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Purpose: Hepatic venous transplant anastomotic pressure gradient measurement and transjugular liver biopsy are commonly used in clinical decision-making in patients with suspected anastomotic hepatic venous outflow obstruction. This investigation aimed to determine if sinusoidal dilatation and congestion on histology are predictive of hepatic venous anastomotic outflow obstruction, and if it can help select patients for hepatic vein anastomosis stenting. Materials and Methods: This is a single-center retrospective study of 166 transjugular liver biopsies in 139 patients obtained concurrently with transplant venous anastomotic pressure gradient measurement. Demographic characteristics, laboratory parameters, procedure and clinical data, and histology of time-zero allograft biopsies were analyzed. Results: No relationship was found between transplant venous anastomotic pressure gradient and sinusoidal dilatation and congestion (P = 0.92). Logistic regression analysis for sinusoidal dilatation and congestion confirmed a significant relationship with reperfusion/preservation injury and/or necrosis of the allograft at time-zero biopsy (OR 6.6 [1.3–33.1], P = 0.02). Conclusion: There is no relationship between histologic sinusoidal dilatation and congestion and liver transplant hepatic vein anastomotic gradient. In this study group, sinusoidal dilatation and congestion is a nonspecific histopathologic finding that is not a reliable criterion to select patients for venous anastomosis stenting. [ABSTRACT FROM AUTHOR]

Abstract Purpose To evaluate efficacy, safety, and outcomes of endovascular treatment of hepatic vein stenosis post major liver resection. Methods A retrospective data analysis was performed including all interventional treatments of hepatic vein stenosis post major liver resection since 2010. Post procedural course and clinical parameters including amount of ascites accumulation and relevant laboratory values were assessed during the follow-up period. Primary and primary assisted hepatic venous patency time were calculated. Results Twelve patients (median age 55.5, IQR 49.75 to 61.5 years) undergoing a total of 16 interventions were included. Interventions were primary stent placement (n = 3), primary balloon angioplasty (n = 8), three re-interventions and two aborted interventions (no significant pressure gradient). Technical success was 100% (16/16). Permanent reduction and / or complete resolution of ascites was achieved in 72% (8/11). Laboratory parameters related to liver function did not show significant improvement after intervention. Median follow-up period was 6 months (IQR: 1.5 to 18 months). The median primary patency time for patients with balloon angioplasty was 11 months (IQR: 1.375 to 22.25 months) and assisted patency time was 13.25 months (IQR: 4.5 to 22.25 months). The median primary patency time for patients with angioplasty and stent placement was 1 months (IQR: 1.0 to 1.5 months) and assisted patency time was 2.0 months (IQR: 1.5 to 2.5months). Conclusion An endovascular approach for the treatment of hepatic venous stenosis post major liver resection is safe and efficient to reduce and / or resolve refractory ascites. However, liver function parameters seem not to be improved by the procedure. Stent placement can be a reasonable option in patients with significant residual stenotic disease post angioplasty.

Purpose: To evaluate efficacy, safety, and outcomes of endovascular treatment of hepatic vein stenosis post major liver resection. Methods: A retrospective data analysis was performed including all interventional treatments of hepatic vein stenosis post major liver resection since 2010. Post procedural course and clinical parameters including amount of ascites accumulation and relevant laboratory values were assessed during the follow-up period. Primary and primary assisted hepatic venous patency time were calculated. Results: Twelve patients (median age 55.5, IQR 49.75 to 61.5 years) undergoing a total of 16 interventions were included. Interventions were primary stent placement (n = 3), primary balloon angioplasty (n = 8), three re-interventions and two aborted interventions (no significant pressure gradient). Technical success was 100% (16/16). Permanent reduction and / or complete resolution of ascites was achieved in 72% (8/11). Laboratory parameters related to liver function did not show significant improvement after intervention. Median follow-up period was 6 months (IQR: 1.5 to 18 months). The median primary patency time for patients with balloon angioplasty was 11 months (IQR: 1.375 to 22.25 months) and assisted patency time was 13.25 months (IQR: 4.5 to 22.25 months). The median primary patency time for patients with angioplasty and stent placement was 1 months (IQR: 1.0 to 1.5 months) and assisted patency time was 2.0 months (IQR: 1.5 to 2.5months). Conclusion: An endovascular approach for the treatment of hepatic venous stenosis post major liver resection is safe and efficient to reduce and / or resolve refractory ascites. However, liver function parameters seem not to be improved by the procedure. Stent placement can be a reasonable option in patients with significant residual stenotic disease post angioplasty. [ABSTRACT FROM AUTHOR]

