Sagban et al.

Orphanet Journal of Rare Diseases (2015) 10:3

DOI 10.1186/s13023-014-0223-4

RESEARCH Open Access

Elevated risk of thrombophilia in agenesis of the

vena cava as a factor for deep vein thrombosis
Tolga Atilla Sagban1*, Rüdiger E Scharf2, Markus U Wagenhäuser1, Alexander Oberhuber1, Hubert Schelzig1,
Klaus Grabitz1 and Mansur Duran1

Abstract
Background: Congenital absence of the inferior vena cava (AIVC) is a rare malformation which may be associated
with an increased risk for deep vein thrombosis (DVT). However, the role of thrombophilia in AIVC and DVT is
unknown.
Methods: Between 1982 and 2013 41 patients (12 female, 29 male, mean age 28 S.D. 11 years) were detected at
the University of Düsseldorf, Germany, with AIVC. Based on medical history, clinical examination, imaging and
coagulation studies, we performed on this collective a risk characterisation. Extensive literature research added
further 123 published cases during 1993 and 2013. AIVC-patients were compared with iliocaval DVT-patients without
AIVC (n = 168) treated during the same period in our clinic (90 female, 78 male, mean age 38 S.D. 17 years).
Results: In contrast to classical DVT younger men were more often affected. Factor-V-Leiden-mutation,
5,10-methylenetetrahydrofolate reductase (MTHFR) polymorphism and hyperhomocysteinemia individually are
associated with an increased risk of DVT in patients with AIVC. Aplasia/hypoplasia of the right or left kidney is also
associated with IVCA.
Conclusions: AIVC should be considered in young patients who present with DVT involving the vena cava.
Analysis of publications with AIVC and our patients yielded a typical spectrum of AIVC-associated DVT
characteristics: AIVC occurs in young male adults, is revealed by proximal DVT, not necessarily accused by
precipitating factors like immobilisation, and is mostly located bilateral. Hereditary coagulation abnormalities seem
to be more often a contributing factor for DVT in AIVC.
Keywords: Vena cava anomaly, Agenesis, Deep venous thrombosis, Thrombophilia

Background which drain into the PCV. Between the two SV and the
The formation of the inferior vena cava (IVC) in the hu- omphalomesenteric system numerous anastomoses exist,
man embryo is a complex process with fusion and regres- which give rise to the intrahepatic segment of the IVC. At
sion of three paired veins which develop around the sixth this stage the involution of the PCV and SV begins,
gestational week [1]. The posterior cardinal veins (PCV) though the SV are only partially obliterated, maintaining
drain the venous flow from the medial and caudal portions their distal portion, which gives rise to the corresponding
of the dorsal wall of the embryo into the venous sinus of gonadal vein on the left and to the renal and postrenal
the primitive heart through Cuvier’s veins. On the other segments of the IVC on the right. At the same time as the
hand, the omphalomesenteric veins, give rise to hepatic si- regression of the SV and PCV, a new system of SV de-
nusoids, which lead directly to the coronary sinus via the velops designed to drain the dorsal wall of the embryo.
hepatocardiac ducts. The primitive kidney or mesoneph- These later veins develop later to the azygos and hemiazy-
ros is drained by the ipsilateral subcardinal veins (SV), gos venous systems.
Malformations of the IVC are unusual and include an
interruption of the IVC with azygos and hemiazygos vein
* Correspondence: drsagban@yahoo.de
1
Department of Vascular and Endovascular Surgery, Heinrich Heine University
continuation [2,3]. 90% of those cases involve suprarenal de-
Düsseldorf, Moorenstraße.5, 40225 Düsseldorf, Germany fects and only 6% involve the renal or infrarenal segments.
Full list of author information is available at the end of the article

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unless otherwise stated.
Sagban et al. Orphanet Journal of Rare Diseases (2015) 10:3 Page 2 of 7

