Intermittent edema of the upper and lower extremities and the abdominal wall caused by membranous stenosis of the superior vena cava and membranous obstruction of the inferior vena cava

We report on a 38-year-old patient with intermittent edema of the lower legs, arms and abdominal wall. The cause for his tendency to develop edema was a membranous obstruction of the inferior vena cava and a membranous stenosis of the superior vena cava. The etiology of these anomalies of the vena cava suggests a congenital malformation. In consideration of the cases of inferior and/or superior vena cava-anomalies published to date the patient received an anticoagulant therapy (coumarin) and treatment with graduated compression stockings. He now complains from time to time of a sensation tension in the lower legs after prolonged standing or sitting. Edema of the upper and lower extremities and the abdominal wall have disappeared.     

Changes in the activities of antioxidant enzymes of the lymphoid organs of 21-day pregnant rats due to administration of fish oil by gavage

Budd-Chiari syndrome (BCS) due to membranous obstruction of the hepatic vein and the inferior vena cava is rare in children. We report a child with BCS that had a membranous obstruction at the level of the hepatic veins. The web was successfully dilated percutaneously by balloon catheters. Symptoms and signs of obstruction improved without any complication. As percutaneous catheterization is an effective, safe and repatable procedure, we recommend this technique for treatment of children and adults with BCS due to membranous obstruction of the hepatic veins.     

Budd-Chiari syndrome: a common complication of Beh?et's disease

OBJECTIVE: The Budd-Chiari syndrome is characterized by venous outflow obstruction of the liver, usually occurring as a consequence of thrombosis of the hepatic veins. Vasculitis is a major component of Beh?et's syndrome. The aim of this study was to determine the incidence of hepatic vein thrombosis in patients with Beh?et's disease and to estimate the effect of this entity upon the clinical features and course of Beh?et's syndrome. METHODS: During an 8-yr period from 1985 to 1994, from a total of 493 patients with Beh?et's disease seen at Hacettepe University Hospital, the incidence and effect of hepatic vein thrombosis on the clinical course of Beh?et's syndrome was investigated. The hepatic vein thrombosis in each case was documented by hepatic venography and confirmed by digital subtraction angiography, computed tomography, ultrasonography, and liver biopsy. Coagulation parameters including protein C, protein S, and anti-thrombin III levels were easured in each case. The survival of cases with Beh?et's syndrome complicated by Budd-Chiari syndrome and the effect of the Budd-Chiari syndrome on the survival of individuals with Beh?et's syndrome were determined using the Kaplan-Meier technique. RESULTS: Of the 493 cases of Beh?et's syndrome, 53 (10.8%) were found to have one or more large vessel thrombosis. Of these 53 patients, 14 (26.4%) had hepatic vein thrombosis. Of these 14 patients, 8 had an additional inferior vena cava thrombosis and 4 had portal vein as well as total inferior vena cava thrombosis. Only two patients with isolated hepatic vein thrombosis were identified. These two patients and two additional patients with hepatic vein thrombosis plus thrombosis of the hepatic portion of the inferior vena cava are currently alive. Of the 10 patients with total inferior vena cava and hepatic vein thrombosis (4 also had portal vein thrombosis), all 10 died with a mean survival of 10.3 months. During the same time period, 37 patients obtained from a total of 1494 patients with clinical evidence of either portal hypertension, hepatic venous outflow obstruction or inferior vena caval obstruction without Beh?et's syndrome were found to have a Budd-Chiari syndrome. Of these 37 patients, 19 (51%) had an identifiable underlying disorder responsible for their hepatic vein thrombosis. CONCLUSION: Based upon this experience, it appears as if Budd-Chiari syndrome is a relatively frequent complication of Beh?et's disease. When individuals with Beh?et's syndrome have BCS, concurrent thrombosis of the portal vein and inferior vena cava are often found, if the patency of these vessels is assessed. The clinical course of patients with Beh?et's syndrome complicated by Budd-Chiari syndrome is poor. The extent of the vascular thrombosis within the inferior vena cava rather than the presence of the hepatic vein thrombosis per se is the major determinant of survival.     

Inferior vena cava stenosis and pancreatic ascites complicating chronic calcific pancreatitis: a case report

A 19-year-old Thai male, who was a regular drinker, presented with massive ascites, back pain and leg edema for four months. On examination there was obvious clinical evidence of an inferior vena cava obstruction. Inferior vena cavography showed narrowing of the hepatic portion of IVC with collateral circulation. Surgical dilatation of the inferior vena cava was performed. The ascites were diagnosed four months later as pancreatic ascites with a very high ascitic amylase level. Computerised axial tomography and endoscopic retrograde pancreatography showed evidence of chronic calcific pancreatitis and pseudocyst. After further medical treatment, ascites and inferior vena cava stenosis subsided which was confirmed by repeated vena cavography, computerised axial tomography and magnetic resonance imaging. The cause of inferior vena cava stenosis and clinical obstruction in this case most likely resulted from phlebitis secondary to pancreatitis. The etiology of chronic calcific pancreatitis in this case might be alcoholic abuse and/or nutritional tropical pancreatitis. Inferior vena cava stenosis and associated pancreatic ascites complicating chronic calcific pancreatitis has not been previously reported in Thailand.     