Budd-Chiari syndrome (BCS) is a rare hepatic vascular disorder defined by the presence of partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Several conditions can lead to BCS, from hypercoagulable states to malignancies. Primary BCS is the most common subtype, and usually bartends hypercoagulability states, while secondary BCS involves tumor invasion or extrinsic compression. A combination of clinical and imaging features leads to the diagnosis of BCS, including (1) direct signs: occlusion or compression of the hepatic veins and/or inferior vena cava, and the presence of venous collaterals; (2) indirect signs: morphological hepatic changes with caudate lobe enlargement; inhomogeneous enhancement, and hypervascular nodules. From a clinicopathological point of view, two forms of BCS can be distinguished: acute and subacute/chronic BCS, although asymptomatic and fulminant forms are also possible. Acute presentations are rare, and symptoms include hepatomegaly, ascites, and hepatic insufficiency. Subacute/chronic forms are the most common presentation, with dysmorphic liver and variable degrees of fibrosis deposition. Patients with chronic BCS can develop benign regenerative nodules (large regenerative nodules or FNH [Focal Nodular Hyperplasia]-like lesions), but are also at a higher risk of hepatocellular carcinoma (HCC). The radiologist role is therefore fundamental in both diagnosis and surveillance of BCS. The aim of this review is to present all clinical and imaging signs that can help to reach the diagnosis of BCS, with their clinical significance, providing tips and tricks for the cross-sectional diagnosis of this condition. [ABSTRACT FROM AUTHOR]

Budd-Chiari syndrome (BCS) is the clinical spectrum presenting with hepatic venous outflow obstruction from the hepatic veins to the junction of the inferior vena cava (IVC) and the right atrium. This syndrome is rare in infants and children, which leads to misdiagnoses or delayed diagnoses. Clinical presentations may be non-specific. A high index of suspicion and imaging findings may help in early diagnosis of this condition. We report a rare case of BCS in a 6-week-old male infant who presented with jaundice and gradual abdominal distension. He was successfully treated with rivaroxaban.

Introduction: Budd-Chiari syndrome is a rare condition which can be defined as hepatic venous outflow obstruction occurring at any level between the small hepatic veins and junction of the inferior vena cava and the right atrium. Symptoms may vary from mild or even asymptomatic for many years to fulminant hepatic failure. Imaging is the mainstay of diagnosis of BCS. Laboratory and ematological tests are invaluable in recognizing hematological and coagulation disorders that may be identified in up to 75% of the patients. The recommended therapeutic approach to BCS is based on a stepwise algorithm beginning with medical treatment, endovascular treatment to restore vessel patency, placement of transjugular portosystemic shunt (TIPS), and orthotopic liver transplantation as a last resort rescue treatment. The aim of the study: This study aims to gather current knowledge about clinical manifestations, diagnosis and treatment of Budd-Chiari syndrome. Materials and methods: This review was based on available data collected in the PubMed database and Google Scholar web search engine, using the keywords: ’Budd-Chiari syndrome’ ,’hepatic venous outflow obstruction’. Conclusions: The treatment of Budd-Chiari syndrome requires interdisciplinary cooperation, which has resulted in the increasing curability of this rare disease over the last years.

Abstract Background Migration of the left hepatic lobe into the potential space following right lobe resection can result in torsion and hepatic venous outflow obstruction with compromised venous return from the IVC. If untreated, significant morbidity and mortality can develop. Case presentation We report a case of a 29-year-old female with Lynch syndrome who underwent right lobe resection for a metastatic hepatic tumor. There was subsequent migration of the liver remnant, torsion of the IVC, and impaired hepatic outflow, successfully treated with thrombectomy and stenting. Conclusion Following right hepatectomy, hepatic venous outflow obstruction should be consdered in the setting of hepatorenal failure and hemodynamic instability. Endovascular stenting is a viable treatment option.

Budd–Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review. [ABSTRACT FROM AUTHOR]

Budd-Chiari syndrome (BCS) is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction. Abernethy malformation is a congenital vascular malformation defined by diversion of portal blood away from the liver. Both conditions are rare vascular diseases. We report here the first case of a patient with combined type II Abernethy malformation and BCS from China. The inferior vena cava obstruction was treated with percutaneous balloon angioplasty; close follow-up was elected for the Abernethy malformation.

• Hepatic venous outflow obstruction (HVOO) is a rare complication of hepatectomy. • HVOO might occur irrespective of whether the left triangular ligament is divided. • The treatment for HVOO is repositioning and/or stenting into the hepatic vein. Hepatic venous outflow obstruction (HVOO) is a rare complication of hepatectomy. We report a case of HVOO caused by remnant liver migration into the subphrenic space after hepatectomy, which was successfully managed by repositioning of the remnant liver. A 55-year-old Japanese man was diagnosed with a liver tumor on ultrasound. Contrast-enhanced CT revealed early enhancement in the arterial phase, followed by a washout in the late phase. Preoperative diagnosis was hepatocellular carcinoma, and hand-assisted laparoscopic extended posterior sectionectomy was performed. On postoperative day 1, middle hepatic vein (MHV) flow was not detected on ultrasound, and the portal flow was hepatofugal. CT during arterial portography revealed absence of the portal flow to the medial and anterior sections, and remnant liver migration into the subphrenic space. Therefore, we suspected that HVOO was caused by the remnant liver migration and performed redo laparotomy to reposition the remnant liver with suturing of the falciform ligament to the anterior abdominal wall. Postoperatively, contrast-enhanced CT demonstrated that the remnant liver remained in the anatomical position, and the medial and anterior sections were well enhanced. HVOO might occur irrespective of whether the left triangular ligament is preserved. We believe that it is necessary to fix the remnant liver to the abdominal wall in cases with poor venous blood flow confirmed by intraoperative ultrasound. If kinking of the hepatic vein persists, stent insertion should be performed. HVOO after hepatectomy is rare but potentially fatal, and prevention and countermeasures should be discussed. [ABSTRACT FROM AUTHOR]

