Budd Chiari syndrome, visceral arterial occlusions, recurrent fetal loss and the "lupus anticoagulant" in systemic lupus erythematosus

A 30-year-old Chinese woman had been diagnosed as having systemic lupus erythematosus (SLE) at age 25 after 4 spontaneous abortions, all between 8 and 12 weeks of gestation. A year later she developed mild jaundice and at laparotomy, nodular enlargement of the right lobe of the liver and splenomegaly were seen. She was found to have a "lupus anticoagulant" and false positive test for syphilis (VDRL), but persistently negative antibodies to cardiolipin. Angiography demonstrated occlusion and stenoses of visceral abdominal arteries and hepatic venography also showed evidence of hepatic venous occlusions. She subsequently developed thyrotoxicosis which was treated with carbimazole, followed by radioactive iodine. The SLE and lupus anticoagulant were treated with systemic steroids (prednisolone) and anticoagulation (warfarin).     

Transformation of inferior vena caval thrombosis to membranous obstruction in a patient with the lupus anticoagulant

A 24-yr-old woman with hemolytic anemia developed multiple thrombosis of the hepatic vein and inferior vena cava. She was found to have circulating lupus anticoagulant that could have been causally related to the thrombosis and hence the Budd-Chiari syndrome. On her first admission to the hospital vena cava and hepatic vein catheterizations revealed partial thrombotic occlusion of the cava at the level of the diaphragm, which was subsequently transformed into complete membranous obstruction. The right hepatic vein, which was patent on the first admission, was also completely occluded. These observations support the theory that membranous obstruction of the inferior vena cava is a sequela to inferior vena caval thrombosis rather than a congenital anomaly.     

Occlusion of small hepatic veins associated with systemic lupus erythematosus with the lupus anticoagulant and anti-cardiolipin antibody

We report the case of a woman with lupus anticoagulant-positive systemic lupus erythematosus who developed small hepatic vein occlusion. Since the age of 34, she had been known to have hepatomegaly. A definitive diagnosis of systematic lupus erythematosus was made eight years later. Histological evaluation of the liver biopsy specimen was not fully diagnostic of prominent hepatomegaly during this period. Occlusion of the small hepatic veins was confirmed by hepatic venography, but the lumen of the large hepatic veins showed a smooth appearance. The lupus anticoagulant and anti-cardiolipin antibody were both positive. Since a high incidence of thromboembolic diseases in patients with the lupus anticoagulant or anti-cardiolipin antibody has been reported, the presence of this type of anticoagulant may provide an explanation for hypercoagulability and subsequent development of hepatic vein thrombosis in this patient. This is the first report of a patient with systemic lupus erythematosus who developed an occlusion of small hepatic veins attributable to the lupus anticoagulant and anticardiolipin antibody. This case suggested that a systematic search for hepatic vein occlusion should be made in patients with systemic lupus erythematosus who have developed inexplicable hepatomegaly, especially in those with positive tests for the lupus anticoagulant and/or anti-cardiolipin antibody.     

Caval occlusion technique for hepatic venous sampling: a new approach to estimating splanchnic substrate balance in conscious dogs.

We have proposed a new technique for sampling hepatic venous blood in conscious dogs. Sub-hepatic vena caval blood flow was temporarily occluded by a previously implanted inflatable snare so that all blood entering the inferior vena cava was hepatic venous effluent. Hepatic venous blood samples were collected from the inferior vena cava 8 seconds after beginning caval occlusion, with the total interval of flow occlusion lasting 12 to 15 seconds. No behavioral or metabolic alterations were observed when or metabolic alterations were observed when hepatic venous effluent was repetitively sampled using the caval occlusion technique. Net splanchnic glucose balance (NSGB) was measured in conscious dogs receiving saline, glucose or glucagon infusions. NSGB measurements made with the caval occlusion technique were in accord with previous results obtained via tracer methodology or arterio-venous difference techniques utilizing hepatic vein catheterization. The caval occlusion technique thus provides a method for collecting hepatic venous blood samples from conscious animals without the difficulties associated with hepatic vein catheterization.     

