Extensive portal vein thrombosis related to abdominal trauma

Abdominal trauma is a classic but very rare cause of portal vein thrombosis. We report the case of a patient with portal vein thrombosis and cavernoma associated with portal hypertension. Anamnesis identified a serious thoraco-abdominal trauma related to a bicycle accident 6 months before. Biological screening identified an inherited heterozygous G20210A factor II gene mutation which supports a recent notion that portal vein thrombosis most often occurs when both local and systemic aetiological factors are combined.     

Transjugular intrahepatic portosystemic shunt in the treatment of portal vein thrombosis: a critical review of literature

Reports of successful transjugular intrahepatic portosystemic shunt (TIPS) surgery in patients with portal vein thrombosis (PVT) are considered anecdotal owing to the technical difficulty of the procedure and potential procedure-related complications. A literature review was undertaken to determine the feasibility and safety of TIPS in the treatment of PVT. All studies in which TIPS was attempted in patients with PVT were identified by searching through the PUBMED and MEDLINE databases. A total of 424 PVT patients undergoing TIPS were reported in 54 articles. The success rate of TIPS insertion was 67–100% in 19 case series. Further, 85 patients with portal cavernoma underwent successful TIPS insertions. Three therapeutic strategies of TIPS placement were used: (1) TIPS placement followed by portal vein recanalization via the shunt, (2) portal vein recanalization via percutaneous approaches followed by TIPS placement, and (3) TIPS insertion between a hepatic vein and a large collateral vessel without portal vein recanalization. Four approaches were used to access the portal vein: transjugular, transhepatic, transsplenic, and transmesenteric. Intra-abdominal hemorrhage secondary to hepatic capsule perforation was lethal in only three patients. No episode of pulmonary embolism was reported. Other procedure-related complications were reversible. The overall incidence of shunt dysfunction and hepatic encephalopathy was 8–33% and 0–50%, respectively. In conclusion, the reviewed studies uniformly support the feasibility and safety of TIPS for PVT even in the presence of portal cavernoma. Further, several major issues that remain unresolved are discussed.     

Gore-Tex jump graft for portal vein thrombosis following living donor liver transplantation

Portal vein thrombosis is a rare surgical complication following liver transplantation, which remains a cause of graft loss and death. We describe here the treatment of portal vein thrombosis following living donor liver transplantation using an extended left lobe graft. The patient was treated with a Gore-Tex vascular jump graft extra-anatomically interposed between the recipient superior mesenteric vein and the donor umbilical vein. This technique allowed the hepatic hilum to be left untouched and supplied suitable blood flow to the hepatic allograft. Our experience suggests that this innovative technical solution can be helpful in the effort to rescue cases of hepatic allograft with vascular complications.     

Isolated splenic vein thrombosis: an unusual cause and review of the literature.

Isolated obstruction (mainly due to thrombosis) of the splenic vein usually results in left-sided portal hypertension and isolated fundal varice formation. This syndrome is a rare cause of gastrointestinal bleeding. Pancreatic diseases are among the most common etiologies of splenic vein obstruction. Renal disorders are rarely reported as a cause of splenic vein thrombosis. In the present article, a case of a 26-year-old woman with a perirenal abscess presenting with gastrointestinal bleeding as a complication of an isolated splenic vein thrombosis is described. The thrombosis could not be visualized with ultrasonography and angiography because of its extremely proximal localization. Fundal varices disappeared following splenectomy and nephrectomy. Follow-up at one year revealed the patient to be well both clinically and endoscopically.     

Portal vein thrombosis associated with hilar bile duct carcinoma and liver abscess

As most portal vein occlusion in hilar bile duct carcinoma is caused by tumor invasion to the portal vein, other mechanisms of its occlusion are very rare. We report the case of a 69-year-old man who underwent surgical resection for an advanced hilar bile duct carcinoma associated with unusual portal vein occlusion. Preoperative diagnosis was advanced hilar bile duct carcinoma with liver abscess and right portal vein occlusion due to tumor invasion. Extended right hepatectomy combined with resection of caudate lobe was performed. Intraoperatively, tumor invasion to the portal vein was not evident and resected margin of the right portal vein showed thrombosis and no evidence of malignancy histologically. To our knowledge, this is the first reported case of a patient with a combination of portal vein thrombosis and liver abscess in hilar bile duct carcinoma. Although portal vein occlusion due to thrombosis is an unusual complication in hilar bile duct carcinoma, the presence of liver abscess may be a useful diagnostic implication of this occlusion.     