Refractory ascites post–liver transplantation can be a challenging problem. Causes of refractory ascites include venous outflow anastomotic stenosis, vessel kinking by the regenerating liver, pre‐existing graft disease, and positional outflow obstruction. We present a case report of a child presenting with high drain output and refractory ascites post–LDLT secondary to a positional kinking. Repeating the Doppler studies with patients both supine and sitting may be helpful. [ABSTRACT FROM AUTHOR]

Hepatic venous outflow obstruction (HVOO) is rare but serious complication after right lobe living donor liver transplantation (LDLT). Failure to identify this complication early can result in graft failure and even death. The early diagnosis and management of HVOO is very important. We report a case with this complication treated by endovascular stent placement in the early period after right lobe LDLT and review-related reports to explore the possible mechanism. A 43-year-old male with chronic liver disease underwent right lobe LDLT. On postoperative day 2, his liver function deteriorated. Hepatic venography showed an obstruction and thrombosis of mechanical heart valve with an element of torsion of the venous drainage proximal to the anastomosis of the right hepatic venous orifice and inferior vena cava. The obstruction was successfully treated by insertion of an expandable metallic stent. The result demonstrates that stent placement for obstruction is safe and effective.

Patients suffering from polycystic liver disease may develop Hepatic Venous Outflow Obstruction, Portal Vein Obstruction and/or Inferior Caval Vein Syndrome because of cystic mass effect. This can cause portal hypertension, leading to ascites, variceal haemorrhage or splenomegaly. For this review, we evaluate the evidence to provide clinical guidance for physicians faced with this complication. Diagnosis is made with imaging such as ultrasound, computed tomography or magnetic resonance imaging. Therapy includes conventional therapy with diuretics and paracentesis, and medical therapy using somatostatin analogues. Based on disease phenotype various (non‐)surgical liver‐volume reducing therapies, hepatic or portal venous stenting, transjugular intrahepatic portosystemic shunts and liver transplantation may be considered. Because of complicated anatomy, use of high‐risk interventions and lack of empirical evidence, patients should be treated in expert centres. [ABSTRACT FROM AUTHOR]

Background: The successful application of ex vivo liver resection and autotransplantation (ERAT) has gained widespread attention for the treatment of end-stage hepatic alveolar echinococcosis, which is considered to be unresectable by conventional methods due to extensive invasion of the extra- and intrahepatic vasculature. However, data on remnant liver volume (RLV) are limited, and the safe volume limit of remnant liver is still unclear.Methods: To determine the effect of liver volume in the technically developed era, we investigated the impact of the remnant liver-to-standard liver volume ratio (RLV/SLV) on the outcomes of ERAT.Results: From February 2014 to May 2018, 56 ERAT procedures were performed. Eleven patients with an RLV/SLV < 40% (group S) were compared with 45 patients with an RLV/SLV ≥ 40% (group L). Serial changes in postoperative serum total bilirubin, alanine aminotransferase, aspartate aminotransferase, and international normalized ratio were comparable in both groups. The incidences of postoperative complications did not significantly differ between the two groups. Three patients died of intra-abdominal bleeding, acute cerebral hemorrhage, and severe liver dysfunction. In RLV estimation analysis, the actual RLV and RLV/SLV were significantly smaller than the expected RLV and RLV/SLV as determined by preoperative three-dimensional reconstruction software in patients with hepatic venous outflow obstruction.Conclusion: Patients with a smaller RLV/SLV did not have outcomes inferior to those with a larger RLV/SLV. Further studies are warranted to clarify the factors that contribute to preoperative volumetric estimation and the safe lower limits for ERAT. [ABSTRACT FROM AUTHOR]

Outflow obstruction is a rare but critical vascular complication in liver transplantation, which may lead to graft loss and mortality. We report a case of caval vein outflow obstruction due to retrohepatic compression after living donor liver transplantation (LDLT), which was managed by temporary implantation of a vena cava filter. A 63-year-old male with end stage liver disease presented with caval vein outflow obstruction and massive ascites 12 days after right lobe LDLT. We opted for a minimally invasive approach and implanted a vena cava filter at the compressed site through transjugular route. The patient's ascites drainage significantly decreased and graft function maintained stable after the intervention. On day 50 posttransplant, the filter was successfully removed and the patient was discharged without complications. Outflow obstruction after liver transplantation can result from anastomotic stenosis, graft size mismatch, thrombosis or compression of the outflow tract. Various management strategies have been employed both peri- and posttransplant, ranging from surgical interventions to minimally-invasive techniques. The treatment strategy should be tailored to the individual case, considering the timing of presentation and the specific cause for the obstruction. We successfully managed a case of compressive outflow obstruction by temporary implantation of a vena cava filter after LDLT. The vena cava filter was safely removed under angiography. • Outflow obstruction caused by graft compression is a rare but severe complication in liver transplantation. • The treatment strategy of outflow obstruction should be individualized, based on the timing and cause of the obstruction. • The implantation of vena cava filter is a safe and effective method to overcome outflow obstruction. [ABSTRACT FROM AUTHOR]