Hepatic sinusoidal dilatation with portal hypertension during azathioprine treatment after kidney transplantation

An unusual hepatic disease developed in 3 patients with a well-functioning kidney graft 16-24 months after transplantation. Vague abdominal pain, increased bleeding tendency and edema were initial complaints, and hepato- or splenomegaly and ascites were found as well. Liver function tests were not or only mildly disturbed; hemolysis and pancytopenia were always present. Colloid uptake was absent at liver scintigraphy and the hepatic venous wedge pressure was increased. Esophageal varices were demonstrated. Liver biopsy showed extensive midzonal and pericentral sinusoidal dilatation. After discontinuation of azathioprine the symptoms and the extent of sinusoidal dilatation disappeared gradually, but after 1-3 years fibrosis or micronodular cirrhosis had developed and splenomegaly with hypersplenism remained. These observations strongly suggest an association between chronic use of azathioprine and the development of venous congestion of the liver with sinusoidal dilatation, eventually resulting in chronic liver disease.     

Budd-Chiari syndrome with fatal outcome in a patient with polycythemia vera and antiphospholipid antibody syndrome

We report the case of a 41-year-old woman, affected by Vaquez syndrome, admitted to our hospital for a severe pain in the right hypochondrium, suddenly followed by hepatomegaly and ascites. The clinical and laboratory data were suggestive of hepatic insufficiency and abdominal ultrasonography, integrated by color Doppler and computed tomography, revealed an interrupted hepatic venous outflow. In addition a spontaneous prolonged partial thromboplastin time was present and the patient was found to be positive for lupus anticoagulant. A transient clinical improvement, with a partial reperfusion of suprahepatic veins, was achieved with medical treatment by using anticoagulants, diuretics and paracentesis. However, the patient showed a subsequence of suprahepatic venous thrombosis, although two transjugular intrahepatic portosystemic shunts with stent placement and local thrombolysis were performed. The polycythemia vera is a disease mainly associated with Budd-Chiari syndrome but, in our patient, the thrombotic event occurred in spite of normal values of hematocrit and platelet count. Certainly in this case the lupus anticoagulant positivity represents an additional thrombogenic factor. Nowadays the antiphospholipid antibody syndrome is a recognized and not unusual cause of Budd-Chiari syndrome but, to our knowledge, this is the first case characterized by the presence of polycythemia vera and antiphospholipid antibody syndrome to be reported.     

Budd-Chiari syndrome with inferior vena cava obstruction associated with systemic lupus erythematosus

The Budd-Chiari syndrome is an uncommon condition in which hepatic venous outflow is obstructed by thrombosis of the major hepatic veins. Many of the cases are idiopathic, but it has been described in association with vena caval webs, abdominal trauma, retroperitoneal neoplasms, and hypercoagulable states. A patient with systemic lupus erythematosus (SLE) who developed the Budd-Chiari syndrome with inferior vena cava thrombosis led to a review of the possible association between SLE and the Budd-Chiari syndrome. The therapy of the Budd-Chiari syndrome with associated vena cava thrombosis is also discussed.     

Monocrotaline-induced Budd-Chiari syndrome in monkeys

Budd-Chiari syndrome was produced in adultMacaca speciosa monkeys within 3 months by the parenteral administration of the pyrrolizidine alkaloid monocrotaline. These animals developed ascites, distended abdominal veins, hypoproteinemia, and an increase in portal venous pressure. The vascular lesions included partial to complete occlusion of hepatic veins throughout the liver. These venous alterations were initiated by endothelial lysis. As a result of the altered permeability, blood components collected throughout the vessel wall, producing marked disruption. Occlusion of the affected vessels followed the encroachment of the thickened edematous wall on the lumen. Eventually these hepatic veins underwent fibrous connective tissue repair, giving rise to widespread focal fibrosis throughout the livers.Supported by USPHS FR-0167 and HE-10941.Presented in part at the Sixty-Seventh Annual Meeting of the American Association of Pathologists and Bacteriologists, St. Louis, March 8, 1970.     

LUPUS ANTICOAGULANT MASQUERADING AS AN ACUTE ABDOMEN WITH MULTIORGAN INVOLVEMENT

A 45-yr-old male patient developed acute abdominal pain, ileus, and microscopic hematuria with biochemical evidence of pancreatitis and a marked increase in liver alkaline phosphatase; CT demonstrated swelling of the pancreas, bilateral adrenal hemorrhage, and a suggestion of renal hemorrhage. ERCP was negative and renal arterial and venous blood flow normal. A coagulation profile demonstrated the presence of lupus anticoagulant, but tests for anticardiolipin antibodies and collagen vascular diseases were negative. Treatment with corticosteroids and anticoagulation resulted in improvement in clinical and all biochemical indices. Thus, Iupus anticoagulant syndrome may masquerade as an acute abdominal illness with multiorgan involvement.     

