Endovascular treatment of symptomatic hepatic venous outflow obstruction post major liver resection.

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.

Positional outflow obstruction as a cause of early refractory ascites post-pediatric living donor liver transplantation.

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.

An Update on the Management of Budd-Chiari Syndrome.

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.

Management of portal hypertension and ascites in polycystic liver disease.

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.

Type II Abernethy Malformation in a Patient with Primary Budd-Chiari Syndrome.

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.

Modified triangular hepatic vein reconstruction for preventing hepatic venous outflow obstruction in pediatric living donor liver transplantation using left lateral segment grafts.

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.

Diagnosis, treatment and outcome of hepatic venous outflow obstruction in paediatric liver transplantation: 24-year experience at a single centre.

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.

Hepatic vena cava syndrome: New concept of pathogenesis.

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.

Optimizing hepatic venous outflow reconstruction for hepatic vein stenosis with indwelling stent in living donor liver retransplantation.

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.

Long-term follow-up after endovascular treatment of hepatic venous outflow obstruction following liver transplantation.

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.

Impact of post-transplant flow cytometric panel-reactive antibodies on late-onset hepatic venous outflow obstruction following pediatric living donor liver transplantation.

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, FlowPRA 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.

Histologic Findings of Sinusoidal Dilatation and Congestion in Liver Grafts Do Not Correlate with Hepatic Venous Anastomotic Gradients.

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.

Long-term outcome of percutaneous interventions for hepatic venous outflow obstruction after pediatric living donor liver transplantation: experience from a single institute.

PURPOSE: To evaluate retrospectively the long-term outcome of percutaneous interventions for hepatic venous outflow obstruction (HVOO) occurring after pediatric living donor liver transplantation (LDLT). MATERIALS AND METHODS: Between October 1997 and December 2012, 48 patients (24 boys, 24 girls; median age, 6 y) who had undergone LDLT were confirmed to have HVOO using percutaneous hepatic venography and manometry. All patients underwent percutaneous interventions, including balloon angioplasty with or without stent placement. Technical success, clinical success, patency rates, stent placement, and major complications were evaluated. RESULTS: Technical success was achieved in 92 of 93 sessions (99.0%) and in 47 of 48 patients (97.9%), and clinical success was achieved in 41 of 48 patients (85.4%). During the follow-up period (range, 1-182 mo; median, 51.5 mo), 28 patients were treated with a single session of balloon angioplasty, and 20 patients who developed recurrent stenosis were treated with repeated percutaneous interventions. The rates of primary and primary-assisted patency at 1, 3, 5, and 10 years after balloon angioplasty were 64%, 57%, 57%, and 52% (primary patency) and 98%, 95%, 95%, and 95% (primary-assisted patency). Of six patients with stent placement, four had no recurrent HVOO after the stent placement, but two developed recurrent stenosis. The stent migrated to the right atrium in one patient. CONCLUSIONS: Percutaneous interventions were effective treatments for HVOO after LDLT.

Successful percutaneous transluminal balloon dilatation for hepatic venous outflow obstruction after pediatric liver transplantation: A series of cases.

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.

Outflow obstruction after living donor liver transplantation managed with a temporary vena cava filter: A case report.

INTRODUCTION: 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. PRESENTATION OF CASE: 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. DISCUSSION: 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. CONCLUSION: 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.

Budd-Chiari Syndrome Imaging Diagnosis: State of the Art and Future Perspectives.

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.

MR imaging features and long-term evolution of benign focal liver lesions in Budd-Chiari syndrome and Fontan-associated liver disease.

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 ±â€¯11 [SD] years; age range: 15-66 years) and 84 benign lesions in 14 patients with FALD (2 men, 12 women; mean age, 31 ±â€¯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.

Do Natural Portosystemic Shunts Need to Be Compulsorily Ligated in Living Donor Liver Transplantation?

BACKGROUND: Natural portosystemic shunt ligation practices in liver transplant vary widely across transplant centres and are frequently undertaken to prevent the serious consequence of portal steal phenomenon. No concrete indications have so far been convincingly identified for their management in living donor liver transplant. METHODS: We retrospectively studied the outcome of 89 cirrhotic patients who either did (n = 63) or did not (n = 25) undergo shunt ligation during living donor liver transplantation between 2017 and 2020. RESULTS: The incidence of early allograft dysfunction/nonfunction (P = 1.0) and portal venous complications (P = 0.555) were similar between the two groups. Although overall complications, biliary complications, and the composite of Grade III and IV complications were significantly higher in the nonligated group (P = 0.015, 0.052 and 0.035), 1- year graft and patient survival were comparable between them (P = 0.524). CONCLUSION: We conclude that shunt ligation in living donor liver transplantation may not always be necessary if adequate portal flow, good vascular reconstruction, and good graft quality have been ensured.

A case of hepatic venous outflow obstruction caused by migration of the remnant liver into the subphrenic space after extended posterior sectionectomy of the liver.

INTRODUCTION: 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. PRESENTATION OF CASE: 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. DISCUSSION: 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. CONCLUSION: HVOO after hepatectomy is rare but potentially fatal, and prevention and countermeasures should be discussed.