Background: Vascular disorders of the liver are relatively rare in clinical practice. Budd Chiari syndrome (BCS) is a potentially life-threatening condition. It describes a group of disorders characterized by hepatic venous outflow obstruction at any level from the small hepatic veins up to the junction of the inferior vena cava and the right atrium. The prevalence of Budd Chiari Syndrome is greatly influenced by geographical differences. There is a dearth of data in Nigeria regarding this condition. Case report: We report the case of a thirty seven year-old woman presenting with an eight year history of recurrent abdominal swelling, jaundice and progressive weight loss. The patient was promptly screened for viral hepatitis B and C, which were both negative. The laboratory work-up revealed mild hepatic dysfunction and an abdominal ultrasound scan showed mild hepatomegaly and ascites. Abdominal Computed Tomography (CT) scan findings were consistent with Budd Chiari Syndrome, based on the findings of an extensive thrombus, involving the common iliac and hepatic veins, in addition, the inferior vena cava (IVC) and right atrium. Conclusion: This case report highlights the importance of the knowledge of Budd Chiari Syndrome and the diagnostic approach. This condition should be considered whenever investigating the aetiology of chronic or acute liver disease in Gastroenterology practice in Nigeria. [ABSTRACT FROM AUTHOR]

Abstract: HVOO can be a critical complication in pediatric LDLT. The aim of this study was to evaluate a modified triangular technique of hepatic vein reconstruction for preventing HVOO in pediatric LDLT. A total of 298 pediatric LDLTs were performed using a left lateral segment graft by 2 methods for reconstruction of the hepatic vein. In 177 recipients, slit‐shaped anastomosis was indicated with partial clamp of the IVC. A total of 121 recipients subjected to the modified triangular anastomosis with total clamp of the IVC. We compared the incidence of hepatic vein anastomotic complications between these 2 methods. Nine of the 177 cases (5.3%) treated with the conventional technique were diagnosed with outflow obstruction. All 9 cases underwent hepatic vein reconstruction with the slit‐shaped hepatic vein anastomosis. In contrast, there were no cases of outflow obstruction in the 121 cases treated with the modified triangular anastomosis. The modified triangular technique of hepatic vein reconstruction with total clamping of the IVC was useful for preventing HVOO in pediatric LDLT. [ABSTRACT FROM AUTHOR]

Background: Hepatic venous outflow obstruction after paediatric liver transplantation is an unusual but critical complication.Objectives: To review the incidence, diagnosis and therapeutic modalities of hepatic venous outflow obstruction from a large national liver transplant unit.Materials and Methods: During the period from October 1992 to March 2016, 917 liver transplant procedures were performed with all types of grafts in 792 children. Transplants suspected to have early or delayed venous outflow obstruction were confirmed by percutaneous venography or surgical revision findings. Therapeutic intervention, recurrence and outcome were evaluated.Results: Twenty-six of 792 children (3.3%) experienced post-transplant hepatic venous outflow obstruction. These patients had been diagnosed from 1 day to 8.75 years after transplantation. Six occurred during the early post-transplant period; in three of them, the graft was lost. Seventeen patients were initially treated by balloon angioplasty with success; 11 of these experienced recurrences. Four stents were implanted; one was complicated by definitive occlusion. Three of the five surgical revisions were successful. The initial stenosis involved the inferior vena cava in 10 grafts, in isolation or associated with hepatic vein involvement. Mean follow-up was 79 months after transplantation. Eight grafts were lost.Conclusion: Acute postoperative hepatic venous outflow obstruction was associated with poor prognosis. Diagnostic venography should be performed if there is any suspicion of venous outflow obstruction, even if first-line examinations are normal. Stenosis frequently involved the inferior vena cava. Angioplasty was a safe and efficient treatment for venous outflow obstruction despite frequent recurrence. [ABSTRACT FROM AUTHOR]

Hepatic venous outflow obstruction (HVOO) is common in developing countries. It is a serious condition that clinically manifests with ascites from sinusoidal hypertension, and carries the risk of high mortality or development of liver cirrhosis. In the past, the eponym Budd–Chiari Syndrome (BCS) was used synonymously for HVOO. In the West, hepatic vein (HV) thrombosis caused by prothrombotic disorders is the main cause of HVOO. In the East, obliterative disease of hepatic portion of inferior vena cava induced by bacterial infection, now renamed hepatic vena cava syndrome, is the common cause of HVOO. These two diseases with different etiology, epidemiology, and natural history are at present grouped together under BCS causing much confusion. Sinusoidal obstruction syndrome, another important cause of HVOO at the level of the sinusoid and terminal HV, was left out in the classification of HVOO. In this article, the pathophysiology of sinusoidal hypertension is described, the term BCS is redefined, and a new classification of HVOO is suggested.

