Patients with inferior vena cava thrombosis frequently present with lower back pain and bilateral lower-extremity deep vein thrombosis
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Vasa 2013; 42: 275 – 283 C. Kraft et al.: Clinical presentation of IVC thrombosis
© 2013 Hans Huber Publishers, Hogrefe AG, Bern DOI 10.1024/0301-1526/a000288
Original communication 275
Patients with inferior vena cava thrombosis
frequently present with lower back pain and bilateral
lower-extremity deep vein thrombosis
Christiane Kraft, Carola Hecking, Jan Schwonberg, Marc Schindewolf, Edelgard Lindhoff-Last,
and Birgit Linnemann
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J. W. Goethe University Hospital Frankfurt/Main, Germany
Summary Zusammenfassung
Background: Inferior vena cava (IVC) thrombosis is rare, and Klinische Charakteristika von Patienten mit Thrombose der
data about the clinical presentation of patients are scarce. Vena cava inferior
Therefore, we reviewed all cases of IVC thrombosis consecu- Hintergrund: Thrombosen mit Beteiligung der Vena cava in-
tively registered in the MAISTHRO (MAin-ISar-THROm- ferior (VCI) sind extrem selten und es fehlen Daten, wie sich
bosis) database and described patients’ characteristics in Patienten mit dieser Thromboseform klinisch präsentieren.
terms of their clinical presentations in the acute setting of Anhand unseres prospektiven Thromboseregisters MAIS-
IVC thrombosis. THRO haben wir ein Kollektiv von VCI-Thrombosepatienten
Patients and methods: From the MAISTHRO registry, which zusammengestellt und stellen hier die klinischen Charakte-
enrolled 1470 consecutive patients with documented histo- ristika dieser Patientengruppe dar.
ries of venous thromboembolism, we identified 60 patients Patienten und Methoden: Aus dem MAISTHRO-Register,
(0,4 %; females 60 %) with IVC thrombosis and 888 patients in dem 1470 konsekutive Patientenfälle mit venöser
(60.4 %; females 55 %) with isolated lower-extremity deep vein Thromboembolie erfasst sind, konnten wir 60 Patienten
thrombosis (LE-DVT). (0,4 %; Frauenanteil 60 %) mit VCI-Thrombose und 888 Pa-
Results: The median age at the time of IVC thrombosis mani- tienten (60,4 %; Frauenanteil 55 %) mit isolierter tiefer Bein-
festation was 36.5 years (9 to 83). IVC thrombosis was the venenthrombose identifizieren.
initial VTE event in 47 patients (78 %). In the majority of cases, Ergebnisse: Bei den VCI-Thrombosepatienten lag das Mani-
IVC thrombosis extended to the lower-extremity veins, and festationsalter im Median bei 36.5 Jahren (Range 9–83). In
both lower extremities were affected in 17 cases (28 %). The 47 Fällen (78 %) war die VCI-Thrombose das erste Throm-
initial clinical symptom of IVC thrombosis was lower back or boseereignis. In der Mehrzahl der Fälle dehnte sich die
abdominal pain which preceded typical symptoms of LE-DVT VCI-Thrombose auf die Becken- und Beinvenen aus; eine
in 29 (48 %) patients. Symptomatic pulmonary embolism was beidseitige Thrombose fand sich in 17 Fällen (28 %). Gefragt
more frequently observed in IVC thrombosis patients when nach der Initialsymptomatik gaben 29 (48 %) der Patienten
compared to a sex- and age-matched subgroup of LE-DVT Rücken- und Bauchschmerzen an, für die sich kein ande-
patients, although the difference was not significant (27 % vs. res pathologisches Korrelat finden ließ. Erst mit zeitlicher
12 %; p = 0.064). Malignant disease was the only established Latenz trat die typische Klinik einer Beinvenenthrombose
VTE risk factor with a higher prevalence among IVC throm- hinzu. Symptomatische Lungenembolien waren häufiger bei
bosis patients than patients with isolated LE-DVT (27 % vs. VCI-Thrombose als bei isolierter Beinvenenthrombose, auch
9 %; p = 0.015). Congenital IVC anomalies were identified in wenn der Unterschied nicht signifikant war (27 % vs. 12 %;
another eight IVC thrombosis patients (13 %). p = 0,064). Eine maligne Erkrankung war der einzige etab-
Conclusions: IVC thrombosis should be considered a differen- lierte Risikofaktor, der häufiger bei VCI-Thrombosepatienten
tial diagnosis for inexplicable lower back or abdominal pain als bei Patienten mit isolierter Beinvenenthrombose vorkam
especially in young patients. Malignant disease and congenital (27 % vs. 9 %; p = 0,015). Angeborene Anomalien der Vena
IVC anomalies seem to be predisposing factors for thrombosis cava inferior fanden sich hingegen bei 8 Patienten mit einer
involving the inferior vena cava. VCI-Thrombose (13 %).