Occlusion of the inferior vena cava--a rare, but severe disease picture, easily diagnosed with ultrasound

Vena cava inferior thrombosis can occur in many disease patterns. In the literature the vena cava inferior thrombosis is described in several case reports. Thrombosis of the vena cava inferior often leads to pulmonary embolism and influences the prognostic and therapeutic way definitively. This is important in the case of a real thrombus and also for "thrombotic tumorous material", which is not differentiated in the literature. Even if the pattern of a vena cava inferior thrombosis is very rare (in our patients 1%), early and safe diagnosis is very important. Abdominal ultrasound supplies a definitive clue to the diagnosis. The high diagnostic ranking in comparison to other imaging techniques is discussed. This is a definitive imaging technique which influences the therapeutic concept and prognosis.     

Proposal of a new nomenclature for Budd-Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion

Budd-Chiari syndrome (BCS) was initially defined as a symptomatic occlusion of the hepatic veins, but subsequent reports on various obliterative changes that occur in the hepatic portion of the inferior vena cava (IVC) and hepatic vein orifices have resulted in a broadened and ambiguous definition. Membranous obstruction of the inferior vena cava has been regarded by many as a congenital vascular malformation, but its relation to the classical BCS has remained obscure. With modern imaging and recent histological study of new cases, membranous obstruction of the IVC is now considered to be a sequela to thrombosis. How to classify various forms of occlusion and stenosis of the IVC and hepatic vein ostia is a major challenge. In this review, we emphasize that primary hepatic vein thrombosis (classical Budd-Chiari) and an obliterative disease predominantly affecting the hepatic portion of the IVC, both of which account for most patients with venous outflow block, are clinically quite different. In the West, the former is more common than the latter, which constitutes the vast majority of cases of outflow block in developing countries such as Nepal, South Africa, China, and India. The latter is frequently complicated by hepatocellular carcinoma (HCC), and primary hepatic vein thrombosis is not. The major cause of thrombosis is a hypercoagulable state in hepatic vein thrombosis, but more of the latter cases are idiopathic. The clinical presentation of the latter is milder, and onset is frequently inapparent, whereas the former is more severe, sometimes causing acute hepatic failure. Markedly enlarged subcutaneous veins over the body trunk characterize the latter. We propose that these two disorders be clinically distinguished with a suggested term "obliterative hepato-cavopathy" for the latter against classical BCS.     

Use by visitors of the services of the Queen Elizabeth Hospital, Barbados, W.I

The successful excision of genitourinary malignancies extending to the inferior vena cava relies heavily on accurate preoperative imaging. For the majority of these patients magnetic resonance imaging, inferior venacavography, abdominal ultrasound or abdominal computerized tomography will reliably predict the extent of inferior vena caval involvement by tumor. However, occasionally the results of these studies will conflict or be called into question intraoperatively. We report on 8 patients considered to be at risk for inferior vena caval involvement by tumor and for whom intraoperative ultrasound was obtained to clarify the presence or extent of thrombus. Five patients had renal cell carcinoma and 3 had adrenal carcinoma. In all patients concern as to the extent or presence of tumor was based on either inconclusive preoperative studies or unexpected intraoperative findings. In each case intraoperative ultrasound clearly visualized the inferior vena cava and established the presence or extent of tumor invasion. In 4 patients venacavotomy was avoided as a consequence of these findings. Intraoperative ultrasound is a useful tool that can accurately assess the inferior vena cava for possible tumor invasion, especially when the presence or extent of tumor involvement is not definitively established preoperatively.     

Calcified thrombus in the inferior vena cava in infants and children

Calcified thrombi in the prerenal (suprarenal) segment of the inferior vena have a characteristic radiographic appearance that permits accurate "plain film" diagnosis. Most have been fortuitously discovered in infants and young children. None of the affected individuals has had clinical evidence of venous obstruction. Vena caval obstruction is usually incomplete. Uncalcified clot caudal to the calcified thrombus caused complete obstruction of the inferior vena cava and renal veins in one of our patients, a healthy infant; since collateral flow was adequate surgery was not advised. We believe that aggressive diagnostic and therapeutic measures are unnecessary in the management of infants and children with calcified thrombi in the inferior vena cava.     

