Membranous obstruction of the inferior vena cava and systemic lupus erythematosus

A 37-year-old woman who presented with systemic lupus erythematosus was found to have a membranous obstruction of the inferior vena cava above the hepatic veins, with an extensive collateral circulation. The obstruction was relieved by a renal vein-right atrial interposition graft, and this was followed by disappearance of the anti-nuclear and anti-DNA antibodies. We postulate that the systemic lupus erythematosus might have been provoked by life-long immune stimulation due to bowel-derived antigens bypassing the hepatic reticuloendothelial system and reaching the systemic circulation, or by immune hyperreactivity associated with the abnormal abdominal venous circulation.     

Budd-Chiari syndrome caused by membranous obstruction of the inferior vena cava associated with coeliac disease

Ten cases of Budd-Chiari syndrome associated with coeliac disease have been reported in the literature, most of them in North African subjects. Supporting this association, we report a new case in a young Spanish Caucasian man in whom the cause of the syndrome was the membranous obstruction of the inferior vena cava, an infrequent cause of Budd-Chiari syndrome in Western countries. A percutaneous balloon angioplasty was performed, with satisfactory outcome.     

Budd-Chiari syndrome associated with obstruction of the inferior vena cava. A report of seven cases

Seven patients with the Budd-Chiari syndrome associated with obstruction of the inferior vena cava are described. Six of these showed membranous obstruction. In these patients elevation of venous pressure in the lower limbs, abnormal bromsulfalein tests with minor changes in the other liver function tests, hepatomegaly and insidious onset of symptoms were the common features. Edema of the legs, ascites and dilation of the superficial veins were also found in most cases, but abdominal pain and jaundice were rarely observed. Duration from initial symptoms to admission was relatively long (one to seventeen years).     

A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction

Transjugular intrahepatic portosystemic shunt (TIPS) is the standard treatment of Budd-Chiari syndrome (BCS) non responsive to medical therapy. However, patients with inferior vena cava (IVC) obstruction proximal to the atrium do not benefit from TIPS and a surgical approach is mandatory. We report the case of BCS due to intrapericardial IVC obstruction. We describe a novel surgical approach using a fresh caval homograft. An attempt to balloon dilatation of the IVC obstruction was complicated by right atrial disruption with tamponade and ventricular fibrillation. Lately, the patient successfully underwent a reconstruction of the cavo-atrial continuity by the interposition of a fresh caval homograft, a novel surgical approach never described before for BCS. Further follow-up revealed progressive reduction and resolution of ascites, and overall clinical improvement. IVC obstruction near to the atrium can be surgically approached with a new technique consisting in inferior vena cava resection and replacement with a caval homograft.     

Renal vein thrombosis and inferior vena cava thrombosis in systemic lupus erythematosus

Phlebography of the inferior vena cava with selective study of the renal veins was performed in 43 patients with systemic lupus erythematosus (SLE). Inferior vena cava thrombosis (IVCT) or renal vein thrombosis (RVT) was found in 3 of 11 patients (27%) with nephrotic syndrome, in 8 of 13 (61.5%) with previous thrombophlebitis, and in 3 of 4 (75%) with suggestive acute clinical picture. In contrast, none of the 20 control patients with SLE had IVCT or RVT. These results show that SLE patients with thrombophlebitis have a very high risk of developing IVCT or RVT; patients with nephrotic syndrome have a smaller risk. Neither IVCT nor RVT was found in SLE patients without antecedent thrombophlebitis or nephrotic syndrome.     

Histopathology of membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome.

It is generally believed that membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome is caused by congenital malformation. However, it does not explain the late onset of the disease. In the current study, hepatic portion of the inferior vena cava and hepatic veins were studied in 17 autopsy cases of the Budd-Chiari syndrome, 16 of which had no demonstrable cause (idiopathic). A sufficient amount of vena cava tissue was available for evaluation in 15 cases. Nine had membranous obstruction, with thickness varying from 3 to 8 mm. Thrombus formation was recognized in 7 of 9 cases. Occlusion of hepatic vein orifices of varying degree was present in 8 cases. In these occluding lesions, the basic structure of the venous wall was maintained. The intima was transformed into a fibrous laminar structure, and organized thrombi of varying ages were recognized; they were a mixture of fresh thrombi, organized thrombi, fibrous tissues, recanalizations, and calcifications. It is concluded that in these cases of the Budd-Chiari syndrome, occluding and stenosing lesions in the inferior vena cava and hepatic veins were thrombosis and its sequelae. There was no indication of congenital malformation.     

Chronic Budd-Chiari syndrome due to obstruction of the intrahepatic portion of the inferior vena cava

Clinical features of obstruction of the intrahepatic portion of the inferior vena cava were observed in five out of the 11 patients with the Budd-Chiari syndrome seen during the last four years. These patients apparently formed a distinct group from the remaining six and resembled in clinical course, biochemical features, haemodynamic findings, and radiological investigations the patients described as cases of membranous obliteration of the intrahepatic portion of the inferior vena cava. The present study reports five such patients diagnosed with the help of venous catheterization, percutaneous hepatography, and haemodynamic studies. The value of distinguishing these patients from those with obstruction due to hepatic vein occlusion is highlighted because of the difference in the prognosis and treatment.     