Schlussfolgerungen: Eine Thrombose der Vena cava inferior
sollte als Differenzialdiagnose in Betracht gezogen werden bei
zunächst unerklärlichen Rücken- oder Bauchschmerzen, ins-
besondere wenn jüngere Patienten betroffen sind. Malignome
Key words: Venous thrombosis, inferior vena cava, lower und angeborene VCI-Anomalien scheinen prädisponierende
back pain, congenital anomaly, malignant disease Faktoren für die Entstehung einer VCI-Thrombose zu sein.C. Kraft et al.: Clinical presentation of IVC thrombosis Vasa 2013; 42: 275 – 283
© 2013 Hans Huber Publishers, Hogrefe AG, Bern
276 Original communication
Introduction March 2000. Since then, 1470 con- bosis. All ultrasound studies were
secutive patients with acute or previ- performed in the supine position for
Deep vein thrombosis of the lower ous venous thromboembolism (VTE) the examination of the inferior vena
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extremity (LE-DVT) is estimated who were referred to our university cava, the iliac veins and the femoral
to occur with an incidence of 1 per hospital’s outpatient department were veins and in the sitting position for
1000 per year [1]. Typically, LE-DVT enrolled. We identified patients with the examination of the popliteal and
is suspected in cases of unilateral leg thrombosis involving the IVC and calf veins. From the common femoral
swelling and pain. Less frequently, pa- compared the patients’ character- vein to the distal calf veins, the diag-
tients present with tenderness along istics to the total cohort of patients nosis of venous thrombosis was based
the course of the deep veins or with with isolated lower-extremity DVT. on the absence of complete venous
a prominence of superficial veins Because of the difference in the distri- compressibility. The IVC and iliac
functioning as collaterals. In con- bution of manifestation age, further veins were screened by colour-coded
trast, thrombosis involving the infe- analysis was performed comparing ultrasound, and thrombosis was de-
rior vena cava (IVC) is a rare event, the IVC thrombosis cohort with a fined as the absence of spontaneous
and data on IVC thrombosis and its randomly selected LE-DVT subgroup or provoked blood flow within these
clinical presentation are scarce. In matched for sex and age (± 2 years) venous segments. If an examination
the literature, case reports and stud- in a 1 : 1 approach. In 3 adolescent was not conclusive or it was impos-
ies dealing with this infrequent form IVC thrombosis patients – 9, 12 and sible to determine the proximal ex-
of thrombosis primarily involve small 14 years of age – no corresponding tension of thrombosis, an additional
numbers of patients. match partners were available. imaging test was performed (Table I).
