI) is the preferred treatment in children with Budd-Chiari syndrome (BCS). We studied the comparative long-term outcome of BCS children, with and without RI and utility of liver and splenic stiffness measurement (LSM, SSM) by 2-dimensional shear wave elastography (2D-SWE) in assessing response. METHODS: Sixty children (40 boys, median age 10.5 [6.5-15.25] years) with BCS (29 newly diagnosed, 31 follow-up) were evaluated. LSM and SSM by 2D-SWE and vascular patency were monitored pre- and postprocedure (≥ 6 months postprocedure) in those undergoing RI. Medical therapy without anticoagulation and monitoring was done in subjects without RI. The RI and no-RI groups were compared. RESULTS: Ascites (54,90%), hepatomegaly (56,93%) and prominent abdominal-veins (42,70%), were the commonest features. The majority (46,78%) had isolated hepatic vein block. 44 (73%) cases underwent RI, while 16 (27%) were managed conservatively. Both groups were similar at baseline. Post-RI subjects showed significant improvement in clinical findings, liver functions and portal hypertension. LSM [33 (32-34.5) to 19.2 (18-20.67) kPa] and SSM [54.5 (52.3-57.6) to 28.9 (27.6-30.25) kPa] showed a significant decline from baseline value over a follow-up of 12 (6-13) months. Gradual reduction occurred in the LSM and SSM over 1-5 years, with near-normal LSM [10.2 (9.2-11.5) kPa] and SSM [22.3 (20.5-24.3) kPa] values in patients (n-16) with > 5 years follow-up. Patients without RI showed worsening in LSM and SSM. Hepatopulmonary syndrome and hepatocellular carcinoma developed in 4 (8%) and 1 (1.7%) cases respectively. CONCLUSION: RI leads to clinical recovery and reduction with near normalization of LSM and SSM over long-term follow-up in children with BCS. 2D-SWE is a promising tool to monitor outcomes.

Stenting of Inferior Right Hepatic Vein in a Patient with Budd-Chiari Syndrome: A Case Report.

A Japanese man in his 20s was referred to our hospital with a two-month history of abdominal fullness and leg edema. Abdominal computed tomography revealing massive ascites and ostial blockage of the main hepatic veins, and angiographic evaluation demonstrating obstruction of the main hepatic veins yielded a diagnosis of Budd-Chiari syndrome (BCS). Diuretic agents were prescribed for the ascites but failed to provide relief. The patient was referred to our department for further evaluation and treatment. Angiography showed ostial obstruction of the main hepatic veins, with most of the portal hepatic flow draining from an inferior right hepatic vein (IRHV) into the inferior vena cava (IVC) thorough an intrahepatic portal venous and venovenous shunt. Access between the main hepatic veins and IVC was impossible, but cannulation between the IRHV and IVC was achieved. Because of the venovenous connection between the main hepatic vein and the IRHV, metallic stents were placed into two IRHVs to decrease congestion in the hepatic venous outflow. After stent placement followed by balloon expansion, the gradient pressure between the hepatic vein and IVC improved remarkably. The ascites and lower leg edema improved postoperatively, and long-term stent patency (6 years) was achieved.

Hepatic venous outflow obstruction after adult living donor liver transplantation.

Hepatic venous outflow obstruction (HVOO) is a rare but critical vascular complication after adult living donor liver transplantation. We categorized HVOOs according to their morphology (anastomotic stenosis, kinking, and intrahepatic stenosis) and onset (early-onset < 3 mo vs. late-onset ≥ 3 mo). Overall, 16/324 (4.9%) patients developed HVOO between 2000 and 2020. Fifteen patients underwent interventional radiology. Of the 16 hepatic venous anastomoses within these 15 patients, 12 were anastomotic stenosis, 2 were kinking, and 2 were intrahepatic stenoses. All of the kinking and intrahepatic stenoses required stent placement, but most of the anastomotic stenoses (11/12, 92%) were successfully managed with balloon angioplasty, which avoided stent placement. Graft survival tended to be worse for patients with late-onset HVOO than early-onset HVOO (40% vs. 69.3% at 5 y, p = 0.162) despite successful interventional radiology. In conclusion, repeat balloon angioplasty can be considered for simple anastomotic stenosis, but stent placement is recommended for kinking or intrahepatic stenosis. Close follow-up is recommended in patients with late-onset HVOO even after successful treatment.