Portal and mesenteric vein thrombosis after portal vein embolization in a patient with protein S deficiency

Portal vein embolization can be performed safely, and so far no major complications have been reported. We report an extremely rare complication of portal vein embolization, a case of portal and mesenteric thrombosis in a 65-year-old patient with protein S deficiency. Right portal vein embolization was carried out prior to extended right hepatectomy for advanced gallbladder carcinoma involving the hepatic hilus. Computed tomography 14 days after embolization revealed massive thrombosis of the portal and the superior mesenteric veins. A protein S deficiency was found by means of an extensive workup for hypercoagulable state. Portal vein embolization may have triggered a cascade of events that was expressed as portal and mesenteric vein thrombosis resulting from deficiency of protein S. It may be better to determine the concentrations of such coagulation regulators prior to portal vein embolization.     

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.     

Extrahepatic portal vein thrombosis

Noncirrhotic, nontumoral portal vein thrombosis (PVT) is the second most-frequent cause of portal hypertension in the world. General thrombophilic factors can be identified in approximately 60% of patients. PVT may manifest as an acute process. However, the acute episode more frequently is asymptomatic or paucisymptomatic and portal vein thrombosis is misdiagnosed until the development of complications secondary to portal hypertension, such as variceal bleeding or portal biliopathy. Although no randomized controlled trials have been performed, after the diagnosis of acute PVT early initiation of anticoagulation (within 30 days of the onset of symptoms) is recommended to achieve recanalization. In patients with portal cavernoma, anticoagulation is aimed to prevent the progression and recurrence of thrombosis. Because of the lack of data in this specific population, variceal bleeding is managed as in cirrhotic patients. Ursodeoxycholic acid has been proposed empirically for the treatment of patients with symptomatic portal biliopathy. Choledocholithiasis might be present, complicating a bile duct stenosis. Accordingly, an endoscopic retrograde cholangiopancreatography with sphincterotomy, extraction with balloon catheter, and stent placement is indicated. Mortality among patients with PVT is low (5-year mortality rate of 5 to 10%) and is mainly related to associated diseases rather than to complications of portal hypertension.     

Pancreatic pseudocyst causing portal vein thrombosis and pancreatico-pleural fistula.

Portal vein thrombosis and pancreatico-pleural fistula are unusual complications of chronic pancreatitis. We describe a patient with chronic alcoholic pancreatitis in whom erosion of the splenic vein led to portal vein thrombosis and to the development of a pancreatico-pleural fistula. We suggest that fistula formation may occur over a considerable time period as the portal vein thrombosis was diagnosed three years before the amylase-rich pleural effusions.     

Thrombosis of the portal and superior mesenteric veins in a patient with a congenital and familial deficiency in antithrombin III (author's transl)]

Thrombosis of the visceral veins is an extremely rare condition in cases of congenital and hereditary deficiency of antithrombin III, associated with recurring venous thorbosis of the limbs. The authors report such a case in a man of 40 years of age, who had this deficiency, associated with thrombosis of the portal and superior mesenteric veins and a portal cavernoma. They stress the frequent association of recurring peripheral vein thrombosis, portal vein thrombosis in adults, and thrombosis of the mesenteric vein, and the importance of systematic measurements of antithrombin III levels in these pathological conditions.     

Laparoscopic cholecystectomy in patient with portal cavernoma and portal hypertension.

Successful laparoscopic cholecystectomy has been reported in patients with cirrhosis of liver with portal hypertension; the procedure has, however, not been reported in patients with portal vein thrombosis, portal cavernoma and portal hypertension. We report an 18-year-old man with portal hypertension due to portal vein thrombosis and portal cavernoma who had symptomatic gallstone disease and was successfully treated with laparoscopic cholecystectomy.     

Combined liver vein and spleen pulp pressure measurements in patients with portal or splenic vein thrombosis