Absence of the infrarenal segment of the IVC (AIVC) is an topics addressed herein and to answer several questions im-
extremely rare anomaly [4]. The reasons for such a develop- portant in clinical practice: demographic data of the AIVC
mental failure are unclear. Most researchers believe that the population, clinical DVT presentation, imaging for AIVC
cause lies in embryonic dysgenesis affecting separate seg- diagnosis, additional anatomical anomalies, contribution of
ments or the entire IVC [5-7]. Others suggest that AIVC is thrombophilia screening and therapeutic approach.
not embryonic in origin, rather the result of intrauterine or All patients treated in our clinic, had an objectively di-
perinatal thrombosis [8,9]. agnosed episode of DVT diagnosed by Doppler ultrason-
Until now only case reports or small collectives were ography, computed tomography or magnetic resonance
reported for AIVC. Normally these malformations are imaging and/or by venography. These patients were
well collateralized because of their presence since em- treated with anticoagulation or when feasible by surgery
bryonic development. But some of them come to med- in combination with anticoagulation [13].
ical attention because of deep vein thrombosis (DVT).
Although association with hyperhomcysteinemia [10] Laboratory tests
and factor-V-Leiden have been reported [11], in litera- All patients in our clinic were screened for inherited and ac-
ture described collectives are very small and partly con- quired defects in blood coagulation. We determined the ac-
trary [13]. Only limited data is available on the role of tivities of lupus anticoagulant and hyperhomocysteinemia in
thrombophilia associated with AIVC [10-12]. To enlarge plasma, and performed a genetic analysis to determine
the small cohort of our AIVC patients (n = 41), we per- factor-V-Leiden-gene-mutation, the G20210A prothrombin-
formed a Medline research reporting about cases with gene-mutation, and the gene encoding C677T 5,10-methy-
AIVC and could identify 124 additional AIVC patients lenetetrahydrofolate reductase (MTHFR)-mutation. Lupus
with tested thrombophilia to ours (Additional file 1). anticoagulant was measured with the DVV test and DVV
confirm test (American Diagnostica, Greenwich, Conn.).
Methods
This study was approved by the local ethics committee Genetic analysis
of the University Hospital of Düsseldorf, Germany and DNA was extracted from peripheral-blood leukocytes
informed consent was obtained from every single patient according to standard protocols with the use of the Che-
treated and investigated in our department, which in- lex system (Bio-Rad, Munich, Germany). The presence
cluded permission to use data from chart review and of factor-V-Leiden was determined by allele-specific
genetic investigations for thrombophilc factors. The restriction-enzyme analysis [14]. For confirmation of the
study was performed in accordance with the Helsinki genotypes, an oligonucleotide-ligation assay was per-
Declaration. formed [15]. The results of the oligonucleotide-ligation
assay were in 100 percent concordance with the results
Subjects of genotypic analysis, as determined by an allele specific
We studied 209 consecutive patients between January restriction-enzyme assay. The G20210A prothrombin-
1982 and June 2013 with a history of ileo-femoral DVT gene-mutation was identified by allele-specific restriction-
with involvement of the vena cava. In this collective, 41 enzyme analysis [16]. Screening for the presence of the
individuals with an AIVC were identified. Patients with C677T MTHFR-polymorphism was performed by the
ilio-femoral, vena cava involving DVT without AIVC method described by Frosst et al. [17].
(NoAIVC; n = 168) served as control group.
All patients were evaluated for determination of Statistical analysis
thrombophilic and other abnormalities. In both, the pa- The SPSS statistical package (SPSS for Windows, version
tients with a history of DVT and/or patients with an 19.0 SPSS Inc., Chicago, IL., USA) was used for all statis-
AIVC, blood samples were obtained after the diagnosis tical analyses. Measure of variation is reported as stand-
of DVT or/and AIVC. ard deviation. Depending on the type of data, the
We compared and added our results with those reported Wilcoxon rank-sum test, chi-square analysis, or Fisher’s
available on Medline database with sufficient relevant data. exact test (two-tailed) was used to assess the differences
References were identified through search of the Medline between the groups. Multivariate analyses, including the
database using the terms: “inferior vena cava”, “absence”, calculation of predictive values, were performed with the
“anomaly”, “agenesis” and “atresia”. 68 publications could use of a stepwise logistic-regression procedure.
be identify, mostly case reports from 1993 until 2013, add-
ing information of 124 patients with AIVC to our collective Results
(Additional file 1). Publications without information about The characteristics of all patients are presented in
patients’ thrombophilia were disclosed. The references Table 1 (details of patients derived by literature research
finally retained were chosen based on their relevance to is presented under Additional file 1).
Sagban et al. Orphanet Journal of Rare Diseases (2015) 10:3 Page 3 of 7

