A rare case of idiopathic retroperitoneal fibrosis involving obstruction of the mesenteric arteries, duodenum, common bile duct, and inferior vena cava

Idiopathic retroperitoneal fibrosis (IRF), usually affects the ureter, although the biliary tree, duodenum and vasculature may also be susceptible. This case report describes a 64-year-old man with IRF, who presented painless watery diarrhea, radiological features of obstructive jaundice and duodenal obstruction, and ultimately an obstruction of the inferior vena cava. We employed tamoxifen for his treatment, but the disease progressed and the patient died of multiple organ failure two years after the onset. While the cause of IRF in this patient was obscure, we suspected his painless watery diarrhea indicated chronic ischemia of the small bowel, and the findings of an abdominal CT scan were extremely valuable in indicating IRF.     

Primary Budd-Chiari syndrome: its significance for the choice of surgical management of a patent right inferior hepatic vein

Budd-Chiari syndrome is still a major problem and the overall prognosis for the patients is dismal and disappointing. The case history of a patient with not only outflow obstruction of the hepatic vein but also complete obstruction of the retrohepatic inferior vena cava is presented. She had a patent right inferior hepatic vein which partially decompressed the liver via a rich network of venous collaterals. Management included an inferior vena cava to inferior vena cava shunt using a Goretex graft. The patient fared well and the postoperative course was satisfactory. The case history of this patient illustrates the importance of precise pre-operative investigations for the choice of the type of surgical management. Each patient has to be considered individually on the basis of his or her mechanical peculiarities.     

Kidney tumour mimicking cardiac mass

The discovery of a mass in the right atrium obliges the clinician to perform a broad differential diagnosis between a primary cardiac tumour (with myxoma being the most frequent), invasion of an extracardiac tumour, vegetations on the tricuspid valve, and an atrial thrombus. We describe the case of a patient who was admitted to our service with a diagnosis of suspected myxoma based on the chance transthoracic echocardiographic discovery of a right atrial mass. A transesophageal echocardiogram showed the process to be extracardiac, and magnetic resonance imaging showed it to originate at the renal level extending via the inferior vena cava to the right atrium. Tumour extension with thrombosis of the vena cava is a relatively frequent complication of renal carcinoma, but only exceptionally does it reach the right atrium. It is also exceptional that this was a chance finding in an asymptomatic patient.     

Budd-Chiari syndrome as a late complication following closure of an atrial septal defect

A case of a Budd-Chiari syndrome in a 19-year-old female patient is reported who had undergone surgical closure of a secundum atrial septal defect 13 years before. 8 months before the development of the Budd-Chiari syndrome she started to take oral contraceptives. The clinical picture of the Budd-Chiari syndrome developed within several days. The inferior vena cava did not fill with contrast dye when an angiography was performed using the right vena iliaca approach. The contrast dye disappeared through collateral veins (vena azygos, vena hemiazygos). After 4 days of treatment with systemic streptokinase she underwent open-heart surgery. The orifice of the inferior vena cava was occluded to a diameter of 6 mm. No thrombi were found. The lesion was corrected with two patches, one in the right atrium and the other in the inferior vena cava. This case report demonstrates that a Budd-Chiari syndrome is a possible late complication after closure of an atrial septal defect which should be treated by surgery.     

Successful surgical management of a recurrent leiomyosarcoma of the inferior vena cava

The leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumour of the venous system. The recurrence of the tumour after previous initial surgical resection is common and occurs in more than half of the patients. Surgical resection of a local recurrence is poorly described in the literature and the available data are restricted to a small number of cases. We report the case of a 62 year old woman, who was referred to our vascular surgical unit for recurrence of a leiomyosarcoma of the inferior vena cava, 35 months after diagnosis and initial surgical treatment. We performed an extensive local resection and circumferencial replacement of the IVC. 18 months after the second operation and adjuvant radiotherapy, the patient is in a very good physical condition and CT-scans show no evidence of tumour recurrence.     