BACKGROUND: Patients with thrombosis of the portal or splenic vein may develop portal hypertension with bleeding from oesophageal or gastric varices. The relevant portal pressure cannot be measured by liver vein catheterization or transhepatic puncture of the portal vein because the obstruction is peripheral to the accessible part of the portal system. METHODS: Liver vein catheterization was combined with percutaneous splenic pressure measurement in 10 patients with portal or splenic vein thrombosis and no cirrhosis, and 10 cirrhotic patients without thrombosis. The splenic pressure was measured by percutaneous puncture below the curvature of the ribs with an angle of the needle to skin of 30 degrees in order to minimize the risk of cutting the spleen if the patient took a deep breath. RESULTS: None of the patients in whom the described procedure was followed had complications. Pressure measurements in the spleen pulp and splenic vein were concordant. The pressure gradient across the portal venous system (splenic-to-wedged hepatic vein pressure) was -1.3 to 8.5 mmHg (median, 2.8 mmHg) in cirrhosis patients and 0-44 mmHg (median, 18 mmHg) in thrombosis patients, the variation reflecting various degrees of obstruction to flow in the portal venous system. Peripheral portal pressure (splenic-to-free liver vein pressure gradient) was 1.1-28 mmHg (median, 17 mmHg) in cirrhotic patients and 11-52 mmHg (median, 23 mmHg) in thrombosis patients. CONCLUSIONS: Liver vein catheterization combined with percutaneous splenic pressure measurement is feasible in quantifying pressure gradient across a thrombosis of the portal/splenic vein and in quantifying portal pressure peripheral to this kind of thrombosis.     

Portal vein thrombosis associated with a myeloproliferative disorder, prothrombin G20210A mutation, antiphospholipid syndrome, with repermeation during anticoagulant therapy

Portal vein thrombosis, except in hepatocellular carcinoma and severe cirrhosis, is due to one or several prothrombotic disorders with or without a local precipitating factor. We report a case of a portal and splenic vein thrombosis, without cavernoma and varices which occurred in a 72-year-old man with abdominal pain and weakness. Three prothrombotic states including latent myeloproliferative disorder, antiphospholipid syndrome, and factor II G202101 mutation, were observed. Anticoagulant treatment resulted in complete repermeation of the portal and splenic veins without a hemorrhagic event. This illustrates that several prothrombotic states may occur in a single patient with portal vein thrombosis. Early anticoagulant therapy, in recent portal vein thrombosis, can result in repermeation.     

Pylephlebitis and pyogenic liver abscesses: a complication of hemorrhoidal banding.

Hemorrhoidal banding is a well-established and safe outpatient procedure. Septic complications of hemorrhoidal banding are rare but can be fatal. The first case of pylephlebitis (septic portal vein thrombosis) and pyogenic liver abscess following hemorrhoidal banding in a 49-year-old man with diabetes is reported in the present study. Risk factors, management and the role of prophylaxis in immunocompromised patients are discussed. Caution against hemorrhoidal banding in immunosuppressed patients, including patients with diabetes, is warranted.     

Portal or hepatic vein thrombosis as the first presentation of a myeloproliferative disorder in patients with normal peripheral blood counts

Myeloproliferative disorders (MPD) are associated with an increased risk of thrombotic complications. We describe three patients with portal or hepatic vein thrombosis and normal peripheral blood counts who had MPD on bone marrow morphology and growth factor-independent megakaryocyte or erythroid colony growth in vitro. The peripheral blood counts have become abnormal subsequently in two patients. Patients presenting with unexplained portal or hepatic vein thrombosis should be investigated systematically for the presence of a MPD, which may not be apparent using conventional diagnostic criteria.     

Recent portal or mesenteric venous thrombosis: increased recognition and frequent recanalization on anticoagulant therapy

Characteristics and outcomes of recent portal or mesenteric venous thrombosis are ill-known. We intended to compare these features with those of patients with portal cavernoma, and also to assess the incidence of recanalization of recent thrombosis on anticoagulation therapy. All patients seen between 1983 and 1999 were enrolled into this retrospective study if recent portal or mesenteric venous thrombosis or portal cavernoma had been documented, and if cancer of the liver, pancreas, or bile duct, intrahepatic block including cirrhosis, and obstruction of the hepatic veins had been ruled out. The proportion of recent thrombosis was 7% in patients seen before 1990 and 56% after 1994 (P <.05). Patients with recent thrombosis (n = 33) or cavernoma (n = 108) did not differ with regard to age, sex ratio, or prevalence of prothrombotic states and of previous thrombotic events. In patients with recent thrombosis, septic pylephlebitis was more common and the incidence of gastrointestinal bleeding was lower (2.4 vs. 12.7/100 patient-years). Recanalization occurred in 25 of 27 patients given anticoagulation and 0 of 2 patients not given anticoagulation. The probability of recanalization was related to the extent of thrombosis (P =.003). In conclusion, mesenteric or portal venous thrombosis is increasingly recognized at an early stage. The features differentiating recent thrombosis and cavernoma are related to silent onset precluding early recognition and therapy in the latter. Frequent association with prothrombotic states and frequent recanalization on anticoagulation support the recommendation of early anticoagulation therapy in all patients with recent portal vein thrombosis.     

