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OBJECTIVES In this study, we share our approach for care of patients with hepatic venous outlet obstruction after living-donor liver transplant. MATERIALS AND METHODS We retrospectively examined the demographic, clinical, and radiologic data of 35 patients who developed hepatic venous outlet obstruction after living-donor liver transplant. Patients were subgrouped on the basis of onset (8 patients with early onset [< 30 days posttransplant] and 27 patients with late onset [≥ 30 days posttransplant]) and postoperative survival (24 survivors, 11 nonsurvivors). RESULTS Patients ranged in age from 1 to 61 years (24 adults and 11 children). All adult patients had undergone right lobe living-donor liver transplant. In the pediatric group, 8 had undergone left lateral segment and 3 had undergone left lobe living-donor liver transplant. Nineteen adult patients and all 11 pediatric patients underwent hepatic venous reconstruction, with all procedures based on common large-opening drainage models using various vascular graft materials. Development of hepatic venous outlet obstruction occurred at mean posttransplant day 233 ± 298.5 in the adult patients and mean posttransplant day 139 ± 97.8 in the pediatric patients. After development of obstruction, the patients underwent 1-6 sessions (1.5 ± 1.1 sessions) of balloon angioplasty. After the first balloon angioplasty procedure, 25% of the adults and 36.3% of the pediatric patients developed recurrence. The early-onset and late-onset subgroups showed statistically significant differences in serum albumin (P = .01), underlying causes (P < .001), time from transplant to obstruction (P = .02), and time from transplant to last visit (P = .02). The survivor and nonsurvivor subgroups showed statistically significant differences in total bilirubin (P = .03) and time from transplant to last visit (P = .03). CONCLUSIONS Common large-opening reconstruction minimizes hepatic venous outlet obstruction development after living-donor liver transplant. Balloon angioplasty and/or stenting is almost always the first option in the care of this complication.

Orthotopic liver transplantation can be a surgically complex undertaking, with hepatic venous outflow obstruction occurring at a rate of 1%-6% due to inferior vena cava (IVC) torsion, compression, or anastomotic stenosis. In this report, we present 2 cases of immediate postoperative hepatic venous outflow obstruction in the setting of Budd-Chiari syndrome successfully treated with immediate IVC stenting. Although IVC stenting has been reported for management of long-term IVC anastomotic stenosis after orthotopic liver transplantation, use of stenting to address immediate postoperative caval outflow obstruction is less commonly described. We describe the potential utility of immediate stenting to improve outflow from the transplanted liver and highlight the value of this approach in addressing early postsurgical IVC pathology

Hepatic sinusoidal obstruction syndrome (SOS), historically known as veno-occlusive disease (VOD), is a disorder characterized by a hepatic venous outflow obstruction in the intrahepatic sinusoidal venules. Sequelae includes congestive hepatopathy, portal hypertension, and acute liver failure (1). SOS is traditionally seen in those undergoing chemoradiation therapy prior to hematopoietic stem cell transplantation (HSCT), or in the post-hematopoietic cell transplant setting manifesting as an obliterative venulitis (1).

Highlights • 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., 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.

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.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.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 P0.01). 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%]; P0.01). At follow-up, changes were more frequent in BCS nodules with more frequent disappearance (P0.01), changes in size, signal intensity on T2-weighted, portal, and delayed phase, and in the depiction of washout and capsule (all P ≤ 0.03).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.

Budd-Chiari syndrome (BCS) occurs when there is hepatic venous outflow obstruction. Chronic BCS may result in liver cirrhosis due to long-standing obstruction and tend to present late. We present the first case of BCS secondary to Janus tyrosine kinase 2 (JAK2) mutation resulting in "pseudocirrhosis" rather than cirrhosis of the liver. Pseudocirrhosis clinically and radiologically mimics cirrhosis without the classical histopathological changes, and it is usually associated with metastatic cancers.

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 Budd-Chiari syndrome from China. The inferior vena cava obstruction was treated with percutaneous balloon angioplasty; close follow-up was elected for the Abernethy malformation.

