Retroperitoneal Venous Diseases

González, Javier; Ciancio, Gaetano

doi:10.1007/978-3-642-37078-6_151

Javier González² &
Gaetano Ciancio³

682 Accesses

Abstract

The abdominal aorta and inferior vena cava are the great vessels of the retroperitoneum providing vascular supply to the abdominal organs and lower extremities. While the abdominal aortic disorders and its implications are described elsewhere, this review of “retroperitoneal venous diseases” centers the discussion on the anatomic variants of this vascular structure, and those major groups of entities which produce obstruction of the inferior vena cava.

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Introduction

The venous caval system is the major collecting blood network of the human body. It performs several important physiological functions, contributing substantially to maintain homeostatic conditions in the fluids of the extracellular space for optimal survival and functions of the cells. The impact of physiological significance of this system has been revised in recent decades, as new scientific discoveries and their practical applications have verified the multiple roles it plays in a number of disease mechanisms.

Interest in venous diseases and their clinical implications has a very long history, sometimes motivated by erroneous beliefs and misunderstandings. Nowadays, however, this interest is intensified due to special problems related to high morbidity and mortality caused by venous disorders in general, and inferior vena cava (IVC) in particular.

The fully developed IVC is a complex structure derived from different segments of an array of multiple paired longitudinal embryonic veins and the interconnections between them. Although the majority of these congenital variants are of little physiological consequence they do have implications of relevance to both surgeons and interventional radiologists. For this reason, a section on the embryologic development , normal anatomy, and anatomic variations which may be encountered in the IVC is presented.

On the contrary, obstruction of IVC is most commonly an acquired condition, typically caused by intrinsic and extrinsic factors, which give rise a wide variety of clinical scenarios depending on the extent and location of the obstruction. Although not all-inclusive, the foregoing chapter provides a detailed review of many of the known clinical situations in which this condition may be evident, incorporating short discussions of specific etiological factors where appropriate, an organized clinical anatomic discussion by areas of potential involvement (i.e., lower, middle, and upper thirds of the IVC), and a separate discussion of the venous collateral development seen in the event of chronic occlusion . A description of the techniques commonly used for diagnostic purposes in this process, and the treatment strategies to deal with all the above mentioned conditions have been also included.

Anomalies in the Development of the Inferior Vena Cava

The IVC is formed in a complicated series of developmental stages . These variations are fairly common and usually result in developmental anomalies , given that the embryonic caval development may be disrupted , arrested , or misdirected at any stage by factors that are not yet fully understood. All abnormal configurations of the IVC system are based on the persistence or anomalous involution of the embryonic vascular channels .

Although venous embryology is one of the most neglected areas of basic science in medicine, it presents a critical ability to explain many of the obscure conditions that may affect the IVC, and its knowledge is, therefore, essential for the recognition and interpretation of these anatomic variants and anomalies.

Development of the Primitive Venous System

The heart and blood vessels develop from the mesoderm as isolated masses and cords of mesenchymal cells as early as 15 days in order to rapidly deliver sufficient nutrients to the exponentially proliferating cells and dispose of waste products via connection with maternal blood vessels in the placenta (Langman 1985; Warwick and Williams 1989; Hamilton and Mossman 1972). By the beginning of the fourth week of gestation, an extensive network of blood vessels has formed from the mesenchyme as clusters of angiogenetic cells throughout the embryonic body to establish a communication with extra-embryonic vessels and to create a primitive vascular system . At this moment, three set of veins drains the sinus horn into the heart: (i) the umbilical veins , (ii) the vitelline veins returning blood from the placenta and yolk sac , and (iii) the common cardinal veins returning blood from the head and trunk (Langman 1985; Warwick and Williams 1989; Hamilton and Mossman 1972; Figs. 1 and 2).

Embryogenesis of the Inferior Vena Cava

The development of the IVC is a complex process involving the formation of several anastomoses between three paired symmetric embryonic veins : (i) the posterior cardinal veins , (ii) the subcardinal veins , and (iii) the supracardinal veins , which arise in a chronological order between 4 and 8 weeks of gestation (Giordano and Trout 1986; Bass et al. 2000; Moore and Persaud 2008). Meyer et al. (1998) described the morphology of the IVC as “the result of a dynamic process of development, regression, anastomosis and replacement initially of three paired venous blood conduits” (Fig. 3).

The infrahepatic IVC develops as a composite structure from these three pairs of veins through the processes of fusion, regression, and formation of midline anastomoses between them. The posterior cardinal (postcardinal) veins , which arise on the posterolateral aspect of the fetus, are the earliest to form and are the dominant system at 6 weeks of embryonic life. They give rise to only the most distal portion of the IVC, at the level of the iliac confluence draining the body wall caudal to the heart. Blood from the viscera is conveyed via the vitelline vein, which drains the yolk sac.

During the seventh week of embryonic life, the subcardinal veins start to predominate. They are ventromedial and parallel to the posterior cardinal veins . Ultimately, the right subcardinal vein forms the prerenal/suprarenal segment of the IVC (it is customary for its counterpart on the left to involute completely).

The supracardinal veins start to develop at 6 weeks and predominate at 8 weeks. They are dorsomedial to the regressing posterior cardinal veins and lateral to the subcardinal veins. They extend above the diaphragm to become the azygos and hemiazygos veins. The left supracardinal vein regresses, while the right supracardinal vein forms the postrenal/infrarenal segment of the IVC.

During their ascent from the pelvis, the kidneys drain their blood through a network of anastomotic channels between the paired subcardinal and supracardinal systems. Later, these vessels recede to form a vascular ring around the aorta connecting the left kidney to the IVC, and the retroaortic component usually regresses leaving paired single renal veins (McClure and Butler 1925; Giordano and Trout 1986; Shingleton et al. 1994; Soltes et al. 1992).

The final adult form of the IVC is formed of four segments: the hepatic segment (from the vitelline vein); suprarenal segment (from the subcardinal-hepatic vein anastomosis); renal segment (from the right supracardinal-subcardinal vein anastomosis); and infrarenal segment (from the right supracardinal vein) (Fig. 4).

Inferior Vena Cava and its Tributaries

The common iliac veins join approximately one finger breath below and right to the aortic bifurcation to form the adult IVC. The lower part of the IVC is overlapped in its anterior surface by the right common iliac artery and rests over the body of the fifth lumbar vertebra.

The IVC ascends on the right side of the aorta and parallel to it until reaching the level of the lower pole of the right kidney, where it diverges slightly to the right from the abdominal aorta and travels in a groove on the posterior surface of the liver. The IVC enters the thoracic cavity though the vena cava foramen in the diaphragm (at the level of the 10th thoracic vertebra) and immediately opens into the right atrium of the heart (Warwick and Williams 1989; Fig. 5).

The anterior surface of the IVC is covered in its lower portion by peritoneum (primary parietal peritoneum) up to the level of the mesenteric root. As it ascends, the IVC loses its peritoneal covering (secondary retroperitoneal structure) and is apposed to the duodenum and pancreas. Ascending further in the posterior wall of the epiploic foramen , the IVC is again covered with peritoneum and is adherent to the bare area of the liver.

The IVC has both parietal and visceral tributaries. The parietal tributaries include: (i) the common iliac veins ; (ii) the median sacral vein , which follows the course of the homonymous artery and may be empty in the left common iliac vein; (iii) the lumbar veins , whose arrangement corresponds to that of the homonymous arteries; and (iv) the inferior phrenic veins which enter the inferior vena cava just before it pierces the diaphragm (Table 1).

Table 1 Tributaries of the inferior vena cava

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The visceral tributaries are as follows: (i) the right gonadal (testicular or ovarian) vein, which opens in the inferior vena cava just below the entrance of the right renal vein; (ii) the renal veins ; (iii) the right suprarenal vein , and (iv) the major and minor hepatic veins , generally entering the cava in its anterior aspect along the hepatic groove (Table 1).

Congenital Anomalies of the Inferior Vena Cava

The complex embryological development of the IVC may give rise to a wide variety of congenital anomalies. The majority of these variants are of little physiological consequence. However, they do have significant implications from a diagnostic, as well as a practical perspective, which is of relevance to both surgeons and interventional radiologists.

Today, with the widespread use of cross-sectional imaging , congenital anomalies of the IVC and its tributaries are more frequently encountered in asymptomatic patients (Bass et al. 2000; Zhang et al. 2007; Moore and Persaud 2008), and have been described more frequently (0.6–2 %) in those individuals with other cardiovascular defects.

Classification of venous malformations and IVC anomalies. From a general standpoint, venous malformations are classified according to the embryological stage at which the defective development occurs. Venous malformations originating during the early stage of embryogenesis are termed extratruncular , while those originating during the late stage of embryogenesis are classified as truncular . A defect at any point in the complex development stages of the evolution and involution of multiple paired embryonic veins can result in various conditions of defective venous trunk.

When defective development occurs in the “early” stage of embryogenesis, the embryonic vessels remain in the form of reticular networks and do not evolve into the vascular trunk formation. After birth these networks can remain as independent clusters of primitive venous tissue without direct involvement of the main venous trunk itself. These primitive vascular structures maintain the mesenchymal cell properties and its evolutional ability to proliferate when stimulated by exogenous or endogenous factors (Bastide and Lefebvre 1989; Lee et al. 2007a, b).