Fractured Inferior Vena Cava Filter Strut Presenting with ST-Segment Elevation and Cardiac Tamponade

The fracture of an inferior vena cava filter strut and its migration to the heart is a rare sequela of implanted inferior vena cava filters. Perforation through the right ventricle into the pericardium with resultant cardiopulmonary compromise is even less frequent. We report the case of a 53-year-old man who presented with chest pain and hypotension consequent to cardiac tamponade. A fractured inferior vena cava filter strut had migrated and perforated his right ventricle. The fractured strut was successfully removed by means of cardiac surgery. Inferior vena cava filters should be placed when necessary to minimize the risk of pulmonary embolism, and regular radiologic monitoring should be performed; however, the eventual extraction of retrievable filters should be considered. In addition to discussing the patient''s case, we briefly review the relevant medical literature.     

Contrast echocardiography of right atrial mass due to hepatocellular carcinoma

We describe one patient suffering from hepatocellular carcinoma who presented with a right atrial metastatic tumour as a result of invasion of the inferior vena cava and extension into the right atrium. Two-dimensional echocardiography disclosed the right atrium tumour and SonoVue contrast agent echocardiography was employed to assess the local extension of the mass and to exclude an important obstruction due to a mass in the right ventricular inflow. Surgical management in the presence of metastatic right atrial tumour thrombus is described in the literature with poor results and this type of treatment should be reserved only for selected cases in which acute and severe cardiovascular distress due to obstruction of right ventricular inflow is present.     

Trauma-induced thrombosis of the inferior vena cava

A 25 year old foreign patient, in his native country had a blunt polytrauma of the abdomen and of the thigh. The consequence was a complete thrombosis of the vena cava inferior in a distal position to the junction with the liver veins. The diagnosis was realized with phlebography, angiography and computertomography. The diaphragmatic part of the vena cava inferior and the liver veins were not affected by the thrombosis. The increasing edemas of the shank and a stasis ulcer which were the reason for the patients admission to the hospital could be treated successfully by Furosemid and Phenprocoumon within a period of 4 weeks.     

Right intra-atrial extension of hydatid cyst mimicking cardiac thrombosis. Apropos of a case]

The authors describe an unusual case of hydatid cyst inserted in the inferior vena cava and extending into the right atrium. The transoesoesophageal echocardiographic appearances were similar to those of a thrombus: the tumour was very mobile, echogenic, polylobular with a cord-like pedicle in the inferior vena cava. The pathological examination revealed a ruptured hydatid cyst. The mass and its insertion were not visible on CT scan or cavography. Transoesophageal echocardiography would therefore seem to be a very useful diagnostic method for tumours arising in the inferior vena cava and extending into the right atrium.     

Inferior vena cavectomy for renal cell carcinoma with intracaval tumor thrombus

Excision of the inferior vena cava for renal cell carcinoma with intracaval tumor thrombus is infrequently performed. Herein the authors report a 60-year old woman with a right renal cell carcinoma and massive occluding tumor thrombus of the inferior vena cava. Following a negative metastatic workup, this patient underwent surgery to remove the tumor and thrombus. Thrombectomy occurred via excision of the affected portion of inferior vena cava and proximal left renal vein. Reconstruction of the vena cava was not undertaken. The patient did not suffer any morbidity during recovery in hospital. Her renal function was normal upon discharge. All resection margins were negative for tumor. This experience is compared to those reported in the literature. Postoperative morbidity may be minimized by careful patient selection. Suitable patients should have a right-sided tumor with an occlusive subhepatic vena caval tumor thrombus.     

Leiomyosarcoma of the inferior vena cava: a case report

Leiomyosarcoma of the inferior vena cava is an extremely rare tumor that is characterized by a poor prognosis and nonspecific symptoms, a fact that may delay the diagnosis for several years. The only therapeutic modality proven to prolong the survival of patients is total surgical resection of the tumor. In this study, the authors report the case of a 50-year-old patient with a diagnosis of leiomyosarcoma of the inferior vena cava, affecting the middle and distal thirds, who was submitted to surgical treatment.     