Contains fulltext : 215273.pdf (Publisher’s version ) (Open Access) 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.

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. 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. From February 2014 to May 2018, 56 ERAT procedures were performed. Eleven patients with an RLV/SLV

AIM To assess the efficacy and safety of balloon dilatation for the treatment of hepatic venous outflow obstruction (HVOO) following pediatric liver transplantation. METHODS A total of 246 pediatric patients underwent liver transplantation at our hospital between June 2013 and September 2016. Among these patients, five were ultimately diagnosed with HVOO. Seven procedures (two patients underwent two balloon dilatation procedures) were included in this analysis. The demographic data, types of donor and liver transplant, interventional examination and therapeutic outcomes of these five children were analyzed. The median interval time between pediatric liver transplantation and balloon dilatation procedures was 9.8 mo (range: 1-32). RESULTS Five children with HVOO were successfully treated by balloon angioplasty without stent placement, with seven procedures performed for six stenotic lesions. All children underwent successful percutaneous intervention. Among these five patients, four were treated by single balloon angioplasty, and these patients did not develop recurrent stenosis. In seven episodes of balloon angioplasty across the stenosis, the pressure gradient was 12.0 ± 8.8 mmHg before balloon dilatation and 1.1 ± 1.5 mmHg after the procedures, which revealed a statistically significant reduction (P < 0.05). The overall technical success rate among these seven procedures was 100% (7/7), and clinical success was achieved in all five patients (100%). The patients were followed for 4-33 mo (median: 15 mo). No significant procedural complications or procedure-related deaths occurred. CONCLUSION Balloon dilatation is an effective and safe therapeutic option for HVOO in children undergoing pediatric liver transplantation. Venous angioplasty is also recommended in cases with recurrent HVOO.

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. Keywords: Budd Chiari Syndrome, Thrombosis, Hepatic Venous Outflow Obstruction, Ascites

Context.—Cardiac hepatopathy and Budd-Chiari syndrome are 2 forms of hepatic venous outflow obstruction with different pathophysiology but overlapping histologic findings, including sinusoidal dilation and centrilobular necrosis. Objective.—To determine whether a constellation of morphologic findings could help distinguish between the 2 and could suggest the diagnoses in previously undiagnosed patients. Design.—We identified 26 specimens with a diagnosis of cardiac hepatopathy and 23 with a diagnosis of Budd-Chiari syndrome. Slides stained with hematoxylin and eosin and with trichrome were evaluated for several distinctive histologic findings. Results.—Features common to both forms of hepatic outflow obstruction included sinusoidal dilation and portal tract changes of fibrosis, chronic inflammation, and bile ductular reaction. Histologic findings significantly more common in cardiac hepatopathy included pericellular/sinusoidal fibrosis and fibrosis around the central vein. Only centrilobular hepatocyte dropout/necrosis was significantly more common in Budd-Chiari, regardless of duration. Conclusions.—The finding of pericellular/sinusoidal fibrosis in cardiac hepatopathy compared with Budd-Chiari is not unexpected, given the chronic nature of most cardiac hepatopathy. Portal tract changes are common in both forms of hepatic outflow obstruction and should not deter one from making the diagnosis of hepatic outflow obstruction. Fibrosis along sinusoids and around the central vein may be suggestive of cardiac hepatopathy in biopsies from patients without a prior diagnosis.

Hepatic venous outflow obstruction in Budd Chiary syndrome can be at various level causing symptoms due to hepatic congestion. Effective treatment strategy to relieve the obstruction by combination of medical therapy, transcatheter angioplasty and stent placement has good clinical outcome.