On the contrary, when defective development occurs in the vascular trunk formation period, in the “later stage” of embryonic development, the defects involve “named” vessels and are limited to the vessel trunk itself. These are “embryologically mature” lesions, which no longer possess the evolutionary capacity to proliferate. However, truncular lesions present with more serious hemodynamic consequences compared with extratruncular defects due to their direct involvement with the truncal venous system (i.e., inferior vena cava stenosis/occlusion) (Lee 2006). As all truncular lesions involve the already formed, established venous trunk to varying degrees, they present as either hypoplastic or hyperplastic vessels causing obstruction or dilatation , respectively (Vaket et al. 1958; Zamboni et al. 1990). It should be noted that intraluminal defects within the vein (i.e., webs or membranes) may result in similar conditions of stenosis or obstruction (Rao et al. 1989; Croquet et al. 1999) (Table 2).

Table 2 The modified Hamburg classification of vascular malformations

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Many classification systems have been advanced to improve the understanding of the different anomalies that may develop from aberrations in the development of the IVC. The classification most commonly used is based on the segment of the final vena cava that is abnormal. Using this classification system, a few major anomalies may be identified (Bass et al. 2000; Mathews et al. 1999).

Anomalies of the supracardinal veins : Isolated left inferior vena cava and double inferior vena cava . The normal right-sided inferior vena cava and the anomalous left-sided cava are two ends of a spectrum: right versus left-sided dominance of the supra-cardinal system. The double IVC represents the “midpoint” of this type of anomaly. The suprarenal IVC only exists from the union of right-sided vessels (the right subcardinal and hepatic veins ). Therefore, left-sided vessels cross over at one of the midline anastomoses at the level of renal vein or at a variable distance inferiorly (Fig. 6).

An isolated left IVC occurs in 0.2–0.5 % of the population (Bass et al. 2000). The main clinical significance of a left vena cava is its potential to be misdiagnosed as left para-aortic lymphadenopathy (Siegfried et al. 1983). There have been also reports of spontaneous rupture of abdominal aortic aneurysms into a left vena cava (Davachi et al. 1965) and of left IVC causing chronic intermittent mesenteric angina, due to compression of the coeliac trunk , as the vena cava crosses in front of the aorta (Wartmann et al. 2011). A left IVC may make transjugular access for caval filter placement dificult (Savader and Ronsivalle 2000).

A double IVC occurs in 1–3 % of the population (Hirsch and Chan 1963; Zhang et al. 2007). Other associated variations with this anomaly include, right double IVC , double IVC with the retroaortic right renal vein , and double IVC with hemiazygos continuation of the left vena cava (Nagashima et al. 2006).

The clinical implications of a double vena cava are similar to those of a left vena cava in being misdiagnosed as adenopathy, especially if contrast enhancement of the vein is poor for technical reasons. In addition, recurrent pulmonary emboli despite adequate anticoagulation and the presence of a caval filter should raise this suspicion (Kouroukis and Leclerc 1996). From a practical point of view, this variant should ideally be excluded with a venogram performed just prior to filter deployment in patients with venous thrombosis. In this case inserting a single filter in the common suprarenal IVC has been described and should be considered (Mano et al. 2004). Other options are insertion of a filter in each IVC (Sartori et al. 2006) as well as coil embolization of the smaller vena cava (Smith et al. 1992). It is also important to be aware of this variant during retroperitoneal surgery (Shingleton et al. 1994).

Anomalies of the subcardinal veins: Azygos and hemiazygos continuation of the IVC . This variant occurs in up to 0.6 % of the population (Anderson et al. 1961). It results from failure of the right subcardinal-hepatic vein anastomosis to form with resultant atrophy of the right subcardinal vein. Consequently, blood is shunted from the supracardinal vein anastomosis through the retrocrural azygos vein, which is partially derived from the right supracardinal vein. The infrarenal vena cava continues as the azygos vein and, in cases of left inferior vena cava, as the hemiazygos vein. Azygos continuation is more common than hemiazygos. The renal segment of the vena cava receives venous return from both kidneys and passes posterior to the diaphragmatic crura to enter the thorax as the azygos vein. The azygos vein joins the superior vena cava in the normal location within the right paratracheal space. The hepatic segment (often termed the post-hepatic segment) is not truly absent; rather it drains directly into the right atrium.

Although azygos and hemiazygos continuation of the IVC has been associated with situs anomalies and other congenital heart anomalies, as well as asplenia and polysplenia syndromes, these anomalies have become increasingly recognized in the absence of these conditions since the advent of CT (Bass et al. 2000).

This anomaly may be suspected on the frontal chest radiograph. The enlarged azygos vein is seen at the confluence with the superior vena cava and can be misinterpreted as a right paratracheal mass or retrocrural adenopathy. Preoperative knowledge of this anomaly is important in planning cardiopulmonary bypass surgery.

Anomalies of the renal segment of the inferior vena cava. Anomalies of the renal segment of the IVC include all variants of the left renal vein. A circumaortic renal vein occurs in up to 8.7 % of the population (Wijdicks and Roseman 2007). Two left renal veins are present. Normally, the left renal vein is derived from the subcardinal-subcardinal anastomosis , which courses anterior to the aorta. Persistence of both the posteriorly located supracardinal-supracardinal vein anastomosis and the anteriorly located subcardinal-subcardinal anastomosis results in a circumaortic venous ring, with one vein coursing anterior and the other posterior to the aorta. If the midline anastomosis is at the level of the left renal vein, the “collar” configuration is clear. However, the midline anastomosis can occur at variable distances inferiorly, leading to retention of a longer segment of the left supracardinal vein . The left adrenal vein drains into the superior left renal vein, which crosses the aorta anteriorly. The left gonadal vein drains into the left inferior renal vein and then crosses posterior to the aorta approximately 1–2 cm inferior to the normal anterior left renal vein. The significance of this is in misdiagnosis as adenopathy and in preoperative planning of nephrectomy and in renal vein catheterization during procedures such as testicular vein embolization. Rarely, the renal vein can be compressed during its retroaortic course (“nutcracker” phenomenon) leading to periureteric varices, hypertension, and haematuria (Gibo and Onitsuka 1998).

The left renal vein is retroaortic in up to 3.4 % (Karaman et al. 2007). As with the circumaortic renal vein, retroaortic renal vein results from persistence of the posterior supracardinal-supracardinal anastomosis. However, in this variant, the ventral arch (subcardinal-subcardinal vein anastomosis) regresses so that a single renal vein passes posterior to the aorta.

Anomalies of the posterior cardinal veins: circuncaval or retrocaval ureter. A retrocaval ureter is also known as a circumcaval ureter. It is one of the few congenital anomalies that can be symptomatic, as the proximal ureter becomes trapped posterior and medial to the IVC (Hadzi-Djokic et al. 2009). This event can lead to significant compression, resulting in hydronephrosis or recurrent urinary tract infections. On intravenous urography , the ureter has an abnormal course as it projects over or medial to the lumbar pedicles. The resulting hydronephrosis is seen as a characteristic “fish hook ” or “reverse J ” appearance. Treatment consists of surgical relocation of the ureter anterior to the IVC (Fig. 7).

A circumcaval ureter almost always occurs on the right, although there are reports of left circumcaval ureter associated with situs inversus or bilateral circumcaval ureters associated with a double IVC (Brooks 1962; Pierro et al. 1990; Chou et al. 2006).

Congenital absence of inferior vena cava. Absent IVC is an extremely rare anomaly. Controversy exists as to whether an absent cava has a true embryonic etiology or whether it is the result of perinatal caval thrombosis causing regression and disappearance of the once present IVC (Ramanathan et al. 2001).

There has been one previous report in the literature of an absent IVC associated to left renal hypoplasia and a right hypertrophic, probably compensating, kidney (Dougherty et al. 1996). A more common association recognized is right renal aplasia. As suggested in a review by Gayer et al. all the nine patients included in their series with complete absence of the inferior vena cava had an absent or very small right kidney (Gayer et al. 2003). The association of an absent or hypoplastic kidney is related to perinatal renal vein thrombosis. Veen et al. proposed the name “KILT ” (kidney and inferior vena cava abnormalities with leg thrombosis) for this syndrome when associated with deep vein thrombosis (Van Veen et al. 2002).

Membranous lesions of the inferior vena cava. Focal or segmental membranous lesions can cause suprahepatic stenosis of the inferior vena cava along the proximal terminal segment, a condition known as primary Budd-Chiari syndrome . This has a profound hemodynamic impact, not only on the lower extremities where it causes chronic venous hypertension, but also on the liver where it results in severe portal hypertension due to hepatic venous outlet obstruction. This congenital anomaly most frequently involves Asian and African races (Benbow 1986; Koc and Oguzkurt 2007).

Inferior Vena Cava Occlusion

For many years thrombosis of the calf veins has been considered to be an event of primary importance in venous thrombo-embolism , and present-day clinicians continue to place particular stress on the prevention, diagnosis and management of this condition. It has been known for a long time that thrombus formation in the calf veins is a common event. Homans (1934) and Bauer (1940) suggested that from an initial site in the calf venous plexuses, by extension in continuity the posterior tibial, popliteal and femoral veins are involved in clotting, the iliac veins and the inferior vena cava also being affected on less frequent occasions. From a global standpoint, strictly, IVC occlusion represents a subset of deep vein thrombosis. However, the wide variety of different clinical scenarios leading to inferior vena caval thrombosis, their complexity and particular implications for adequate management, merit specific attention.

Epidemiology

Obstruction of the lumen of the IVC, whether partial or complete, is not a common phenomenon. The exact number of patients who have inferior vena caval thrombosis remains elusive because of the clinical variability in presentation. Caval occlusion is often asymptomatic, and even when symptoms are present, they are often nonspecific or mistakenly attributed to other etiologies.

It is estimated that deep vein thrombosis (DVT) occurs 1:1,000 patient-years (White 2003). By compiling information from several epidemiologic and various population-based studies that investigated DVT prevalence it can be estimated that the frequency of inferior vena caval thrombosis in patients with deep vein thrombosis is 4–15 % (Hansson et al. 1997). However, these figures are subjected to a variable geographic distribution depending on the diversity of underlying etiological factors (Prandoni et al. 1999).