High output heart failure 8 months after an acquired arteriovenous fistula

Congestive heart failure (CHF) due to hyperkinetic states can occur in systemic diseases and in arteriovenous fistulas. An 18 year old Turkish male patient complaining of dyspnea and palpitations, who had suffered a stab wound to his abdomen eight months earlier, was admitted to our clinic. Auscultation revealed a systolodiastolic murmur over the entire abdomen. Chest x-rays demonstrated significant cardiomegaly. Echocardiography revealed biatrial enlargement and significant mitral and tricuspid regurgitation accompanied by dilatation of the inferior vena cava. Right heart catheterization showed increased oxygen saturation at the inferior vena cava. A diagnosis of an aortocaval fistula was made by aortography. The symptoms subsided and valvular regurgitations ceased alter surgical correction. This rare case demonstrates the significance of routine physical examination and history of the patient.     

Combined resection of the inferior vena cava for hepato-biliary and pancreatic malignancies

Seven cases of hepato-biliary and pancreatic malignancies that underwent partial resection of the inferior vena cava) were reviewed. Histological findings of inferior venca cava involvement were direct invasion in 5 cases, tumor thrombus in 1 case, and adhesion in 1 case. Correct preoperative diagnosis of inferior vena cava involvement was made in only 2 cases. A retrospective study on enhanced CT revealed that irregular deformity of the inferior vena cava had suggested inferior vena cava involvement. Total occlusion of the inferior vena cava was employed temporarily for inferior vena cava resection in 3 cases. A saphenous vein graft was used for reconstruction in 2 cases. Complications due to inferior vena cava resection are, as yet, unknown. One patient is alive, without recurrence, 24 months after the operation. One case underwent re-resection of liver metastasis, and is alive 17 months after the operation. Although advanced hepato-biliary and pancreatic malignancies involving inferior vena cava have been regarded as having a poor prognosis, an aggressive surgical approach may be applicable in some cases.     

Massive deep vein thrombosis after cesarean section treated with a temporary inferior vena cava filter: a case report

A 25-year-old woman suffered a massive deep vein thrombus in her left common iliac vein extending to the inferior vena cava after an abdominal cesarean section. The massive and floating inferior vena cava thrombus was considered to pose a high risk of pulmonary thromboembolism. After placement of a temporary inferior vena cava filter via the left brachial vein, thrombolytic therapy and anticoagulation therapy were instituted. The filter successfully prevented pulmonary thromboembolism during thrombolytic therapy. This patient was confined to bed because the filter moved vertically with left shoulder joint abduction. Although a temporary inferior vena cava filter is very useful for the prevention of pulmonary thromboembolism in a patient with deep vein thrombus, the appropriate range of activity for such a patient needs careful consideration.     

Massive intrahepatic haemorrhage responsible for an inferior vena cava syndrome: an exceptional complication of hepatocellular carcinoma

Intralesional massive haemorrhage responsible for inferior vena cava syndrome is a very rare complication of hepatocellular carcinoma. A 36-year-old man with no past medical history was referred to hospital for abdominal pain with clinical and biological signs of internal bleeding. An abdominal CT scan showed a huge right liver mass, with regular margins and a fluid content, indicating a large intratumoral haemorrhage complicating hepatocellular carcinoma. Due to haemodynamic stability and the signs of inferior vena cava compression conservative management was chosen prior to surgical treatment of the tumour. Three months later, the huge liver mass remained unchanged but the inferior vena cava syndrome had decreased and the patient's condition improved. After evaluation of liver function and the extent of the tumour, a surgical procedure was performed via a subcostal incision with midline extension and sternotomy to control the intrapericardial inferior vena cava and perform a veno-venous bypass with the technique used for orthotopic liver transplantation. Then, right hepatectomy (segments V to VIII) was performed with an anterior approach. The postoperative course was uneventful. This two-step strategy allowed successful surgery for this rare complication of a hepatocellular carcinoma on a normal liver and could be recommended for the management of any unruptured intratumoral haemorrhages.     