Portal thrombosis due to intrahepatic cholangiocarcinoma following successful treatment for hepatocellular carcinoma

A 57-year-old man, who had undergone hepatic arterial infusion chemotherapy with right portal occlusion for hepatocellular carcinoma was admitted to our hospital because of severe abdominal pain. Contrast-enhanced computed tomograms revealed that most areas of the liver were not enhanced, a finding suspicious for perfusion disturbance in the liver. Angiography revealed an interrupted right hepatic artery. Arterial portograms revealed complete obstruction of the right portal vein and a small left branch of the portal vein. Despite anticoagulant therapy with urokinase for portal vein thrombosis, the patient died from hepatorenal failure. Autopsy revealed that cholangiocarcinoma occupied almost the entire parenchyma of the right lobe, although the treated hepatocellular carcinoma lesion was completely necrotic. The right hepatic artery was obstructed due to direct invasion of tumor. There were diffuse thrombi in the left portal branches surrounded by tumor infiltrating along Glisson's sheath to the peripheral portion of the left lobe.     

Aortic occlusion in systemic lupus erythematosus associated with antiphospholipid antibodies.

The patient, a Caucasian woman of 22 years, developed malignant hypertension at the age of 16 years. An abdominal bruit was found on routine examination. She had two spontaneous abortions and systemic lupus erythematosus was diagnosed at age 21 years. She was found to have a persistently low positive VDRL and antibodies to mitochondria were also present on repeated examinations, to a titre of 1/160. Because of angiographic findings, demonstrating an occlusion of the aorta, an endarterectomy of the descending thoracic and upper abdominal aorta was performed. This showed mainly intimal thickening and the presence of thrombus. She then had four further spontaneous abortions (with good blood pressure control). The lupus anticoagulant and antibodies to cardiolipin were found to be positive at this time.     

Detection of caval obstruction by magnetic resonance imaging after intraatrial repair of transposition of the great arteries

Vena caval obstruction may cause significant morbidity after intraatrial repair of transposition of the great arteries (TGA). Two noninvasive methods of diagnosing vena caval obstruction were compared with cardiac catheterization. Echocardiographically gated magnetic resonance imaging (MRI) and echocardiographic evaluation (2-dimensional saline contrast echocardiography and pulsed Doppler flow measurement) were performed on 15 patients 0.7 to 13.5 years after intraatrial repair of TGA (8 Mustard, 7 Senning). At catheterization, complete superior vena cava or partial caval obstruction (gradient greater than 5 mm Hg from cava to systemic venous atrium) was present in 7 of 15 patients. Superior vena cava obstruction was directly visualized by MRI in both patients with catheterization-proved complete superior vena cava occlusion. A dilated azygous/hemiazygous venous complex (greater than or equal to 5 mm cross-sectional diameter) was seen by MRI in 5 of 7 patients with complex or partial vena caval obstruction and in no patient without vena caval obstruction. MRI showed superior vena caval dilatation (ratio of superior vena caval diameter to aortic diameter greater than 1.45) in 3 of 5 patients with partial vena caval obstruction and in 0 of 8 without vena caval obstruction. Direct visualization of narrowing within the atrium was unreliable for any MRI plane because of the 3-dimensional nature of the intraatrial baffle. Two-dimensional saline contrast echocardiography, successfully performed in 12 of 15 patients, detected complete superior vena caval obstruction only in the 2 patients with catheterization-proved complete superior vena cava occlusion. Contrast echocardiography failed to identify any of the 5 patients with partial vena caval obstruction.(ABSTRACT TRUNCATED AT 250 WORDS)     