Predisponent Factors: The Virchow’s Triad

Although the cause of deep vein thrombosis remains uncertain, in up to 80 % of patients who are affected of venous thromboembolism, a risk factor can be identified. To a large degree, the etiology of IVC thrombosis mirrors that of deep vein thrombosis. However, specific situations relate to the inferior vena cava only, but the wide variety of these situations all relate in one or more ways to Virchow’s classic description. Virchow’s triad (Virchow 1862) of vessel wall disease, change in the composition of the blood (hypercoagulability), and slowing of the blood stream (stasis) remains the foundation for our understanding of the pathophysiology of deep vein thrombosis in general and for inferior caval obstruction in particular as covering most of the aetiological possibilities (Brotman et al. 2004; Fig. 8).

Etiology

Regarding the location of the etiological factor leading to caval blockage , thrombosis of the inferior vena cava maybe divided into (i) intrinsic, when the cause is primarily related with a disorder arising directly from the inferior vena cava (i.e., developmental anomalies, or primary caval wall tumors), or (ii) extrinsic, in which the disorder secondarily manifests as caval occlussion.

Intuitively, any structure that is anatomically related to the IVC can generate an extrinsic caval occlussion either by direct compression or vascular invasion. However, a number of different conditions leading to general status impairment (i.e., dehydratation or compsumtion syndrome) may also precipitate the development of a caval thrombosis without the need for an spatial relationship with the inferior vena cava (i.e., infectious diseases).

Therefore, inferior vena caval obstruction may result from a wide variety of diverse causes, and with some exception the course will depend upon the etiology and the level of obstruction. Table 3 summarizes the reported causes of inferior vena cava occlusion.

Table 3 Classification of IVC anomalies

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Inferior Vena Cava Thrombosis due to Intrinsic Factors

Anomalies in the development of the inferior vena cava. Inferior vena cava anomalies are recognized as a possible risk factor for deep vein thrombosis, especially in young adults (Takehara et al. 2005). The pathophysiology of deep vein thrombosis in patients with inferior vena cava anomalies may be due to slow flow in spite of prominent collateral vessels. This inadequate venous return may lead to venous hypertension of the lower extremities resulting in stasis and subsequent thrombosis, which is typically bilateral in more than 50 % of patients (Lambert et al. 2010). This bilateral propensity is in contrast to the reported incidence of less than 10 % in patients with DVT with a normal IVC.

Ruggeri et al. reported four cases of absent inferior vena cava over a 5-year period that presented with idiopathic deep vein thrombosis in patients younger than 30 years (2001). This figures represent an estimated 5 % of idiopathic DVT in young people. Chee et al. similarly noted that up to 5 % of 20- to 40-year-old patients presenting with DVT had an IVC anomaly, 75 % of which had a complete absence of inferior vena cava (Chee et al. 2001).

Primary tumors of the inferior vena cava. Primary tumors of the IVC are a rare group of neoplasms. Endotheliomas, endochondromas, and leiomyomas present low grade clinical malignancy and they rarely metastasize.

Leiomyosarcomas of the inferior vena cava are rare, slow growing, malignant tumors. Only 2 % of leiomyosarcomas are vascular in origin, yet tumors of the inferior vena cava account for at least half them. Women make up for 75–90 % of the cases, and a white-to-black ratio of 5:1 can be observed (Kulaylat et al. 1997). They originate from smooth muscle cells of the media layer of the inferior vena cava, and the microscopic picture varies from a benign appearance with monotonous, cellular uniformity and little mitotic activity to a highly malignant one (40 % of cases) with hyperchromatism, hypercellularity, bizarre-shaped nuclei, and numerous mitoses. In the early stages it remains confined to the wall of the vein as a mural growth. As it grows in size, the tumor expands extraluminally (75 %), intraluminally (25 %), or in a bidirectional fashion as a dumbbell growth. In the predominantly intraluminal growth type, the inferior vena cava is expanded and leads to caval obstruction. Tumor necrosis and size did not reflect the biologic behavior of the tumor. Furthermore, prognosis following complete resection varied according to the histologic features of the primary tumor (Bower and Stanson 2000; Abis et al. 2006). Half of these tumors recur locally or metastasize. However, the tumor metastasized late in the course of the disease. The spread pattern is mainly systemical, affecting most commonly the liver and lungs, but no organ is exempt. Lymph node metastases occur less frequently. The presence of a capsule cannot be considered a sign of benignity. However, once breached recurrence is common (Mingoli et al. 1991, 1996).

According to their location, they can be classified as arising from the superior (above the hepatic veins), middle (from the hepatic to the renal veins), and inferior segment (below the renal veins) of the inferior vena cava. This classification has important clinical and prognostic implications (the ones arising in the middle segment have better prognosis than the ones arising from the lower segment: 34.4 % versus 0.0 % 10-year survival rate). In a recent review, the level of origin of the tumor, the pattern of growth, and the extent of the intracaval growth have been seen to be important prognostic factors (Bower and Stanson 2000).

The diagnosis is often delayed. Despite its large size, the tumor remained asymptomatic for a long time. Because of its rarity, it is not suspected as the culprit in the genesis of the symptomatology in the majority of cases. The diagnosis is easier to suspect in cases of intralluminal growth, although differential diagnosis includes hepatocellular carcinoma when the intrahepatic portion of the inferior vena cava is involved. In the predominantly extraluminal presentation, the retroperitoneal mass appearance is less specific and the differential diagnosis includes all other retroperitoneal masses such as lymphoma, other sarcomas, metastasis, inflammatory pseudotumor, and desmoid tumor. Computerized tomography and magnetic resonance confirmed the presence of a tumor, its pattern of growth, relationship to the surrounding structures, and the presence of caval obstruction. Percutaneous needle biopsy allowed identification of the histologic subtype of the tumor but not the organ of origin. In some cases, the precise origin of these tumors cannot be determined preoperatively (Abis et al. 2006).

The treatment of choice, based on available literature, is radical en-bloc surgical excision, with negative resection margins as it poses the only chance for a long-term cure. When radical surgery is performed, 5- and 10-year survivals are 49.4 % and 29.5 %, respectively. The mean survival time is about 1 month in inoperable patients and 34 months in those surgically treated (Blum et al. 1995; Bower and Stanson 2000; Abis et al. 2006).

Complete resection entails excision of the tumor, a portion of the inferior vena cava, the intracaval extension, concomitant metastatic disease, and involved nearby structures. Tumor thrombectomy alone is able to prevent death from liver failure and right heart outflow tract obstruction in some cases. Resection of intrahepatic IVC tumors had been classically performed utilizing standard vascular techniques (control of the IVC and its tributaries proximal and distal to the tumor). In the late 60s extracorporeal circulation techniques were added to this armamentarium to facilitate resection and replacement of supra- and retrohepatic IVC, disobliteration of occluded hepatic veins, and to perform extensive liver resection. More recently, with the objective of avoiding the disadvantages associated with these techniques (Chiappini et al. 2002), Hassan et al. have proved that complete excision can be accomplished without the need for extracorporeal circulation even when the intracaval extension reaches the right atrium (Hassan et al. 2010).

The extent of vein resection (tangential or segmental) depends on the patency of the IVC and degree of involvement by tumor. Patency of the IVC following tangential excision is maintained with lateral venorrhaphy or patch angioplasty. Ligation of the IVC following segmental resection is commonly performed as the collateral channels that develops as a result of gradual occlusion of the cava allows venous drainage. Graft replacement (autologous or synthetic) can be used in cases of circumferential resection and inadequacy of collateral circulation. The right kidney is often removed en-bloc with the tumor when venectomy is deemed necessary, or in selected cases, may be preserved by pelvic auto-transplantation. The most common intraoperative complication following resection is hemorrhage, which occurred in 3.5 % of cases. The postoperative morbidity ascends to 15 %, the most common being phlebitis. Death in the postoperative period has been shown to occur in up to 6 % of cases (Kulaylat et al. 1997).

When complete resection is deemed not possible, debulking combined with radiation therapy has proved to provide good palliation. Nevertheless, recurrence rates even in cases of complete or almost-complete excision are as high as 50 %, thus surgery may only simply provide palliation. Local-only-recurrence develops in aproximatelly 10 % of cases, while systemic disemination can be seen in 80 % of these patients. These recurrences develop in the period between 6 months and 5 years following resection, but the great majority occurred within 30 months. The role of neoadjuvant therapy is not clear; it may be given preoperatively to downsize the tumor and increase resectability rate. In the postoperative period, adjuvant therapy may be indicated for high risk tumors (Bower and Stanson 2000; Abis et al. 2006).

Inferior Vena Cava Trombosis Due to Extrinsic Factors

Dysfunctional coagulation system. The balance between the coagulation system and the fibrinolytic system is delicate and dynamic. Disorders that disrupt this balance can cause a situation in which thrombus formation may occur. Hypercoagulable states have often been associated with thrombosis of the inferior vena cava. Such a mechanism may play a more prominent role than is presently recognized. “Visceral thrombophlebitis migrans ” (Gerber and Mendelowitz 1949) often involves the inferior vena cava as well as other abdominal and visceral venous systems.

Both primary and secondary polycythemia have been associated with inferior as well as superior vena cava thrombosis (Ragings and Coe 1943; Retzlaff and Mongé 1973; Harris 1976; Muranishi et al. 2009). Extension of peripheral thrombophlebitis into the inferior vena cava should always be suspected when the phlebitis is recurrent or bilateral. Embolization to the upper part of the inferior vena cava may occur from the deep leg veins or from the lower segment of the IVC.