Cholestasis as presenting symptom of portal cavernoma

A 51-year-old man with a history of portal vein thrombosis, was examined because of elevated liver tests and a tumoral mass in the liver hilus. Computed tomography (CT) scan and magnetic resonance imaging confirmed the portal vein thrombosis and an infiltrating mass in the porta hepatis with compression on the common bile duct. Endoscopic retrograde cholangiography showed an irregular narrowing of the mid-part of the common bile duct. The patient was referred for explorative laparotomy, which revealed a hypervascular mass in the liver hilus surrounded by many blood vessels. The diagnosis of portal cavernoma was made. Further haematological examination for the cause of portal vein thrombosis revealed an anti-phospholipid syndrome as well as myeloproliferative disease. Oral anticoagulant treatment is started. In conclusion, we report a case of biliary stricture due to portal vein thrombosis and cavernoma (portal biliopathy) which was not diagnosed preoperatively. Biliary strictures associated with portal vein thrombosis are due to extrinsic compression by collaterals and can also be induced by ischemic injury secondary to venous and arterial thrombosis of the choledochal vascular plexus.     

New thrombolytic modality — regional administration of tissue plasminogen activator for hepatic artery and portal vein thromboses after liver transplantation: Case report

A 2 1/2-year-old boy with biliary atresia underwent orthotopic living-related liver transplantation. On the 7th postoperative day, he had an episode of hepatic arterial thrombosis following disseminated intravascular coagulation (DIC) due to severe intraabdominal sepsis. Tissue plasminogen activator was administered regionally and the hepatic arterial flow recovered promptly. On postoperative day 33, portal vein thrombosis occurred and direct tissue plasminogen activator injections into the portal vein improved portal blood flow. However, the patient eventually died of poorly controlled DIC. Throughout the course, color Doppler ultrasonogram and arterial ketone body ratio were good indicators of hepatic arterial and portal blood flow. When hepatic arterial thrombosis and portal vein thrombosis occur, retransplantation is often inevitable. Thus, while the patient is awaiting a suitable donor, it could be possible to maintain blood flow to the graft with this new thrombolytic therapy.     

Portal vein thrombosis in patients with thrombophilia--own observations

Portal vein thrombosis is one of the main prehepatic causes of portal hypertension. The most frequent causes of thrombosis in this localization, apart from hepatic cirrhosis, are the following: acute inflammatory diseases and abdominal cancers, traumas, proliferative diseases of the hematopoietic system. In recent years attention was given to disorders in hemostasis, such as thrombophilia, in the course of which thrombosis development is particularly common. The authors present 10 patients after an incident of portal vein thrombosis, in which primary hepatic pathology was excluded and tests directed at thrombophilia were performed. In seven patients abnormalities in the examined parameters were found, and what is more, in two cases they had a complex character and involved more than one parameter. In five patients hyperhomocysteinemia was found. Among them, in two patients there was also a decreased protein S activity and in one of them there was also APC-resistance. In the next two patients there were abnormalities in one of the examined parameters - APC-resistance. Hyperhomocysteinemia was found in all patients with idiopathic thrombosis, and in one of them there were concurrent changes in protein S activity and APC-resistance. In patients with the history of portal vein thrombosis diagnostics of thrombophilia should be performed.     

The postoperative splenic/portal vein thrombosis after splenectomy and its prevention--an unresolved issue

Patients undergoing splenectomy have an increased risk of splenic/portal vein thrombosis. We used several databases to identify publications dealing with this risk and analyzed incidence, risk factors and outcome. The risk of splenic portal vein thrombosis has been addressed in prospective and retrospective randomized or non-randomized studies. All studies combined, the overall risk is 3.3%. Risk factors are big spleens (i.e. myeloproliferative disorders) and hereditary hemolytic anemias, whereas the risk is low in autoimmune thrombocytopenia and trauma. The incidence is approximately the same in laparoscopic and open splenectomy. The median time from splenectomy to symptomatic splenic vein thrombosis is 8-12 days. Postoperative antithrombotic prophylaxis ranged from no prophylaxis to heparin for seven days or longer. Treatment of symptomatic splenic vein thrombosis with heparin and warfarin leads to complete resolution of thrombosis in 67%, to partial resolution in 13%, but persistent occlusion, portal hypertension or cavernoma occurred in 20%.The long-term outcome of treatment failures is unknown. Well-designed randomized studies on the prophylaxis of venous thromboembolism after splenectomy are urgently needed.