Rectal arterio-portal fistula: An unusual cause of persistent bleeding per rectum following a proximal spleno-renal shunt

Gastrointestinal arterio-venous malformations are a known cause of gastrointestinal bleeding. We present a rare case of persistent rectal bleeding due to a rectal arterio-portal venous fistula in the setting of portal hypertension secondary to portal vein thrombosis. The portal hypertension was initially surgically treated with splenectomy and a proximal splenorenal shunt. However, rectal bleeding persisted even after surgery, presenting us with a diagnostic dilemma. The patient was re-evaluated with a computed tomography mesenteric angiogram which revealed a rectal arterio-portal fistula. Arterio-portal fistulas are a known but rare cause of portal hypertension, and possibly the underlying cause of continued rectal bleeding in this case. This was successfully treated using angiographic localizationand super-selective embolization of the rectal arterioportal venous fistula via the right internal iliac artery. The patient subsequently went on to have a full term pregnancy. Through this case report, we hope to high-light awareness of this unusual condition, discuss the diagnostic workup and our management approach.     

Positron emission tomography/computed tomography with (18)F-fluorodeoxyglucose identifies tumor growth or thrombosis in the portal vein with hepatocellular carcinoma

Patients suffering from hepatocellular carcinoma (HCC) with tumor thrombus in the portal vein generally have a poor prognosis. Portal vein tumor thrombus must be distinguished from portal vein blood thrombus, and this identification plays a very important role in management of HCC. Conventional imaging modalities have limitations in discrimination of portal vein tumor thrombus. The application of positron emission tomography (PET) with 18F-fluorodeoxyglucose (18F-FDG) for discrimination between tumor extension and blood thrombus has been reported in few cases of HCC, while portal tumor thrombosis and portal vein clot identified by 18F-FDG PET/CT in HCC patients has not been reported so far. We present two HCC cases, one with portal vein tumor thrombus and one thrombosis who were identified with 18F-FDG PET/CT. This report illustrates the complimentary value of combining the morphological and functional imaging in achieving a correct diagnosis in such clinical situations.     

Liver function impairment in liver transplantation and after extended hepatectomy

Extended hepatectomy,or liver transplantation of reduced-size graft,can lead to a pattern of clinical manifestations,namely"post-hepatectomy liver failure"and"small-for-size syndrome"respectively,that can range from mild cholestasis to irreversible organ non-function and death of the patient.Many mechanisms are involved in their occurrence but in the recent past,high portal blood flow through a relatively small liver vascular bed has taken a central role.Therefore,several techniques of inflow modulation have been attempted in cases of portal hyperperfusion first in liver transplantation,such as portocaval shunt,mesocaval shunt,splenorenal shunt,splenectomy or ligation of the splenic artery.However,high portal flow is not the only factor responsible,and before major liver resections,preoperative assessment of the residual liver function is necessary.Techniques such as portal vein embolization or portal vein ligation can be adopted to increase the future liver volume,preventing posthepatectomy liver failure.More recently,a new surgical procedure,that combines in situ splitting of the liver and portal vein ligation,has gradually come to light,inducing remarkable hypertrophy of the healthy liver in just a few days.Further studies are needed to confirm this hypothesis and overcome one of the biggest issues in the field of liver surgery.     

A half century (1961-2011) of applying microsurgery to experimental liver research

The development of microsurgery has been dependent on experimental animals. Microsurgery could be a very valuable technique to improve experimental models of liver diseases. Microdissection and microsutures are the two main microsurgical techniques that can be considered for classifying the experimental models developed for liver research in the rat. Partial portal vein ligation, extrahepatic cholestasis and hepatectomies are all models based on microdissection. On the other hand, in portacaval shunts, orthotopic liver transplantation and partial heterotopic liver transplantation, the microsuture techniques stand out. By reducing surgical complications, these microsurgical techniques allow for improving the resulting experimental models. If good experimental models for liver research are successfully developed, the results obtained from their study might be particularly useful in patients with liver disease. Therefore experimental liver microsurgery could be an invaluable way to translate laboratory data on liver research into new clinical diagnostic and therapeutic strategies.     

