Iliofemoral Thrombosis Secondary to Iliac Compression ... · rent DVT and post-thrombotic syndrome because sole treatment using anticoagulation has been proven inef - fective in patients

CASE REPORT AND REVIEW

Vascular Disease Management® April 2015 54

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

Copyri

ght H

MP Com

munica

tions



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.

Copyri

ght H

MP Com

munica

tions



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.

Copyri

ght H

MP Com

munica

tions



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.

Copyri

ght H

MP Com

munica

tions



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.

Copyri

ght H

MP Com

munica

tions



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.

Copyri

ght H

MP Com

munica

tions



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

REFERENCES1. Aljabri B, MacDonald PS, Satin R, Stein LS, Obrand

DI, Steinmetz OK. Incidence of major venous and renal anomalies relevant to aortoiliac surgery as dem-onstrated by computed tomography. Ann Vasc Surg.

2001;15(6):615-618.2. May R, Thurner J. The cause of predominately sinis-

tral occurence of thrombosis of the pelvic veins. Angi-ology. 1957;8(5):419-427.

3. Bass JE, Redwine MD, Kramer LA, Huynh PT, Har-ris JH Jr. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 2000;20(3):639-652.

4. Milani C, Constantinou M, Berz D, Butera JN, Col-vin GA. Left sided inferior vena cava duplication and venous thromboembolism: case report and review of literature. J Hematol Oncol. 2008;1:24.

5. Mathews R, Smith PA, Fishman EK, Marshall FF. Anomalies of the inferior vena cava and renal veins: embryologic and surgical considerations. Urology. 1999;53(5):873-880.

6. Balridge ED Jr, Canos AJ. Venous anoma-lies encountered in aortoiliac surgery. Arch Surg. 1987;122(10):1184-1188.

7. Chee YL, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk factor for deep ve-nous thrombosis in the young. Br J Haematol. 2001;114(4):878-880.

8. Gayer G, Luboshitz J, Hertz M, et al. Congenital anomalies of the inferior vena cava revealed on CT in patients with deep vein thrombosis. AJR Am J Roent-genol. 2003;180(3):729-732.

9. Obernosterer A, Aschauer M, Schnedl W, Lipp RW. Anomalies of the inferior vena cava in pa-tients with iliac venous thrombosis. Ann Intern Med. 2002;136(1):37-41.

10. Marcy PY, Magne N, Frenay M, Bruneton JN. Re-nal failure secondary to thrombotic complications of suprarenal inferior vena cava filter in cancer patients. Cardiovasc Intervent Radiol. 2001;24(4):257-259.

11. Matchett WJ, Jones MP, McFarland DR, Ferris EJ. Suprarenal vena caval filter placement: follow-up of four filter types in 22 patients. J Vasc Interv Radiol. 1998;9(4):588-593.

12. Becker DM, Philbrick JT, Selby JB. Inferior vena cava filters. Indications, safety, effectiveness. Arch In-tern Med. 1992;152(10):1985-1994.

13. Hann CL, Streiff MB. The role of vena caval filters in the management of venous thromboembolism. Blood Rev. 2005;19(4):179-202.

14. Krupski WC, Bass A, Dilley RB, Bernstein EF,

Copyri

ght H

MP Com

munica

tions



Otis SM. Propagation of deep venous thrombosis identified by duplex ultrasonography. J Vasc Surg. 1990;12(4):467-474; discussion 74-5.

15. Cockett FB, Thomas ML, Negus D. Iliac vein compression.--Its relation to iliofemoral thrombo-sis and the post-thrombotic syndrome. Br Med J. 1967;2(5543):14-19.

16. AbuRahma AF, Perkins SE, Wulu JT, Ng HK. Ilio-femoral deep vein thrombosis: conventional therapy versus lysis and percutaneous transluminal angioplasty

and stenting. Ann Surg. 2001;233(6):752-760.17. Kim JY, Choi D, Guk Ko Y, Park S, Jang Y, Lee do

Y. Percutaneous treatment of deep vein thrombosis in May-Thurner syndrome. Cardiovasc Intervent Radiol. 2006;29(4):571-575.

18. Koster T, Rosendaal FR, de Ronde H, Briët E, Van-denbroucke JP, Bertina RM. Venous thrombosis due to poor anticoagulant response to activated protein C-Leiden Thrombophilia Study. Lancet. 1993;342(8886-8887):1503-1506.

Copyri

ght H

MP Com

munica

tions

Documents

Iliofemoral Thrombosis Secondary to Iliac Compression ... · rent DVT and post-thrombotic syndrome because sole treatment using anticoagulation has been proven inef - fective in patients