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Iliofemoral Thrombosis Secondary to Iliac Compression Syndrome in Double Inferior Vena Cava
Duplication of the inferior vena cava (D-
IVC) is an uncommon congenital anomaly
with an incidence of 0.3% to 3.0% in the
population.1 May-Thurner syndrome (MTS), which
is also known as iliac compression syndrome, is a
rare condition that results in compression of the left
common iliac vein by the right common iliac artery,
crossing anterior to the vein.2 This narrowing of the
left common iliac vein is a significant risk for devel-
oping deep venous thrombosis (DVT), especially of
the left lower limb. We describe successful treatment
of a very rare combination of pathology of D-IVC,
heterozygous mutation of factor V Leiden and MTS
in a case of extensive left lower limb DVT.
CASE PRESENTATIONA 30-year-old female presented with a 1-week history
of left lower extremity swelling, pain, and erythema.
The pain was continuous and dull in nature with pain
score of 9/10. The pain was aggravated by movement
and slightly relieved by rest and cold compression.
She did not experience any shortness of breath, chest
pain, or fever. On physical examination, she was he-
modynamically stable and was not in distress. Her left
lower extremity was warm, erythematous, swollen,
and tender. Her risk factors for DVT included a signifi-
cant family history of recurrent DVT and use of oral
contraceptive pills for 1 year. There was no history of
trauma or recent long-distance travel. Her complete
See-Wei Low, MD1; Kapildeo Lotun, MD2; Kwan Seung Lee, MD2
From the 1University of Arizona and 2Section of Vascular Medicine, Sarver Heart Center, University of Arizona, Tucson, Arizona.
ABSTRACT: Duplication of the inferior vena cava (D-IVC) joined by a pelvic connector is a rare
congenital anomaly. May-Thurner syndrome (MTS), also known as iliac compression syndrome, is
caused by a localized stricture at the point where the left common iliac vein is crossed by the right
common iliac artery resulting in left common femoral deep venous thrombosis. The combination
pathology of D-IVC and MTS is rarely described. We describe a case of a patient with iliofemoral
thrombosis precipitated by MTS with D-IVC, heterozygous mutation of factor V Leiden, and describe
our successful treatment approach.
VASCULAR DISEASE MANAGEMENT 2015;12(4):E54-E61 Key words: deep venous thrombosis, thrombectomy, interventional cardiology
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blood count and basic metabolic profile were within
normal limits. However, her prothrombin and activated
prothromboplastin times were 14.8 seconds and 36.9
seconds respectively, with an INR of 1.2. Her fibrinogen
level was significantly elevated at 856 mg/dL.
Initial imaging with lower extremity Doppler re-
vealed a large, occlusive DVT extending from the left
tibial vein to the left common iliac vein. There was
also evidence of D-IVC on the initial Doppler imaging
study. A computed tomography (CT) scan confirmed
D-IVC and a filling defect in the left common iliac vein
and no pulmonary embolism. Screening for throm-
bophilia revealed a heterozygous mutation for factor
V Leiden. She was started on anticoagulation therapy
with intravenous heparin but continued to have sig-
nificant pain and swelling of the leg with preservation
of arterial pulses.
Considering the thrombus burden and increased
risk of post-thrombotic syndrome, a decision to per-
form venous thrombectomy was made. Pelvic iliac
venography was performed, which showed the D-IVC
anomaly. A connection was noted between the right
common iliac vein and the left inferior vena cava (IVC).
A decision was made at this point to deploy IVC filters
into both right and duplicate left IVC in the infrarenal
positions, as there was dynamic flow between the left
IVC into the right IVC at both iliac and suprarenal
levels. There was also left thoracic azygos flow from
the left IVC. Two OptEase filters (Cordis Corpora-
tion) were deployed, with one in the left IVC and
another in the right IVC in the infrarenal position.
Venography performed after the deployment of filters
confirmed the appropriate deployment level. Access
was then obtained at the left posterior popliteal vein
Figure 1. Computed tomography of pelvis (axial plane) showed compression of the left common iliac vein between the left common iliac artery and lumbar vertebrae.