Objectives Hepatic vein outflow obstruction in liver transplantation can lead to graft or patient loss. We used an intrauterine balloon to overcome this complication in 13 liver transplant recipients. Here, we report the results of these cases; our report, as far as we know, involves the highest number of patients on this issue. Materials and methods Positional hepatic vein outflow obstruction was diagnosed in 13 of 651 liver transplant recipients between January 2014 and December 2016. The grafts were repositioned by intrauterine balloon placed to the right subdiaphragmatic area. Data of donors, recipients, and grafts and postoperative courses were analyzed. Results Of the 13 patients, 9 were men, with age range of patients of 22 to 70 years. The amount of saline used to inflate the balloon was variable (200-450 cm3), and hepatic vein outflow obstruction was relieved after balloon implantation in all patients. There were no balloon-related complications. Removal was done at bedside, without any additional sedation or any additional skin incision on days 2 to 15. Doppler ultrasonography scans were performed before and after the balloon removal. There were no vascular complications after removal. Conclusions Intrauterine balloon can be safely and efficiently used for hepatic vein outflow obstruction during liver transplant when needed.

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Background Our aim in this study was to evaluate long-term efficiency of hepatic venous balloon angioplasty (BA) and stent placement (SP) for hepatic venous outflow obstruction (HVOO) in pediatric liver transplantation (LT). Methods From January 1999 to September 2016, 262 pediatric patients underwent LT at our hospital. Ten were diagnosed with HVOO, which included 8 living donor grafts and 2 split liver grafts. BA and SP were used in management of these 10 patients with HVOO. After intervention, Doppler ultrasound (DUS) was the major follow-up modality for comparing efficiency of BA and SP. Results The incidence of HVOO was 3.8% (10 of 262) in our pediatric LTs. Of the 10 HVOO cases, 5 had SP, 3 had BA once, 1 had BA twice, and 1 had BA twice along with SP. The patent hepatic vein was maintained after a mean follow-up of 7.4 (range, 0.04–17) years. Recurrent rate of HVOO after BA was 42%. Neither recurrent HVOO nor stent migration occurred after SP and throughout long-term follow-up. Conclusion Hepatic venous SP was found to be more effective and safe than BA for treatment of HVOO in pediatric LT for long-term follow-up.

Backgrounds/Aims Preservation of the native inferior vena cava using a large graft during adult whole liver transplantation is associated with a potential risk of hepatic venous outflow compression/obstruction, which may adversely affect both graft and short-term patient outcomes. Intraoperative placement of materials to restore adequate hepatic venous outflow can overcome this complication. Methods Data of patients who underwent liver transplantation between 2011 and 2016 were retrospectively reviewed. All cases of hepatic venous outflow obstruction due to large graft size managed via intraoperative intervention were analyzed. The literature was searched for studies reporting adult cases of hepatic venous outflow obstruction following whole liver transplantation managed extrahepatically. Results Three patients diagnosed with intraoperative hepatic venous outflow obstruction due to large graft size were managed via retro-hepatic placement of breast implants (2 cases) or abdominal pads (1 case). It was successfully carried out in all cases. Four studies including 15 patients were identified in the literature search. Different types of materials such as inflatable materials (Foley catheter, Blakemore balloon), surgical gloves or breast implants, were used. Conclusions Placement of inflatable materials leads to gradual deflation in the postoperative period, which might obviate the need for reoperation. Breast implants could be left in place indefinitely due to their bio-inert nature.

HVOO is a rare complication after LT and an important cause of graft failure. Balloon venoplasty is the first-line treatment for HVOO, but the effect of repeated balloon venoplasty and stent placement for HVOO recurrence after pediatric LDLT remains unclear. Between 1998 and 2016, 147 pediatric patients underwent LDLT in our institution. Among them, the incidence of HVOO and the therapeutic strategy were retrospectively reviewed. Ten patients were diagnosed with HVOO. All the patients underwent LLS grafts. Median age at the initial endovascular intervention was 2.7 years (range, 5 months-8 years). The median interval between the LDLT and the initial interventional radiology was 2.7 months (range, 29 days-35.7 months). Four patients experienced no recurrence after a single balloon venoplasty; 6 underwent balloon venoplasty more than 3 times because of HVOO recurrence; and 2 underwent stent placement due to the failure of repeated balloon venoplasty. All patients are alive with no symptoms of HVOO. The HVOO recurrence-free period after the last intervention ranged from 20 days to 15.5 years (median, 8.9 years). Repeated balloon venoplasty may prevent unnecessary stent placement to treat recurrent HVOO after pediatric LDLT.