The patient was a boy of 7 years and 5 months of age, who underwent LDLT for acute liver failure at 10 months of age. HV stent placement was performed 8 months after LDLT because of intractable HV stenosis. At 7 years of age, his liver function deteriorated due to chronic rejection. The patient therefore underwent living donor liver retransplantation from his father. The HV was transected with the stent in situ. The IVC was resected due to stenosis. The pericardial cavity was opened and detached around the IVC to elongate the IVC. The divided ends of the IVC were joined by suturing to the posterior wall of the IVC. A new triangular orifice was made by adding an incision on the anterior wall of the IVC. The graft HV was then anastomosed to the new orifice with continuous sutures in the posterior wall and interrupted sutures in the anterior wall using 5-0 non-absorbable sutures. Doppler ultrasound showed a triphasic waveform. We successfully performed HV reconstruction without a vascular graft. This is a feasible procedure for overcoming HV stenosis in LDLT patients with an indwelling stent. [ABSTRACT FROM AUTHOR]

Hepatic vena cava syndrome, also known as membranous obstruction of inferior vena cava (IVC), was considered a rare congenital disease and classified under Budd-Chiari syndrome. It is now recognized as a bacterial infection-induced disease related to poor hygiene. Localized thrombophlebitis of the IVC at the site close to hepatic vein outlets is the initial lesion which converts on resolution into stenosis or complete obstruction, the circulatory equilibrium being maintained by development of cavo-caval collateral anastomosis. These changes persist for the rest of the patient's life. The patient remains asymptomatic for a variable period until acute exacerbations occur, precipitated by bacterial infection, resulting in deposition of thrombi at the site of the lesion and endophlebitis in intrahepatic veins. Large thrombus close to hepatic vein outlets results in ascites from hepatic venous outflow obstruction, which is followed by development of venocentric cirrhosis. Endophlebitis of intrahepatic veins results in ischemic liver damage and development of segmental stenosis or membrane. Acute exacerbations are recognized clinically as intermittent jaundice and/or elevation of aminotransferase or ascites associated with neutrophil leukocytosis and elevation of C-reactive protein; sonologically, they are recognized as the presence of thrombi of different ages in IVC and thrombosis of intrahepatic veins. Development of liver cirrhosis and hepatocellular carcinoma is related to severity or frequency of acute exacerbations and not to duration or type of caval obstruction. Hepatic vena cava syndrome is a common co-morbid condition with other liver diseases in developing countries and it should be considered in differential diagnosis in patient with intermittent elevation serum bilirubin and or aminotransferase or development of ascites and cirrhosis. [ABSTRACT FROM AUTHOR]

For hepatic venous outflow obstruction, alcoholic liver injury, and nonalcoholic fatty liver disease, the term "centrizonal injury disease" (CID) is used, because injury patterns in all three entities are similar. To elucidate CID-related CD34+ vessels (sinusoids and/or microvessels) and keratin 7+ hepatocytes (K7+ Hs), we examined a series of 41 liver tissue specimens obtained at autopsy and surgery, consisting of 32 CID cases and 9 controls. Centrizonal scars were found in 21 CID cases, and these were associated with centrizonal CD34+ vessels (P = 0.009) and centrizonal K7+ Hs (P < 0.001). Centrizonal coexistence of CD34+ vessels and K7+ Hs was observed in 22 CID cases (P = 0.057). These findings suggest close centrizonal proximity of scar, CD34+ vessels, and K7+ Hs in CID. However, centrizonal K7+ Hs without CD34+ vessels were observed in 21 CID cases. CD34+ vessels were detectable in all control samples and may represent the normal vascular bed. In 29 CID cases, centrizonal CD34+ vessel density was higher than that in controls. However, most appeared to be continuous with periportal and/or interlobular CD34+ vessels, and those CD34+ vessels restricted to centrizonal regions were focal and limited in seven CID cases. Centrizonal CD34+ vessels were associated with venoportal adhesions (P = 0.027). Our findings suggest that CID induces both venoportal adhesion-related structural distortion and expansion of normally present CD34+ vessels, which may result in increased centrizonal CD34+ vessel density. [ABSTRACT FROM AUTHOR]