Figure 2. Venography showed duplicated inferior vena cava anomaly with communication above pelvic level and confluence at L1, suprarenal position.
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and a 5 Fr Cragg-McNamara thrombolytic infusion
catheter (Covidien Inc) was advanced into the left
common iliac position. Unfractionated heparin fol-
lowed by tissue plasminogen activator (tPA) infusion
(2mg/hour for 6 hours followed by 1mg/hour) was
administered via the Cragg-McNamara catheter in an
ICU setting for 24 hours.
She was then brought back to the catheterization
laboratory the next day for venography and her re-
sidual thrombus burden was assessed. Venography was
performed with demarcation of the limits of residual
thrombus and showed some improvement in blood
flow. An 8 Fr, 30 cm therapeutic length Trellis device
(Covidien Inc) was then advanced with the distal port
in the left common iliac vein and proximal port in
the left deep femoral vein. Trellis thrombectomy was
performed for 10 minutes with a total of 10 mg of tPA
administered. Thrombus material was aspirated. The
therapy was once again repeated, from the left deep
femoral vein to the left popliteal vein. Final images
were obtained which showed marked improvement in
blood flow. Her left lower extremity swelling along
with symptoms of DVT significantly improved within
24 hours and she was discharged home after being
started on warfarin oral anticoagulation therapy.
Three weeks later, she was brought back to the cardiac
Figure 4. Venography post catheter-directed thrombolysis showed residual thrombus in the left external iliac vein in prone position.
Figure 3. Venography showing bilateral OptEase infrarenal inferior vena cava filter placement.
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catheterization laboratory for venography and for pos-
sible removal of the IVC filters. Venography revealed
re-occlusion of the left iliac system secondary to throm-
bus. A decision was made to proceed with balloon
angioplasty of the left iliac system. After multiple bal-
loon dilatations, intravascular ultrasound (IVUS) was
performed, which revealed compression of the left iliac
vein by the right iliac artery. Two self-expanding 12
mm x 60 mm and 10 mm x 60 mm Protégé GPS self-
expanding stents (Covidien Inc) were then deployed
into the left common iliac venous system. There was
excellent flow through the stents via both wide-open
bilateral IVC and pelvic connectors. We chose not to
remove the IVC filters at this juncture because of the
presence of extensive clot burden. The pelvic connec-
tor between left IVC and right iliac vein was noted
to be behind the aortoiliac bifurcation and was com-
pressed. IVUS of the pelvic connector was performed,
which confirmed the suspicion of compression. After
performing balloon dilatation of the compression, a 12
mm x 40 mm Protégé GPS self-expanding stent was
deployed which subsequently showed excellent flow
in the connector and in bilateral IVC.
Two weeks later, she underwent venous Doppler,
Figure 5. Trellis mechanical thrombectomy of left iliofemoral venous system.
Figure 6. Venography post Trellis thrombectomy showed restoration of flow.
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which revealed normal flow in bilateral IVC, the iliac
veins and bilateral lower extremity. She also under-
went repeat venography, which confirmed patent stents
with good blood flow through the left iliac system
and no recurrence of thrombus formation. Both IVC
filters were removed. She was continued on warfarin
anticoagulation and was followed up in an outpatient
clinic after 1 month without recurrence of symptoms.
One year later, she became pregnant and carried the
pregnancy with no complications under the care of he-
matology, obstetrics and gynecology, and cardiology.
During her pregnancy, she was treated with enoxapa-
rin sodium injections daily and this was later substi-
tuted with heparin in the 36th week of her pregnancy.
She had an uneventful pregnancy and delivery with
no recurrence of DVT. At 2 years, she has remained
asymptomatic with no recurrence.