Our interest in this special form of Information about the clinical pre- During routine follow-up when at-
DVT arose through observations of sentation of VTE was provided by pa- tending our outpatient department,
several young patients in our outpa- tient interviews using a standardised all patients underwent at least one
tient department that presented with questionnaire and by medical reports complete ultrasound examination
extensive LE-DVT involving the IVC. sent by general practitioners or ad- after a standard protocol including
Some of these patients suffered from mitting hospitals. The interview was the iliac veins and inferior vena cava
inexplicable lower back pain for sev- performed after a median period of with the intention to screen for con-
eral days to weeks before thrombosis 31 months (interquartile range 13 genital anomalies.
manifestation. Consultations with an to 71) from the acute VTE event. Diagnostic procedures for pulmo-
orthopaedic specialist and X-rays of When the family histories were as- nary embolism (PE) in the acute
their spines did not reveal the causes sessed, only patients with VTE events DVT situation were only performed
of the pain. Therefore, the symptoms in first-degree relatives were consid- if patients presented with the typical
of these patients were treated with an- ered to have a positive family history. clinical symptoms that suggest PE.
algesics until leg swelling occurred, The study protocol was approved by For the diagnosis of acute PE, patients
which led to the diagnosis of exten- the local ethics committee and has underwent multi-detector spiral CT
sive thrombosis involving the inferior been registered in ClinicalTrials. scan or ventilation/perfusion lung
vena cava. Gov (NCT00631423). All patients scintigraphy (Table I).
With a retrospective case-control enrolled in this study provided writ-
study design, we aimed to describe ten informed consent. Clinical presentation
clinical characteristics of patients For detailed information about the
with thrombosis involving the IVC Diagnosis of VTE clinical presentation of IVC throm-
and compare them to a cohort of pa- IVC thrombosis was diagnosed by bosis, we contacted all patients again
tients with isolated LE-DVT matched performing a colour-coded duplex and asked them to specify the symp-
for sex and age. ultrasound examination, conven- toms at the time of the venous throm-
tional venography, contrast-medi- bosis manifestation. We registered
ated computed tomography (CT) or the first symptom and the symptoms
Patients and methods magnetic resonance imaging studies at the time when thrombosis was con-
(MRI). LE-DVT was diagnosed by firmed by an imaging technique. In
Study population either compression ultrasonography addition, patients were asked about
Patient data were obtained from the or conventional venography. Ultraso- the time interval between the start
MAISTHRO (MAin-ISar-THROm- nography was the preferred method of symptoms and the time of diagno-
bosis) registry [2], which began in in cases of suspected venous throm- sis. Furthermore, we aimed to assessVasa 2013; 42: 275 – 283 C. Kraft et al.: Clinical presentation of IVC thrombosis
© 2013 Hans Huber Publishers, Hogrefe AG, Bern
Original communication 277
Table I: Diagnostic procedures in cases of IVC thrombosis and isolated LE- ered positive for antiphospholipid an-
DVT tibodies if at least two tests performed
at a 12 week interval showed patho-
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IVC Isolated logic results. As all recruited patients
Thrombosis LE-DVT suffered from VTE, patients with
(N = 60) (N = 57) confirmed antiphospholipid antibod-
N (%) N (%) ies fulfilled the diagnostic criteria of
an antiphospholipid syndrome [4].
Diagnosis of DVT
Duplex ultrasound 53 (88) 49 (86) Statistical analysis
Conventional venography 22 (37) 19 (33)
Statistical analysis was performed us-
CT scan 43 (72) 4 (7)
MRI scan 7 (12) 0 (0) ing the Statistical Package for Social
Sciences (SPSS, version 20.0; Chica-
Diagnosis of PE go, IL, USA). In addition to the de-
Spiral multidetector CT scan 22 (37) 11 (18)
scriptive statistics with frequencies,
Lung scintigraphy 9 (15) 4 (7)
median and range, we also performed
Echocardiography 21 (36) 15 (26)
the U-Test of Mann and Whitney to
compare the metric variables and the
Chi-squared test in cross-tabulations
which medical discipline was first tation or was diagnosed during an for the categorical variables. The cri-
involved with the patient. Thus, data acute VTE course. In cases of IVC terion for statistical significance was
from fifty-three IVC thrombosis pa- thrombosis, external compression of a p-value less than 0.05.