Table 1 Characteristics of the patients with an aivc in this paper and in literature, compared with noaivc
Characteristics Patients with AIVC and Patients with AIVC in Patients with P Value of AIVC in this
DVT in this paper (n = 41) literature research (n = 124) NoAIVC (n = 168) paper and in literature
(n = 165) vs. NoAIVC (n = 168)
Sex male/female (male in %) 29/12 (70.7%)† 93/31 (75.0%)† 78/90 (46.4%) <0.0001
Mean age at appearance of
DVT (years ± SD) 28 ± 11.1† 28 ± 11.0† 38 ± 17.3 <0.0001
Range (min., max.) 16-58 8-67 8-84
preciptating factors for DVT -
No. (%) 25/41 (61.0%)† 52/122 (42.6%)†* 130/168 (77.4%) <0.0001
† †
Left sided DVT (%) 4/41 (9.8%) 26/124 (21.0%) 83/168 (49.4%) <0.0001
Right sided DVT (%) 12/41 (29.3%)† 30/124 (24.2%)† 28/168 (16.7%) 0.049
† †
Both sided DVT (%) 25/41 (61.0%) 59/124 (47.6%) 57/168 (33.9%) 0.002
DVT deep vein thrombosis. AIVC inferior vena cava agenesia. NoAIVC iliofemoral DVT with involvement of inferior vena cava without AIVC. SD standard deviance.
*in two cases not reported in literature.
†
The difference between the AIVC groups was not significant.

