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 angled–tip 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 patient’s 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, Arrivé 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, THAT’S 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, Arrivé 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.

REFERENCES

1. Takehara N, Hasebe N, Enomoto S, Takeuchi T, Takahashi F, Ota T, Kawamura Y, Kikuchi K. Multiple and recurrent systemic thrombotic events associated with congenital anomaly of inferior vena cava. J Thromb Thrombolysis. 2005 Apr;19(2):101-3.

2. Koc Z, Oguzkurt L. Interruption or congenital stenosis of the inferior vena cava: prevalence, imaging, and clinical findings. Eur J Radiol. 2007 May;62(2):257-66.

3. Lambert M, Marboeuf P, Midulla M, Trillot N, Beregi JP, Mounier-Vehier C, Hatron PY, Jude B. Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature. Vasc Med. 2010 Dec;15(6):451-9.

4. Häusler M, Hübner D, Delhaas T, Mühler EG. Long term complications of inferior vena cava thrombosis. Arch Dis Child. 2001 Sep;85(3):228-33.

5. Porter D, Rundback JH, Miller S. Sharp recanalization using a subintimal reentry device, angioplasty, and stent placement for severely symptomatic iliofemoral deep venous thrombosis secondary to congenital aplasia of the inferior vena cava. J Vasc Interv Radiol 2010;21:1765-9.

6. Mindikoglu AL, Miller JS, Borge MA, Van Thiel DH. Post-transplant IVC occlusion and thrombosis treated with tPA, heparin, and sharp recanalization. J Gastroenterol 2005;40:302-5.

7. te Riele WW, Overtoom TT, van den Berg JC, van de Pavoordt ED, de Vries JP. Endovascular recanalization of chronic long-segment occlusions of the inferior vena cava: midterm results. J Endovasc Ther. 2006 Apr;13(2):249-53.

8. Razavi MK, Hansch EC, Kee ST, Sze DY, Semba CP, Dake MD. Chronically occluded inferior venae cavae: endovascular treatment. Radiology 2000;214:133-8.

9. Neglén P, Oglesbee M, Olivier J, Raju S. Stenting of chronically obstructed inferior vena cava filters. J Vasc Surg. 2011 Jul;54(1):153-61.

10. McGee H, Maudgil D, Tookman A, Kurowska A, Watkinson AF. A case series of inferior vena cava stenting for lower limb oedema in palliative care. Palliat Med. 2004 Sep;18(6):573-6.

11. Tarazov P, Prozorovskij K, Rumiantseva S. Pregnancy after embolization of an ovarian varicocele associated with infertility: report of two cases. Diagn Interv Radiol. 2011 Jun;17(2):174-6.

12. Patterson BO, Hinchliffe R, Loftus IM, Thompson MM, Holt PJ. Indications for catheter-directed thrombolysis in the management of acute proximal deep venous thrombosis. Arterioscler Thromb Vasc Biol. 2010 Apr;30(4):669-74.

13. Kahn RS. The Post-Thrombotic Syndrome. Hematology. 2010 Dec:2010(1):216-220.

A painful swollen legChief Complaint & HPIRelevant HistoryWorkupDiagnostic WorkupDiagnosisInitial TreatmentInterventionInterventionClinical Follow UpSummary & Teaching PointsSummary & Teaching PointsQuestionCORRECT!SORRY, THAT’S INCORRECT.References