Budd-Chiari-like syndrome in a dog secondary to a gunshot wound treated with balloon angioplasty and endovascular stent placement.

A 6-year-old female spayed Chihuahua mix presented with chronic recurrent ascites. Computed tomographic angiography revealed an isolated stenosis of the caudal vena cava secondary to a metallic foreign body, resulting in Budd-Chiari-like syndrome. Balloon angioplasty and endovascular stent placement successfully resolved the obstruction with long-term resolution of ascites.

Quantitative liver magnetic resonance imaging: correlation between conventional magnetic resonance imaging, laboratory values, and prognostic indices in Budd-Chiari syndrome.

PURPOSE: In Budd-Chiari syndrome (BCS), unevenly distributed parenchymal changes and perfusion abnormalities occur due to hepatic venous outflow obstruction. This study aimed to evaluate the changes in the liver parenchyma in BCS using the quantitative magnetic resonance (MR) techniques of MR elastography, T1 and T2 mapping, and diffusion imaging and correlate the quantitative MR parameters through biochemical results and prognostic indices. METHODS: Fourteen patients with BCS (seven men and seven women) were examined retrospectively. Liver stiffness (kPa), T1 relaxation times (ms) were achieved using the modified Look-Locker inversion recovery (MOLLI) 3(2)3(2)5 sequence and B1-corrected variable flip angle methods, T2 relaxation times (ms), and apparent diffusion coefficient (ADC) values (mm2/s) were measured using regions of interest placed in the same region in all quantitative methods. Measurements were repeated at the precontrast and postcontrast hepatobiliary phases. The reduction rate (RR; %) and adjusted postcontrast T1 (%) were calculated. The values obtained from different liver parenchyma areas (whole liver, caudate lobe, pathological T2 hyperintense tissue, and relatively preserved normal-appearing tissue) were compared using the Wilcoxon signed-rank test. Spearman's correlation coefficient was used to investigate the correlation between quantitative MR parameters and biochemical parameters/ prognostic scores (Child-Pugh score, Clichy score, and Rotterdam index). RESULTS: The parenchymal stiffness and precontrast T1 values of the caudate lobe were significantly lower than those of the remainder of the parenchyma, whereas the adjusted postcontrast T1 percentages (MOLLI) were significantly higher (P ≤ 0.027). The parenchymal stiffness value, T1 and T2 values, percentages of RR (MOLLI), and adjusted postcontrast T1 values for the pathological tissue and relatively normal tissue were significantly different (P < 0.028). No significant difference was found in terms of ADC values between any of the distinct regions of the liver. A strong correlation was detected between the Child-Pugh score, Clichy score, and precontrast T1 values obtained through the MOLLI sequence (r = 0.867, P = 0.012, r = 0.821, P = 0.023, respectively). No correlation was found between the whole liver stiffness values and the laboratory parameters, fibrosis markers, prognostic indices, or MR parameters. A significant correlation was identified between creatinine levels and several T1 parameters and the T2 relaxation time (r ≥ 0.661, P ≤ 0.052). CONCLUSION: Tissue stiffness and T1 relaxation values are high in the areas identified as fibrosis compared with those in the relatively preserved parenchyma. The T1 relaxation time can offer quantitative information for assessing segmental functional changes and prognosis in BCS.ion for assessing segmental functional changes and prognosis in BCS.

Budd-Chiari syndrome in Behçet's disease.