tients were available. The information the IVC or pelvic veins by benign or
obtained from patient interviews was malignant tumours, liver cirrhosis or
reconciled with that from the medi- inflammatory or infectious disease in Results
cal records of the VTE event. Only anatomical sites of venous drainage
one patient could not be contacted (the liver, gall bladder, pancreas, intes- Baseline characteristics
for follow-up, and six IVC throm- tines or genitourinary tract) defined Between March 2000 and Febru-
bosis patients and four patients with venous thrombosis as risk-associated. ary 2008, 1470 consecutive patients
isolated LE-DVT had died. In these If thrombosis was not related to one with documented histories of venous
cases, we only analysed data from of the aforementioned factors, the thromboembolism were registered in
medical records. condition was considered to be an the MAISTHRO registry. Of those, 60
unprovoked DVT. Thus, according (0.4 %) had thrombosis involving the
VTE risk factors to these criteria, carriers for throm- IVC, and 888 suffered from isolated
Venous thrombosis was classified as bophilic disorders were also classified lower-extremity DVT (60.4 %). The
risk-associated if the thrombosis was as having an unprovoked VTE. IVC thrombosis cohort was com-
related to at least one of the follow- prised of 36 women (60 %) and 24
ing risk factors: long-term travel (for Testing for thrombophilia men (40 %). The patient age at IVC
more than 6 hours), surgical interven- Screening for thrombophilia includ- thrombosis manifestation ranged
tion within the last 4 weeks, immobili- ed testing for the factor V Leiden from 9 to 83 years (median 36.5), and
sation for longer than 3 days, active mutation (FVL), the prothrombin 58 % experienced their IVC throm-
malignant disease or treatment for G20210A mutation (PT), antiphos- bosis before the age of 40. Thirteen
malignoma by the time of VTE mani- pholipid antibodies (APL) and the IVC thrombosis patients had suffered
festation, acute or chronic inflamma- antithrombin (AT), protein C (PC), another VTE previously (22 %). For
tory disease, hormonal treatment or protein S (PS) and factor VIII (FVIII) 47 patients (78 %), the IVC throm-
pregnancy. Inflammation consisted of activities, as previously described bosis was the first VTE event. These
either an acute infection or a chronic [3]. If measuring the antithrombin, patients averaged 31.5 years of age at
inflammatory disease (e.g., inflam- protein C, protein S or factor VIII the time of thrombosis manifestation.
matory bowel disease, rheumatoid activities revealed values outside of During the same time interval, we
arthritis or systemic lupus erythema- the reference range, the measurement registered 888 patients (55 % women)
tosus). Malignant disease was either was repeated at least once to confirm with a first isolated lower-extremity
present at the time of VTE manifes- thrombophilia. Patients were consid- DVT (LE-DVT) not extending theC. Kraft et al.: Clinical presentation of IVC thrombosis Vasa 2013; 42: 275 – 283
© 2013 Hans Huber Publishers, Hogrefe AG, Bern
278 Original communication
the diagnoses of thrombosis were
confirmed. The time interval be-
tween the start of symptoms and the
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confirmation of thrombosis ranged
from 1 to 35 days (median 5 days) in
the case of IVC thrombosis and from
one to 90 days (median 7 days) in the
case of LE-DVT (p = ns). Among pa-
tients with IVC thrombosis, 18 (30 %)
reported lower back pain to be the
first clinical sign, whereas 11 (18 %)
mentioned abdominal pain as their
first symptom. Nine patients (15 %)
reporting lower back pain were ini-
Figure 1: Age distribution at the time of VTE manifestation among 60
tially seen by an orthopaedic special-
patients with IVC thrombosis and 888 patients with LE-DVT registered in
ist. In these cases, a clinical assess-
the MAISTHRO database.
