Successful renal transplantation in a child with thrombosis of the inferior vena cava and both iliac veins

An 8-year-old girl who was born premature in the 24th gestational week suffered a septic venous thrombosis due to an indwelling central line during the early perinatal period. As a result the inferior vena cava including the intrahepatic segment and both iliac veins was obliterated. The right kidney was primarily dysplastic, and the left kidney developed a partial infarction. Renal function was compensated until the age of 6 years. Magnetic resonance angiography at that time showed a collateral system via the azygos vein. The venous pressure and its variation with breathing as measured invasively showed normal values. During pretransplant initiation of immunosuppressive therapy, the child developed cerebral convulsions after the third dose of cyclosporine. Therefore we utilized a regimen of rapamycin, mycophenolate mofetil, and steroids. The transplantation was performed using a living donor graft from the child's mother. The relatively long vein from the left kidney was used for anastomosis with a large presacral collateral vein. Twelve months after transplantation the kidney function is stable with a serum creatinine of 0.5 mg/dL. The recipient thrombosis of the caval and iliac veins is not a principal contraindication for successful renal transplantation. MR angiography and invasive pressure measurements facilitated evaluation of the collateral venous system. The living donation setting allowed the initiation of an immunosuppressive regimen that was tailored to the concomitant diseases of the child.     

Congenital anomalies of inferior vena cava in young patients with iliac deep venous thrombosis

Venous thromboembolism (VTE) in young patients is frequently associated with hereditary biological thrombophilia, autoimmune disorders, or neoplasia. Advances in venous ultrasound and contrast-enhanced computed tomography have allowed for the identification of inferior vena cava (IVC) anomalies as newly considered etiologic factor. We present two cases of VTE in young patients: the first case involves left IVC in a 22-year-old man and the second involves IVC atresia in a 39-year-old man. IVC anomalies should be identified in young patients with spontaneous VTE involving the iliac veins because they are at a high risk for thrombotic recurrence and adaptation to long periods of antithrombotic therapy.     

Perinatal inferior vena cava thrombosis and absence of the infrarenal inferior vena cava

We report a case of infrarenal absence of the inferior vena cava (IVC) presenting as a major iliofemoral deep venous thrombosis in an adolescent. This is the first report of infrarenal IVC absence in which IVC thrombosis has been demonstrated in the perinatal period. We propose an association between perinatal IVC thrombosis and subsequent infrarenal IVC absence. In addition, the case demonstrates the importance of assessment for anatomical anomalies in patients presenting with apparently idiopathic deep venous thrombosis.     

Vena cava filters and inferior vena cava thrombosis

OBJECTIVE: Retrievable vena cava filters (R-VCF) are a recent addition to the therapeutic armamentarium for the prevention of pulmonary embolism. However, unlike permanent vena cava filters (P-VCF), outcomes data are limited regarding complication rates. METHODS: This was a retrospective comparative analysis of consecutive patients undergoing placement of R-VCF vs P-VCF at Wake Forest University School of Medicine from January 2000 to December 2004. Data collected included demographics, procedural specifics, filter type, indications, and complications. Summary data are expressed as number (percentage) or mean +/- SD. Continuous and categorical variables were analyzed by using t and Fisher exact testing, as appropriate. Four additional patients with vena cava thrombosis were also referred to our institution for treatment during the study period, all with opposed biconical VCFs (OptEase and TrapEase filters) recently placed at other facilities. This last group of patients is described but not included in the analysis. RESULTS: A total of 189 VCF (165 P-VCF and 24 R-VCF) cases were examined. No significant differences in VCF groups were observed according to age, documented hypercoagulability, or concomitant anticoagulation. Significant differences were observed according to sex (30.3% of P-VCF vs 62.5% of R-VCF patients were female), morbid obesity (4.2% of P-VCF vs 25% of R-VCF patients), active malignancy (20% of P-VCF vs 41.7% of R-VCF patients), and indication for VCF placement. Over a median follow-up of 8.5 months, no case of significant hemorrhage, no VCF migration, and four cases of vena cava thrombosis were observed. Vena cava thrombosis was observed more frequently in the presence of R-VCF when compared with P-VCF (12.5% vs 0.6%; P = .007). All observed vena cava thromboses were associated with severe clinical symptoms and occurred in patients who received opposed biconical VCF designs. CONCLUSIONS: In our experience, both P-VCF and R-VCF can be placed safely. Among both permanent and retrievable devices, however, opposed biconical designs seem to be associated with an increased risk for vena cava thrombosis. Although causative factors remain unclear, filter design and resultant flow dynamics may play an important role, because all episodes of vena cava thrombosis occurred in patients with a single-filter design.     

