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.     

Role of echo Doppler ultrasonography in the evaluation of postprandial hyperemia in cirrhotic patients

AIM： TO assess the role of echo-Doppler ultrasonography in postprandial hyperemia in cirrhotic patients by comparing the results with the hepatic vein catheterization technique.
METHODS： Patients with cirrhosis, admitted to the portal hemodynamic laboratory were included into the study. After an overnight fast, echo-Doppler ultrasonography （basal and 30 min after a standard meal） and hemodynamic studies by hepatic vein catheterization （basal, 15 min and 30 min after a standard meal） were performed. Ensure Plus （Abbot Laboratories, North Chicago, IL） was used as the standard liquid meal. Correlation analysis of the echo- Doppler and hepatic vein catheterization measurements were done for the basal and postprandial periods.
RESULTS： Eleven patients with cirrhosis （5 Child A, 4 Child B, 2 Child C） were enrolled into the study. After the standard meal, 8 of the 11 patients showed postprandial hyperemia with increase in portal blood flow, portal blood velocity and hepatic venous pressure gradient. Hepatic venous pressure gradient in the postprandial period correlated positively with postprandial portal blood velocity （r = 0.8, P 〈 0.05） and correlated inversely with postprandial superior mesenteric artery pulsatility index （r = -1, P 〈 0.01）.
CONCLUSION： Postprandial hyperemia can be efficiently measured by echo-Doppler ultrasonography and the results are comparable to those obtained with the hemodynamic studies.     

Extrahepatic portal venous obstruction of different pathogenesis in pancreatic diseases: reports of 4 autopsy cases with chronic pancreatitis and pancreatic carcinoma

Four autopsy cases of extrahepatic portal venous obstruction associated with pancreatic diseases, 1 case of pancreatitis and 3 cases of pancreatic carcinoma, are presented. The pathogenesis of portal obstruction was different in each case; old thrombosis with recanalization due to chronic pancreatitis with pseudocysts formation in 1 case, fresh thrombosis due to intraportal venous catheterization for pancreatic carcinoma in 1 case, fresh thrombosis probably due to pancreatitis accompanying pancreatic carcinoma in 1 case, and direct invasion of pancreatic carcinoma into the portal vein in the remaining 1 case. Morphologic evidence for portal hypertension was present in each case. In the pancreatitis case and one pancreatic carcinoma case with portal tumor invasion, both of which had chronic portal obstruction, there were many thin-walled vascular channels (cavernous transformation) around the occluded portal vein. Their endothelia were positive for factor VIII-related antigen and Ulex europaeus lectin I, implying that these vessels were hepatopetal blood vascular collaterals. It was shown that pancreatic diseases resulted in portal venous obstruction by several different mechanisms and chronic portal obstruction in pancreatic diseases led to the formation of hepatoperal blood vascular collaterals.     

Effectiveness of combined anticoagulant therapy for extending portal vein thrombosis in Crohn's disease

PURPOSE: Portal vein thrombosis is a rare complication of Crohn's disease, and its precise cause and appropriate treatment are not known. We describe a patient with extending portal vein thrombosis in Crohn's disease who was successfully treated with combined anticoagulant therapy. METHOD: Urokinase and tissue plasminogen activator were administered from a catheter inserted into the superior mesenteric artery, and heparin and a serine protease inhibitor also were given intravenously. RESULTS: On admission, thromboembolic occlusion was observed throughout the entire portal venous system in association with massive ascites and remarkable intestinal edema. After administration of combined anticoagulant therapy, thrombus rapidly decreased in size, and color Doppler ultrasonography showed a gradual increase in portal venous flow. The patient had no recurrence of symptoms while receiving warfarin after resolution of thrombus. CONCLUSION: This case report suggests that combined anticoagulant therapy is effective for patients with severe portal vein thrombosis in Crohn's disease and that color Doppler ultrasonography is useful for evaluation of portal venous flow.     