Primary Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow obstruction from small hepatic veins to the inferior vena cava (IVC), due to thrombosis or fibrous stenosis of these veins. In Western countries, one or multiple prothombotic diseases are usually identified, mainly myeloproliferative neoplasms. New noninvasive diagnostic tools facilitate the diagnosis of these causes which may be difficult to detect in the presence of portal hypertension (PH) or liver failure.

Objectives To evaluate the safety and patency of self-expanding stents to treat hepatic venous outflow obstruction after orthotopic liver transplantation. To evaluate differences in the response between patients with early obstruction and patients with late obstruction. Material and methods This is a retrospective analysis of 16 patients with hepatic venous outflow obstruction after liver transplantation treated with stents (1996–2011). Follow-up included venography/manometry, ultrasonography, CT, and laboratory tests. We did a descriptive statistical analysis of the survival of patients and stents, technical and clinical success of the procedure, recurrence of obstruction, and complications of the procedure. We also did an inferential statistical analysis of the differences between patients with early and late obstruction. Results The mean follow-up period was 3.34 years (21–5331 days). The technical success rate was 93.7%, and the clinical success rate was 81.2%. The rate of complications was 25%. The survival rates were 87.5% for patients and 92.5% for stents. The rate of recurrence was 12.5%. The rate of primary patency was 0.96 (95% CI 0.91–1) at 3 months, 0.96 (95% CI 0.91–1) at 6 months, 0.87 (95% CI 0.73–1) at 12 months, and 0.87 (95% CI 0.73–1) at 60 months. There were no significant differences between patients with early and late obstruction, although there was a trend toward higher rates of primary patency in patients with early obstruction ( P = 0.091). Conclusions Treating hepatic venous outflow obstruction after orthotopic transplantation with self-expanding stents is effective, durable, and effective. There are no significant differences between patients with early obstruction and those with late obstruction.

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.

The aim of this study was to analyze the outcomes of children added to the waiting list for deceased donor liver transplantation (DDLT) and the results of DDLT in a single Japanese center. Forty-seven children were listed on the organ allocation system for DDLT. The priority points related to the medical status of each patient were evaluated and stratified into four categories; 10, 8, 6, and 3 points. The clinical data were collected from the medical records, and the outcomes were analyzed. There were 10 priority points in 25 patients, 6 points in 13 and 3 points in 9. Ten recipients (21.3 %); 7 patients with 10 points and 3 patients with 6 points, underwent DDLT. Seven out of the 10 recipients received split/reduced liver grafts. The surgical complications consisted of biliary stricture, hepatic venous outflow obstruction, intraabdominal abscess and intraabdominal bleeding. Two recipients, who were critically-ill before DDLT, died due to sepsis. The one-year graft survival rate was 70.0 %, with a median follow-up period of 6.4 months. The initial experience with pediatric DDLT in our series was satisfactory. Split LT of deceased donor organs may have the potential to resolve the serious organ shortage in Japan.

Budd-Chiari syndrome (BCS) or hepatic venous outflow obstruction, is a rare and complex clinical entity, with diverse aetiology and a wide range of presentations. Its rarity and heterogeneity create significant challenges for evidence-based clinical decision-making, particularly in the even more infrequent setting of acute BCS presentation. Here we present the case of a young woman with acute, severe BCS, where treatment alternatives were limited due to a significant IVC thrombus burden. A clearly defined acute onset allowed the prompt use of systemic thrombolysis, conducted in consultation with a multi-disciplinary team of clinicians, with close monitoring for both treatment response and complications. Her treatment resulted in resolution of hepatic vein occlusion and prevention of the potentially significant clinical sequelae. We review the literature and discuss current opinion in the management of BCS.

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.