Intestinal permeability in the pathogenesis of liver damage: From non-alcoholic fatty liver disease to liver transplantation

The intimate connection and the strict mutual cooperation between the gut and the liver realizes a functional entity called gut-liver axis.The integrity of intestinal barrier is crucial for the maintenance of liver homeostasis.In this mutual relationship,the liver acts as a second firewall towards potentially harmful substances translocated from the gut,and is,in turn,is implicated in the regulation of the barrier.Increasing evidence has highlighted the relevance of increased intestinal permeability and consequent bacterial translocation in the development of liver damage.In particular,in patients with non-alcoholic fatty liver disease recent hypotheses are considering intestinal permeability impairment,diet and gut dysbiosis as the primary pathogenic trigger.In advanced liver disease,intestinal permeability is enhanced by portal hypertension.The clinical consequence is an increased bacterial translocation that further worsens liver damage.Furthermore,this pathogenic mechanism is implicated in most of liver cirrhosis complications,such as spontaneous bacterial peritonitis,hepatorenal syndrome,portal vein thrombosis,hepatic encephalopathy,and hepatocellular carcinoma.After liver transplantation,the decrease in portal pressure should determine beneficial effects on the gut-liver axis,although are incompletely understood data on the modifications of the intestinal permeability and gut microbiota composition are still lacking.How the modulation of the intestinal permeability could prevent the initiation and progression of liver disease is still an uncovered area,which deserves further attention.     

Can a fibrotic liver afford epithelial-mesenchymal transition?

The question whether epithelial-mesenchymal transition(EMT) occurs during liver fibrogenesis is a controversial issue. In vitro studies confirm that hepatocytes or cholangiocytes undergo EMT upon transforming growth factor β(TGF-β) stimulation, whereas in vivo experiments based on genetic fate mapping of specific cell populations suggest that EMT does not occur in fibrotic animal models. In this review we present current data supporting or opposing EMT in chronic liver disease and discuss conditions for the occurrence of EMT in patients. Based on the available data and our clinical observations we hypothesize that EMT-like alterations in liver cirrhosis are a side effect of high levels of TGF-β and other pro-fibrotic mediators rather than a biological process converting functional parenchyma, i.e., hepatocytes, into myofibroblasts at a time when essential liver functions are deteriorating.     

Clinical characteristics of idiopathic portal hypertension

Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism（s） is （are） unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that ＂not all varices mean cirrhosis＂.     

Complications arising in simple and polycystic liver cysts

Liver cysts are common,affecting 5%-10% of the population.Most are asymptomatic,however 5% of patients develop symptoms,sometimes due to complications and will require intervention.There is no consensus on their management because complications are so uncommon.The aim of this study was to perform a collected review of how a series of complications were managed at our institutions.Six different patients presenting with rare complications of liver cysts were obtained from Hepatobiliary Units in the United Kingdom and The Netherlands.History and radiological imaging were obtained from case notes and computerised radiology.As a result,1 patient admitted with inferior vena cava obstruction was managed by cyst aspiration and lanreotide;1 patient with common bile duct obstruction was first managed by endoscopic retrograde cholangiopancreatography and stenting,followed by open fenestration;1 patient with ruptured cysts and significant medical co-morbidities was managed by percutaneous drainage;1 patient with portal vein occlusion and varices was managed by open liver resection;1 patient with infected cysts was treated with intravenous antibiotics and is awaiting liver transplantation.The final patient with a simple liver cyst mimicking a hydatid was managed by open liver resection.In conclusion,complications of cystic liver disease are rare,and we have demonstrated in this series that both operative and non-operative strategies have defined roles in management.The mainstays of treatment are either aspiration/sclerotherapy or,alternatively laparoscopic fenestration.Medical management with somatostatin analogues is a potentially new and exciting treatment option but requires further study.     