A 36-year-old man was admitted to the emergency department due to a 30-day history of abdominal distention and epigastralgia. He had described a non-intentional 10kg weight loss, dry cough, and fever 6 months before his admission. He had a history of tobacco and cocaine abuse and reported recurrent oral and genital ulcers. Physical examination showed an extensive area of venous collateral circulation on the abdominal wall, hepatomegaly, signs of a moderate ascites, and lower limb edema. Liver and renal function tests were normal. The ascitic fluid analysis did not show an inflammatory or infectious pattern. Upper flexible endoscopy revealed esophageal fine-caliber varices and colonoscopy showed an isolated terminal ileal ulcer. Abdominal imaging revealed hepatomegaly, voluminous ascites, and thrombosis of hepatic veins, inferior and superior vena cava (Figure 1). Infections and coagulation or lymphoproliferative disorders were excluded. Thereafter, the diagnosis of Budd-Chiari Syndrome in Behçet disease was established and immunosuppression treatment was started with good initial clinical evolution.

Assessing the Complicated Venous Hemodynamics and Therapeutic Outcomes of Budd-Chiari Syndrome with Respiratory-gated 4D Flow MR Imaging During the Expiratory and Inspiratory Phases.

A man in his 50s with Budd-Chiari syndrome diagnosed with the suprahepatic inferior vena cava (IVC) obstruction on CT was assessed using 4D Flow MRI before and after balloon angioplasty. 4D Flow MRI acquired in two respiratory phases, depicted complex hemodynamic and respiratory variability, and a jet stream at the narrowed channel of the membranous IVC. Post-interventional 4D Flow MRI showed that the IVC blood flow increased with corrected flow directions in the infrarenal IVC.

The change in the liver volume after direct intrahepatic portocaval shunt in patients with Budd-Chiari syndrome with diffuse hepatic vein occlusion.

PURPOSE: To evaluate the changes in the liver volume and function after direct intrahepatic portocaval shunt (DIPS) in patients with Budd-Chiari syndrome (BCS) with diffuse hepatic vein (HV) occlusion. METHODS: The clinical data of 29 patients with BCS who underwent DIPS for intractable ascites caused by diffuse hepatic vein occlusion in the Affiliated Hospital of Xuzhou Medical University were analysed retrospectively; the patients included 8 males and 21 females, with an average age of 33.3 ± 6.3 years. The patients underwent abdominal CT scanning and liver function examinations before DIPS, and 1 week, 3 months, 6 months and 12 months after DIPS. The changes of the liver volume and liver function before and after DIPS were compared. RESULTS: All 29 patients underwent DIPS successfully. 28 patients survived during the follow-up of 12-33 months, with a median follow-up of 16 months. The patients' liver function were significantly improved at 3, 6 and 12 months after the operation compared to before the operation (P < 0.05). The liver volumes measured before the operation and 1 week, 3 months, 6 months and 12 months after the operation were 2124.586 ± 420.889 cm3, 1926.263 ± 372.268 cm3, 1480.592 ± 183.061 cm3, 1461.904 ± 153.027 cm3 and 1469.286 ± 148.549 cm3, respectively. Compared with the preoperative liver volume, the liver volume had decreased significantly at 1 week, 3 months, 6 months and 12 months after the operation (P < 0.05). However, there was no significant difference in the liver volumes at 6 and 12 months after the operation (P = 0.35). CONCLUSIONS: Direct intrahepatic portocaval shunt has achieved satisfactory clinical results in the treatment of BCS with diffuse hepatic vein occlusion. The congestive hepatomegaly was gradually reduced after the operation. The liver volume (which was defined as the clinical efficacy in this study) remained stable after 6 months.

Percutaneous Sclerotherapy for Budd-Chiari Syndrome Secondary to Giant Hepatic Venous Malformations (Hemangiomas).