Traumatic thrombosis of the inferior vena cava

Traumatic thrombosis of vena cava is rare. Thrombosis of the inferior vena cava diagnosed by uroscanner after blunt abdominal trauma involving the kidney is reported. The Doppler exam confirmed the floating character of the clot. Three days after the initiation of anticoagulant therapy, the thrombus disappeared without any clinical or radiological signs of pulmonary embolism. Fifteen cases of traumatic thrombosis of the inferior vena cava have been described in the literature. Mechanisms, clinical pictures and the management are discussed.     

Renal carcinoma with inferior vena cava malignant thrombosis

During an 11-year period a total of 314 patients underwent surgery for renal carcinoma; 70 had venous extension of the tumour, 31 had extension to the main renal vein and were staged V1 and 39 had involvement of the inferior vena cava and were staged V2. Special attention was paid to the latter group, which was divided into 2 subgroups: V2a for caval extension without ingrowth and V2b for caval extension with infiltration of the caval wall. Thirty-eight patients with caval involvement underwent surgery, with a 13% post-operative mortality rate. Most of the patients with malignant caval ingrowth (V2b) had concomitant lymph node and distant metastases. However, some had negative lymph nodes and no metastases at the time of operation. Perifascial nephrectomy associated with caval tumour removal or lateral subhepatic caval resection for patients staged V2aNOMO significantly increased the survival rate when compared with that of patients with no surgery on the obstructed vena cava. Total resection of the completely obstructed subhepatic vena cava for patients staged V2bNOMO has limited indications but, in selected cases, may prolong survival. This retrospective study supports the reintroduction of indicator V in the TNM staging of renal carcinoma and suggests the splitting of stage V2 into V2a for patients with free-floating caval extension and V2b for caval thrombus with ingrowth and caval wall infiltration.     

Risk of pulmonary embolus with inferior vena cava thrombosis

The authors have evaluated the risk of pulmonary embolism both as a primary event or as a secondary embolus despite adequate anticoagulation in 39 patients with phlebographically documented inferior vena caval (IVC) thrombosis. Twenty-six of these patients had thrombi characterized as free floating, and 13 had thrombi that were adherent to the IVC wall without a free-floating component. The incidence of initial pulmonary embolism confirmed by either pulmonary arteriography or high probability ventilation-perfusion lung scanning was 50 per cent (13/26) in those patients with free-floating IVC thrombi, but 15 per cent (2/13) in those with closely adherent mural thrombi (P less than 0.05). Pulmonary embolism despite adequate anticoagulation occurred in 27 per cent (7/26) of patients with free-floating clots, but in only 17 per cent (1/8) of cases with adherent thrombi (P greater than 0.05). These data strongly suggest that patients with documented free-floating inferior vena caval thrombi are at a significant risk for pulmonary embolism as an initial event and perhaps also as a recurrent embolus, even in the presence of adequate anticoagulation. When such thrombi are identified, the overall incidence of pulmonary embolus is high and conventional anticoagulant treatment with heparin may not be sufficient.     

Massive pulmonary embolism caused by internal iliac vein thrombosis with free-floating thrombus formation in the inferior vena cava

Nowadays, compression ultrasonography (CUS) is the gold standard for the routine diagnosis of deep venous thrombosis (DVT). The drawback of CUS is the low sensitivity concerning the diagnosis of isolated pelvic vein thrombosis, especially referring to isolated internal iliac vein and ovarian vein thromboses. Therefore, magnetic resonance (MR) venography has become a valuable alternative. We present the case of a 45-year-old female patient with a massive pulmonary embolism with the indication for thrombolytic therapy due to severe right ventricular overload. We were not able to detect a DVT in the lower limbs of this patient with CUS. However, further DVT workup by MR venography showed a free-floating thrombus formation originating from the right internal iliac veins into the inferior vena cava. Owing to the fact that this thrombus was free floating, surgical removal of the thrombus was scheduled and performed successfully. In some patients it might be important to look for so-called rare causes of pulmonary embolism, even when CUS of the lower limbs does not reveal any DVTs. The diagnostic procedure of choice for these patients seems to be MR phlebography, as iliac and pelvic veins can be evaluated without radiation exposure with this procedure.     