Budd-Chiari syndrome (BCS) is the liver disease resulting from hepatic venous outflow obstruction comprising a triad of abdominal discomfort, hepatomegaly and ascites. Advances in the management of this disorder over the last three decades have dramatically improved survival. We present a review of the management of BCS followed by a case which illustrates some key points in the diagnosis and treatment of this condition.

To investigate Doppler US and catheter venogram correlates to improve detection of transplant HVOO and avoid unnecessary invasive imaging procedures.A retrospective review was performed in all pediatric OLT patients undergoing catheter venography of the hepatic veins between 2007 and 2017 at a single large tertiary pediatric liver transplant institution.Forty-four transplant hepatic venograms in 32 OLT patients were included (mean 1.38, range 1-4 venograms per patient). All venograms were preceded by an independent Doppler US examination. Twenty-one (47.7%) venograms were performed for the investigation of suspected HVOO based on Doppler US alone, 19 (43.2%) were performed for TJLB without suspected HVOO, 4 (9.1%) were performed for both. Sixteen (36.3%) instances of50% anastomotic stenosis were identified. Mean peak anastomotic velocities were 208 cm/s and 116 cm/s in the presence and absence of a50% venographic stenosis, respectively (P 0.004). In all cases where there was a monophasic waveform seen on Doppler US, there was a 50% stenosis seen on hepatic vein venogram. In all cases where a triphasic waveform was seen on Doppler US, there was no stenosis seen on hepatic vein venogram.While a Doppler US velocity threshold providing both high sensitivity and specificity has yet to be identified, increasing peak anastomotic velocity and decreasing intrahepatic venous velocity correlate strongly with venographic outflow stenosis. The presence of a triphasic intrahepatic waveform provides good NPV.

Hepatic venous outflow obstruction is a rare but serious complication after liver transplantation. We report ultrasound-guided percutaneous transhepatic stent placement in a 9-month-old infant with a left lateral split liver transplantation with near-complete hepatic vein obstruction.

Budd-Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atriocaval junction. BCS is a complex disease with a wide spectrum of aetiologies and presentations. This article reviews the current literature with respect to presentation, management and prognosis of the disease. Medical, interventional and surgical management of BCS is discussed. Particular attention is paid to interventional and surgical aspects of management. The review is augmented by images, which provide a clinical corollary to the text.

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.

Background: Hepatic venous outflow obstruction (HVOO) is a rare disorder that occurs predominantly due to a hypercoagulable state. The syndrome may result from hepatic vein obstruction, inferior vena cava obstruction or a combination of both and manifests with post-sinusoidal portal hypertension. The presentation may be fulminant with poor prognosis or as either acute, subacute or chronic forms with relatively better prognosis. Portosystemic shunt surgery (PSS) has thus far been the mainstay of treatment for HVOO. However, over the last decade, transjugular intrahepatic portosystemic shunt (TIPS) and liver transplantation have emerged as viable options. This review aims to evaluate the available treatment options and the current relevance of PSS for the management of HVOO. Methods: A literature review on investigations and treatment was performed using Medline and additional library searches. Results: Portosystemic shunts form the mainstay of treatment for patients with subacute presentation (preserved liver function) with medically intractable ascites or recurrent variceal hemorrhage. Excellent results with 5-year survival of more than 90% have been reported from specialized centers. The main limitation for PSS is the reported perioperative mortality of 10–20% and a declining technical expertise for such surgery. Liver transplantation with disease-specific 5-year survival between 50 and 95% is presently the treatment of choice for patients with fulminant presentation, end-stage liver disease (ESLD), unshuntable anatomy or decompensation after PSS. TIPS may be preferable for sick patients with acute presentation with isolated hepatic vein thrombosis or as a temporizing measure for those with ESLD awaiting transplantation. The drawback of TIPS is late shunt dysfunction that occurs in more than 50% of patients at 1 year. Conclusions: Due to rarity of the disorder there is a lack of trials comparing the different treatment modalities. Hence, the current treatment recommendations are based on retrospective studies. In a select group of HVOO patients (subacute presentation with preserved liver function), PSS remains the treatment of choice with excellent long-term results.