AIVC in our clinic and in literature NoAIVC

41 patients with AIVC could be identified at the Univer- 168 patients with NoAIVC served here as control group.
sity of Düsseldorf, Germany. Mean age was 28 ± 11.1 years Mean age was 38.0 ± 17.3 years (min. 8, max. 84 years).
(min. 16, max. 58 years; 29 male and 12 female). Medline AIVC patients compared to NoAIVC patients were in
database research revealed additional 124 AIVC patients average 10 years younger (p < 0.0001). Male dominance
reported in 68 articles. Mean age here was 28.0 ± 11.0 years was also notable in AIVC (73.9% vs. 46.4%; p < 0.0001).
(min. 8, max. 67 years; 93 male). Age difference and male In NoAIVC, right sided DVT was seen in 16.7% vs.
dominance in both groups was not statistical significant. 25.4% in AIVC, both sided in 33.9% vs. 50.9% in AIVC.
While in our department 36.6% of AIVC patients (15/41 Atypical site of DVT was significant for AIVC (right
patients) were surgical treated with a prosthetic bypass, sided: p = 0.049; both sided: p = 0.002), whereas left sided
anticoagulation and compressing stockings [13], in litera- DVT was more often observed in NoAIVC (49.4% vs.
ture only three reported bypasses could be identified 18.3% in AIVC; p < 0.0001; Table 1).
[9,18,19]. Thrombolysis was reported as treatment of first
choice in 14 patients [20-28]. Three publications implied Other organ anomalies in AIVC and NoAIVC
no information about their treatment, whereas the major- In many reported cases, AIVC was associated with other
ity of the published cases were treated conservatively with anatomic disorders. The most recognized anomaly in
anticoagulation and compressing stockings (n = 104/124). AIVC was aplasia of the right kidney (6.0% vs. 0% in
DVT of the legs was present in all AIVC cases in our NoAIVC; p = 0.002), secondly, hypoplasia of the left kid-
clinic. A left sided DVT was evaluable in 9.8% (4/41 pa- ney in 2.7% of AIVC patients (vs. 0% in NoAIVC; p =
tients), right sided in 29.3% (12/41 patients). Both sided 0.042). Polysplenia was just as well seen in 2.0% of AIVC
affection was dominant with 61.0% in our AIVC patients patients (vs. 0% in NoAIVC; p = 0.080; n.s.). May-
(25/41 patients). There was a trend toward a higher Thurner syndrome was only observed in five NoAIVC-
prevalence of both sided DVT in our patients with AIVC patients (3.0% vs. 0% in AIVC; p = 0.026). A preduodenal
than among the AIVC patients in literature (prevalence, portal vein was only observed in two cases of AIVC and
61.0% vs. 47.6%; p = 0.10) but reached no significance. not statistical significant represented (p = 0.176; Table 2).
In the here employed literature, left sided DVT could
be diagnosed in 21.0% (n = 26/124 patients), right sided Prevalence of inherited risk factors
in 30/124 patients (24.2%). Both sided affections of DVT There was no significant difference in the prevalence of
was present in 59/124 patients (47.6%). Compared to factor-V-Leiden, prothrombin-gene-mutation, MTHFR-
our AIVC-group, DVT pattern was not significant differ- gene-mutation, and hyperhomocysteinemia between the
ent in literature. 41 AIVC patients in our clinic and the 124 AIVC pa-
Precipitating factors like immobilization, use of oral tients derived from literature research. Homozygoty for
contraceptives, etc. were more often documented in factor-V-Leiden or prothrombin-gene-mutation was not
NoAIVC (130/168) than in AIVC (77/163; 77.4% vs. detected. In AIVC, factor-V-Leiden-mutation was het-
47.3%; p < 0.0001). erozygous in 27/165 patients and in 8/168 in NoAIVC.
Sagban et al. Orphanet Journal of Rare Diseases (2015) 10:3 Page 4 of 7

Table 2 Prevalence of combined organ anomalies

Organ or system anomalies Patients with AIVC Patients with AIVC in Patients with P Value of AIVC in this
in this paper (n = 41) literature (n = 108/124; NoAIVC (n = 168) paper and in literature
16/124 NR†) (n = 149) vs. NoAIVC (n = 168)
Hypoplasia/aplasia of left kidney (%) 1/41 (2.4%) 3/108 (2.8%) 0/168 (0.0%) 0.042
Hypoplasia/aplasia of right kidney (%) 2/41 (4.9%) 7/108 (6.5%) 0/168 (0.0%) 0.002
Polysplenia (%) 1/41 (2.4%) 2/108 (1.9%) 0/168 (0.0%) 0.080
Preduodenal portal vein (%) 1/41 (2.4%) 1/108 (0.9%)‡ 0/168 (0.0%) 0.176
May-Thurner syndrome (%) 0/41 (0.0%) 0/108 (0.0%) 5/168 (3.0%) 0.026
AIVC inferior vena cava agenesia. NoAIVC iliofemoral DVT with involvement of inferior vena cava without AIVC. NR not reported.
†
In 16 of 124 reported cases in literature organ anomalies were not reported (NR); comparisons were calculated with 108 patients.
‡
Absence of portal vein.

