A PAINFUL SWOLLEN LEG

Resident: Daniel K. Powell MD

Attending: Joseph Shams, MD
Program: Mount Sinai Beth Israel , New York, NY

Originally Posted: December 15, 2014
CHIEF COMPLAINT & HPI
Chief Complaint and/or reason for consultation
28 year old female presents with severe right leg pain.
History of Present Illness
A 28 year-old woman presented to the emergency department in October 2010 with
new right leg swelling and severe leg pain, acutely worsening over one day, requiring
bed rest after one hour of walking, and right flank pain. There was no history of
trauma.
RELEVANT HISTORY
Past Medical and Surgical History
On Coumadin for deep venous thrombosis (DVT)
Premature birth at 5 months
Two spontaneous abortions (2010, and 2007) and infertility, dating back approximately 5
years (normal HSG)
Allergies
NKDA

WORKUP
Grey-scale and Doppler ultrasound showed extensive right popliteal to
common femoral vein DVT. The patient was switched to Lovenox, but
returned 19 days later with chest pain and shortness of breath.

Initially suspicious for underlying congenital or autoimmune causes of
hypercoagulability. Hypercoagulability workup was performed, which was
negative.




DIAGNOSTIC WORKUP
Non-Invasive Imaging
Image A: CT venogram showed
iliofemoral thrombosis (yellow arrows)
and apparent absence of the inferior
vena cava (IVC).

Image B: 3D reformatted image from CT
venogram was performed for further
evaulation, which showed right
common femoral vein drainage into an
ovarian and other collateral veins
without a well-formed infra-renal IVC
(black arrow), a diminutive draining
collateral (white arrow), a dilated
abdominal-pelvic paraspinal azygos
feeder (bottom arrowhead)
communicating with the hepatic IVC
and azygos (top arrowhead).

A
B
DIAGNOSIS
Absent or chronically thrombosed IVC
INITIAL TREATMENT
Catheter Venogram:
The patient was referred for thrombolysis.
Catheter venogram confirmed persistent
clots in the iliofemoral veins (yellow arrows),
despite anti-coagulation. The bilateral iliac
veins and the IVC were occluded and an
atretic IVC or draining collateral was seen
(white arrow).
The superior aspect of the right common iliac
vein could not be crossed with a wire despite
multiple attempts.
Mechanical saline pump rheolytic
thrombectomy and multi-side hole catheter
directed tissue plasminogen activator (tPa)
thrombolysis was performed with clot
aspiration overnight, two days in a row, with
three sessions of thrombectomy, due to
residual thrombus on the second morning.


INTERVENTION
Two weeks later the patient presented with increasing leg pain and recurrent
iliofemoral DVT on ultrasound with femoral vein occlusion. The patient was
presented at vascular conference and endovascular IVC reconstruction was
presented to the patient , including a discussion of the risk of life-threatening
hemorrhage and chronic back pain from presence of the stents. The patient
was eager for resolution of her pain, which limited her mobility. Risk of
retroperitoneal hemorrhage was considered minimal from the low-flow in
these atretic veins.
Two weeks later the patient underwent planning angiography.
Simultaneous inferior-approach right common iliac venogram and separate
superior-approach cavogram.
AP projections: Chronic thrombus was seen in the common right iliac vein.
Multiple dilated collateral vessels were again demonstrated from below,
including a markedly dilated right ovarian vein, draining in an arc over the
liver to the supra-hepatic IVC, and ascending lumbar veins. The inferior
catheter (yellow arrow) was advanced superiorly into a collateral vessel
running anteromedial to the ascending lumbar veins, possibly a parallel
retroperitoneal vein or ureteric vein (white arrow). Upon injection from
above, a dilated azygos and accessory hemiazygos with a large paraspinal
abdominopelvic feeding vessel (arrow head) were seen which appeared to
drain into the relatively preserved supra-renal IVC and the azygos vein.
Lateral and oblique projections: performed to evaluate the proximity of the
distal right common iliac collateral (white arrow) and the adjacent dilated
paraspinal azygos tributary (arrow head), draining into the suprarenal IVC.
Proximity of these vessels was demonstrated and recanalization was
planned.

AP AP Lateral Oblique Oblique
Top row of images:
A subsequent procedure was performed to create a drainage pathway via controlled
venous perforation from the right common iliac vein collateral (white arrow) into the
azygos system and IVC. Access was obtained through the right IJ and profunda
femoris, due to femoral vein occlusion.