To evaluate retrospectively the endovascular management of hepatic venous outflow obstruction after piggyback orthotopic liver transplantation.The study was performed with the approval and under the guidelines of the institutional review board and complied with the Health Insurance Portability and Accountability Act. Informed consent from patients was not required by the institutional review board for this retrospective study. From 1995 to 2003, 13 patients (eight male, five female), including 12 adults and one adolescent (age range, 14-67 years; median age, 52 years), underwent endovascular treatment of hepatic venous outflow obstruction after piggyback orthotopic liver transplantation. Patients gave informed consent for all procedures. Eleven patients received whole livers, and two received living-related donor right liver lobes. Four underwent repeat piggyback orthotopic liver transplantation prior to intervention. Primary stent placement was performed in 12 patients. One patient refused primary stent placement and chose venoplasty alone, but required a stent 5 months later. Short balloon-expandable stents (mean diameter, 14.6 mm +/- 1.1 [standard deviation]) were used to minimize jailing of branch vessels and to resist recoil. Pre- and post-procedural pressure gradients were measured. Follow-up included venography, cross-sectional imaging, and laboratory tests. The Wilcoxon signed rank test or the sign test was performed to compare pre- and post-procedural pressure gradients, body weights, and laboratory values.Technical success (pressure gradientor = 3 mm Hg) was achieved in 13 of 13 patients, and clinical success, in 12 of 13. Mean pre- and post-procedural pressure gradients were 13.0 mm Hg +/- 1.4 and 0.8 mm Hg +/- 0.3. Mean interval from transplantation to intervention was 348 days +/- 159. Mean follow-up was 678 days (range, 16-2880 days). Technical success did not result in clinical improvement in one patient. Biopsy demonstrated severe hepatic necrosis, likely from prolonged venous congestion, and the patient required repeat transplantation. Only one patient required reintervention for stent migration, and no other complications occurred. No significant restenosis was encountered after stent placement.Hepatic venous outflow obstruction is an uncommon but potentially fatal complication of piggyback orthotopic liver transplantation. Endovascular treatment with balloon-expandable stents is effective, safe, and apparently durable.

Hepatic venous drainage in liver transplantation may be reduced to the level of caval anastomosis producing an obstruction degree and leading to serious vascular complication such as the acute Budd-Chiari syndrome, which may result in organ loss. Outflow obstruction may be caused by lack of technique in caval anastomosis or by allograft malposition as a consequence of anatomical graft and recipient conditions. Fixation of the round ligament, placement of bowel loops and use of tissue expanders have been described to stabilize graft position during liver transplantation with related procedure complications. We report our experience of a simple homemade device using a surgical glove expander that allowed us to successfully avoid outflow obstruction in all of nine treated patients. No device related complications occurred. In malposed liver allografts, we strongly suggest the use of this simple and safe device to avoid hepatic venous outflow obstruction on condition that the device is early removed within 48 hours.

Vascular complications after orthotopic split liver transplantation in children result in significant post-operative complications.A review of children undergoing liver transplantation from 2004 to 2006 was undertaken. The data was obtained based on a proforma-based analysis.Seven of 106 transplants were identified to have hepatic venous outflow obstruction (HVOO) of whom five were males and two were females. Median age at transplant was 8 years (range 3 years 4 months-15 years). The median donor-to-recipient weight ratio was 2.78 (range 0.97-6.15). ANASTOMOTIC TECHNIQUE: Hepatic vein-IVC in four, Hepatic vein-hepatic vein (HV-IVC) confluence in two and cavo hepatic in one. Ascites was the commonest post op manifestation of HVOO. Although Doppler USG was useful in identifying the venous outflow obstruction, venography confirmed the exact site of obstruction aiding in therapeutic dilatation. Three of seven cases had early onset (1 month) while 4/7 had late onset (1 month). 5/7 underwent venography and dilatation, of whom three are well and one is awaiting a repeat venography and dilatation. 2/7 died without intervention and 1/7 died in the waiting list for retransplantation.The diagnosis of HVOO requires a high index of suspicion, prompting early venography to manage HVOO successfully. Technical steps to avoid HVOO are to keep the hepatic vein-caval anastomosis short and wide with triangulation and to avoid graft rotation at the hepato caval junction.