Hepatic venous outflow obstruction ( HVOO) is a rare complication after liver transplantation ( LT) associated with significant morbidity and reduced graft survival. Endovascular intervention has become the first-line treatment for HVOO, but data on long-term outcomes are lacking. We have analysed outcomes after endovascular intervention for HVOO in 905 consecutive patients who received 965 full-size LT at our unit from January 2007 to June 2014. There were 27 (3%) patients who underwent hepatic venogram for suspected HVOO, with persistent ascites being the most common symptom triggering the investigation ( n = 19, 70%). Of those, only 10 patients demonstrated either stricture or pressure gradient over 10 mmHg on venogram, which represents a 1% incidence of HVOO. The endovascular interventions were balloon dilatation ( n = 3), hepatic vein stenting ( n = 4) and stenting with dilatation ( n = 3). Two patients required restenting due to stent migration. The symptoms of HVOO completely resolved in all but one patient, with a median follow-up period of 74 (interquartile range 39-89) months. There were no procedure-related complications or mortality. In conclusion, the incidence of HVOO in patients receiving full-size LT is currently very low. Endovascular intervention is an effective and safe procedure providing symptom relief with long-lasting primary patency. [ABSTRACT FROM AUTHOR]

Hepatic venous outflow obstruction (HVOO) is rare but serious complication after right lobe living donor liver transplantation (LDLT). Failure to identify this complication early can result in graft failure and even death. The early diagnosis and management of HVOO is very important. We report a case with this complication treated by endovascular stent placement in the early period after right lobe LDLT and review-related reports to explore the possible mechanism. A 43-year-old male with chronic liver disease underwent right lobe LDLT. On postoperative day 2, his liver function deteriorated. Hepatic venography showed an obstruction and thrombosis of mechanical heart valve with an element of torsion of the venous drainage proximal to the anastomosis of the right hepatic venous orifice and inferior vena cava. The obstruction was successfully treated by insertion of an expandable metallic stent. The result demonstrates that stent placement for obstruction is safe and effective. [ABSTRACT FROM AUTHOR]

This paper aimed to systematically review the survival of Budd-Chiari syndrome and to identify the most robust prognostic predictors. Overall, 79 studies were included. According to the treatment modalities, the median 1-, 5- and 10-year survival rate was 93, 83 and 73% after interventional radiological treatment; 81, 75 and 72.5% after surgery other than liver transplantation; 82.5, 70.2 and 66.5% after liver transplantation and 68.1, 44.4% and unavailable after medical therapy alone. According to the publication years, the median 1-, 5- and 10-year survival rate was 68.6, 44.4% and unavailable before 1990; 75.1, 69.5 and 57% during the year 1991-1995; 77, 69.6 and 65.6% during the year 1996-2000; 86.5, 74 and 63.5% during the year 2001-2005 and 90, 82.5 and 72% after 2006. Bilirubin, creatinine and ascites were more frequently identified as significant prognostic factors in univariate analyses. But their statistical significance was less frequently achieved in multivariate analyses. [ABSTRACT FROM AUTHOR]

Redundancy of hepatic vein in the venous outflow reconstruction of partial graft in paediatric liver transplantation may occur when the graft hepatic vein is longer than anticipated. We describe two cases with outflow obstruction due to hepatic vein redundancy. The correction of the redundancy was made by pulling the graft caudally and to the left or right side of the abdominal cavity as determined by Doppler ultrasonography.

The development of late-onset hepatic venous outflow obstruction ( LOHVOO) following pediatric living donor liver transplantation ( LDLT) can lead to uncontrollable fibrotic damage in liver grafts, even long-term patency of the graft outflow is achieved with appropriate therapeutic modalities. The aim of this study was to verify our hypothesis that some immunological responses, particularly cellular and/or antibody-mediated rejection (AMR), are associated with LOHVOO, which occurs following damage to liver sinusoidal endothelial cells in zone 3 of liver grafts. One hundred and eighty-nine patients underwent LDLT between May 2001 and December 2010 at our institute. Nine patients (4.8%) were identified as having LOHVOO. The preoperative factors, operative factors, and mortality, morbidity, and survival rates were examined and compared between the groups with and without LOHVOO. No statistical differences were observed between the groups with regard to preoperative factors, technical factors, or postoperative complications. However, Flow PRA reactivity was found to be a statistically significant risk factor for LOHVOO ( P = 0.006). The patients with both class I- and class II-reactive antibodies also had a significant risk of developing LOHVOO ( P = 0.03) and exhibited significantly higher retransplant rates. In conclusion, although further studies are needed to clarify this phenomenon, the pathophysiological mechanism underlying the development of LOHVOO after LDLT may be explained by immune-mediated responses that facilitate damage in zone 3 of liver grafts. [ABSTRACT FROM AUTHOR]