The abdominal manifestations of the antiphospholipid syndrome

OBJECTIVES: To study the abdominal manifestations of the antiphospholipid syndrome (APS). METHODS: We reviewed the medical literature from 1968 to 2006 using MEDLINE and the key words: APS, anticardiolipin antibodies, lupus anticoagulant, antiphospholipid (aPL) antibodies, catastrophic antiphospholipid syndrome, liver, hepatic biliary, pancreas, spleen, gastrointestinal and abdominal. RESULTS: Liver involvement is the most frequent abdominal manifestation associated with APS. Various hepatic manifestations have been reported including Budd-Chiari syndrome, hepatic-veno-occlusive disease and occlusion of small hepatic veins, nodular regenerative hyperplasia, hepatic infarction, cirrhosis, portal hypertension, autoimmune hepatitis and biliary cirrhosis. Acute intestinal infarction, intestinal angina, and intestinal bleeding have also been reported in association with aPL in addition to few sporadic cases of splenic infarction and acute pancreatitis. CONCLUSION: A high index of suspicion for any signs of abdominal involvement should be considered in patients with APS. In addition screening for aPL should be carried out in patients who present with hepatic vein occlusion and unexplained signs of intestinal angina.     

经颈静脉肝内门体分流术及肝静脉成形术治疗肝静脉型Budd-Chiari综合征的临床意义

目的分析经颈静脉肝内门体分流术(TIPS)及经皮肝或经颈静脉途径肝静脉成形术等介入手段治疗肝静脉型Budd-Chiari综合征(BCS)的临床意义。方法回顾性分析2000年5月至2012年10月收治的32例肝静脉型BCS的临床资料。其中男15例,女17例;平均年龄(38±6)岁;肝功能Child-Pugh评分(9.6±2.2)分;肝静脉近段闭塞8例,肝静脉闭塞合并下腔静脉狭窄4例,全肝静脉闭塞11例,肝小静脉闭塞9例;急性3例,亚急性或慢性29例。患者主要临床表现为顽固性腹水和食管静脉曲张破裂出血,采用彩色多普勒超声、CT血管造影或MRI血管造影、上消化道钡餐及内镜检查明确诊断。治疗方法包括:TIPS 9例,改良TIPS 11例,单独经皮肝或经颈静脉途径肝静脉成形术8例,经皮肝肝静脉成形术联合下腔静脉成形术4例。结果所有患者均成功完成介入或手术治疗,其中TIPS或改良TIPS术后,患者出血控制,腹水逐渐消退,肝功能明显好转;门静脉压力由(42±8)cm H2O(1 cm H2O=0.098 kPa)下降至(27±5)cm H2O(t=20.20,P=0.001),门静脉血流速度由(18±6)cm/s增加至(52±10)cm/s(t=15.02,P=0.001)。住院期间因肝功能不全死亡1例,肝门分流道急性阻塞1例。术后随访3~241个月,平均随访(102±26)个月,分流道狭窄性扩张内支撑2例,肝静脉狭窄再扩张2例。结论经皮肝或经颈静脉途径肝静脉成形术与TIPS均是治疗肝静脉型BCS的有效方法,可根据患者病情酌情选择治疗方法。     

The Simon nitinol filter: evaluation by MR and ultrasound.

In this prospective blinded study of inferior vena caval (IVC) patency, 18 patients underwent 25 duplex ultrasound (US) and magnetic resonance (MR) angiography examinations over an eight-month period following Simon nitinol filter placement. Clinical examination for lower extremity venous stasis and plain abdominal radiography were also performed. Twenty-three of 24 MR examinations and 11 of 24 US examinations were judged technically adequate by the blinded observers. One technically adequate US exam was false positive for intraluminal caval thrombus. Thirteen technically inadequate US examinations missed 3 complete caval occlusions and 2 partial occlusions. MR identified all patients with complete or partial caval occlusion. The authors conclude that duplex US reliably confirms IVC patency only when strict criteria for technical adequacy and interpretation are met (good visualization of filter and IVC above and below filter). MR, although expensive, more reliably identifies nonoccluding intraluminal thrombus and caval occlusion. It should be the noninvasive study of choice in symptomatic patients with venous stasis and patients with recurrent pulmonary emboli.     

Caval thrombosis in a young athlete.

We here describe a clinical case of caval thrombosis of sudden and unexpected onset, in an athlete without signs of venous insufficiency. Pre-existing caval hypoplasia was observed through imaging examinations. Following a review of the literature, the authors interpret the pathophysiological mechanism of thrombosis as such: a combination of intense physical activity and the malformation led to an overwhelming venous flow and a consequent congestion. Caval thrombosis distal to the malformation is an absolute indication to lifelong oral anticoagulant therapy.     