A superior-approach 4 Fr angledtip diagnostic catheter (arrow heads) roadmap
angiography of the IVC provided a target. An inferior-approach catheter (yellow
arrows) was advanced over a guide wire through the common iliac collateral until
resistance was met superiorly. A stiff straight-tip hydrophilic 0.035 guide wire was
then advanced/perforated with minimal resistance (through the walls of the
adjacent vessels) into the renal IVC. The catheter was slowly advanced and intra-
luminal position was confirmed with contrast injection.

Bottom row of images:
Balloon dilatation of the tract was performed followed by placement of multiple
14mm x 6cm overlapping nitinol Smart stents from the external iliac vein to the
renal IVC, sequentially dilated with 8mm, 10mm and 12mm balloons. 12mm x 6cm
overlapping nitinol Smart stents were placed across external iliac vein to the mid-
common femoral vein. Finally a 10mm x 4cm Smart stent was deployed from the
profunda femoris, partially overlapping the common femoral stent do to poor inflow
into the common femoral. The stents were balloon dilated and good angiographic
results were demonstrated.

INTERVENTION
AP
Inferior
injection
Inferior
& superior
injections
Inferior
Injection
opacifying
above
After
perforation
into renal IVC
Oblique
CLINICAL FOLLOW UP
Images A and B:
At three weeks, improved right flank pain and resolution of right leg pain and
swelling. 2 weeks later the patient returned to the emergency department with
pelvic pain. Cavogram demonstrated minimal neo-intimal hyperplasia and
slight separation of the stents at the common femoral-profunda junction,
without pseudoaneurysm. Balloon angioplasty was performed along the length
of the stents (12mm x 4cm balloon in the external iliac and proximal common
iliac and 14mm x 4cm balloon in the IVC). At two week follow-up, complete
resolution of pain.
Over the next year, physical exam, CTV and US were unremarkable on regular
follow-up evaluations. She complained of mild vague lower extremity pain.
Coumadin treatment was ceased after one year and Plavix was initiated.
Images C and D:
Two years post-procedure, the patient was admitted to the hospital with leg
pain. Cavogram demonstrated high-grade stenosis at the junction of the
inferior and middle thirds of the stent tract (arrow), at the level of the venous
anastomosis / recanalization. Angioplasty with 12, 14 and 16mm balloons was
performed with good technical results, symptomatic relief and unremarkable
follow-up physical exam and Doppler evaluations.
Images E and F:
Three years post-procedure, the patient presented with vague right flank and
leg pain. CTV showed multifocal stent narrowing. Cavogram showed recurrent
high-grade stenosis at the same segment (arrow) and angioplasty was
performed with a 14mm balloon. Technical success and symptom relief were
achieved. 1-month follow-up CTV and 2-month follow-up cavogram showed
wide stent patency.
A B
D
E F C
Post balloon
angioplasty
Post balloon
angioplasty
Post balloon
angioplasty
SUMMARY & TEACHING POINTS
Successful nitinol Smart stent reconstruction in a young patient with absence/chronic thrombosis of the infra-renal IVC, from a distal right common iliac vein collateral into a preserved renal
IVC to treat extensive iliofemoral and popliteal thrombosis. There were no early complications of the procedure. Three secondary angioplasties were required: 1 month later with minimal
diffuse neo-intimal hyperplasia as well as at two and three years post-procedure for focal stenoses at the point of venous anastomosis /recanalization. Otherwise, the patient had no
recurrence of DVT, severe lower extremity pain or skin changes associated with chronic venous stasis. There was incidental minimal separation of the overlapping stents at the junction of
the common femoral and profunda veins at one-month follow-up without pseudoaneurysm as well as chronic vague non-specific lower extremity, pelvic and right flank pain, likely due to the
presence of the stents.
The variations of IVC absence, including atresia, agenesis, anomalous and interruption are rare (1).
Independent risk factor for DVT (1,2).
Agenesis is the predominant theory. Some authors suggest that it is the results of intra-uterine or infantile thrombosis (3)
0.15% incidence has been reported. As high as 5% in young patients with lower extremity DVT (2).
Prematurity and likely central venous catheterization were features of our case.
Extensive infantile IVC thrombosis has shown to persist in 80% of children (4).
30% of infants with IVC thrombosis may have post-thrombotic syndrome at 10 year follow-up (4).
Prior reports of sharp recanalization of the IVC for IVC hypoplasia (5) and for post transplant occlusion (6). In small studies, recanalization and self-expanding Wallstent repair of chronic IVC
obstruction has been described with 78% 1-year primary patency (7) and 87% 1-2 year patency (8).
IVC stent revascularization has 40% primary and 86% secondary patencies for incorporated occluded IVC filters (9) and has successfully relieved debilitating LE edema in patients with
metastatic hepatic IVC obstruction (10).
Our patients infertility was never explained. No correlation between pelvic congestion and infertility although there are two reports of successful pregnancy after ovarian varicocele coiling
(11).
SUMMARY & TEACHING POINTS
Regarding indications for DVT thrombolysis, the exact population is not defined. According to the American College of Chest Physicians, the indications are:
>1 year life expectancy
Good functional status
Extensive iliofemoral thrombosis
Presenting soon after symptoms, up to 14 days
Angioplasty plus stenting in the case of reversible causes
Mechanical and chemical thrombectomy/thrombolysis
50% of patients with DVT will develop post thrombotic syndrome (PTS) and 50% of those will be severe, 10% will ulcerate. The degree of risk reduction with thrombolysis
is not defined but may be up to 50% (despite the significant risk of bleeding complications). The ideal timing is also not defined, with 10 days suggested as the optimal
time after onset, although the ATTRACT and CaVenT trials used 14 and 21 days as cutoffs.
Most trials are focusing on the use of rTPA.
Stents may be useful in the cases of abnormal anatomy, such as May-Thurner or in the case of obstructing tumors with 1-year patency from 80-100%.
Villalta Scale clinical scoring system of signs and symptoms (either binary, yes/no; categorical, none, mild, moderate, severe; or continuous, 0-33), which has been
validated as a reliable measure of PTS.
Post thrombotic syndrome (PTS) signs and symptoms:
Signs: peri-malleolar (classically medial) telangiectasia, brown pigmentation, venous eczema, varicose veins, skin thickening lipodermatosclerosis, ulcers, venous
hypertension, venous reflux, obstruction, and valve dysfunction.
Symptoms: pain, swelling, heaviness, and claudication worsened by walking and standing.
Complications: DVT recurrence, PE, phlegmasia cerulea dolens (capillary thromboses and cyanosis), venous infarction, compartment syndrome, and limb loss (12,13).