Aim Whether percutaneous transluminal balloon dilatation ( PTBD) or stent placement should be used in children with hepatic venous outflow obstruction ( HVOO) is still controversial. The aim of the present study was to retrospectively describe experience in diagnosis and treatment of HVOO and to evaluate the outcome of PTBD in HVOO patients after pediatric liver transplantation ( P-LT). Methods From January 2001 to January 2011, 54 children received P-LT at our center. The clinical features of children with HVOO analyzed included demography, type of donor and liver transplant, the new-onset symptoms, liver function test, interventional examination, and treatment and outcome. Results Three children were treated successfully with PTBD without stenting. All patients received percutaneous interventional management successfully. In the total of eight episodes of PTBD across the stenosis, the mean pressure gradient ± standard deviation was 16.6 ± 7.90 mmHg before PTBD and 6.8 ± 2.27 mmHg after PTBD. The difference was significant ( P < 0.05). All of the three HVOO patients were still surviving with primary graft functioning normally until the last follow up. Conclusion HVOO after P-LT should be taken seriously. PTBD is an effective and safe treatment for HVOO in younger patients subjected to P-LT and re-venoplasty is recommended even in patients with recurrent HVOO. [ABSTRACT FROM AUTHOR]

Background: Pregnancy management is a crucial issue in women with Budd-Chiari Syndrome (BCS) and there are no established guidelines on the management. Aim: To report our experience of pregnancy outcome with BCS. Material and Methods: We report outcome of 13 pregnancies in three women, with favourable outcome after the diagnosis of the condition and its treatment using intervention to bypass obstruction and anticoagulant therapy during pregnancy. Results: Three women had a total of 13 pregnancies; three after the diagnosis and decompressive treatment of the disease. Disease was diagnosed during index pregnancy in two women. Anticoagulation was given in all the three pregnancies (Robertson et al., Br J Haematol, 132:171-196, ). Pregnancies prior to diagnosis and treatment resulted in a live birth. Conclusion: Pregnancy does not seem to be a contraindication in well treated and controlled BCS. Maternal outcome is good with close multidisciplinary surveillance. Foetal outcome, however, may still be poor due to underlying prothrombotic condition. [ABSTRACT FROM AUTHOR]

Sakamoto S, Nakazawa A, Shigeta T, Uchida H, Kanazawa H, Fukuda A, Karaki C, Nosaka S, Kasahara M. Devastating outflow obstruction after pediatric split liver transplantation. Abstract: HVOO is a rare complication after pediatric LT, which may lead to graft failure. There are various causes of HVOO, such as mechanical anastomotic obstruction and SOS. A 10-month-old female underwent split LT from a deceased donor for ALF. Her postoperative course was uneventful. However, her liver function suddenly deteriorated a month later. A liver biopsy revealed centrilobular injury, and D-US suggested outflow obstruction. Venography was performed to reveal hepatic venous narrowing inside the graft. She received another graft from a living donor because of progressive graft failure in spite of successful venoplasty with stent insertion. The macroscopic findings of the explanted graft did not show an anastomotic stricture of the hepatic vein, although the pathological findings revealed necrosis of the first graft due to SOS. SOS might cause severe consequences with concomitant mechanical outflow obstruction after pediatric LT. [ABSTRACT FROM AUTHOR]

We present a 3-year-old child with Budd Chiari syndrome having idiopathic complete occlusion of all three major hepatic veins and small hepatic venules. Adequate antegrade flow in right hepatic vein was established by transfemoral balloon angioplasty followed by stenting of the same. Long term antiplatelet therapy was instituted. Medium term follow up reveals satisfactory antegrade flow and regression of symptoms. This strategy highlights an effective nonsurgical approach of restoring physiological pattern of hepatic sinusoidal blood flow in Budd Chiari syndrome. [ABSTRACT FROM AUTHOR]

HVOO may lead to graft dysfunction in LDLT. Balloon angioplasty is the first treatment for HVOO. However, some cases with recurrent HVOO need multiple interventions and require stent placement. The authors describe a pediatric case with recurrent HVOO requiring multiple stent placements. Her symptoms related to HVOO finally disappeared after the third stenting. A year later, follow-up liver biopsy did not show any dramatic change in perivenular fibrosis. From a review of our pediatric cases with HVOO requiring stent placement, the majority of them lost the grafts, because the timing of stent placement was too late to prevent the progression of fibrosis. In conclusion, stent placement should be considered in select cases of HVOO. Serial liver biopsies evaluating the degree of fibrosis are essential in determining the timing of stent placement. [ABSTRACT FROM AUTHOR]

: Background/AimsTo review our experience of thrombolytic therapy in patients with acute Budd Chiari syndrome (BCS).: MethodsRecords of 10 patients with BCS, treated by thrombolysis over a 12-year period were retrospectively analysed for demographics, clinical presentation/duration, primary disease, thrombolytic regimen, and follow-up. The same characteristics were also studied in previously reported patients. The agent used was recombinant tissue plasminogen activator (tPA) in all patients.: ResultsThrombolysis was used 12 times in 10 patients. Infusion was made systemically in three patients, into the hepatic artery in one patient, locally into a hepatic vein and/or IVC in four patients and locally within TIPS/portal vein in two patients. Only one infusion made systemically was partially successful. Adjunctive balloon angioplasty and/or stent insertion was undertaken for all eight procedures (in six patients) where local infusion was into the hepatic vein or TIPS. Six of these were ultimately successful (in five patients) and two were unsuccessful. Thrombolysis was more likely to be successful in the presence of a short history of thrombosis, when the thrombolytic agent was locally infused and when it was combined with a successful radiological procedure. Mean follow-up was 4.5 years (range 1–10 years). No serious bleeding complication occurred.: ConclusionsWe observed no benefit from thrombolysis when delivered systemically or arterially except in one case. Thrombolysis was useful in adjunctive management of BCS when the drug was infused locally into recently thrombosed veins that had appreciable flow following partial recanalisation. Thrombolysis was clearly of benefit in the repermeation of occluded/partially occluded hepatic veins/TIPS when early detection of new thrombus followed interventional procedures such as balloon angioplasty or stenting of hepatic veins. [Copyright &y& Elsevier]