ment and an x-ray of the spine did
not reveal any explanation for the
pain. The median age of these patients
inguinal ligament proximally. The had a so-called tumour thrombosis was 26 years, and 6 patients were even
patient ages at LE-DVT thrombosis progressing into the IVC. In only one younger than 18 years. Another 12
manifestation ranged from 17 to 87 case, IVC thrombosis was limited to patients (20 %) were initially seen
years (median 44.0 years). The distri- the IVC (1.6 %). In this case, symp- by general practitioners, surgeons
butions of age at the time of throm- tomatic hydronephrosis in a horse- or gynaecologists for lower back or
bosis manifestation for both groups shoe kidney led to the diagnosis of abdominal pain without finding the
are presented in Fig. 1. Patients who a circumscribed IVC thrombosis at source of the symptoms. In contrast,
suffered IVC thrombosis as the first the same time. In the majority of no patient with LE-DVT suffered
thrombotic event were significantly cases, IVC thrombosis extended to from lower back or abdominal pain as
younger at the time of thrombosis the iliac and lower-extremity veins. an initial symptom of DVT. Remark-
manifestation than LE-DVT patients Bilateral LE-DVT was detected in 17 ably, only a minority of IVC throm-
(31.5 vs. 44.0 years; p = 0.001). Twen- cases (28 %), whereas involvement of bosis patients (30 %) reported classic
ty-four out of 60 (40 %) IVC throm- the left or the right lower extremity symptoms such as pain or swelling
bosis patients were younger than 30 veins was observed in 26 (44 %) and of the lower extremities as their first
years. No significant difference was 17 (28 %) cases, respectively. IVC symptom.
observed between IVC thrombosis thrombosis distally extended to the However, at the time when IVC
and LE-DVT patients with regard to iliac veins in 57 cases (95 %), to the thrombosis was finally diagnosed,
sex (60 % and 55 % females, respec- femoropopliteal veins in 48 cases the situation had changed. There were
tively). (80 %) and to the calf veins in 22 53 IVC thrombosis patients (88 %)
cases (37 %). In contrast, no patient who had developed leg symptoms
Localisation and extension of with an isolated LE-DVT had a bi- such as pain or swelling indicating
thrombosis lateral thrombosis. Among patients thrombosis and resulting in specific
IVC thrombosis was located in the with isolated LE-DVT, there were 36 diagnostic procedures. At the same
suprarenal segment in 2 cases (3 %) cases (63 %) with DVTs of the femo- time, 28 (52 %) and 16 patients (30 %)
and the infrarenal segment in the ropopliteal veins and 21 cases (37 %) also reported lower back and abdomi-
remaining 58 cases (97 %). Suprare- with isolated calf vein thromboses. nal pain, respectively. In contrast,
nal IVC thrombosis occurred in one leg pain and swelling were the most
patient with Budd-Chiari syndrome Clinical presentation prevalent first symptoms in LE-DVT
involving the hepatic veins and ex- Table II presents the prevalence of patients (89 %).