Prothrombin-gene-mutation was heterozygous analysed MTHFR-gene-mutation were included in the same model,
in 9/165 AIVC and in 3/168 NoAIVC patients. Except of only the presence of factor-V-Leiden-gene-mutation, hyper-
2/168 NoAIVC individuals, all MTHFR-mutations were homocysteinemia and MTHFR-gene-mutation could be
heterozygous (in AIVC 25/165; in NoAIVC 4/168). None identified as independent risk factors (Table 5). Localisation
of the patients with a history of AIVC had both, factor- of DVT was also analysed. There was a statistical differ-
V-Leiden and prothrombin-gene-mutation. A combin- ence with regard to the prevalence or relative risk of both
ation of two or more thrombophilic factors in-between or right sided DVT between AIVC and NoAIVC patients
the groups showed no significant difference (Table 3). (p = 0.049 resp. p = 0.002). Nevertheless, left sided DVT
A strong statistic trend was present for lupus anticoagu- was predominantly associated with NoAIVC than with
lant, detected in 6/165 AIVC patients vs. 1/168 in AIVC (prevalence, 18.2% among AIVC vs. 49.4% in
NoAIVC (3.6% vs. 0.6%; p = 0.053), but reached no signifi- NoAIVC; p < 0.0001). Aplasia of the right and hypoplasia
cance (Table 3). Significant difference could be calculated of the left kidney were statistical significant in multivariate
for prevalence of factor-V-Leiden and hyperhomocysteine- analyses for AIVC (Table 5).
mia between AIVC and NoAIVC patients (prevalence of
factor-V-Leiden, 16.4% vs. 4.8%; p = 0.001; prevalence of Discussion
hyperhomocysteinemia, 12.1% vs. 3.0%; p < 0.0001; Tables 3 The factors that predispose to venous thrombosis were ini-
and 4). The prevalence of prothrombin-gene-mutation tially described by Virchow in 1856 [29]. Individuals with
among the AIVC patients compared with NoAIVC pa- DVT usually have a predisposing risk factor, such as a gen-
tients was not significant (prevalence, 5.5% vs. 1.2%; p = etic abnormality causing hypercoagulability, pregnancy, etc.
0.171; Table 4). By contrast, the MTHFR-gene-mutation AIVC should also be considered as a potential associated
was predominantly associated with AIVC (prevalence, anatomic abnormality, lacking of a main venous conduit,
12.7% vs. 3.6% in NoAIVC patients; p = 0.005; Table 4). which influences blood flow in the venous system [11]. Our
In a logistic-regression analysis in which adjustments were results showed that precipitating factors like immobilization
made for age and gender (Table 5) and in which hyperho- were less often an activator for DVT in AIVC patients.
mocysteinemia, presence of factor-V-Leiden- polymorphism, Younger male with atypical DVT (right or both sided
the prothrombin-gene-mutation, lupus anticoagulant and DVT) are more often affected by AIVC presenting with

Table 3 Prevalence of hereditary coagulation defects

Patients with AIVC in Patients with AIVC Patients with P Value of AIVC in
this paper (n = 41) in literature research NoAIVC (n = 168) this paper and in
(n = 124) literature (n = 165)
vs. NoAIVC (n = 168)
Factor V Leiden (%) 11/41 (26.8%) 16/124 (12.9%) 8/168 (4.8%)† 0.001
Prothrombin gene mutation (%) 1/41 (2.4%) 8/124 (6.5%) 2/168 (1.2%) 0.146
MTHFR gene mutation (%) 15/41 (36.6%) 6/124 (4.8%) 6/168 (3.6%)‡ 0.005
Homocysteinemia (%) 15/41 (36.6%) 5/124 (4.0%) 5/168 (3.0%) <0.0001
Lupus anticoagulant (%) 2/41 (4.9%) 4/124 (3.2%) 1/168 (0.6%) 0.053
Two thrombophilic factors positive (%) 9/41 (22.0%) 7/124 (5.6%) 4/168 (2.4%) 0.075
Three and more thrombophilic factors positive (%) 8/41 (19.5%) 1/124 (0.8%) 3/168 (1.8%) 0.086
AIVC inferior vena cava agenesia. NoAIVC iliofemoral DVT with involvement of inferior vena cava without AIVC.
†
One case homozygous for factor-V-Leiden-gene-mutation. ‡Two cases homozygous for MTHFR-gene-mutation.
Sagban et al. Orphanet Journal of Rare Diseases (2015) 10:3 Page 5 of 7