DISCUSSIONWe highlight a case of a patient who presented with
DVT in her left lower leg, who was subsequently found
to have D-IVC, MTS, and a heterozygous mutation
of factor V Leiden. A D-IVC arises from persistence
of both right and left supracardinal veins.3 In D-IVC,
a left-sided IVC ascends to the level of the renal veins
to join the normal right IVC along the right side of
the spine through a vascular structure, in this case
through a pelvic connector that may pass either ante-
rior or posterior to the aorta at the level of the renal
vein.4 D-IVC is an uncommon congenital anomaly
with an incidence of 0.3% to 3.0% in the population.1
It can cause major venous hemorrhage during vascu-
lar surgeries, thromboembolic complications, tumor
extension, and diagnostic error.5 D-IVC is usually
Figure 7. Venography showing re-occlusion of left iliac system at 3-week follow up for filter removal.
Figure 8. Venography post angioplasty and stenting of left iliac venous system with Protégé GPS self-expanding stents.
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discovered incidentally during a radiologic work-up
via CT, MRI,3 or during abdominal surgeries.6 Some
studies have identified D-IVC to be a risk factor for
DVT in patients younger than 30 years of age with
DVT7,8 by promoting venous stasis.9
It is clinically important to recognize the presence
of D-IVC in the therapy of DVT with IVC filters
because connectors are often present between both,
necessitating the accurate placement of IVC filters to
prevent pulmonary embolism. The typical site of IVC
filter placement is at the level of the L3 vertebral body,
caudal to the renal veins. However, in some cases, su-
prarenal (T12-L1) placement of the IVC filters is indi-
cated, such as in the presence of thrombus in the renal
veins, with poor placement of infrarenal filter, or in
pregnant patients.10,11 Indications for use of IVC filters
are protection against recurrent DVT when there are
contraindications to anticoagulation therapy or failure
of anticoagulant therapy.12 The use of retrievable IVC
filters as compared to permanent IVC filters lowers the
risk of filter thrombosis and occlusion, filter migration,
filter fracture, and vena caval thrombosis.13
May-Thurner syndrome is also known as iliac vein
compression syndrome and is characterized by the oc-
currence of left common iliac obstruction secondary to
compression of the left iliac vein by the right common
iliac artery. May-Thurner syndrome is seen in 22%
of autopsies. However, this syndrome is often under-
recognized as a cause of left iliofemoral DVT.2 Failure
to diagnose MTS increases the patient’s risk of recur-
rent DVT and post-thrombotic syndrome because sole
treatment using anticoagulation has been proven inef-
fective in patients with underlying iliofemoral venous
obstruction.14,15 In young patients who have DVT with
underlying MTS, it is recommended the patient be
treated with thrombolysis or mechanical thrombec-
tomy combined with angioplasty and stenting of the
Figure 9. Venography showing stenosis at confluence of stented left common iliac and pelvic vein connector.
Figure 10. Final results post pelvic connector stenting and dual inferior vena cava filter retrieval demonstrating patency.
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iliac vein stenosis in order to reduce the risk of post-
thrombotic syndrome.16 The additional use of stenting
or catheter-guided thrombolysis and angioplasty is to
achieve long-term patency of the iliac vein, to reduce
the recurrence of thrombosis.17
In addition to her risk factors, the presence of het-
erozygosity for factor V Leiden mutation along with
the intake of oral contraceptive pills contributed to her
thrombotic risk. Patients with factor V Leiden muta-
tions have an increased risk of DVT approximately 7
times that of noncarriers. This risk is further increased
by a factor of 35 when taking oral contraceptive pills.18
This is a rare case that illustrates successful treatment
with catheter-directed thrombolytic therapy, adjunc-
tive mechanical thrombectomy, angioplasty, and stent-
ing as combination therapy for extensive iliofemoral
thrombosis secondary to MTS in our patient with
D-IVC and heterozygous mutation of factor V Leiden
and highlights the special considerations necessary in
successful care of this combination. n
Editor’s note: Disclosure: The authors have completed
and returned the ICMJE Form for Disclosure of Potential
Conflicts of Interest The authors report no disclosures related
to the content herein.
Manuscript received November 12, 2014; manuscript ac-
cepted February 20, 2015.
Address for correspondence: Dr. Kwan S Lee, 2800 E Ajo
Way, Tucson, AZ 85713, United States. Email: klee@
shc.arizona.edu
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