This prospective study evaluated the safety and effectiveness of percutaneous sclerotherapy in the treatment of secondary Budd-Chiari syndrome due to hepatic venous malformations (HVMs). Four patients (mean age, 40 years; 3 women) with 5 HVMs underwent 7 sessions of percutaneous sclerotherapy with a mixture of bleomycin and lipiodol. All patients had chronic Budd-Chiari syndrome, determined based on imaging findings, with the main symptom being abdominal discomfort and distention. On physical examination, 2 patients had ascites and the other 2 had an epigastric mass. The indication for treatment was intractable abdominal symptoms due to hepatic and/or inferior vena cava (IVC) outflow compression. All procedures were technically successful, with no major complications. Three patients underwent a second session because of incomplete IVC decompression. The patients' symptoms completely resolved at 6 and 12 months of follow-up. There was a significant reduction in lesion volume (P = .007) and an increase in IVC luminal area (P = .018) at 12 months of follow-up.

Efficacy and Safety of Transjugular Intrahepatic Portosystemic Shunt Creation for Budd-Chiari Syndrome: A Systematic Review and Meta-Analysis.

PURPOSE: To assess the critical role of transjugular intrahepatic portosystemic shunt (TIPS) in the management of Budd-Chiari syndrome (BCS), as the data with respect to the safety and outcome of TIPS in patients with BCS are scarce because of the rarity of the disease. MATERIALS AND METHODS: A comprehensive search of literature of various databases from 2000 to October 2021 was conducted for studies evaluating the outcome of TIPS in patients with BCS. The primary outcomes of the analysis were technical and clinical success, adverse events and mortality associated with TIPS, dysfunction of TIPS, need for TIPS revision, need for liver transplantation (LT), and 1-year survival. RESULTS: A total of 33 studies (1,395 patients) were included in this meta-analysis. The pooled rates and 95% confidence intervals of various outcomes were 98.6% (97.6-99.7) for technical success, 90.3% (86.0-94.6) for clinical success, 10.0% (6.5-13.6) for major adverse events, 0.5% (0.2-1.0) for TIPS-related mortality, 11.6% (7.8-15.4) for post-TIPS hepatic encephalopathy (HE), 40.1% (32.5-47.7) for TIPS dysfunction, 8.6% (4.9-12.4) for the need for TIPS revision, 4.5% (2.8-6.2) for the need for LT, and 94.6% (93.1-96.1) for 1-year survival. Publication bias was seen with all outcomes except for post-TIPS HE, TIPS dysfunction, and the need for LT. CONCLUSIONS: The existing literature supports the feasibility, safety, and efficacy of TIPS in the treatment of BCS. Deciding the optimal timing of TIPS in BCS needs further studies.

Exceptional association of Budd-Chiari syndrome with pheochromocytoma.

We are reporting a case revealed by an abdomino-thoracic painful syndrome associated with multi-organ failure. A 61-year-old hypertensive woman was hospitalized for the management of hypertensive emergency associating acute coronary syndrome and abdominal pain. A thoraco-abdomino-pelvic CT-scan showed a right adrenal mass associated with hepatic vein thrombosis. After medical preparation, an adrenalectomy was performed by way of open surgery; the study of the surgical specimen found a pheochromocytoma score PASS 4. The follow-up was marked by the normalization of blood pressure and biological abnormalities.

Hepatic Venous Outflow Obstruction in Adult Deceased Donor Liver Transplantation: Classic Piggyback Implantation Versus a Modified Technique that Widens the Ostium of the Recipient's Left and Middle Hepatic Veins.