The nephrotic syndrome is another classic example. Patients with this syndrome have urinary protein losses. Both renal vein and inferior vena cava thrombosis have been described (Pollak et al. 1956; Baird and Buchanan 1962). The exact mechanism of the hypercoagulability of patients with the nephrotic syndrome has not been fully delineated. However, these patients have massive urinary protein loss, and diminished levels of antithrombin III have been observed (Kuhlmann et al. 1981).

Infections. A number of generalized and local infections involving abdominal organs have been associated with caval thrombosis (Myhre and Ylvisaker 1954). Among those frequently listed are pelvic inflammatory disease and puerperal sepsis, appendicitis, particularly with abscess formation, perforated peptic ulcer with local abscess, typhoid, scarlet fever, small pox, pneumonia, erisipelas, cholera, meningitis, tuberculosis, vertebral osteomyelitis, perirectal abscess, bacterial endocarditis and septic aortic aneurysms. As most of these cases occurred before adequate therapy for infectious diseases was known, one may assume that such factors as debitation, dehydration, prolonged bed rest, and overwhelming toxicity from uncontrolled sepsis played a significant role.

External compression. When trombosis occur due to external pressure on the vena cava, there is impaired flow and sludging, resulting in retrograde trombosis behind the level of the occlusion. The distortion of the normal caval anatomy by extrinsic compression generates both venous stasis and turbulent flow. This situation increases the likelihood of thrombus formation. The spectrum of medical diagnoses that can cause compression of the IVC is determined by those structures anatomically adjacent to IVC.

In this way, obstruction of the IVC by external pressure may be physiologically induced during the later states of pregnancy by enlargement of the uterus, or pathologically conditioned by other visceral enlargement. Pleasants (1911) demonstrated that a likely place for external obstruction of the inferior vena cava was in the passage behind the liver, near the diaphragm and the head of the pancreas. According to Pleasants’ description the pathologic enlargement of liver may cause caval compression (Okuda 2002). The most common causes for this enlargement are hepatic abscesses (Arakura et al. 2009), either from amebae or echinococci, benign tumors (i.e., hepatic hemangioma) (Paolillo et al. 1993), primary or metastatic malignancy, and cirrhosis, but before adequate therapy for syphilis existed, liver gumma was also a common cause (Plessier et al. 2012; De Stefano and Martinelli 2012).

Other retroperitoneal organ systems that have been shown to cause caval thrombosis include the pancreas and the kidneys. Polycystic disease of the right kidney has reportedly been clinically associated with thrombosis of the IVC (O’Sullivan et al. 1998). Pancreatic pseudocysts and acute pancreatitis have been reported also among the causes of IVC thrombosis. The pathophysiology of the evolution of the thrombosis may reflect either the local impact of inflammation of the pancreatic head or the impact of a hypercoagulable state on the inferior vena cava. Although caval thrombosis in the setting of pancreatitis is uncommon, this clinical entity may account for an unexplained deterioration in the status of a patient with acute pancreatitis.

Retroperitoneal tumors , as well as enlarged lymph nodes along the course of the inferior vena cava may lead to external compression and eventual blockage of the inferior vena cava. In the past, abdominal lymphadenopathy was commonly a sequel of tuberculosis and typhoid fever. Today, such lymphadenopathy is more commonly the result of malignant lymphoma (Harris 1976).

External occlusion may result from fibrous adhesions, intrabdominal hemorrhage, previous surgery, and a variety of intra-abdominal neoplasms such as listed in Table 4. Other aspects of compression can be attributed to the presence of a large hematoma adjacent to the cava or the iliac systems.

Table 4 Etiology of inferior vena cava obstruction

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Aneurysms of the abdominal aorta can compress the vena cava and cause thrombosis. Although this clinical situation is somewhat uncommon, the implications for surgical repair of the aneurysm are significant. The surgeon must be prepared for enlarged venous collaterals and the possibility of unusual configurations of the tissue planes. One reported case described incorporation of the inferior vena cava into the aneurysm (Flynn and Zammit-Maempel 1998). In this particular case, the caval wall was actually part of the wall of the aneurysm. Abdominal aortic aneurysms may, on rare occasions, occlude the inferior vena cava. Ten Eyck and Wellman (1959) reported an interesting case of Salmonella infection in an abdominal aortic aneurysm. Rupture of the mycotic aneurysm caused occlusion of the inferior vena cava and death.

Trauma . Unique among causes, trauma combines the limbs of the Virchow’s triad. Stasis, vessel injury, and hypercoagulability may all exist in the same clinical situation. Direct trauma to the IVC may be the result of either penetrating or blunt trauma (Campbell et al. 1981; Nagy and Duarte 1990; Takeuchi et al. 1995; Peck et al. 1998). In the absence of venous laceration, blunt endothelial damage has been postulated to cause thrombosis. Other mechanisms observed secondary to trauma include extension of hepatic venous thrombosis and thrombus formation after perihepatic surgical packing.

Tumors invading the inferior vena cava. Malignancy itself is a risk factor for DVT and thus represents a risk factor for its extension into the IVC. Additionally, numerous malignancies have been associated with IVC thrombosis. Retroperitoneal leiomyosarcoma (Hartman et al. 1992), adrenocortical carcinoma (Hedican and Marshall 1997), hepatocellular carcinoma (Okada et al. 2003) and renal angiomyolipoma (Bernstein et al. 1997) have all been reported as presenting in association with caval thrombosis. Testis cancers can also be associated with intraluminal caval extension in approximately 2–6 % of advanced stage germ cell malignancies (Spitz et al. 1997; Beck and Lalka 1998).

The incidence of clinically apparent (i.e., symptomatic) venous thromboembolism in cancer patients has been reported to be approximately 15 %, with reported incidence rates ranging from 3.8 % to 30.7 % (Prandoni et al. 1999). Cancer patients are more likely than non-cancer patients to present with proximal DVT. In addition, cancer patients have been shown to present with a greater initial thrombus burden, to experience greater clinical deterioration despite anticoagulant therapy, and to have less venographic improvement in response to standard treatment when compared with non-cancer patients (Breddin et al. 2001).

Venous thrombosis has been traditionally associated with aerodigestive tract adenocarcinomas involving the pancreas, stomach, and lungs. Nevertheless, thrombosis can affect the clinical course of all histologic types, of all stages, of all grades, and during any and all treatments (Levitan et al. 1999).

Adrenocortical carcinoma (ACC) is a rare tumor with a poor prognosis. Its incidence is 0.6–1.67 cases per million people per year (Ross and Aron 1990). It occurs at all ages and is more frequent after 45 years of age (Venkatesh et al. 1989; Luton et al. 1990). The majority (80 %) of ACC patients debut with an endocrine syndrome, usually Cushing’s syndrome, either in isolation or associated with virilizing features. Hyperaldosteronism and feminization syndromes are rare (Wajchenberg et al. 2000; Ng and Libertino 2003). Typically the entire adrenal gland has been replaced by a heterogeneous mass that displaces the adjacent organs such as the kidney or liver and invades the adrenal vein and IVC (Gahan et al. 2011). A very rare primary adrenal tumor is the adrenal leiomyosarcoma (Kato et al. 2004). They usually present as a large mass invading the kidney and adrenal gland with tumor thrombus invading the adrenal veins and inferior vena cava indistinguishable from adrenal cell carcinoma. Typically, the pathologist makes the diagnosis.

Hepatocellular carcinoma (Okada et al. 2003) typically invades the portal veins, although rare advanced cases can invade the hepatic veins and spread into the intrahepatic vena cava. Tumoral invasion of the inferior vena cava can also be seen with intrahepatic cholangiocarcinoma and metastasis from other primary tumors.

Special comment should be made concerning renal cell carcinoma (RCC), one of the most frequent causes of malignant invasion of the inferior vena cava. RCC accounts for 2–3 % of all malignant diseases in adults, representing the third most frequent and the most lethal genitourinary cancer (Kim et al. 2004; Gupta et al. 2008). This malignancy has the propensity to infiltrate the surrounding adjacent structures with unique proclivity for vascular invasion. The particular tropism shown for the venous system facilitates its propagation into the renal vein and inferior vena cava, generating a specific form of a locally advanced renal tumor. Intracaval neoplastic extension is present in 4–10 % of patients with RCC, and a subpopulation of 1 % has extension into the right atrium (Marshall et al. 1988; Abreu and Gill 2003; Boorjian and Blute 2009; Pouliot et al. 2010). There has been much debate about the prognostic significance of this entity. Currently, most authors agree that the presence of thrombus itself, in absence of caval wall infiltration, has no specific detrimental impact on survival if it can be successfully removed, though it seems obvious that more advanced thrombi are associated with more advanced tumor stages and, thus, a negative impact on survival rates (Martínez-Salamanca et al. 2010).

The recently revisited Union for International Cancer Control/American Joint Cancer Committee (UICC/AJCC) TNM staging system for renal carcinoma has supplanted Robson RCC staging classification for prognostic and, thus, therapeutic purposes. In previous TNM classifications , the pT3b group included both renal vein and IVC invasions. As the result of many studies into the independent prognostic value of vena cava compared to renal vein invasion alone (Thompson et al. 2005; Moch et al. 2009; Wagner et al. 2009), these two groups have now been separated in the latest version of the TNM classification (Sobin et al. 2009). For surgical considerations, the most widely used classification remains that proposed by Neves and Zincke (1987) (Fig. 9).