Membranous obstruction of the inferior vena cava and invasion of the inferior vena cava by tumour in hepatocellular carcinoma

A 28 yr old Zulu presented with a painful swelling in the right hypochondrium and severe swelling of the legs of short duration. The serum alpha-fetoprotein concentration was over 2 X 10(5) ng/ml and imaging showed a large hepatic mass-lesion. Radionuclide venography revealed no flow through the inferior vena cava but flow through a large collateral vessel. Contrast venography showed the upper portion of the inferior vena cava to be occluded: large collateral vessels arose from the lower vena cava and the iliac veins. The histological features were those of longstanding hepatic venous outflow obstruction with irregular centrizonal and portal fibrosis: severe acute centrizonal congestion was not seen. This combination of findings indicates the presence of both membranous obstruction of the inferior vena cava, a rare developmental abnormality which predisposes to hepatocellular carcinoma formation, and invasion by the tumour of the inferior vena cava via the hepatic veins, an uncommon complication of hepatocellular carcinoma.     

Congenital familial vascular anomalies: a study of patients with an anomalous inferior vena cava, and of their first-degree relatives

With magnetic resonance angiography and computed tomography, congenital anomalies of the inferior vena cava are diagnosed more frequently than they used to be. Accessory renal arteries identified by magnetic resonance angiography in a patient with an anomalous inferior vena cava indicated a combination of arterial and venous abnormalities. The study was initiated to screen consecutive patients with an anomalous inferior vena cava for concomitant abdominal and pelvic arterial abnormalities, and their first-degree relatives for congenital vascular anomalies. Magnetic resonance angiography identified in 2 of 5 patients with an anomalous inferior vena cava concomitant accessory renal arteries and in 5 of 11 first-degree relatives major abdominal vascular anomalies including accessory renal arteries, accessory renal veins, and anomalies of the hepatic artery. None of the relatives showed abnormalities of the inferior vena cava. The familial occurrence of vascular anomalies strongly suggests an underlying pathogenetic component in affected family members. In patients with a congenital anomaly of the inferior vena cava, concomitant arterial abnormalities should be considered. First-degree relatives may be at risk for congenital vascular anomalies.     

Resection of renal cell carcinomas with inferior vena caval extension using deep hypothermic circulatory arrest.

Renal cell carcinoma with tumour thrombus extension into the inferior vena cava presents a difficult surgical challenge. The conventional surgical approach, which involves isolating the inferior vena cava, incising its wall and removing the thrombus, can have high incidences of perioperative mortality and embolization of the tumour thrombus compounded by severe hemorrhage. Four patients with renal cell carcinomas extending into the inferior vena cava were supported with cardiopulmonary bypass and deep hypothermic circulatory arrest during tumour excision. All of the operations were successfully performed with no mortality and minimal morbidity. The technique allowed the surgeon to operate in a bloodless field, thereby improving visibility and allowing complete tumour excision without significantly prolonging operative time. It is believed that this technique has improved the safety and technical feasibility of what had previously been a complicated and risky surgical procedure.     

Paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome: therapeutic challenge with bone marrow transplantation, transjugular intrahepatic portosystemic shunt, and vena cava stent

Stenosis of inferior vena cava can be a cause of or a consequence of Budd-Chiari syndrome (BCS). However, its occurrence after transjugular intrahepatic portosystemic shunt (TIPS) insertion has only been twice reported. We report the case of a 23-year-old man who presented BCS of the three suprahepatic veins. The cause of BCS was paroxysmal nocturnal hemoglobinuria, which was treated by bone marrow transplantation. A few months later, it was necessary to insert a TIPS because of refractory ascites and severe denutrition. However, refractory ascites was persistent and esophageal varices bleeding occurred. A TIPS desobstruction was needed, and during this angiography a stenosis of the suprahepatic vena cava was found and was treated with dilatation and stent. In a few weeks, the patient's clinical and nutritional state improved and, after 9 months hospitalization, the patient was discharged. One year later, this patient had no symptoms with an excellent permeability of TIPS and vena cava stent. This case report confirms that TIPS is a good treatment for BCS, but stenosis of inferior vena cava can occur after such a treatment.