Interventional treatment for symptomatic acute-subacute portal and superior mesenteric vein thrombosis

AIM: To summarize our methods and experience with interventional treatment for symptomatic acute-subacute portal vein and superior mesenteric vein thrombosis (PV-SMV) thrombosis. METHODS: Forty-six patients (30 males, 16 females,aged 17-68 years) with symptomatic acute-subacute portal and superior mesenteric vein thrombosis were accurately diagnosed with Doppler ultrasound scans, computed tomography and magnetic resonance imaging.They were treated with interventional therapy, including direct thrombolysis (26 cases through a transjugular intrahepatic portosystemic shunt; 6 through percutaneous transhepatic portal vein cannulation) and indirect thrombolysis (10 through the femoral artery to superior mesenteric artery catheterization; 4 through the radial artery to superior mesenteric artery catheterization).RESULTS: The blood reperfusion of PV-SMV was achieved completely or partially in 34 patients 3-13 d after thrombolysis. In 11 patients there was no PV-SMV blood reperfusion but the number of collateral vessels increased significantly. Symptoms in these 45 patients were improved dramatically without severe operational complications. In 1 patient, the thrombi did not respond to the interventional treatment and resulted in intestinal necrosis, which required surgical treatment.In 3 patients with interventional treatment, thrombi re-formed 1, 3 and 4 mo after treatment. In these 3 patients, indirect PV-SMV thrombolysis was performed again and was successful.CONCLUSION: Interventional treatment, including direct or indirect PV-SMV thrombolysis, is a safe and effective method for patients with symptomatic acutesubacute PV-SMV thrombosis.     

Interventional treatment for symptomatic acute-subacute portal and superior mesenteric vein thrombosis

AIM: To summarize our methods and experience with interventional treatment for symptomatic acute-subacute portal vein and superior mesenteric vein thrombosis (PV-SMV) thrombosis. METHODS: Forty-six patients (30 males, 16 females,aged 17-68 years) with symptomatic acute-subacute portal and superior mesenteric vein thrombosis were accurately diagnosed with Doppler ultrasound scans, computed tomography and magnetic resonance imaging.They were treated with interventional therapy, including direct thrombolysis (26 cases through a transjugular intrahepatic portosystemic shunt; 6 through percutaneous transhepatic portal vein cannulation) and indirect thrombolysis (10 through the femoral artery to superior mesenteric artery catheterization; 4 through the radial artery to superior mesenteric artery catheterization).RESULTS: The blood reperfusion of PV-SMV was achieved completely or partially in 34 patients 3-13 d after thrombolysis. In 11 patients there was no PV-SMV blood reperfusion but the number of collateral vessels increased significantly. Symptoms in these 45 patients were improved dramatically without severe operational complications. In 1 patient, the thrombi did not respond to the interventional treatment and resulted in intestinal necrosis, which required surgical treatment.In 3 patients with interventional treatment, thrombi re-formed 1, 3 and 4 mo after treatment. In these 3 patients, indirect PV-SMV thrombolysis was performed again and was successful.CONCLUSION: Interventional treatment, including direct or indirect PV-SMV thrombolysis, is a safe and effective method for patients with symptomatic acutesubacute PV-SMV thrombosis.     

Percutaneous transhepatic catheterization of the portal venous system

During recent years, percutaneous transhepatic catheterization of the portal venous system has become the most accurate procedure for investigation of the portal system. The procedure can be performed under local analgesia, is relatively simple, and complications are rare. The success rate is high, approximately 90%, especially when the liver hilum is localized by ultrasonography prior to catheterization. The free portal pressure can be measured. Selective catheterization of all portal tributaries can be performed. The indications are: portography in patients with cirrhosis of the liver and portal hypertension for delineation of collateral vein systems including gastro-oesophageal varices; visualization of veins that may be used for portosystemic shunt operations; postoperative control of shunt patency; diagnosis of portal and hepatic vein thrombosis; localization of stenosis in the portal vein system; pre-operative evaluation of patients with tumours in the biliary tract and pancreas; obliteration of bleeding oesophageal varices; and verification and localization of endocrine pancreatic tumours making curative resection possible. Further, transhepatic catheterization of the portal system may be used in research on the development of portal hypertension, collateral veins, variceal bleeding, and for haemodynamic, metabolic and pharmacologic studies in the gastrointestinal tract.     

Portal vein thrombosis in children: clinical and laboratory study of 26 cases]