tending proximally into the IVC and symptoms at the time of thrombosis Thrombosis manifestation oc-
the right atrium. The other patient manifestation. We differentiated be- curred with symptoms indicative
with suprarenal IVC thrombosis suf- tween the patients’ initial symptoms of PE (e.g., chest pain, dyspnoea)
fered from renal cell carcinoma and and the symptoms at the time when in 13 % and 9 % of patients withVasa 2013; 42: 275 – 283 C. Kraft et al.: Clinical presentation of IVC thrombosis
© 2013 Hans Huber Publishers, Hogrefe AG, Bern
Original communication 279
Table II: Prevalence of the patients’ initial symptoms and the symptoms at the time of diagnosis
Initial symptom* Symptoms at the time of diagnosis*
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IVC Isolated IVC Isolated
Thrombosis LE-DVT Thrombosis LE-DVT
(N = 60) (N = 57) (N = 60) (N = 57)
N (%) N (%) p-value N (%) N (%) p-value
Lower back pain 18 (30) 0 (0)C. Kraft et al.: Clinical presentation of IVC thrombosis Vasa 2013; 42: 275 – 283
© 2013 Hans Huber Publishers, Hogrefe AG, Bern
280 Original communication
Table IV: Prevalence of hereditary and acquired thrombophilia Thus, lower back or abdominal pain
was much more likely to be the ini-
IVC Isolated P value tial symptom of IVC thrombosis than
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Thrombosis LE-DVT leg pain or swelling. At the time of
(N = 60) (N = 57) the definitive diagnosis of DVT,
even more IVC thrombosis patients
N % N % (82 %), suffered from lower back pain
FVL mutation 17/58 29 21/56 38 0.428 and abdominal pain. Until the time
PT mutation 6/57 11 4/56 7 0.742 that DVT was diagnosed, i.e., one to
Elevated FVIII activity 7/49 14 9/53 17 0.789 35 days after the start of symptoms,
AT deficiency 2/60 3 0/60 0 0.496 as many as 89 % of IVC thrombosis
PC deficiency 1/49 2 1/52 2 1.000 patients also developed leg symptoms
PS deficiency 3/47 6 3/49 6 1.000 which led to the suspicion of DVT
Lupus anticoagulant 6/57 11 3/57 5 0.490 and finally to the correct diagnosis.
ACL-IgG/-IgM 2/59 3 1/57 2 1.000 In contrast, 91 % of patients with iso-
lated LE-DVT initially became symp-
FVL = Factor V Leiden; PT = prothrombin; FVIII = factor VIII; AT = antithrombin; tomatic with pain, swelling or cramp-
PC = protein C; PS = protein S; ACL = anticardiolipin.
ing of the leg. No LE-DVT patient
experienced lower back or abdominal
tients without IVC anomalies (35 vs. ease was already known at the time pain as a first symptom.
37 years; p = 0.695). In contrast, no of thrombosis manifestation. In cases where thrombotic obstruc-
IVC anomalies were detected among tion of the IVC occurs, the ascending
patients with isolated LE-DVT (Ta- lumbar veins and lateral sacral veins
ble III). Discussion are important collaterals to achieve
venous blood flow from the lower ex-
Malignant disease In this retrospective case-controlled tremities into the azygous and hemia-
Malignant disease was present in 16 study, we investigated the clinical zygos veins. The extensive blood flow
IVC thrombosis patients (27 %) and characteristics of IVC thrombosis via the ascending lumbar veins also
was located in the kidneys (n = 2), patients compared to patients with affects the vertebral venous system
ovaries (n = 2), testes (n = 2), blad- isolated LE-DVT. The main find- that consists of an internal and exter-
der (n = 1), vulva (n = 1), colon ings of our study are as follows: in nal venous network (Fig. 2). Radicu-
(n = 2), lung (n = 1), mammary the majority of patients, IVC throm- lar veins traverse the intervertebral
(n = 1), and brain (n = 1). External bosis occurs before the age of 40; an foramen and connect the internal and
compression of the IVC by tumour IVC thrombosis often presents with external vertebral veins and plexus.