QUESTION
Which of the following collateral pathways of chronic SVC/IVC obstruction is the
least common?
A. Internal and external mammary pathway
B. Azygos-hemiazygos pathway
C. Lateral thoracic pathway
D. Vertebral pathway
E. Cavoportal collateral pathway


CORRECT!
Which of the following collateral pathways of chronic SVC/IVC obstruction is the least
common?
A. Internal and external mammary pathway This is a common collateral pathway and includes
the internal mammary, superior epigastric, and inferior epigastric veins and superficial veins
of the thorax.
B. Azygos-hemiazygos pathway This pathway predominates unless it is poorly developed or
obstructed at its confluence with SVC
C. Lateral thoracic pathway This is a common collateral pathway and includes the lateral
thoracic, thoracoepigastric, superficial circumflex, long saphenous, and femoral veins to
collaterize to the IVC
D. Vertebral pathway This s a common pathway and includes the innominate, vertebral,
intercostal, lumbar, and sacral veins to collaterize to the azygos and internal mammary
pathways
E. Cavoportal collateral pathway although an important collateral pathway, it is less common
than the others mentioned above. Flow is directed from the vena cava to the portal vein.


Dahan H, Arriv L, Monnier-Cholley L, Le Hir P, Zins M, Tubiana JM. Cavoportal collateral pathways in vena cava obstruction: imaging features. AJR Am J Roentgenol. 1998 Nov;171(5):1405-11.
SORRY, THATS INCORRECT.
Which of the following collateral pathways of chronic SVC/IVC obstruction is the least
common?
A. Internal and external mammary pathway This is a common collateral pathway and includes
the internal mammary, superior epigastric, and inferior epigastric veins and superficial veins
of the thorax.
B. Azygos-hemiazygos pathway This pathway predominates unless it is poorly developed or
obstructed at its confluence with SVC
C. Lateral thoracic pathway This is a common collateral pathway and includes the lateral
thoracic, thoracoepigastric, superficial circumflex, long saphenous, and femoral veins to
collaterize to the IVC
D. Vertebral pathway This s a common pathway and includes the innominate, vertebral,
intercostal, lumbar, and sacral veins to collaterize to the azygos and internal mammary
pathways
E. Cavoportal collateral pathway although an important collateral pathway, it is less common
than the others mentioned above. Flow is directed from the vena cava to the portal vein.


Dahan H, Arriv L, Monnier-Cholley L, Le Hir P, Zins M, Tubiana JM. Cavoportal collateral pathways in vena cava obstruction: imaging features. AJR Am J Roentgenol. 1998 Nov;171(5):1405-11.
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