Portal vein thrombosis represents one of the most frequent causes of portal hypertension in childhood. The aim of the present study was to describe the clinical and laboratorial characteristics of portal vein thrombosis in pediatric patient. We studied 26 children with diagnosis of portal vein thrombosis through splenoportography (two patients) and ultrasound scan (24 patients) which ages varied from 2 months to 11 years and 4 months (median-5 years and 3 months). Data of the patient history, physical and laboratories examination were used to a retrospective study which was done through medical record analysis. The main complaint of the examination was hematemesis, which was found in 57.6%. In 26.9% a possible risk factor for portal vein thrombosis was found [catheterization of the umbilical vein (four), sepsis (two), omphalitis (one)]. Splenomegaly was present in all cases and the associated illness to portal vein thrombosis were: hepatoportal sclerosis (three), cytomegalovirus infection (two), blastomycosis (two), virus C (two), virus B (one) and virus A (one). The time between the first bleeding and the examination at University of Campinas Hospital, in Campinas, SP, Brazil, varied from 0.23 months to 54 months with a median of 12 months. Only 11.5% of patients underwent the endoscopy with sclerotherapy before going to University of Campinas Hospital. Aminotransferases' activities were considered normal in 20 patients. We could conclude that: 1. The most frequent initial symptom was hematemesis. 2. The known risk factors for portal vein thrombosis were present in about 1/3 of the cases. 3. Laboratorial exams usually indicated absence of hepatocitic lesions. 4. The efforts towards sending the patient to a reference center were late with a delayed diagnostic and with delayed effective therapeutic conduct. 5. In about 50% of the cases there was PVT associated with other hepatic diseases.     

Left-Sided gallbladder with intrahepatic portal venous anomalies

The congenital anomaly in which the gallbladder is found on the left of the round and falciform ligaments (left-sided gallbladder) is rare. We report two patients with left-sided gallbladder in whom intrahepatic portal venous anomalies were identified. Computed tomography and intraoperative ultrasonography were used to define the portal venous anomaly. A long straight left main portal vein was demonstrated, which did not have the typical umbilical portion. The right anterior segmental portal branch (case 1), or the right main portal vein (case 2) were shown to course in a ventral direction and terminate as a cul de sac. The round ligament (right round ligament) was attached to this venous termination, forming the right umbilical portion. The left medial segmental portal venous branches originated from the right umbilical portion, and coursed to the left. In contrast, cholangiography disclosed that the left medial segmental bile duct coursed to the right after arising from the left hepatic duct (case 1), or the common hepatic duct (case 2). The essence of this anomalous condition is not a left-sided gallbladder, but a right round ligament, which is an embryologic abnormality of the umbilical vein. A review of the English language literature revealed no reports of left-sided gallbladder with intrahepatic portal venous anomalies.     

Recent portal and mesenteric venous thrombosis associated with Fusobacterium bacteremia

SUMMARY: Septic pylephlebitis is usually a complication of intraabdominal infection in the region drained by the portal venous system. We report two cases of portal and mesenteric venous thrombosis associated with Fusobacterium necrophorum bacteremia, which did not show any obvious intra-abdominal source of infection with noninvasive imaging procedures. In one case, early anticoagulation treatment was associated with repermeation of the portal vein and its right branch. As in Bacteroides bacteremia, portal and/or mesenteric venous thrombosis should be searched for in case of Fusobacterium bacteremia of unknown origin. Repermeation of the portal vein and relief of extrahepatic portal hypertension can be achieved in these cases with early anticoagulation.     

Recurrent pulmonary embolism from left subclavian thrombosis: a case report

A 38-year-old woman was admitted to our hospital because of pulmonary thromboembolism. Thrombolysis therapy resulted in initial improvement in symptoms and laboratory data. However, 4 months later, pulmonary thromboembolism recurred despite antiplatelet and anticoagulation therapy. Contrast venography and venous ultrasonography of both upper and lower extremities revealed subtotal occlusion and venous thrombosis of the left subclavian vein with collateral vessels, but no evidence of lower extremity venous thrombosis. She had no history of subclavian venous catheterization, neoplasm, hypercoagulability or other predisposing cause of thrombus formation. Operative ligation of the left subclavian vein was performed at the junction with the internal jugular vein. White thrombus was identified within the venous lumen. She was well without recurrent pulmonary thromboembolism or venous insufficiency for 10 months after the operation. Surgical interruption of the subclavian vein may be effective to prevent recurrent pulmonary thromboembolism in patients with recurrent pulmonary thromboembolism due to venous thrombosis of the upper extremity despite therapeutic anticoagulation.     