masses was observed in only 2 cases, lower back or abdominal pain; IVC Thus, IVC thrombosis may lead to
and in 2 patients with renal cell car- thrombosis frequently involves both enlargement of these collateral veins
cinoma, a tumour invasion into the iliac or lower-extremity veins; in the and compression of the neighbour-
renal vein caused a so-called tumour acute situation, the risk of symptom- ing neural nerve roots. Consequently,
thrombosis. Additionally, three IVC atic pulmonary embolism is high; patients can present with radicular
thrombosis patients suffered from and regarding the prevalence of es- pain and sciatica. In most cases, lower
haematologic malignancies. The tablished VTE risk factors, malignant back pain associated with radicular
prevalence of malignant disease was disease is more frequent among IVC pain is caused by prolapse of an inter-
higher when compared to LE-DVT thrombosis patients when compared vertebral disc. However, compression
patients (27 % vs. 9 %; p = 0.015). In to those with isolated LE-DVT. Fur- of the peripheral nerve roots due to
the LE-DVT group, cancer was lo- thermore, a considerable number of vascular pathologies has also been
cated in the mammary (n = 2), the IVC thrombosis patients presented recognised as a possible cause of back
ovaries (n = 2) and the lung (n = 1). with a congenital anomaly of the and radicular pain. In particular, the
In 4/16 IVC thrombosis patients and IVC (i.e., segmental hypoplasia or enlargement of the epidural veins has
2/5 LE-DVT patients, malignant dis- aplasia). been associated with the occurrence
ease was first diagnosed during the Approximately every second patient of lower back pain, lumbar radicu-
diagnostic work-up for DVT. In the with IVC thrombosis suffered from lopathy, claudication, and even cauda
remaining patients, malignant dis- initial symptoms atypical for DVT. equina syndrome [5 – 8]. Paksoy etVasa 2013; 42: 275 – 283 C. Kraft et al.: Clinical presentation of IVC thrombosis
© 2013 Hans Huber Publishers, Hogrefe AG, Bern
Original communication 281
ment of DVT despite a compensatory
enlargement of the lumbar, azygous,
hemiazygos and other collateral veins
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that allow adequate venous outflow
from the pelvis and lower extremities
[10, 11, 14].
Besides congenital anomalies, ma-
lignant disease was observed more
frequently in IVC thrombosis pa-
tients when compared to patients
with isolated LE-DVT (27 % vs. 9 %;
p = 0.015). Among IVC thrombosis
patients, 15 patients had solid tu-
mours located in different organs
Figure 2: Anatomy of the paravertebral venous system (from [5]) (i.e., genitourinary and gastrointes-
tinal tract, lung, mamma, or brain). In
addition, IVC thrombosis occurred
al. analysed MRIs from 9640 patients of IVC anomalies in the general pop- in three patients with haematologic
experiencing back pain or sciatica ulation. In the majority of cases, IVC malignancies. It may be possible that
and found that 13 (0.13 %) had IVC anomalies usually are asymptomatic an obstruction of the IVC due to a
obstruction or occlusion [5]. In ten due to a patent compensatory system neighbouring tumour process or a
of these patients, thrombosis of the of venous collaterals and are detected tumour thrombosis due to invasive
infrarenal IVC was detected, and in only when imaging studies are per- tumour growth could explain the
three patients, external compression formed for other reasons. However, difference between groups. How-
of the IVC due to a neighbouring the anomaly most often observed in ever, only 2 cases of external IVC
pathologic process was diagnosed. conjunction with IVC thrombosis compression by tumour masses and
MRI scans demonstrated enlarge- or proximal LE-DVT is a segmen- 2 cases of tumour thrombosis from
ment of the epidural venous plexus in tal aplasia or hypoplasia of the IVC renal cell carcinoma were identified
all of these cases. All of these patients [10 – 22], which was also observed in in our cohort. Therefore, the majority
presented with acute onset of lower our IVC thrombosis cohort. of malignancy-associated VTE can
back pain followed shortly thereafter Remarkably, a congenital anomaly be attributed to malignancy-induced
by acute radicular symptoms. of the IVC was present in 13 % of hypercoagulability.
The development of symptoms, i.e., the IVC thrombosis cases; however, Patients with IVC thrombosis did not
lower back pain as an initial symp- these patients were not younger at differ from those with isolated LE-
tom, favours the hypothesis that in a IVC thrombosis manifestation than DVT with regard to the other VTE
considerable number of patients, the those without anomalies. In contrast, risk factors or the prevalence of he-
thrombotic process initially involves no IVC anomalies were detected reditary or acquired thrombophilias.
the IVC and secondarily descends to among patients with isolated LE- However, the patient numbers were
the pelvic and lower extremity veins. DVTs. Unfortunately, the majority of too small to draw definitive conclu-
Bilateral thrombosis is a frequent LE-DVT patients were diagnosed by sions.