Leiomyosarcoma of the inferior vena cava presenting as Budd-Chiari syndrome

A case of a 72-year-old male with a leiomyosarcoma of the inferior vena cava causing Budd-Chiari syndrome is described. Percutaneous decompression of the portal system was attempted but with no success. At autopsy, a large tumor arising from and completely obstructing the inferior vena cava was found, histologically proven to be a leiomyosarcoma. Over 50 cases of leiomyosarcoma of the inferior vena cava have been reported in the literature, the majority of which occurred in women and 1/3 of which were associated with the Budd-Chiari syndrome. Histologically up to two mitotic figures per 10 high power fields have been scored. This case indicates the unreliability of a low mitotic index as an indicator of benign behavior of smooth muscle neoplasms of the venous system.     

Surgical repair for entire inferior vena cava occlusion in Budd-Chiari syndrome

Successful repair of thrombotic occlusion of the entire inferior vena cava, including the iliac and hepatic veins, was performed on a 51-year-old man with Budd-Chiari syndrome associated with severe liver dysfunction.     

Canada-Cronkhite syndrome associated with systemic lupus erythematosus

A 43-year-old woman with a history of systemic lupus erythematosus manifested severe diarrhea, generalized gastrointestinal polyposis, pigmentation, and onychodystrophy, which were typical of Canada-Cronkhite syndrome. The exacerbation of systemic lupus erythematosus, presenting with hemolytic anemia and subsequently with profound proteinuria, was seen during the course of this syndrome. The patient achieved complete symptomatic remissions of both entities for more than four years following prednisolone therapy; the resolution of the gastrointestinal polyps was confirmed histologically. This case showed that this syndrome could be completely reversible in its course and that it might have an exogenous cause, as previously suggested.     

Moyamoya syndrome associated with systemic lupus erythematosus

Moyamoya disease is a rare, progressive cerebrovascular disorder that is characterized by a stenosis or occlusion of the bilateral internal carotid arteries and the development of collateral vessels. Transient ischemic attacks or seizures are the usual presentation of moyamoya disease in children, whereas cerebral hemorrhage is the most common symptom in adults. We report an 18-year-old female patient with active lupus nephritis who presented with the sudden onset of left hemiparesis. Brain magnetic resonance imaging showed acute infarctions in the right basal ganglia and subcortical white matter of the right frontal lobe. Cerebral angiography showed the stenosis of the bilateral internal carotid arteries with rich basal collateral vessels (moyamoya vessels). There was no evidence of atherosclerosis or antiphospholipid syndrome. Glucocorticoid therapy was used to control the systemic lupus erythematosus. Prophylactic bypass surgery was performed to prevent recurrent ischemic attacks. This case report shows that an underlying cerebrovascular lesion of moyamoya vessels in a patient with systemic lupus erythematosus is susceptible to cerebrovascular accidents.     

Budd-Chiari syndrome and membranous obstruction of the suprahepatic inferior vena cava. Treatment by percutaneous intraluminal angioplasty

A 38-year-old woman, suffering from a Budd-Chiari syndrome due to membranous obstruction of the inferior vena cava, has been treated by percutaneous trans-luminal angioplasty. The result is excellent six months after. The permeability of the vena cava has been studied by manometry and cavography. This innocuous procedure seems worth while in the treatment of Budd-Chiari syndrome with membranous obstruction of the inferior vena cava.     

Ultrasound study in the diagnosis of primary Budd-Chiari syndrome (obstruction of the inferior vena cava)

Primary Budd-Chiari syndrome, obstruction of the hepatic portion of the inferior vena cava (IVC), is a rare disorder, but relatively prevalent in the Far East, northern India and Africa. Ultrasound examination was carried out on 9 patients with primary Budd-Chiari syndrome. There were 5 men and 4 women aged 27–60 years. In all the 9 cases, the diagnosis was confirmed by cavography and liver histology. Moreover, 7 of the 9 subsequently underwent radical operation using a patch graft. Ultrasonic study showed several characteristic findings suggestive of the syndrome, and frequencies of the main findings were as follows: 1) an echogenic obstructing membrane; 22.2%, 2) segmental obstruction of the IVC; 77.8%, 3) occlusion of the major hepatic veins at the juxtacaval portion; 100%, 4) enlarged inferior right hepatic veins; 55.6%, 5) abnormal intrahepatic venous structures and collaterals; 88.9%. Of these findings, 5) was the most prominent and most characteristic in the diagnosis of the syndrome. It is necessary for early detection of this entity to evaluate carefully intrahepatic venous abnormalities and patency of either the IVC or major hepatic veins on ultrasonic examination. The careful examination for Budd-Chiari syndrome should be done in cases with cryptogenic liver cirrhosis, particularly in countries where the prevalence of the syndrome is high.