Left-sided gallbladder： Its clinical significance and imaging presentations

AIM： To assess the importance of preoperative diagnosis and presentation of left-sided gallbladder using ultrasound （US）, CT and angiography.
METHODS： Retrospective review of 1482 patients who underwent enhanced CT scanning was performed. Left-sided gallbladder was diagnosed if a right-sided ligamentum teres was present. The image presentations on US, CT and angiography were also reviewed.
RESULTS： Left-sided gallbladder was diagnosed in nine patients. The associated abnormalities on CT imaging included portal vein anomalies, absence of umbilical portion of the portal vein in the left lobe of the liver, club-shaped portal vein in the right lobe of the liver, and difficulty in identifying segment Ⅳ. Angiography in six of nine patients demonstrated abnormal portal venous system （trifurcation type in four of six patients）. The main hepatic arteries followed the portal veins in all six patients. The segment Ⅳ artery was identified in four of six patients using angiography, although segment Ⅳ was difficult to define on CT imaging. Hepatectomy was performed in three patients with concomitant liver tumor and the diagnosis of left-sided gallbladder was confirmed intraoperatively.
CONCLUSION： Left-sided gallbladder is an important clinical entity in hepatectomy due to its associated portal venous and biliary anomalies. It should be considered in US, CT and angiography images that demonstrate no definite segment Ⅳ ,absence of umbilical portion of the portal vein in the left lobe, and club-shaped right anterior portal vein.     

Comparison between ultrasonographic signs and the degree of portal hypertension in patients with cirrhosis

The sensitivity of ultrasonography (US) for the diagnosis of portal hypertension was assessed in 48 patients with known cirrhosis. These results were compared to the hemodynamic values obtained on the same day by hepatic vein catheterization. The sensitivity of US in detecting portal hypertension was about 40% considering either a greater than 13 mm diameter of the portal vein, or the lack of mild caliber variation of the superior mesenteric vein. The sensitivity was more than 80% considering the presence of portosystemic venous collaterals. Presence of numerous portosystemic shunts was significantly associated with high hepatic venous pressure gradients which reflected the severity of portal hypertension.     

Resistance to activated protein C and portal vein thrombosis: two new cases and review of the literature]

INTRODUCTION: Resistance to activated protein C is the most common inherited factor at the origin of deep venous thrombosis. As portal vein thrombosis is rare, causes such as cirrhosis, intra-abdominal infection, primary hepatocellular carcinoma, myeloproliferative disorders or coagulation abnormalities must be investigated. EXEGESIS: We report two cases of portal vein thrombosis associated with resistance to activated protein C. This association is not frequent, as only 12 cases have been reported in the literature. These studies show that resistance to activated protein C was rarely the only factor, as other prothrombotic abnormalities were present in more than 70% of cases. CONCLUSION: Resistance to activated protein C is rarely associated with portal vein thrombosis. When present, other causes should not be overlooked. The potential existence of resistance to activated protein C should be systematically investigated in case of either portal vein thrombosis in patients with personal or familial thrombosis history, association with multiple thrombosis, or when the disease etiology remains unknown.     

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.     

Portal vein thrombosis successfully treated with a colectomy in active ulcerative colitis

Portal vein thrombosis is a rare complication in ulcerative colitis. We present a patient with portal vein thrombosis in ulcerative colitis who was successfully treated with colectomy. A 38-year-old Japanese female was admitted to our hospital because of an exacerbation of colitis. Abdominal ultrasonography performed because of liver dysfunction showed the thrombus in an umbilical portion of the portal vein. The patient underwent a subtotal colectomy and ileostomy because her colitis did not respond to intensive intravenous therapy. Although portal vein thrombus was treated with an intravenous infusion of urokinase before the operation, no change in the thrombus size was found. Approximately three months after the colectomy, the thrombus of the portal vein disappeared without anticoagulant therapy. Although a resection of an inflamed colon may be theoretically effective in the thrombosis in the inflammatory bowel disease, its benefit has not been confirmed. Our case suggests that the resection of the diseased bowel may have a favorable effect on the course of portal vein thrombosis in acute attacks of ulcerative colitis.     

Hepatic radionuclide angiography and Doppler ultrasonography in the detection and assessment of vascular disturbances in the portal system

BACKGROUND/AIMS: The aim of the study was evaluation of the morphology of the blood vessels, blood flow velocity and direction with Doppler ultrasound (D-US) and correlation with the relative liver parenchymal perfusion assessed by hepatic radionuclide angiography (HRA). METHODOLOGY: Real-time, D-US and HRA were performed in 108 patients. RESULTS: In patients with portal venous aneurysm, hepatopetal blood flow was increased, while portal perfusion did not differ from controls. In portal hypertensive patients, D-US detected dilatation of the portal system veins, with decreased blood flow. In comparison to the portal perfusion in controls and portal venous aneurysm, values were significantly (p < 0.01) lower in chronic active hepatitis and liver cirrhosis and differed between themselves (p < 0.01). In the groups of cirrhotic patients with esophageal varices, sclerosed esophageal varices, recanalized umbilical vein, partial portal thrombosis and cavernous portal vein with hepatofugal, hyperkinetic or slow blood flow, and very low velocities beside the thrombi, portal perfusion was lower (p < 0.01) than in controls, portal venous aneurysm, chronic active hepatitis and liver cirrhosis without collaterals. In complete thrombosis, minimal collateral flow was found with D-US, while HRA proved no portal supply. CONCLUSIONS: D-US and HRA are complementary for the estimation of various liver vascular disorders.     