Surgical treatment of thrombosis of the inferior vena cava

Personal experience of thromboses of the inferior vena cava is presented and pathogenetic and symptomatological aspects stressed. In addition, in relation to the results presented and reported data, it is concluded that the role of surgical therapy is fundamental to integrate the thrombolytic and anticoagulant treatment of these severe forms of deep venous thrombosis.     

Predictors of mortality and management of patients with traumatic inferior vena cava injuries

The aim of this study was to determine factors that predict mortality in patients with traumatic inferior vena cava (IVC) injuries and to review the current management of this lethal injury. A 7-year retrospective review of all trauma patients with IVC injuries was performed. Factors associated with mortality were assessed by univariate analysis. Significant variables were included in a multivariate regression analysis model to determine independent predictors of mortality. Statistical significance was determined at P < or = 0.05. A literature review of traumatic IVC injuries was performed and compared with our institutional experience. Thirty-six IVC injuries were identified (mortality, 56%; mechanisms of injury, 28% blunt and 72% penetrating). There was no difference in mortality based on mechanism of injury. Injuries with closer proximity to the heart were associated with increased mortality (P < 0.001). Univariate analysis demonstrated that nonsurvivors had a higher injury severity scale, a lower systolic blood pressure in the emergency department, a lower Glasgow coma score (GCS), and were more likely to have thoracotomies performed in the emergency department or operating room. Multivariate analysis revealed that only GCS (P = 0.03) was an independent predictor of mortality. Typical factors predicting mortality were identified in our cohort of patients, including GCS. The mechanism of injury is not associated with survival outcome, although mortality is higher with injuries more proximal to the heart. The form of management by IVC level is reviewed in our patient population and compared with the literature.     

Renal vein and inferior vena cava thrombosis associated with acute pancreatitis

Renal vein thrombosis (RVT) is a well-recognized complication of the nephrotic syndrome, but it is extremely rare in patients with acute pancreatitis. Vascular thrombosis complicating pancreatitis is thought to be due to release of proteolytic enzymes from the pancreas and direct vasculitis. Peripancreatic vessels are most commonly involved in the complications associated with pancreatitis. Renal vein and inferior vena cava (IVC) thrombosis, however, is an exceptionally rare complication of pancreatitis. Awareness of this complication will help physicians in its early diagnosis and management. We report a case of renal vein and IVC thrombosis in a patient with acute pancreatitis.     

Compartment syndrome in patients with massive venous thrombosis after inferior vena cava filter placement

Massive venous thrombosis, which can occur acutely after inferior vena cava filter placement, has 2 forms: phlegmasia cerulea dolens and phlegmasia alba dolens. In phlegmasia cerulea dolens, complete occlusion of venous outflow occurs. In the milder phlegmasia alba dolens version, collateral venous flow out of the limb remains despite the venous thrombosis. This article presents, to our knowledge, the first 2 cases of massive venous thrombosis (1 phlegmasia cerulea dolens, 1 phlegmasia alba dolens) below inferior vena cava filters occurring after the acute period. Phlegmasia cerulea dolens and phlegmasia alba dolens can present as compartment syndrome. Prompt fasciotomies were performed, but the underlying massive venous thrombosis was not addressed surgically. Phlegmasia cerulea dolens and phlegmasia alba dolens have high morbidity and mortality. The patient with phlegmasia alba dolens required leg and thigh fasciotomies and eventually required an above-knee amputation. The patient with phlegmasia cerulea dolens developed compartment syndrome in the left leg, right leg, and right thigh. Although he underwent decompression of all of these compartments, he died from multiple organ failure. A multidisciplinary approach with the vascular service and the intensivists is required in the treatment of patients with massive venous thrombosis. Treatment goals include preventing additional propagation of the thrombus via anticoagulation, with strong consideration for catheter-directed thrombolysis or thrombectomy and fasciotomies for compartment syndrome. The orthopedic surgeon should keep phlegmasia cerulea dolens and phlegmasia alba dolens in the differential for compartment syndrome, especially in patients who have had a history of acute or chronic inferior vena cava filter placement.