Blood loss,predictors of bleeding,transfusion practice and strategies of blood cell salvaging during liver transplantation

Blood loss during liver transplantation (OLTx) is a common consequence of pre-existing abnormalities of the hemostatic system,portal hypertension with multiple collateral vessels,portal vein thrombosis,previous abdominal surgery,splenomegaly,and poor "functional" recovery of the new liver.The intrinsic coagulopathic features of end stage cirrhosis along with surgical technical difficulties make transfusion-free liver transplantation a major challenge,and,despite the improvements in understanding of intraoperative coagulation profiles and strategies to control blood loss,the requirements for blood or blood products remains high.The impact of blood transfusion has been shown to be significant and independent of other well-known predictors of posttransplant-outcome.Negative effects on immunomodulation and an increased risk of postoperative complications and mortality have been repeatedly demonstrated.Isovolemic hemodilution,the extensive utilization of thromboelastogram and the use of autotransfusion devices are among the commonly adopted procedures to limit the amount of blood transfusion.The use of intraoperative blood salvage and autologous blood transfusion should still be considered an important method to reduce the need for allogenic blood and the associated complications.In this article we report on the common preoperative and intraoperative factors contributing to blood loss,intraoperative transfusion practices,anesthesiologic and surgical strategies to prevent blood loss,and on intraoperative blood salvaging techniques and autologous blood transfusion.Even though the advances in surgical technique and anesthetic management,as well as a better understanding of the risk factors,have resulted in a steady decrease in intraoperative bleeding,most patients still bleed extensively.Blood transfusion therapy is still a critical feature during OLTx and various studies have shown a large variability in the use of blood products among different centers and even among individual anesthesiologists within the same center.Unfortunately,despite the large number of OLTx performed each year,there is still paucity of large randomized,multicentre,and controlled studies which indicate how to prevent bleeding,the transfusion needs and thresholds,and the "evidence based" perioperative strategies to reduce the amount of transfusion.     

Successful liver allograft inflow reconstruction with the right gastroepiploic vein

Portal vein thrombosis is a common complica-tion in cirrhotic patients. When portal vein thrombectomy is not a suitable option, a large collateral vessel can be used for allograft venous inflow reconstruction. We describe an unusual case of successful portal revascularization using the right gastroepiploic vein. The patient underwent a cadaveric orthotopic liver transplantation with end-to-end anastomosis of the portal vein to the right gastroepiploic vein. Six months after liver transplantation the patient is well with good liver function. The use of the right gastroepiploic vein for allograft venous reconstruction is feasible and safe, with a great advan-tage of avoiding the need of venous jump graft.     

Etiology and consequences of thrombosis in abdominal vessels

The thrombophilia which can be either congenital or acquired in adult life has major implications in the abdominal vessels. The resulting portal vein thrombosis, Budd-Chiari syndrome and mesenteric vein thrombosis have a variety of consequences ranging from acute abdomen to chronic hepatomegaly and even totally asymptomatic patient in whom the only finding is pancytopenia. The complications like esophageal varices, portal gastropathy, ascites, severe hypersplenism, liver failure requiring liver transplantation are well known. Interesting features of collateral venous circulation showing itself as pseudocholangiocarcinoma sign and its possible clinical reflection as cholestasis are also known from a long time. The management strategies for these complications of intraabdominal vessel thrombosis are not different from their counterpart which is cirrhotic portal hypertension, but the prognosis is unquestionably better in former cases. In this review we presented and discussed the abdominal venous thrombosis, etiology and the resulting clinical pictures. There are controversial issues both in nomenclature, and management including anticoagulation problems and follow up strategies. In light of the current knowledge, we discussed some controversial issues in literature and presented our experience and our proposals about this group of patients.     

Portal vein thrombosis: a concise review

Portal vein thrombosis (PVT) is an uncommon cause for presinusoidal portal hypertension. Although several predisposing conditions are known to exist in the background of PVT, there still remains a proportion of patients in whom the etiology is not known and the pathogenesis is unclear. In this review we summarize the literature on PVT and present the current knowledge about the precipitating factors of PVT. Further, we discuss the advances in the radiological diagnosis that have improved diagnostic accuracy and are noninvasive. Finally, we discuss the treatment options for patients who have varying extents of thrombosis in the portal vein and specifically focus on PVT that is encountered before and after liver transplantation.     