Incidence of portal vein thrombosis in liver cirrhosis. An angiographic study in 708 patients

Portal vein thrombosis was thought to be a common complication of liver cirrhosis in the past. The incidence of angiographically demonstrable portal vein thrombosis was studied in 708 consecutive patients with unequivocal cirrhosis seen in the past 10 yr in whom either transhepatic portography or superior mesenteric arterial portography clearly delineated the major portal vein system. Excluding 2 cases that were thought to be associated with past splenectomy, there were 4 cases of portal vein thrombosis related to cirrhosis, all in a decompensated stage. The calculated incidence of portal vein thrombosis was 0.573% of all cirrhotic patients without splenectomy in the past. They constituted 23.5% of the 17 cases of extrahepatic portal vein obstruction encountered during the same period. There were 78 cases of idiopathic portal hypertension similarly studied angiographically, and the incidence of portal vein thrombosis unrelated to splenectomy was 2.86%. A statistical survey based on 247,728 necropsies recorded in the Japan Autopsy Registries of 1975-1982 showed a 0.05489% incidence of portal vein thrombosis and a 6.58857% incidence of cirrhosis of all types among them, suggesting that portal vein thrombosis is not a common complication of cirrhosis in Japan in recent years.     

Cruveilhier-Baumgarten syndrome in which venous hum disappeared following endoscopic variceal sclerotherapy

We report a case of Cruveihier-Baumgarten syndrome associated with portal vein thrombosis that developed, slowly during a 2-year period after, endoscopic variceal sclerotherapy. The thrombosis led to the disappearance of the venous hum and the dilated abdominal wall veins characteristic of this syndrome. A 73-year-old woman was hospitalized for treatment of esophageal varices in April 1988. Her spleen was markedly enlarged, and the histologic findings of her liver were not consistent with hepatic cirrhosis, but with idiopathic portal hypertension. A venous hum was audible in the upper abdomen. Superior mesenteric angiography revealed a porto-systemic shunt vessel under the abdominal wall, originating from the umbilical vein. She was injected four times with a sclerosant, and this brought about disappearance of the esophageal varices. Two years after the first admission, the venous hum was no longer audible, but there was a recurrence of the esophageal varices. More than 2 years later (4 years after the first admission), ultrasonographic study, computed tomography, and angiography showed a large thrombus, which completely obstructed the portal vein at the origin of the umbilical vein, and the development of collateral vessels, seen as a “cavernous transformation”.     

Low prevalence of femoral venous thrombosis after cardiac catheterizations in children: a prospective study

OBJECTIVE: Cardiac catheterization is an important and frequent diagnostic intervention in children, but few systematic studies have explored the associated venous thrombotic complications. We have prospectively evaluated the prevalence of venous thrombosis, diagnosed by ultrasonography, in children catheterized at our department. MATERIALS AND METHODS: We examined 50 children with weight greater than seven kilograms for thrombosis of the femoral vessels. Prophylactic anticoagulation was given to half of the children who were to undergo left-sided catheterization. The enrolled children had ultrasonography of the site of puncture the day before, and the day after, the cardiac catheterization. During catheterization, blood samples were taken from 33 of the children for analysis of thrombophilic agents. RESULTS: The children, with a median age of 34 months, had been catheterized 103 times, comprising the 50 current and 53 previous procedures. Over the course of the current procedure, interventions of various types were performed in 18 children (36%). We diagnosed thrombophilic predispositions in four patients. Despite the thrombophilic tendencies, and a high frequency of interventions, we did not encounter a single case of femoral venous thrombosis. Nor did we find any evidence of arterial thrombosis. CONCLUSION: Our study indicates that catheterization procedures in children are currently associated with a low prevalence of femoral venous thrombosis. Continuous assessment of vascular complications, nonetheless, is still required to prevent late effects, and to ensure the best quality of cardiac catheterisations in children.