Objective: To report the effectiveness of a mesoatrial shunt in the treatment of Budd-Chiari syndrome caused by combined hepatic vein and inferior vena caval block. Design: Retrospective study. Setting: Tertiary care hospital, India. Patients: 10 patients (4 men and 6 women; mean age 28, range 18-45) who had operations for Budd-Chiari syndrome between 1994 and 2000. Intervention: Mesoatrial shunt. Main outcome measures: Graft patency, survival, liver function and symptoms. Results: One patient died. All grafts were patent over a mean follow up period of 40 months (range 6-71). All survivors have normal liver function and were symptom free at the time of writing. Conclusion: Mesoatrial shunt is effective in the treatment of Budd-Chiari syndrome caused by combined hepatic vein and vena caval occlusion. [ABSTRACT FROM AUTHOR]

Abstract Background: Hepatic venous outflow obstruction (Budd–Chiari syndrome) is frequently encountered as a cause of portal hypertension at our centre. Methods and Results: We studied the clinical presentation, therapeutic modalities and outcome of 71 patients with hepatic venous outflow obstruction between 1992 and 1997. Twenty-seven patients presented with acute disease, while 44 had chronic presentation. Abdominal pain, distension, jaundice and upper gastrointestinal bleeding were the commonest presenting symptoms. The majority of patients had distended veins, hepatomegaly, splenomegaly, ascites and ankle oedema. The diagnosis was made on the basis of inferior vena cavography/functional hepatography and pulsed Doppler ultrasonography and/or liver biopsy in 39 patients and pulsed Doppler ultrasonography and/or liver biopsy in 32 patients. Pulsed Doppler ultrasonography accurately detected the site of the block in 31 of 39 patients (79.4%). The obstruction was in the hepatic vein in 20 patients, in the inferior vena cava in 10, and in both in 41 patients. Aetiologically, four had pregnancy-related disease, four tumour-related, three hypercoagulable states, 18 inferior vena cava membranes and 42 were idiopathic. Of 30 patients in whom liver biopsy was carried out, eight had centrizonal congestion and necrosis, 13 had mixed features and nine had established cirrhosis. Seven patients underwent a shunt operation and surgical membranotomy was carried out in one. Three patients (4.2%) died in the hospital. Conclusions: Hepatic venous outflow obstruction is a common problem; patients present with abdominal pain, distension, jaundice, distended veins, ascites and ankle oedema. Chronic presentation is more frequent. Pulsed Doppler ultrasound, venography and liver biopsy are very helpful in diagnosis. [ABSTRACT FROM AUTHOR]

Purpose: To compare the magnetic resonance imaging (MRI) features of benign liver lesions developed on Budd-Chiari syndrome (BCS) with those on Fontan-associated liver disease (FALD) and to describe their long-term progression., Materials and Methods: Patients with BCS or FALD who underwent MRI between 2010 and 2020 were retrospectively included. MRI features of nodules (≥ 5 mm) at baseline and at final follow-up were reviewed. The final diagnosis of benign lesion was based on a combination of clinical and biological data and findings at follow-up MRI examination., Results: Two-hundred and thirty benign liver lesions in 39 patients with BCS (10 men, 29 women; mean age, 36 ± 11 [SD] years; age range: 15-66 years) and 84 benign lesions in 14 patients with FALD (2 men, 12 women; mean age, 31 ± 10 [SD] years; age range: 20-48 years) were evaluated. On baseline MRI, BCS nodules were more frequently hyperintense on T1-weighted (183/230, 80%) and hypointense on T2-weighted (142/230; 62%) images, while FALD nodules were usually isointense on both T1- (70/84; 83%) and T2-weighted (64/84; 76%) images (all P< 0.01). Most lesions showed arterial phase hyperenhancement (222/230 [97%] vs. 80/84 [95%] in BCS and FALD, respectively; P = 0.28) but wash-out was more common in BCS (64/230 [28%] vs. 9/84 [11%]; P < 0.01). At follow-up, changes were more frequent in BCS nodules with more frequent disappearance (P < 0.01), changes in size, signal intensity on T2-weighted, portal, and delayed phase, and in the depiction of washout and capsule (all P ≤ 0.03)., Conclusion: MRI features of benign lesions are different at diagnosis and during the course of the disease between BCS and FALD. Changes in size and MRI features are more frequent in benign lesions developed in BCS., (Copyright © 2021 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved.)