Despite advances in radiation, chemotherapy, and immunotherapy the reference standard for RCC with tumor thrombus remains surgical resection. Previous evidence had shown that aggressive surgical resection may produce long-term freedom from disease, with survival rates as high as 68 % at 5 years in acceptable surgical candidates (Martínez-Salamanca et al. 2010).

The important steps for radical nephrectomy and thrombectomy are (i) renal hilar exposure, (ii) ligation of the renal artery, (iii) successive occlusion of the IVC above the thrombus first, then of the contralateral renal vein and, finally, of the IVC under the thrombus, (iv) opening of the vena cava, (v) nephrectomy and thrombectomy, (vi) verification that no residual thrombus is attached to the IVC, (vii) flushing of the occluded segment with blood and heparin during the IVC closure, keeping the cephalad vascular clamp in place to avoid emboli, and (viii) release of the vascular clamps.

The use of bypass procedures remains required in select retrohepatic and supradiaphragmatic cases, particularly in the presence of a bulky intraatrial thrombus or when a patient is unable to tolerate the cross-clamping of the IVC (Boorjian and Blute 2009). However, because of the need for anticoagulation and the negative implications of CPB with or without DHCA on the blood coagulation system, mesenteric perfusion, and neurological function, it should be used only when necessary. As an alternative, different “special” maneuvers derived from the transplantation surgery including (i) liver mobilization, (ii) Pringle maneouver, (iii) pancreas-spleen complex mobilization, and (iv) thombus manipulation to decrease the thrombus level may enable the safe and complete removal of large thrombi up to the level of the diaphragm, and in some cases beyond (Ciancio et al. 2009, 2011; Gonzalez et al. 2011; Gorin et al. 2013).

Even with the improvements in preoperative diagnostic modalities, anesthesiology, and preoperative care, there is still considerable morbidity and mortality in this type of surgery. It ranges from 2.7 % to 40 %, arising primarily from massive pulmonary embolism and hemorrhage. Complications such as perioperative death, hemorrhage, need for reoperation, rate of transfusion, and sepsis had been shown closely related to the cephalic extension of the IVC tumor thrombus, as recently reported by Kearnes and Blute (2008), with overall complication rates of 12 %, 18 %, 20 %, 26 %, and 47 % for levels 0 to IV, respectively.

In the perioperative period, the major complication associated with tumor thrombectomy is pulmonary embolization due to excessive manipulation that may cause dislodgment of the thrombus, with fatal consequences. Schuch et al. (2009) reported that this complication has an incidence of 1.5 % and is associated with a 75 % mortality rate. Bland thrombus may be present distal, or more often, proximal to the tumor thrombus. In a few instances, the proximal bland thrombus causes a severe inflammatory reaction that would make impossible the complete removal of the thrombus without resection of the vena cava (Gonzalez et al. 2014). In these instances, biopsy of the intracaval bland thrombus is accomplished to confirm the benign nature of the lesion and to place an IVC filter below the ostia of the renal veins to prevent postoperative pulmonary embolization.

In the last 5 years new therapeutic agents, tirosine kinase inhibitors (TKIs), have been shown to be effective for the treatment of metastatic renal cell carcinoma, especially for clear cell subtypes. Sorafenib and sunitinib inhibit tyrosine kinase activity from platelet derived growth factor , vascular endothelial growth factor receptors and other kinases causing inhibition of angiogenesis and tumor growth. An interesting characteristic is their ability to decrease tumor size in up to 36 % of cases for sunitinib (Motzer et al. 2007) Therefore, before performing surgery for RCC with intracaval extension, one should consider the surgical risks for the patient and procedure together with the natural biology of the tumor to allow personalized treatment, especially with the advent of novel therapeutic agents that have the potential to shrink a tumor thrombus.

Iatrogenic. When surgical ligation of the IVC was more frequently employed for recurrent pulmonary embolism, many iatrogenic occlusions were seen (Spencer et al. 1962; Perhoniemi et al. 1986). Failure to prevent pulmonary embolism may have been to the large caliber of collaterals, particularly the ascending lumbar veins, given that large emboli can transverse such collaterals.

Nowadays, patients with a recent history of medical or surgical care may present with iatrogenic caval thrombosis. The expansion of endovascular technology has led to increased recognition of this entity. Interventions that reportedly have identifiable rates of IVC thrombosis include (i) hepatic transplantation, (ii) dialysis access, (iii) femoral venous catheters, (iv) pacemaker wires, and (v) vena caval filtres (Joels et al. 2003; Shang et al. 2011).

Other associated conditions. Numerous other clinical situations have been associated with caval thrombosis. They may meet some classification criteria to be listed in one or more of the categories mentioned above; however, they are noted here for clarity and can include (i) retroperitoneal fibrosis (Rhee et al. 1994), and (ii) oral contraceptives (Dinger 2009).

Although retroperitoneal fibrosis may course with caval obstruction, this feature is only anecdotal and thus not included in its usual presentation. On the contrary, it has been established that oral contraceptive agents with a high oestrogenic component produce a significant increase in the incidence of venous thrombo-embolism. However, the incidence is low with the modern combined oral contraceptives (COC). Nevertheless, venous thrombo-embolism remains a rare but potentially serious complication of COC use (Dinger 2009). This condition typically involves thrombosis in the deep veins of the legs or pelvis and the potential for pulmonary embolism, which has potentially fatal consequences.

Known risk factors for venous thromboembolism include advancing age (Silverstein et al. 1998) antiphospholipid antibodies and hereditary thrombophilia (Levine et al. 2002; Mohllajee et al. 2006), cigarette smoking (Pomp et al. 2008a) surgical procedures (Geerts et al. 2004), trauma, and immobility (such as that associated with travel or hospitalization) (Chandra et al. 2009), obesity (Pomp et al. 2007) and pregnancy and the peripartum period (Ros et al. 2001; Pomp et al. 2008b).

Prospective observational studies have shown that all currently marketed COCs increase the risk of VTE to the range of 9–10/101,000 woman-years of use and that this risk is highest in the first months of use with a fall towards baseline risk thereafter (Heinemann and Dinger 2007; Dinger et al. 2007). Modern COCs offer excellent contraceptive efficacy and good adherence due to their many non-contraceptive benefits.

Pathophysiology, Clinical Manifestations and Diferential Diagnosis

Thrombosis of the IVC is an underrecognized entity with a variety of clinical presentations. It is paradoxical that occlusion of the inferior vena cava is accompanied by few and surprisingly mild clinical signs. Obstruction of many smaller vessels provokes immediate and sometimes devastating changes, but a similar process in the inferior vena cava usually occurs with little clinical “fanfare” and may go unnoticed for many years. Although it is usually a nonfatal or severely incapacitating process, inferior vena cava obstruction is associated with a definite clinical syndrome (so-called “inferior vena cava syndrome ”) consisting of edema of the legs, dilatation of abdominal veins, and, less often, varicosities of the lower extremities, varicocele, and hemorrhoids. When occlusion occurs proximal to the level of the renal veins, albuminuria and ascites may develop (Gonzalez et al. 2014).

The clinical manifestations of the IVC obstruction depend upon several factors: the underlying cause of obstruction, the level of the obstruction, adequacy of collateral circulation, and the presence of intercurrent disease. Occlusion may occur in any portion of the vessel but it is usually caudal to the renal veins; occlusion proximal to this level is more serious because of disturbance in venous return from the kidneys and the liver. These conditions are, however, not necessarily incompatible with life. Because of the propensity of these processes to evolve over time, patients may present without symptoms suggestive of IVC occlusion. They may only demonstrate evidence of the primary process or of collateral venous hypertrophy. The initial presenting symptom may even be pulmonary embolization.

Collateral Circulation Network

The marked variation in the clinical picture of the IVC obstruction syndrome is due to the variable formation of various abdominopelvic collateral venous pathways that result from chronic vascular obstruction (Golub et al. 1992; Duty and Daneshmand 2008). An understanding of the underlying anatomy and hemodynamics of venous collateral pathways has important diagnostic and prognostic implications in patients with obstructing RCC tumor thrombi. Familiarization with these collateral pathways may also aid in evaluating the different treatment options.

The different collateral channels, which may assume certain significance during IVC occlusion, have been outlined by Pleasants (1911). Based on postmortem material, he divided the collateral circulation into four major divisions (i) inferior vena cava to superior vena cava anastomoses, (ii) inferior vena cava to portal vein anastomoses, (iii) portal vein anastomoses to superior vena cava; and (iv) inferior vena cava to superior vena cava anastomoses. However, from a practical standpoint, collateral systems can be arranged into two groups: deep and superficial (Fig. 10).

The deep azygos-hemiazygos system is the earliest channel available and plays a dominant role in venous decompression of IVC obstruction at any level. The vertebral venous plexus communicates in a bidirectional fashion with the IVC and the azygos-hemiazygos system via segmental tributaries . Secondary systems of the deep collaterals comprise the gonadal, ureteral, and, to a lesser extent, the inferior mesenteric vein of the portal system.

The two primary components of the superficial venous network that develop during IVC obstruction are the paired lateral thoracic and internal thoracic systems. The distal tributaries of the lateral thoracic system are the superficial epigastric and circumflex iliac veins, which reach the axillary vein through the thoracoepigastric and lateral thoracic veins. The external iliac veins drain through the inferior and superior epigastric veins into the internal thoracic veins. Communications with the lumbar and intercostal plexuses allow for redistribution of the superficial flow to the primary deep systems and the IVC above the level of the obstruction. Although considered by many to be the least important of all the collaterals, they do take on significance after several weeks of the acute phase. As the obliterating process progressively involves the more common collateral channels, the already known anastomoses between the superficial veins and the retroperitoneal branches of the superior mesenteric vein plays a more important role in venous decompression.