finding among IVC thrombosis pa- ultrasonography or conventional ve- In our cohort, there was a trend to
tients. In a systematic review of 26 nography but did not receive a CT or a higher frequency of symptomatic
cases with IVC anomalies and DVT, MRI scan, so we may have overlooked PE among patients with IVC throm-
Yun et al. observed a bilaterally lo- some cases of IVC anomalies. In the bosis compared to LE-DVT patients.
cated DVT in 16/26 cases (54 %) [9]. general population, IVC anomalies Patients with IVC thrombosis have a
In the presence of an anomaly of the are reported to occur in 0.3 % of oth- larger clot burden than patients with
IVC, bilateral iliofemoral thrombosis erwise healthy subjects and generally isolated LE-DVT which may height-
has been reported in 35 % to 75 % of result from aberrant development en the risk of symptomatic PE. It has
patients, respectively [10 – 13]. How- during the sixth to eighth weeks of previously been shown that patients
ever, these numbers have to be inter- embryogenesis [10]. IVC anomalies with proximal LE-DVT have higher
preted with caution because there is are considered a condition that fa- PE rates than patients with isolated
still uncertainty about the prevalence vours venous stasis and the develop- distal LE-DVT [23].C. Kraft et al.: Clinical presentation of IVC thrombosis Vasa 2013; 42: 275 – 283
© 2013 Hans Huber Publishers, Hogrefe AG, Bern
282 Original communication
It is possible that massive thrombo- Conclusions 4 Miyakis S, Lockshin MD, Atsumi
sis may occur especially in cases of T, BranchDW, Brey RL, Cervera R,
recurrent DVT, but in 78 % of cases, Inferior vena cava thrombosis should Derksen RH, De Groot PG, Koike
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the IVC thrombosis was the first be considered a differential diagno- T, Meroni PL, Reber G, Shoenfeld
VTE event, and these patients were sis in cases of lower back pain or Y, Tincani A, Vlachoyiannopoulos
significantly younger at the time of sciatica, especially if the patient is PG, Krilis SA. International consen-
thrombosis manifestation compared young and no orthopaedic or other sus statement on an update of the
to all patients with isolated LE-DVT. pathology can be identified. Lower classification criteria for definite an-
A similar observation was made by back or abdominal pain is the most tiphospholipid syndrome (APS). J
Joffe et al. who compared patients frequent initial clinical sign of IVC Thromb Haemost 2006; 4: 295 – 306.
with iliac vein thrombosis to patients thrombosis and often precedes the 5 Paksoy Y, Gormus N. Epidural ve-
with isolated LE-DVT and found that development of typical symptoms nous plexus enlargements present-
patients with massive initial DVT of LE-DVT. However, the definitive ing with radiculopathy and back
were younger [24]. The reason that diagnosis of DVT is often not made pain in patients with inferior vena
younger patients tend to develop until the development of typical leg cava obstruction or occlusion. Spine
more massive thrombosis than older symptoms. Bilateral LE-DVT extend- 2004; 29: 2419 – 24.
patients is unknown. In our cohort, ing into the iliac or femoral veins is 6 Dudeck O, Zeile M, Poellinger
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before the age of 40. However, the IVC thrombosis. B. Epidural venous enlargements
time interval between the start of presenting with intractable lower
symptoms and the diagnosis of DVT back pain and scatica in a patient
was similar between both groups. A Conflicts of interest with absence of the infrarenal in-
genetic predisposition seems to be ferior vena cava and bilateral deep
unlikely as hereditary thrombophilia There are no conflicts of interest venous thrombosis. Spine 2007; 32:
and a family history positive for VTE existing. E688 – E691.
was even less frequent among IVC 7 Hammer A, Knight I, Agarwal A.
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Beckman JA, Silverman SG. Throm- risk factor for idiopathic deep vein Submitted: 15.01.2013
bosed interrupted inferior vena thrombosis. Lancet 2001; 357: 441. Accepted after revision: 03.03.2013You can also read