Table 4 Prevalence of hereditary coagulation defects in prevalence of factor-V-Leiden-mutation in NoAIVC is simi-

patients with aivc and in patients with noaivc lar to the normal population. In addition, homozygosis for
Characteristic Prevalence Prevalence P value† a common cytosine-to-thymidine mutation in the gene en-
among patients among patients (NoAIVC coding MTHFR, C677T, is associated with high plasma
with NoAIVC with AIVC vs. AIVC)
(n = 168) (n = 165)* homocysteine concentrations and venous thrombosis.
Percent
There are conflicting results regarding the role of a homo-
zygous 677TT MTHFR genotype as a risk factor for venous
Gender (male) 46.4 73.9 <0.0001
thromboembolism. Homozygosis for this polymorphism
Factor V Leiden 4.8 16.4 0.001
was associated with high plasma homocysteine concentra-
MTHFR gene 3.6 12.7 0.005 tions and venous thrombosis in some studies [17,30,31] but
mutation
not in others [32]. In our study, the 677TT MTHFR-
Homocysteinemia 3.0 12.1 <0.0001 genotype (homozygote) was only present in two cases of
Prothrombin gene 1.2 5.5 0.171 NoAIVC. All other MTHFR-positive testing’s were hetero-
mutation
zygous with the 677CT MTHFR genotype combined with
Lupus anticoagulant 0.6 3.6 0.053 hyperhomocysteinemia. In our opinion this genotype in
AIVC inferior vena cava agenesia. NoAIVC iliofemoral DVT with involvement of combination with metabolic anomaly has an influence on
inferior vena cava without AIVC.
*AIVC in this paper and in literature (n = 165). the risk of DVT; the prevalence of the 677CT MTHFR-
†
P values are for the univariate comparison between patients with NoAIVC and genotype in AIVC was higher than in the group of
those with AIVC.
NoAIVC. Like factor-V-Leiden, MTHFR-gene-mutation
was predominant in AIVC, mostly in combination with hy-
perhomocysteinemia. The G20210A prothrombin-gene-
signs and symptoms of an acute DVT without previous evi- mutation or the presence of lupus anticoagulant seems to
dence of risk factors. Thrombophilia plays also an import- play no role here.
ant role in AIVC patients although other authors published We confirmed the importance of factor-V-Leiden as a risk
contrary findings based on case reports or small groups factor for venous thromboembolism in AIVC. In addition, we
[11,12]. While factor-V-Leiden-gene-mutation can be found found that factor-V-Leiden is a risk factor that is independent
in a normal population roughly in 5%, a similar prevalence of other known determinants of thrombosis; specifically, it
could be shown in our control group (NoAIVC patients is independent of the G20210A prothrombin or MTHFR-
4.8%). In AIVC the prevalence is more than three times gene-mutation, whereas MTHFR-mutation in combination
higher (16.4%). In our opinion this is not debted to a selec- with hyperhomocysteinemia is independently from factor-
tion towards AIVC with DVT, because the here presented V-Leiden-mutation also a risk factor for DVT in AIVC.