Acute Budd-Chiari syndrome with liver failure: the experience of a policy of initial interventional radiological treatment using transjugular intrahepatic portosystemic shunt

BACKGROUND AND AIMS: The optimal management of acute Budd-Chiari syndrome (BCS) with liver failure is controversial. Options include anticoagulation, portal systemic shunting or liver transplantation. In recent years, transjugular intrahepatic portosystemic shunts (TIPS) have been tried in place of shunt surgery. We report our experience with a policy of initial interventional radiological treatment (TIPS) in this patient group. METHODS: A consecutive, non-selected series of five patients with acute BCS with liver failure presenting to our liver unit over a 36-month period from October 2000 were treated with a policy of initial attempt at transjugular shunt. RESULTS: Stents were successfully inserted in four patients. In one patient the hepatic vein remnant could not be cannulated. One of the four stented patients had a hepatic venous web. Rapid recurrent portal and hepatic vein thrombosis occurred in one patient despite anticoagulation and trans-shunt thrombectomy. This patient and the patient with failed stenting underwent successful liver transplantation. One successfully stented patient died unexpectedly at home 2 weeks after TIPS insertion. Four of five patients are alive (80%) at a mean follow-up time of 30 months. CONCLUSION: Initial TIPS, with liver transplantation for treatment failures, may be a reasonable, cost-effective and surgery-sparing treatment for acute Budd-Chiari presenting with liver failure.     

Direct oral anticoagulant administration in cirrhotic patients with portal vein thrombosis: What is the evidence?

In recent years, the traditional concept that cirrhosis-related coagulopathy is an acquired bleeding disorder has evolved. Currently, it is known that in cirrhotic patients, the hemostatic system is rebalanced, which involves coagulation factors, fibrinolysis and platelets. These alterations disrupt homeostasis, skewing it toward a procoagulant state, which can lead to thromboembolic manifestations, especially when hemodynamic and endothelial factors co-occur, such as in the portal vein system in cirrhosis. Portal vein thrombosis is a common complication of advanced liver cirrhosis that negatively affects the course of liver disease, prognosis of cirrhotic patients and success of liver transplantation. It is still debated whether portal vein thrombosis is the cause or the consequence of worsening liver function. Anticoagulant therapy is the mainstay treatment for acute symptomatic portal vein thrombosis. In chronic portal vein thrombosis, the role of anticoagulant therapy is still unclear. Traditional anticoagulants, vitamin K antagonists and low-molecular-weight heparin are standard-of-care treatments for portal vein thrombosis. In the last ten years, direct oral anticoagulants have been approved for the prophylaxis and treatment of many thromboembolic-related diseases, but evidence on their use in cirrhotic patients is very limited. The aim of this review was to summarize the evidence about the safety and effectiveness of direct oral anticoagulants for treating portal vein thrombosis in cirrhotic patients.     

How can portal vein cavernous transformation cause chronic incomplete biliary obstruction?

Biliary disease in the setting of non-cirrhotic portal vein thrombosis (and similarly in portal vein cavernous transformation) can become a serious problem during the evolution of disease. This is mostly due to portal biliary ductopathy. There are several mechanisms that play a role in the development of portal biliary ductopathy, such as induction of fibrosis in the biliary tract (due to direct action of dilated peribiliary collaterals and/or recurrent cholangitis), loss of biliary motility, chronic cholestasis (due to fibrosis or choledocholithiasis) and increased formation of cholelithiasis (due to various factors). The management of cholelithiasis in cases with portal vein cavernous transformation merits special attention. Because of a heterogeneous clinical presentation and concomitant pathophysiological changes that take place in biliary anatomy, diagnosis and therapy can become very complicated. Due to increased incidence and complications of cholelithiasis, standard treatment modalities like sphincterotomy or balloon sweeping of bile ducts can cause serious problems. Cholangitis, biliary strictures and hemobilia are the most common complications that occur during management of these patients. In this review, we specifically discuss important issues about bile stones related to bile duct obstruction in non-cirrhotic portal vein thrombosis and present evidence in the current literature.     