The Budd-Chiari syndrome is a rare but serious condition defined as hepatic venous outflow obstruction. This article presents a case of acute Budd-Chiari syndrome in a 43-year-old woman with activated protein C resistance while she was being treated with intravenous heparin infusion for previously documented deep venous thrombosis. Activated protein C resistance caused by factor V Leiden mutation has emerged as the most prevalent hereditary cause of venous thromboembolism and should be considered as an etiologic factor in patients with Budd-Chiari syndrome.

To describe the outcome of selective management of hepatic venous outflow obstruction (HVOO), based on its presentation and liver function, we reviewed the records of 49 consecutive patients managed at our institution between 1984 and 1993. Twenty-six patients were managed surgically, 12 nonsurgically, and 11 were not treated. Portosystemic shunts (PSS) were performed in 18 patients (patency 83%). Two patients (11%) died postoperatively, 11 (61%) did well (mean follow-up 6.4 years), three (17%) required subsequent orthotopic liver transplantation, and two (11%) died of late liver failure. PSS remained patent if the preoperative pressure gradient between the portal vein and the infrahepatic inferior vena cava was greater than 10 mm Hg and across the intrahepatic inferior vena cava 18 mm Hg or less. All six orthotopic liver transplantations (three as primary treatment and three after failed PSS) were successful (mean follow-up 4.8 years). Five patients underwent other procedures. Nine (75%) of the 12 nonsurgically treated patients did well (mean follow-up 3.8 years). The most important predictor of successful outcome after PSS or medical management was the degree of liver function. All 11 untreated patients died either of end-stage liver failure (n = 7; 63%) or of severe comorbid disease (n = 4; 37%). In patients with preserved liver function, medical management of HVOO can be successful early in the course of the disease; a late presentation necessitates PSS. Orthotopic liver transplantation should be employed in patients with liver failure and may decrease the high mortality rate of HVOO.

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.

Summary 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.

The Budd-Chiari Syndrome is associated with hepatic venous outflow obstruction. This syndrome is rare in infants and children. As this syndrome is uncommon, especially in the paediatric age group, misdiagnoses and delays in the diagnosis are frequent. A high index of clinical suspicion along with a radiological aid and a histopathological correlation can lead to an early diagnosis and an appropriate management in such cases. This case report highlights the occurrence of this rare condition in the paediatric age group as well as it redefines the salient features of this syndrome.

The aim of this study was to evaluate retrospectively the outcome of percutaneous transluminal venoplasty (PTV) after venous pressure measurement in patients with hepatic venous outflow obstruction following living donor liver transplantation (LDLT). We studied 24 consecutive patients suspected of having hepatic venous outflow obstruction after LDLT. Pressure gradients were measured proximal and distal to the lesion, and gradient values >3 mmHg were considered hemodynamically significant. We evaluated the technical success, complications, outcome of venoplasty and recurrence, and the patency rate. In all, 11 female patients manifested a pressure gradient >3 mmHg across the anastomotic site; they underwent subsequent PVT. The initial balloon venoplasty procedure was technically successful in 10 of the 11 patients (91%), and the pressure gradient was reduced from 5.8 to 1.1 mmHg (P < 0.01). Clinical improvement was observed in 9 of these 10 patients; one patient failed to improve and underwent retransplantation. Recurrent obstruction occurred in four patients; they underwent PTV with (n = 2) or without (n = 2) stent placement. There were no major procedural complications. PTV following venous pressure measurement is an effective and safe treatment for venous outflow obstruction in patients subjected to LDLT. In patients with recurrent obstruction, re-venoplasty is recommended.