The determinants of the extent of collateral development are the location or level of the obstructed venous segment, the length of the obstruction, and the number of veins involved. These three elements individually and in combination cause an increase in venous resistance and thus determine the extent of collateralization (Golub et al. 1992).

Deep collateral channels may be adequate in the development of a fully compensated collateral circulation when the IVC is obstructed below the renal veins. In contrast, midlevel IVC occlusion results in congestion of the kidney and usually induces a less compensated state of obstruction. Collateralization from this level of occlusion has been shown to involve the portal system collaterals, wide dilatation of the vertebral veins, and perinephric and capsular drainage into the azygos-hemiazygos system.

In upper level obstructions, such as those involving the major hepatic veins (MHVs), communications between the IVC and superior vena cava develop from the portal systems both deep and superficial. Additionally, the vertebral plexus becomes widely dilated. In this setting, the finding of visceral congestion suggests inadequate collateralization (Fig. 11).

Clinical Presentation

In order to facilitate understanding of the collateral circulation and its relationship with the clinical picture derived from it, the IVC maybe arbitrarily divided into three segments: (i) lower inferior vena cava, below the level of the entrance of the renal veins; (ii) middle inferior vena cava, between the level of the renal veins and the major hepatic veins; and (iii) upper inferior vena cava, above the level of the hepatic veins (Missal et al. 1965).

Manifestations of Lower Segment Occlusion

When the lower segment of the inferior vena cava (below the entrance of the renal veins) is occluded, signs and symptoms are restricted to the lower extremities unless pulmonary embolism (PE) occurs. Iatrogenic occlusion may produce the same clinical picture. Almost immediately after such occlusion of the inferior vena cava, the compromised venous return is manifest as edema of the lower extremities. In about 1 week after complete obstruction, collateral veins begin to form, and by the third week, are well developed. Maximal efficiency of the collaterals is not reached until 3 months. Ray and Burch (1947), in a study of patients with IVC ligations, noted an increased plasma protein concentration that may produce an increase in oncotic pressure, and this would tend to limit edema formation until collateral circulation become effective. They also noted a lack of parallelism between the degree of edema and the venous pressure. Venous pressure persisted increased even 4 years after ligation in their study.

Manifestations of the Middle Segment Occlusion

With the occlusion of the vena cava at a higher level, but below that of the renal veins, low back pain may be the chief complaint. Congestion of the prostatic plexus in the male leads to symptoms suggesting diseases of the prostate and lumbosacral neural plexus. Similarly, in the female, congestion of the pelvic organs may simulate a case of chronic pelvic inflammatory disease. In addition, occlusion of the mesenteric tributaries may lead to gastrointestinal symptoms.

A completely different scenario results from renal veins involvement in the obstruction. Thrombosis of the renal veins usually produces a picture of generalized edema, hypoproteinemia, hipercolesterolemia, and massive proteinuria (i.e., nephrotic syndrome).

Urine may show red and white blood cells, casts of various types, and under polarized light, “maltese crosses ” of cholesterol crystals . Apparently, the rapidity of onset can be correlated with the severity of the symptoms. Sudden bilateral renal vein thrombosis may lead to bilateral hemorrhagic infarction of the kidneys and death. If the occlusion is less abrupt and renal infarction does not occur, but rather engorgement of the kidneys with capsular distension and flank pain or even a membranous glomerulonephritis. Once the nephrotic syndrome has developed, progressive renal failure, uremia and death may occur. Conversely, the occlusion of the middle segment may be only associated with mild proteinuria, microscopic hematuria, and minimal impairment of the renal function.

Manifestations of Upper Segment Obstruction

Sudden occlusion of the upper part of the IVC results in liver congestion, impairment of liver function, decrease in venous return to the heart, congestive heart failure, and death. Gradual occlusion of the upper IVC may result in a chronic Budd-Chiari syndrome with hepatosplenomegaly, ascites, jaundice and hepatic decompensation (Shirodkar et al. 2011). The caput medusae pattern of the venous collaterals may be present.

Metabolic Effects and Chronic Sequelae of Inferior Vena Cava Occlussion

A complete discussion of the pathophysiology of IVC occlusion needs to mention the metabolic defects that are seen within this clinical picture and the post-thrombotic syndrome derived from the chronic obstruction.

The decreased venous return to the heart secondary to caval obstruction may produce compromised perfusion of the kidney. Under this situation, the yuxtaglomerular apparatus becomes hyperplastic, increasing the level of renin delivered to general circulation. Renin catalizes the transformation of angiotensinogen to angiotensin, which stimulates the zona glomerulosa to put forth increased amounts of aldosterone. The resultant sodium retention is an important factor in the role of edema formation. However, great increased renal venous pressure, decreases glomerular filtration rate, and chronic occlusion induce a number of changes that are reversible even when the occlusion persists (Missal et al. 1965).

The post-thrombotic syndrome is an important chronic complication of vein thrombosis seen in more than one-third of patients under this condition. Between 5 % and 10 % of patients develop a severe form of the disease, which can manifest as venous ulcers. The main risk factors for post-thrombotic syndrome are persistent leg symptoms 1 month after the acute phase of venous occlusion, anatomically extensive and recurrent ipsilateral thrombosis, obesity, and older age. Subtherapeutic dosing of initial oral anticoagulation therapy may also be linked to the onset. The cornerstone in its management is compression therapy using elastic stockings. Their daily use for 2 years after proximal thrombosis appears to reduce the risk of post-thrombotic syndrome; however, uncertainty remains regarding optimal duration of use, optimal compression strength, and usefulness after distal venous obstruction. Venoactive medications such as aescin and rutosides may provide short-term relief of symptoms (Kahn 2010).

Diagnosis

Inferior vena cava thrombosis remains a challenging diagnostic process. Timely and accurate diagnosis is imperative because of the unacceptable outcomes associated with a misdiagnosis. Failure to make such a diagnosis can result in significant morbidity and mortality because of recurrent thromboembolism episodes, whereas empirical anticoagulation therapy without a confirmed diagnosis may expose the patient to unnecessary and potentially avoidable risk in the absence of any tangible benefit.

Clinical grounds. Given the wide variety of signs and symptoms that may be present in patients with IVC thrombosis, the diagnosis made on clinical grounds alone is notoriously unreliable. In these cases, the severity of limb edema and pain is often unrelated to the location and extent of thrombosis, whereas the symptoms of pulmonary embolism vary depending on the degree and extent of vessel occlusion, as well as on a patient’s cardiopulmonary reserve. Therefore, any signs and symptoms that are suggestive of proximal thrombosis should be used not as diagnostic endpoints, but simply as a compelling reason to pursue further testing. Therefore, an appropriate objective diagnostic test to confirm or refute the clinical suspicion should be selected.

An important point that cannot be overstated is the clinician awareness of the associated elements that make up the clinical complex of inferior vena cava thrombosis. Contemplating this possibility in various situations (i.e., renal atrophy or agenesis highlighting underlying vascular anomalies) makes the patient more likely to receive prompt diagnosis and intervention avoiding derived management pitfalls and subsequent ensuing sequelae. In addition, different strategies like using a system to classify the symptoms (i.e., predominantly thrombotic in origin or predominantly embolic in nature) may aid in the diagnostic process.

Hematologic Studies. No specific laboratory test includes or excludes the diagnosis of inferior vena caval thrombosis, although evaluation of the clotting and fibrinolytic systems and assessment of the thrombin antagonists (i.e., protein C , protein S , antithrombin III , and anticardiolipin studies ) may be helpful, but many of these assessments can only be made after the fact. Confounding factors include variations caused by heparin and warfarin therapy, and dynamic factors involved with acute thrombosis may also alter measured parameters because of the active consumption of factor by the thrombus.

Imaging studies. Historically, the diagnostic approach to IVC thrombosis and PE has shifted from purely clinical (insensitive and nonspecific) and angiography-based (invasive) to being dependent primarily on noninvasive or minimally invasive objective imaging techniques.

From a global standpoint, contrast venography is the reference standard to show the presence of inferior vena cava thrombosis conclusively. The advantages of contrast venography include (i) limited false-positive study results; (ii) access for therapy, thrombolytic agents, or caval interrupting device; and (iii) access for pulmonary angiography (where indicated). However, this diagnostic tool presents many disadvantages that cannot be overstated. Its invasiveness, the possible need for more than one puncture (i.e., double access through the common femoral and the internal jugular veins) to document the extent of a thrombus in some circumstances, and the possibility of intraoperative partial thrombus dislodgement or postprocedure thrombosis have limited its use. In addition, the caudal extent of the clot may be overestimated because of preferential flow of contrast medium into collaterals and the diagnosis of caval tributaries thromboses (i.e., portal, mesenteric, or ovarian vein) maybe limited by the ability to perform selective contrast injections.

No studies have validated the use of duplex ultrasound for the detection of caval thrombosis. Although visualization of the inferior vena cava and dilated collaterals flow measurements have been described, compression maneuvers are not technically feasible in the abdomen, and the indirect signs of impaired flow and loss of flow phasicity are not specific for caval thrombosis. Moreover, duplex ultrasound cannot differentiate between bland and tumor thrombus, is suboptimal in visualizing the distal renal vein and the infrahepatic vena cava, and its accuracy is also strongly operator-dependent and influenced by the patient’s corporal habitus. On the contrary, other ultrasound modalities such as transesophageal echocardiography with or without doppler measurements have progressively gained a place in diagnosis, particularly in the malignancy setting, aiding the surgeon in the intraoperative assessment of proximal thrombus shape, mobility and size. It is also especially indicated in cases with conflicting findings on magnetic resonance imaging or in determining the presence of thrombus invading the major hepatic veins (Thompson et al. 2004).