Huge extrahepatic portal vein aneurysm as a late complication of liver transplantation

A 60-year-old male underwent orthotopic liver transplantation because of hepatitis C virus related cirrhosis. After 12 d, the patient underwent re-transplantation due to primary graft non function. One year later the patient developed a thrombosis of the main portal vein needing a surgical revision. After 11 years the patient was operated on because of a clinical picture of intestinal occlusion. As an incidental finding, a large aneurysm of the main portal vein was diagnosed. The incidence of intra- and extrahepatic Portal vein aneurysms (PVAs) is not clear. To the best of our knowledge, only one case of intrahepatic PVA in a liver transplant has been reported in the literature. In addition, we have found no documented cases of extrahepatic PVAs in liver transplanted patients.     

Catheter-directed thrombolysis through the operatively recanalized umbilical vein for acute extensive portal vein thrombosis: report of a case

Acute portal vein thrombosis is a rare but severe complication of intra-abdominal infection. It can be life-threatening, given the risk of developing liver abscess and subsequent liver failure. Various types of hereditary thrombophilia are known risk factors for acute portal vein thrombosis. In addition to surgical treatment and potent antibiotic therapy, systemic administration of anticoagulants and locoregional trans-catheter delivery of thrombolytic agents are known to be effective. We present a case report of acute portal vein thrombosis with pylephlebitis caused by acute appendicitis, successfully treated with catheter-directed thrombolysis through the operatively recanalized umbilical vein. The umbilical vein is a promising access route to the portal vein. Therefore, this procedure is an effective and preferred treatment option for portal septic thrombosis, particularly because it does not require puncture of the liver parenchyma or catheterization through an infected peritoneal cavity.     

Use of an interpositional venous graft posterior to the pancreas for LDLT patients with portal vein thrombosis

Portal vein thrombosis is a risk factor in patients who require liver transplantation, because it is often difficult to treat portal vein thrombosis, especially when it involves the confluence of the superior mesenteric vein and splenic vein. Since some transplant centers that perform living-donor liver transplantation do not have cryopreserved cadaveric vein grafts available and do not use graft veins that are long enough for a jump graft, it is difficult to reconstruct the portal vein with interpositional vein grafts in patients with portal vein thrombosis. We describe the treatment of portal vein thrombosis with an interpositional vascular graft posterior to the pancreas in a living-donor liver transplantation patient without using a jump graft. This method provided a shorter rout between the donor and recipient portal vein than a jump graft. Our experience suggests that this solution can be helpful in treating portal vein thrombosis.     

Portal vein thrombosis,revisited

This review article aims to discuss the aetiology, pathophysiology, clinical presentation, diagnostic workup and management of portal vein thrombosis, either as a primary vascular liver disease in adults and children, or as a complication of liver cirrhosis. In addition, indications and limits of anticoagulant therapy are discussed in detail.     

Transjugular intrahepatic portosystemic shunt

The transjugular intrahepatic portosystemic shunt (TIPS) is an interventional treatment resulting in decompression of the portal system by creation of a side-to-side portosystemic anastomosis. Since its introduction 16 years ago, more than 1,000 publications have appeared demonstrating broad acceptance and increasing clinical use. This review summarizes our present knowledge about technical aspects and complications, follow-up of patients and indications. A technical success rate near 100% and a low occurrence of complications clearly depend on the skills of the operator. The follow-up of the TIPS patient has to assess shunt patency, liver function, hepatic encephalopathy and the possible development of hepatocellular carcinoma. Shunt patency can best be monitored by duplex sonography and can avoid routine radiological revision. Short-term patency may be improved by anticoagulation, while such a treatment does not influence long-term patency. Stent grafts covered with expanded polytetrafluoroethylene show promising long-term patency comparable with that of surgical shunts. With respect to the indications of TIPS, much is known about treatment of variceal bleeding and refractory ascites. The thirteen randomized studies that are available to date show that survival is comparable in patients receiving TIPS or endoscopic treatment for acute or recurrent variceal bleeding. Another group comprises patients with refractory ascites and related complications, such as hepatorenal syndrome and hepatic hydrothorax. It has been demonstrated that TIPS improves these complications. Five randomized studies comparing TIPS with paracentesis and one study comparing TIPS with the peritoneo-venous shunt showed good response of ascites but controversial results on survival. In addition, TIPS has been successfully applied to patients with Budd-Chiari syndrome, portal vein thrombosis, before liver transplantation, and for the treatment of ectopic variceal bleeding.