Budd-Chiari syndrome following repair of a giant omphalocele

We report a case of Budd-Chiari syndrome following repair of a giant omphalocele. Thrombosis of hepatic veins and of retrohepatic inferior vena cava may result from direct pressure on the hepatic venous outlet after visceral reduction and final abdominal wall closure.     

Abdominal CT findings when the superior vena cava, brachiocephalic vein, or subclavian vein is obstructed

OBJECTIVE: We evaluated findings on contrast-enhanced abdominal CT scans that suggest obstruction of the superior vena cava, brachiocephalic vein, or subclavian vein. SUBJECTS AND METHODS: We conducted a retrospective review of 22 patients with superior vena caval, brachiocephalic vein, or subclavian vein obstruction and analyzed the upper abdominal images on a chest CT scan or an abdominal CT scan. We assessed collateral vessels in the upper abdomen to answer the following question: Did enhancement approach undiluted IV contrast or were there other findings? In the second part of our study, we conducted a prospective review of abdominal CT scans of 200 patients without known mediastinal disease or known upper extremity venous occlusion to determine the frequency of abnormal enhancement of these vessels in a healthy population. RESULTS: The groups of collateral vessels revealed on abdominal CT scans were azygos or hemiazygos veins, internal mammary veins, lateral thoracic and superficial thoracoabdominal veins, vertebral venous plexus veins, and small mediastinal collateral veins. In the retrospective series, one patient had focal enhancement of the liver and early inferior vena caval enhancement due to collateral vessels. In the prospective series, abdominal CT scans of two patients (1%) revealed dense undiluted enhancement of one or more groups of collateral vessels: One patient had an ipsilateral pacemaker, and the other patient had an anterior neck phlegmon to the upper mediastinum. Both conditions may have been factors in the revealing of the collateral vessels. Two other patients (1%) in the prospective series had mild to moderate vessel enhancement that was less than that from undiluted contrast material. In one of these patients, the enhancement was related to abdominal wall hyperemia after surgery. In the other patient, enhancement may have been the result of ipsilateral axillary nodes. CONCLUSION: On upper abdominal CT scans, dense undiluted contrast material in the collateral vessel groups that we studied suggests possible obstruction of the superior vena cava, brachiocephalic vein, or subclavian vein.     

Anesthetic management of abdominal gunshot wound--a report of three cases

This is a case report of anesthetic management of abdominal gunshot wound. Two patients had upper abdominal wound involving the liver and the inferior vena cava. They died of uncontrolled bleeding. Third patient had lower abdominal injury involving the ascending colon and small intestine. The patient survived the injury and showed good recovery. In a case of the abdominal gunshot injury, prompt diagnosis and laparotomy are mandatory. Multiple intravenous routes are necessary in the upper part of the body for massive infusion and transfusion. Unusual hemostasis methods such as atrio-caval shunt or abdominal clamping of the aorta must be considered in case of injury in the inferior vena cava.     

Replacement of the retrohepatic vena cava in segmental liver transplantation

Reduced grafts represent an important technical development in paediatric liver transplantation. The use of a left lateral segment graft has required preservation of the native inferior vena cava to "piggy-back" the graft onto it. We report four children who underwent left lateral segment transplantation with caval replacement using the donor iliac vein because the native retrohepatic inferior vena cava was small, friable or difficult to preserve. There were no caval or hepatic vein complications post-transplant and the donor iliac vein proved to be a satisfactory interpositional graft. The technique offers the advantages of a wider retrohepatic cava avoiding venous outflow or caval obstruction, provides good tissue to suture and is well suited for the triangulation technique of the left hepatic vein.     

Grouper--creation of marker sets for multiplexed genotyping

We report the successful insertion and subsequent retrieval of a Gunther-Tulip vena cava filter in a patient with an anomalous left-sided inferior vena cava, who developed a right ilio-femoral venous thrombosis prior to planned surgical resection of a right femoral osteosarcoma. The indication was for short-term prophylaxis against pulmonary embolism during manipulative leg surgery.     

Intrahepatic cholangiocarcinoma with marked hypervascularity

Anomalous continuation of the inferior vena cava with an azygous vein is a rare vacular anomaly. The enlarged venous system may simulate adenopathies or mediastinal and retroperitoneal masses on the radiographs. We describe the case of a patient with lung cancer - a pathological condition which may cause adenopathies at these sites - and a dilated azygous-hemiazygous system resulting from failure of formation of the hepatic segment of the inferior vena cava.     