Contrast-enhanced computed tomography (CT) scans are often obtained as part of the diagnostic evaluation for the primary process (i.e., malignancy) in cases of IVC thrombosis. This technique offers an adequate distinction between the different venous filling defects and the presence of extrinsic vessel compression. These both features are required to ensure appropriate treatment. However, this imaging modality remains a non-validated method to assess for the presence of thrombus in the inferior vena cava. Although spiral CT venography has been shown to visualize the intra-abdominal veins accurately, no formal studies have been published. Indirect signs that have been described in these cases include IVC diameter enlargement, reduced lumen density compared with that of the aorta, and rim enhancement. In addition, the presence of a “mass-like” network of collateral veins can be interpreted as suggestive of remote thrombosis. However, these signs are of little value in a patient with a history of thrombosis who is suspected to have a recurrent event, may occur as a result of “contrast flow phenomena ” (mimicking an intraluminal filling defect), and also can be present in patients with neoplastic tumor thrombus. Therefore, any incidental finding of a caval “filling defect” by computed tomography should ideally be confirmed by additional imaging if necessary, and not prompt the initial placement of an IVC filter.

Recent experience suggests that the improved multidetector-CT equipments would represent a valuable option in candidates unsuitable to magnetic resonance imaging (MRI). Doppler ultrasonography and CT have demonstrated good specificity in detecting the presence of intracaval thrombus, with a reported sensitivity of 65–90 %, reaching 87 % when used in combination (Thompson et al. 2004; Table 5).

Table 5 Comparison of multidetector CT and MRI for IVC tumor thrombus imaging

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MRI allows for examination in multiple planes and for estimation of the thrombus age. Reconstructive imaging technology can generate images similar to those seen with venography. The advantages of MRI include its noninvasiveness, lack of any ionizing radiation, and ability to help in determining the proximal extent of thrombosis. However, the disadvantages of this imaging modality include its cost, its accessibility, and the possibility that turbulent flow may be read falsely as a clot. Nevertheless, enhanced magnetic resonance venography has been determined to be the most reliable method of imaging tumor thrombus extent, involvement of vein tributaries, detection and differentiation of tumor thrombus versus blood clot, and assessing for vessel (i.e., caval, renal, and portal) occlusion, particularly when spin-echo and cine MRI techniques are used in combination (Oto et al. 1998).

Imaging Studies in Special Situations

Malignancy. In case of malignancy, appropriate management requires accurate staging. Many cancer patients undergo serial imaging with CT as a means of assessing cancer-therapy efficacy, disease stage or progression, and non-specific abdominal symptoms. Incidental findings of what appears to be a proximal bland thrombosis should not affect patient treatment and prompt the placement of an IVC filter . Prompt and proper diagnostic imaging is especially needed in such a situation. However, in cancer patients not all intraluminal filling defects represent a blood clot, and the distinction between intravascular tumor and bland thrombus might require further investigation with CT or MRI and, in some selected cases, with transvenous catheter-guided biopsy. Likewise, if the column of contrast does not opacify the inferior vena cava, CT or MRI is indicated to assess for the presence of extrinsic compression or invasion by tumor. Operative management should be preceded by very recent imaging (i.e., within 7–14 days), given that a variation in cranial extension due to rapid progression of tumor thrombus, can completely change the surgical approach (Woodruff et al. 2011).

Pulmonary embolism. Helical (spiral) CT and ventilation-perfusion lung scintigraphy remain the first-line imaging modalities for suspected acute pulmonary embolism. Spiral CT is preferred in cases of obvious pulmonary or pleural-based disease. Indeterminate initial studies should prompt performance of additional tests, possibly including the “gold standard”: contrast venography and pulmonary angiography. Evidence to date suggests that D-dimer assays might be unreliable in excluding venous thromboembolism in cancer patients.

Treatment Options and Management

Once the diagnosis has been established, the focus shifts to the different treatment options and the optimal management of the disease. The clinician must choose an appropriate treatment regimen for IVC thrombosis based on the underlying pathophysiology, and the goals of acute treatment of DVT/IVC thrombosis should center on managing the primary impact of thrombosis and avoiding embolization. Goals of treatment can be resumed as follows: (i) arrest growth of the thrombus, (ii) dissolve or remove the thrombus, and (iii) prevent the embolization of dislodged thrombus fragments (Brown 2001).

Both surgical and medical options are available to reach these goals. Medical management can include anticoagulation therapy and thrombolytic agents. In the broadest sense, surgical therapy of IVC thrombosis encompasses IVC interruption, endovenous approaches to IVC occlusion and a number of operative maneuvers to relief obstruction, including thrombectomy and IVC resection/reconstruction procedures.

Medical Management

Anticoagulation. Patients who present with acute IVC occlusion are treated with anticoagulation therapy whenever possible. Nonetheless, anticoagulation therapy does not actually treat IVC obstruction by dissolution of thrombus but instead prevents the propagation of the existing acute thrombosis. Patients diagnosed with DVT/IVC thrombosis and/or PE are initially anticoagulated in the acute setting with appropriate doses of subcutaneous low molecular weight heparin or intravenous heparin drip. Thereafter, they are transitioned to warfarin to undergo a minimum of 3 months of oral anticoagulation with a target international normalized ratio (INR) of 2–3. Patients at a high risk for recurrence should be treated indefinitely.

Complications from anticoagulation include intracranial or retroperitoneal bleeding or requirement for hospitalization/transfusion. However, the risk of major bleeding in patients with an appropriate INR range is estimated at only 4 %. Failure of anticoagulation is a rare event with a 90–95 % success rate if appropriate anticoagulation treatment is administered.

Thrombolysis. Thrombolytic agents are used to dissolve a pathologic intraluminal thrombus or embolus that has not been dissolved by the endogenous fibrinolytic system. They are also used for the prevention of recurrent thrombus formation and rapid restoration of hemodynamic disturbances.

Catheter-directed venous thrombolysis has been proposed as a means of reducing the risk of post-thrombotic syndrome, as this will actually dissolve the acute thrombus, restore venous patency, and, most importantly, restore venous valve function (Wicky 2009). Most thrombolytic agents have been reported in the treatment of IVC thrombosis. However, the merits of thrombolytic therapy must be weighed against the risks of hemorrhagic complications. Up to a 25 % risk of PE during therapy has been reported, and thus some reports advocate using IVC filters above the thrombolysis site, while some others do not. This therapy may play the greatest role as part of combination therapy with endovascular interventions.

Surgical Management

IVC interruption. Interruption of the IVC can be obtained through intraoperative sewing, stapling or ligation. A percutaneous approach can also be used to deploy a vena cava filter (VCF), and thus interrupt venous flow through the IVC. The conventional indication for IVC interruption is a patient with documented venous thromboembolism in whom anticoagulation is contraindicated or has failed. Recently added relative indications include the so-called “free-floating” thrombus and the severely reduced cardiopulmonary reserve (Jones et al. 1998).

VCFs are prophylactic devices that provide no treatment for DVT or PE. Rather, IVC interruption serves to significantly decrease the likelihood of emboli migrating to the pulmonary circulation. IVC interruption with VCFs should be used as an adjunct to prophylaxis in high-risk patients who cannot receive appropriate systemic anticoagulation, regardless of the presence of known venous thromboembolism. Placement of a VCF outside of conventional indication is a decision that should be made based on risk assessment.

IVC filters are also placed to protect against the long-term development of PE in patients with DVT. This is counterbalanced by an increased risk of complications that include iliocaval thrombosis in up to 10 %, access site thrombosis, vessel erosion, migration and fracture. Furthermore, maldeployment in relation to the renal veins may lead to renal failure. VCF may be considered prior to vascular surgery in the presence of a proximal DVT, particularly if there is a history of pulmonary emboli, intolerance to anticoagulation or the patient is regarded as high risk for thromboembolic complications. VCFs are additionally of benefit in patients where IVC involvement by tumor has been detected during preoperative staging. Temporary protection can be provided by retrievable VCFs, which may be removed after 30 days to avoid long-term sequelae (Joels et al. 2003).

Endovascular intervention . Several interventional radiology modalities are available to treat IVC thrombosis. These techniques are emerging as an alternative to open surgery because they are minimally invasive, relatively safe and mid-term IVC patency with symptom relief is excellent. In addition, later open surgery is not precluded.

Optimal results can be obtained by using one or a combination of the following options: (i) percutaneous balloon angioplasty, (ii) wall stents, and (iii) Z stents. The number and type of angioplasty sets and expandable stents are changing as product development continues, but the various stents have limitations both in vessel diameter and length of available stent. Indeed, IVC occlusions are lengthy, and therefore is an intuitive tendency to limit the length of stented segments to minimize thrombosis from stent exposure.

Endovascular techniques are particularly helpful to treat patients with IVC thromboses that have arisen from iatrogenic causes. The numerous clinical scenarios that lend themselves to this approach can include (i) long-term venous access, (ii) hemodialysis access, and (iii) surgery on the IVC, including hepatic transplantation.

Interventional radiology techniques may be applied also as adjunctive procedures prior to or during resection of inaccessible or vascular tumors. Embolization may be performed to prevent haemorrhage from hostile surgical fields. Typically metal coils, glues or particulate agents are deployed via catheter into a feeding vessel to act as a nidus for thrombosis. This is most commonly carried preoperatively in the angiography suite, but may be performed in the operating theatre if imaging facilities are available. The principal complication of embolization is inadvertent maldeployment into a main trunk vessel to cause distal ischaemia. Intraoperative bleeding may be controlled by embolization or intraluminal balloon occlusion catheters, which are positioned using fluoroscopic guidance and removed at the end of surgery. This latter technique should be performed with care to avoid iatrogenic vessel injury or thrombosis.