Table 5 Relative risk of aivc associated with hereditary coagulation defects and anatomic malformations
Risk factors in AIVC Univariate analysis Multivariate analysis†
Relative risk (95% CI) P value Relative risk (95% CI) P value
Age (years) 27.98 (35.65-41.17) <0.0001** 22.31 (32.62-39.89) <0.0001
Gender (male) 3.274 (2.064-5.192) <0.0001* 5.789 (3.131-10.701) <0.0001
Factor V Leiden 3.913 (1.721-8.895) 0.001* 4.890 (1.647-7.517) <0.0001
MTHFR gene mutation 3.354 (1.385-8.123) 0.005* 2.132 (1.016-7.072) 0.005
Homocysteinemia 6.641 (2.243-19.659) <0.0001* 6.916 (2.544-19.814) <0.0001
Aplasia of right kidney 58.4†† 0.002* 64.8†† 0.002
††
Hypoplasia of left kidney 36.1 0.059* 46.7†† 0.042
Right sided DVT 1.707 (0.999-2.918) 0.049* 1.663 (1.025-3.267) 0.049
Both sided DVT 2.019 (1.298-3.141) 0.002* 2.003 (1.305-3.349) 0.002
DVT deep vein thrombosis. AIVC inferior vena cava agenesia. MTHFR 5,10-methylenetetrahydrofolate reductase.
*Univariate analyses were performed with the use of the chi-square test. Multivariate analyses were performed with the use of the stepwise logistic-regression pro-
cedure. CI denotes confidence interval.
**Univariate analyses were performed with the use of the T-test. Multivariate analyses were performed with the use of the stepwise logistic-regression procedure.
CI denotes confidence interval.
†
All stepwise logistic-regression analyses were performed with the following variables: presence of factor-V-Leiden; presence of prothrombin-gene-mutation;
presence of the MTHFR-gene-mutation; hyperhomocysteinemia; presence of Lupus anticoagulant; aplasia of the right kidney; hypoplasia of the left kidney;
polysplenia; right sided DVT; both sided DVT; age; gender; the P values indicate the significance of each risk factor independently.
††
Because no patient with NoAIVC had this combined defect, the estimated relative risk was calculated on the basis of the probability of a combined defect in
these patients. For this reason, no confidence interval is given.
Sagban et al. Orphanet Journal of Rare Diseases (2015) 10:3 Page 6 of 7

The developmental cause of AIVC is debated: it’s maybe Abbreviations

caused by embryonic dysgenesis or by an intrauterine in- AIVC: Inferior vena cava agenesis; DVT: Deep vein thrombosis; IVC: Inferior
vena cava; MTHFR: 5,10-methylenetetrahydrofolate reductase;
sult during the perinatal period [6,33]. AIVC have been NoAIVC: Iliofemoral DVT with involvement of inferior vena cava without AIVC;
associated with other congenital anomalies, like renal hy- PCV: Posterior cardinal veins; SV: Subcardinal veins.
poplasia/agenesis. This anatomic anomaly for the right
kidney was found in our research in 6% of AIVC, but not Competing interests
The authors declare that they have no competing interests.
in NoAIVC patients. The right renal hypoplasia is suggest-
ive of a defect in the formation of the IVC in these seg-
Authors’ contributions
ments, since the embryologic right SV does not drain Conception and design: TAS, MD. Analysis and interpretation: TAS, MD, HS.
the mesonephros [34]. A hypoplastic left kidney was also Data collection: TAS, RS. Writing the article: TAS. Critical revision of the article:
found here in AIVC (2.7%). However, it is not clear HS, MUW, KG, AO. Final approval of the article: TAS, RS. Statistical analysis:
TAS, MUW. All authors read and approved the final manuscript.
whether this represents a true congenital malformation or
atrophy of a previously normal kidney due to long- Author details
1
standing poor perfusion or thrombosis secondary to vascu- Department of Vascular and Endovascular Surgery, Heinrich Heine University
Düsseldorf, Moorenstraße.5, 40225 Düsseldorf, Germany. 2Department for
lar malformation. The question of hereditary is not clearly Hemostasis and Transfusion Medicine, Heinrich Heine University Düsseldorf,
answerable, but two cases in our collective direct to this Moorenstraße.5, 40225 Düsseldorf, Germany.
hypothesis. In one case, the son of a patient with AIVC
Received: 2 November 2014 Accepted: 30 December 2014
was diagnosed with DVT bilateral. Computed tomography
showed here also an AIVC in the same segment like his
father. The second remarkable case was the asymptomatic References
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cannot be representative, but both give rise to a hereditary Demonstration by NMR imaging. J Comput Assit Tomogr. 1984;8:774–6.
4. Shah NL, Shanley CJ, Prince MR, Wakefield WT. Deep venous thrombosis
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