Antiphospholipid syndrome with mesenteric vein thrombosis and hepatic nodular regenerative hyperplasia in a child: A case report

Rationale:Hepatic nodular regenerative hyperplasia (NRH), a nonspecific change in the liver parenchyma, is very rare in children. Hepatic microvascular changes may be the cause, as these vascular changes are uncommon in children. Antiphospholipid syndrome (APS), an autoimmune disease characterized by vascular thromboembolism, is extremely unusual in children.Patient concerns:A 13-year-old girl who presented with abdominal pain and elevated liver enzymes was transferred to our hospital. Abdominal computed tomography and magnetic resonance imaging showed a massive mesenteric venous thrombus and a malignant mass with liver metastasis.Diagnoses:Her immunological profile was positive for antinuclear antibodies (ANA) at a titer of 1/160 (nucleolar pattern), anticardiolipin antibodies (aCL) immunoglobulin G, and anti-histone antibody. A liver biopsy revealed hepatic NRH.Interventions:The patient was initially started on heparin upon hospitalization and switched to warfarin and a vitamin K antagonist and continued treatment with international normalized ratio monitoring.Outcomes:Her symptoms improved after 9 months of anticoagulation therapy.Lessons:In the presence of hepatic NRH or vascular thrombosis in children, we recommend that APS be differentially diagnosed using lupus anticoagulant and aCL and appropriate management be implemented.     

Hepatic venous outflow obstruction in patients with polycystic liver disease: pathogenesis and treatment.

Polycystic liver disease is commonly asymptomatic but may present with hepatomegaly, abdominal distension, and dull abdominal pain. Transudative ascites is a rare manifestation in these patients but may occur when portal hypertension is present resulting from associated hepatic fibrosis or after deroofing procedure of a cyst. Exudative ascites might suggest hepatic venous outflow obstruction. Four cases are described where hepatic venous outflow obstruction occurred in patients with polycystic liver disease. Three patients had orthotopic liver transplantation and one had a mesocaval shunt. Of the two patients that survived orthotopic liver transplantation both have shown considerable improvement in their symptoms. None of the patients had any confirmed procoagulant disorder. The mechanism of hepatic venous outflow obstruction in these patients seems to be mechanical compression of hepatic veins by the cysts and associated formation of thrombi in small hepatic vein tributaries. Patients with severe polycystic kidney/liver disease are at risk of hepatic venous outflow obstruction and the onset of this complication is heralded by tender hepatomegaly and presence of exudative ascites.     

Management of Budd–Chiari Syndrome

Thrombotic occlusion of the hepatic veins leads to liver dysfunction and liver failure requiring liver transplantation in advanced cases. The cause for the occlusion of the hepatic veins is not completely understood. However, several underlying conditions such as polycytemia, factor V Leiden mutation, and protein C and S deficiency are found in these patients. We here report our single-center experience with 18 consecutive patients with Budd–Chiari Syndrome (BCS) who were treated at our institution between August 1992 and June 2003. Twelve patients underwent liver transplantation, three patients received stents into the hepatic veins or vena cava, another patient was treated with TIPSS (transjugular intrahepatic postosystemic stent shunt), and one patient underwent surgical mesocaval shunting. Three patients, among those the patient with TIPSS, were put on anticoagulant therapy and are scheduled for liver transplantation. We outline the indication for an approach tailored to the stage of the disease and the adaption of the procedures with the deterioration of clinical conditions. Surgical aspects and postoperative management with a focus on liver transplantation are outlined. We conclude from our observations that the management of BCS requires an approach that exhausts conservative approaches until clinical conditions deteriorate with respect to portal hypertension or liver function. Conservative management, i.e., interventional and supportive medical therapy, has been used up to 8 years in our series, until the time for liver transplantation is reached. Liver transplantation for BCS had more complications than transplantation for other liver diseases in our series. Therefore, we propose to keep liver function stable using interventional techniques to maintain venous outflow. If venous outflow cannot be interventionally restored and liver function deteriorates or cirrhosis develops during this time course, liver transplantation is the therapy of choice.