Thrombectomy. Thrombectomy is often carried out to gain patency in a previously occluded vein. However, re-thrombosis rates are significant and thrombectomy often fails to completely remove the thrombus. Hence, intravascular prevention devices (i.e., VCFs) are often required after a trombectomy attempt. The operative mortality rate is reportedly 2 %, while the morbidity rate is around 30 %. This procedure is typically performed in conjunction with a distal arteriovenous fistula to maintain high flow (i.e., trying to avoid re-thrombosis due to slow intravascular transit), and it may be specially required for cases of septic thrombus, in which septic clots have to be removed off the vessel (Vaidya et al. 2003; Ciancio et al. 2011; Gonzalez et al. 2011).

Venous resection. Venous resection may be required if a tumor is adherent to or encompassing the IVC. Veins that are chronically occluded may be sacrificed without reconstruction with minimal morbidity; less than a third of patients undergoing resection of occluded IVCs as part of oncological resection suffer long-term lower extremity edema (LEE). In contrast, ligation of a patent major vein is likely to cause significant morbidity from thromboembolism or venous hypertension due to inadequate collaterals (Gonzalez et al. 2014).

It has been suggested that involvement of less than half the circumference of a vein may be managed by either primary closure or venous patch repair. Veins may be patched or bypassed in a manner akin to arteries, although the long-term outcomes are less predictable or assured. This is primarily due to an amplified risk of thrombosis from slow blood flow and competitive flow through developed collateral vessels. Conversely, vessel resection is indicated for lesions involving greater than half the circumference (Duty and Daneshmand 2008; Gonzalez et al. 2014).

A recurring inference in the published literature is that, in selected patients, radical vascular resections do not necessarily amplify perioperative mortality in comparison to more conservative surgical approaches. Today, cancer free survival remains primarily related to resection margin status and tumor biology. Therefore, major vessel involvement by a tumor mass should not necessarily be considered a barrier to an “en-bloc” resection for curative surgical intent, and, in fact, aggressive surgical management may offer the only chance for a potential cure or palliation in these patients.

Given the small proportion of cancer patients with major vascular involvement, current summative evidence is based upon case series that are heterogeneous and often small. The published outcomes for a variety of malignancies suggest that survival is still dependent upon complete clearance of the primary pathology and tumor biology rather than vascular-related complications. Despite the absence of high-level evidence, these reports affirm the achievability of radical surgery most notably for retroperitoneal sarcomas, urological and pancreatic malignancies. Detailed preoperative planning within an extended multidisciplinary team that includes vascular specialists is essential for the management of these complex patients (Duty and Daneshmand 2008; Gonzalez et al. 2014).

Venous reconstruction . These procedures may be considered unnecessary if preoperative imaging demonstrates significant collateralization or there is preexisting occlusion and that tumor excision is not expected to injure venous collaterals. Preoperative planning should consider whether there is radiological evidence of venous occlusion, the extent of collateral vessel development and clinical evidence of LEE. Venous bypass therefore should be considered only if there is expected to be inadequate venous return through collateral vessels and, if performed, close follow-up with serial duplex scanning is advisable although there is no evidence on optimal frequency of scans.

Accordingly, reconstruction typically relies upon use of autologous conduits and short-term anticoagulation postoperatively which is followed by long-term antiplatelet therapy. The use of adjunctive arterio-venous fistulae may be considered to address low flow and pressure states within the deep veins on an individualized basis (Gonzalez et al. 2014).

Managing IVC Obstruction

The standard of care for the treatment of acute DVT/IVC occlusion is anticoagulation therapy. This treatment modality often prevents the formation of new thrombus and reduces the risk of PE but fails to eliminate the clot burden. In addition, treatment with anticoagulation alone, increases the risk of patients developing symptoms of the post-thrombotic syndrome, whereas thrombus removal strategies reduce post-thrombotic morbidity without increasing the risk of embolization (Herrera and Comerota 2011).

Strategies of thrombus removal for acute DVT, such as operative thrombectomy, catheter-directed thrombolysis, and pharmacomechanical techniques, are designed to avoid post-thrombotic morbidity by restoring patency to the deep veins, but there is concern that these techniques may result in fragmentation of clot and PE. Hence, non-obstructive or “free-floating” thrombus in the IVC is a risk factor for procedure-related embolization and is frequently an indication for the use of IVC interruption (Nyamekye and Merker 2012).

DVT and PE warrant prompt institution of antithrombotic therapy to effectively prevent thrombus propagation, embolization, and recurrence; to ameliorate patient symptoms; and to allow thrombus organization, plasmin-mediated lysis, and restoration of venous patency.

Although the abovementioned goals of treatment have remained constant, the initial management of DVT has undergone a series of evolutions during the past decade, affecting both acute treatment and disposition decisions. No specific guidelines exist to date for the treatment of IVC and other intra-abdominal DVT. Whether to use standard anticoagulant therapy as is used for proximal lower extremity DVT or catheter-directed thrombolytic therapy commonly depends on the extent of thrombosis, patient symptoms, and patient bleeding risk. Patients with acute, complete IVC occlusion may develop significant bilateral lower extremity swelling and pain, and are at risk for phlegmasia cerulean dolens (venous limb gangrene). Such patients could benefit most from pharmacologic or mechanical thrombolysis.

Summary

The chapter provides a detailed review on the embryological development, normal anatomy, anatomic variations and developmental anomalies of the IVC system. A review of the etiology, diagnosis and management of the clinical situations in which IVC obstruction may be evident is also provided.

Abbreviations

ACC:: Adrenocortical carcinoma
COC:: Combined oral contraceptives
CT:: Computed tomography
DVT:: Deep venous thrombosis
INR:: International normalized ratio
IVC:: Inferior vena cava
LEE:: Lower extremity edema
MHVs:: Major hepatic veins
MRI:: Magnetic resonance imaging
PE:: Pulmonary embolism
RCC:: Renal cell carcinoma
TKIs:: Tirosine kinase inhibitors
UICC/AJCC:: Union for International Cancer Control/American Joint Cancer Committee
VCF:: Vena cava filter
Virchow triad:: Factors predisposing vascular thrombosis. 1. Changes in the vessel wall. 2. Changes in the pattern of blood flow (flow volume). 3. Changes in the constituents of blood (hypercoagulability). This triad has been attributed to Rudolf Virchow, 19th century German pathologist.
Supracardinal veins::: paired vessels in the embryo, developing later than the subcardinal veins and persisting chiefly as the lower segment of the inferior vena cava.
Inferior vena cava::: Receives the blood from the lower limbs and the greater part of the pelvic and abdominal organs; it begins at the level of the fifth lumbar vertebra on the right side by the merger of the right and left common iliac veins, pierces the diaphragm at the level of the eighth thoracic vertebra, and empties into the posteroinferior aspect of the right atrium of the heart.
Isolated left inferior vena cava::: Caval anomaly consisting of an abnormal regression of the right embryo structures giving rise to a left-sided inferior vena cava.
Double inferior vena cava::: Caval anomaly consisting of a duplication of the inferior vena cava due to an anomalous regression of the embryonic veins.
Subcardinal veins::: paired vessels in the embryo, replacing the postcardinal veins and persisting to some degree as definitive vessels.
Circuncaval ureter (retrocaval ureter)::: the medial deviation of the right ureter in the rare circumstance in which it passes behind the inferior vena cava before entering the pelvis.
Inferior vena cava occlusion::: the blocking of venous return to the heart through the inferior vena cava due to a number of different clinical scenarios.
Collateral circulation::: venous network that carries the redirected blood flow through secondary channels in response to obstruction of the principal channel (i.e., inferior vena cava).

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Servicio de Urología, Hospital Central de la Cruz Roja San José y Santa Adela, Avenida de Reina Victoria 22-26, 28003, Madrid, Spain
Javier González
Department of Urology and Department of Surgery, Division of Transplantation, University of Miami Miller School of Medicine, Jackson Memorial Hospital, Miami Transplant Institute, 012440, Miami, FL, 33101, USA
Gaetano Ciancio

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Javier González
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Correspondence to Javier González.

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Division of Cardiovascular Disease Department of Internal Medicine, Health Care Center Bitterfeld, Bitterfeld-Wolfen, Germany
Peter Lanzer

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González, J., Ciancio, G. (2015). Retroperitoneal Venous Diseases. In: Lanzer, P. (eds) PanVascular Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-37078-6_151

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DOI: https://doi.org/10.1007/978-3-642-37078-6_151
Published: 02 February 2015
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-37077-9
Online ISBN: 978-3-642-37078-6
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Retroperitoneal Venous Diseases

Abstract

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Introduction

Anomalies in the Development of the Inferior Vena Cava

Development of the Primitive Venous System

Embryogenesis of the Inferior Vena Cava

Inferior Vena Cava and its Tributaries

Congenital Anomalies of the Inferior Vena Cava

Inferior Vena Cava Occlusion

Epidemiology

Predisponent Factors: The Virchow’s Triad

Etiology

Inferior Vena Cava Thrombosis due to Intrinsic Factors

Inferior Vena Cava Trombosis Due to Extrinsic Factors

Pathophysiology, Clinical Manifestations and Diferential Diagnosis

Collateral Circulation Network

Clinical Presentation

Manifestations of Lower Segment Occlusion

Manifestations of the Middle Segment Occlusion

Manifestations of Upper Segment Obstruction

Metabolic Effects and Chronic Sequelae of Inferior Vena Cava Occlussion

Diagnosis

Imaging Studies in Special Situations

Treatment Options and Management

Medical Management

Surgical Management

Managing IVC Obstruction

Summary

Abbreviations

References

Further Reading

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