Vena caval resection for bulky metastatic germ cell tumors: an 18-year experience

PURPOSE: The operative management and followup of vena caval resection for bulky metastatic germ cell tumors have been previously described in 3 series. In 1989 Ahlering and Skinner described their experience with 12 patients. We now update this experience with the most recent followup on 19 patients. MATERIALS AND METHODS: From April 1978 to May 1995, 19 men underwent retroperitoneal lymph node dissection for stage B3 (N3) or C (N3, M+) germ cell tumor after induction chemotherapy. In all cases the inferior vena cava was resected because of extensive thrombosis or direct involvement of the vessel wall by a tumor. The inferior vena cava was resected from just below the renal veins to beyond the level of disease involvement. Complete resection of retroperitoneal disease was accomplished in all patients. Morbidity and mortality were examined. RESULTS: The mean hospital stay was 10 days (range 7 to 13) for uncomplicated recoveries (9 patients) versus 19 days (range 6 to 32) for complicated recoveries (10 patients). Followup ranged from 1 month to 16 years. Complications included prolonged ileus, small bowel obstruction, fascial dehiscence and pneumonia with pleural effusion. Chronic edema persisted in 3 of 11 patients with followup of greater than 6 months. Of the 6 patients who died of disease recurrence 4 did not have normalization of tumor markers before surgery, and all 4 had persistence of cancer in the resected specimen. Seven patients are without disease at followup of 24 months to 16 years. All survivors had normalized tumor markers before surgery. Only 1 patient (5%) had retroperitoneal recurrence. CONCLUSIONS: En bloc vena caval resection for tumor involvement or extensive thrombosis can be associated with short and long-term morbidity, is feasible, and may contribute to a prolonged tumor-free interval and a chance for cure.     

Management of inferior vena caval injury in liver trauma

The management of patients with severe hepatic trauma and damage to the inferior vena cava presents many difficulties. Our experience suggests that vena caval injury is more common than has previously been reported. In spite of the severity of the injuries a period for resuscitation is usually available and operation must not be started until full supportive measures are ready. We have evolved a technique of vascular isolation of the liver aboiding the use of internal vena caval shunts that permits repair of the damaged vena cava.     

Postpartum ultrasonographic diagnosis of inferior vena cava thrombus associated with ovarian vein thrombosis

Postoperative ultrasonographic assessment as a result of unremitting fever after cesarean section depicted a large pedunculated thrombus within the inferior vena cava at the level of the right renal vein. Subsequent imaging studies revealed that this thrombus was the distal extension of right ovarian vein thrombosis into the inferior vena cava.     

Responses of systemic and pulmonary veins to the presence of an intravascular stent in a swine model

The outcome of stent implantation for children with pulmonary venous obstruction has been characterized by late reocclusion associated with a marked vessel neointimal proliferation. The purpose of this study was to compare the responses of the systemic vein and pulmonary vein to the presence of an intravascular stent, using a Yorkshire swine (N = 10) model. Under cardiopulmonary bypass, a single Palmaz stent was placed in the inferior vena cava (IVC) and right lower pulmonary vein (PV) with sacrifice at 4.9-6.1 months. Angiography and hemodynamic data were determined at 1 and 3 months post-stent implant and prior to euthanasia. All stents were found to be patent, with no difference in degree of thrombosis or neointimal formation. No statistical difference was found in the initial and final stent diameter for both inferior vena cava and pulmonary vein stents (PV initial 6.8 +/- 0.9; final 7.1 +/- 0.6) (IVC initial 10.4 +/- 1.2; final 10.4 +/- 1.2). Electron microscopy demonstrated smooth endothelialization of both pulmonary and systemic venous stent devices. No thrombosis was found on gross morphology. The data indicate that there is no intrinsic difference in the response of the pulmonary vein to the presence of a stent device. The clinical experience of restenosis following stent implantation for pulmonary vein stenosis appears to be more related to variables of final stent diameter combined with the marked intrinsic abnormal vessel architecture, as seen with this condition.     

Handedness assessment inventory

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.     

Association between catechol O-methyltransferase genotype and violence in schizophrenia and schizoaffective disorder

An 18-month-old dog was examined because of ascites of 1 month's duration. Typical causes of ascites, including hepatic failure, heart failure, and protein-losing enteropathy, were ruled out. The dog's history included being hit by a car 6 months earlier, and the caudal vena cava had an S shape on thoracic radiographs. In addition, the abdominal fluid had a high protein concentration and low cellular content. These findings were all consistent with a diagnosis of postsinusoidal hypertension secondary to obstruction of hepatic venous outflow (Budd-Chiari-like syndrome). During exploratory thoracotomy, the pericardium appeared to have been torn from the heart and was partially wrapped around the caudal vena cava, causing a constriction. The pericardium was removed and the dog recovered without any further complications. Blunt trauma has been previously reported to cause kinking of the caudal vena cava and Budd-Chiari-like syndrome in dogs; but in these dogs, clinical signs of ascites developed a few days to several weeks after the traumatic incident. It appears that, depending on the cause of the hepatic venous outflow obstruction, onset of Budd-Chiari-like syndrome may be delayed for months.