BACKGROUND: The classic piggyback technique uses the union of the 3 hepatic veins to perform the cavo-caval anastomosis. However, due to the lateral localization of the right hepatic vein, the partial clamping of the vena cava in this technique significantly reduces the venous return to the right atrium. To avoid this, we adopted in 2015 a modified piggyback technique, in which we use the common trunk of the middle and left hepatic veins and also perform a lateral incision toward the right in the anterior wall of the vena cava in order to widen the final ostium of the cavo-caval anastomosis. The aim of the study was to analyze the incidence of hepatic venous outflow obstruction between those 2 techniques. METHODS: Retrospective study of liver transplant recipients undergoing venography for suspected hepatic venous outflow obstruction from January 2009 to June 2021. Patients undergoing transplantation with living donors or split grafts and pediatric cases were excluded from the study. RESULTS: From January 2009 to December 2014 and from January 2015 to June 2021, 587 (group 1) and 730 (group 2) deceased-donor liver transplants were performed with the classic and the modified piggyback techniques, respectively. The incidence of cases with suspected hepatic venous outflow obstruction in groups 1 and 2 were 1.87% (n = 11) and 0.95% (n = 7), respectively (P = 0,15). The number of confirmed patients with outflow blockage that required endovascular treatment during venography in groups 1 and 2 were 4 (0.68%) and 5 (0.68%), respectively (P = 0,31). CONCLUSIONS: This modified piggyback technique did not increase the incidence of hepatic venous outflow obstruction at our service.

Evaluating short-term outcomes of the value of sound touch elastography (STE) following the treatment for Budd-Chiari syndrome (BCS): a case series study.

AIM: To investigate the value of sound touch elastography (STE) in the evaluation of short-term therapeutic effect of Budd-Chiari syndrome (BCS) by measuring liver stiffness (LS), and in addition, to analyse the relationships between liver function, pressure gradient of the hepatic veins, and LS. MATERIALS AND METHODS: A case series study was conducted at Affiliated Hospital of Xuzhou Medical University from August 2020 to December 2020. Patients diagnosed with BCS were recruited prospectively and grouped according to Child-Pugh grade before endovascular therapy. LS was measured using STE before and after therapy. Comparisons between the LS and hepatic venous pressure gradient (HVPG) changes of patients were tested with paired sample t-tests. RESULTS: A total of 46 patients (23 males and 23 females) were included in this study. According to the Child-Pugh scoring criteria, 24 patients were classified as grade A, 16 as grade B, and 6 as grade C. LS was significantly different between the three groups (F = 127.01, p<0.001). Post-treatment LS was significantly lower than pre-treatment (p<0.001). The mean HVPG before treatment was 13.02 ± 3.82 mmHg and decreased after intervention (p<0.001). CONCLUSION: The STE is a potential tool for evaluating short-term therapeutic effect of BCS patients.

Establishing a jugular-femoral venous route for recanalization of complicated inferior vena cava in Budd-Chiari Syndrome after transfemoral access failure.

Recanalization of inferior vena cava (IVC) with complete obstruction, old thrombosis or long segmental stenosis/obstruction (complicated IVC) via transfemoral access may fail in patients with Budd-Chiari syndrome (BCS). In this study, 34 consecutive patients with BCS underwent recanalization of complicated IVC occlusion via jugular-femoral venous (JFV) route establishment. BCS with complicated IVC was detected by reviewing preoperative color Doppler ultrasonography or computed tomography (CT) venography, and confirmed by intraoperative venography. Clinical data on technical success, complications, and follow-up outcomes were analyzed. Except for one patient received surgical repair of rupture IVC after recanalization, technical success of IVC recanalization was achieved in remaining 33 (97.1%) patients. No perioperative deaths was found. Three complications were observed during recanalization, for a complication rate of 8.8%. Bleeding of the femoral vein was observed in one patient, and two patients showed bleeding of IVC. The IVC lesion diameter and blood flow of the distal IVC increased significantly after the procedure. Twenty-four patients (77.4%) were clinically cured, and four patients (12.9%) showed clinical improvement. The 1-year, 3-year, 5-year primary patency rates were 85.9%, 76.4% and 70.0%, respectively. The 5-year secondary patency rate was 96.8%. There were three deaths during follow up, and the 5-year survival rate was 90.0%. In conclusion, JFV route establishment and angioplasty for complicated IVC is safe and effective for patients with BCS after transfemoral access failure.