SIR RFS Case Series: A Painful Swollen Leg

8/11/2019 SIR RFS Case Series: A Painful Swollen Leg

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A PAINFUL SWOLLEN LE

Resident: Daniel K. Powell MDAttending: Joseph Shams, MD

Program: Mount Sinai Beth Israel , New York, NY

Originally Posted:



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 approyears (normal HSG)

Allergies

NKDA



WORKUP

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

Initially suspicious for underlying congenital or autoimmune causes of

hypercoagulability. Hypercoagulability workup was performed, whichnegative.



DIAGNOSIS

Absent or chronically thrombosed IVC



INITIAL TREATMENT

Catheter Venogram: The patient was referred for thrombolysis.

Catheter venogram confirmed persistentclots in the iliofemoral veins (yellow arrows),despite anti-coagulation. The bilateral iliacveins and the IVC were occluded and anatretic IVC or draining collateral was seen(white arrow).

The superior aspect of the right common iliacvein could not be crossed with a wire despitemultiple attempts.

Mechanical saline pump rheolyticthrombectomy and multi-side hole catheterdirected tissue plasminogen activator (tPa)thrombolysis was performed with clotaspiration overnight, two days in a row, withthree sessions of thrombectomy, due toresidual 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 waspresented at vascular conference and endovascular IVC reconstruction waspresented to the patient , including a discussion of the risk of life-threateninghemorrhage and chronic back pain from presence of the stents. The patientwas eager for resolution of her pain, which limited her mobility. Risk ofretroperitoneal hemorrhage was considered minimal from the low-flow inthese atretic veins.

Two weeks later the patient underwent planning angiography.Simultaneous inferior-approach right common iliac venogram and separatesuperior-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 theliver to the supra-hepatic IVC, and ascending lumbar veins. The inferiorcatheter (yellow arrow) was advanced superiorly into a collateral vesselrunning anteromedial to the ascending lumbar veins, possibly a parallelretroperitoneal vein or ureteric vein (white arrow). Upon injection fromabove, a dilated azygos and accessory hemiazygos with a large paraspinalabdominopelvic feeding vessel (arrow head) were seen which appeared todrain into the relatively preserved supra-renal IVC and the azygos vein.

Lateral and oblique projections: performed to evaluate the proximity of thedistal right common iliac collateral (white arrow) and the adjacent dilatedparaspinal azygos tributary (arrow head), draining into the suprarenal IVC.

Proximity of these vessels was demonstrated and recanalization wasplanned.

AP AP Lateral Ob



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 theazygos system and IVC. Access was obtained through the right IJ and profundafemoris, due to femoral vein occlusion.

• A superior-approach 4 Fr angled–tip diagnostic catheter (arrow heads) roadmapangiography of the IVC provided a target. An inferior-approach catheter (yellowarrows) was advanced over a guide wire through the common iliac collateral untilresistance was met superiorly. A stiff straight-tip hydrophilic 0.035” guide wire wasthen advanced/perforated with minimal resistance (through the walls of theadjacent 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 therenal IVC, sequentially dilated with 8mm, 10mm and 12mm balloons. 12mm x 6cmoverlapping 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 theprofunda femoris, partially overlapping the common femoral stent do to poor inflowinto the common femoral. The stents were balloon dilated and good angiographicresults were demonstrated.

INTERVENTION

AP

Inferiorinjection

Inferior& superiorinjections

InferiorInjectionopacifyingabove

Afterperforationinto 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 withpelvic pain. Cavogram demonstrated minimal neo-intimal hyperplasia andslight separation of the stents at the common femoral-profunda junction,without pseudoaneurysm. Balloon angioplasty was performed along the lengthof the stents (12mm x 4cm balloon in the external iliac and proximal commoniliac and 14mm x 4cm balloon in the IVC). At two week follow-up, completeresolution of pain.

Over the next year, physical exam, CTV and US were unremarkable on regularfollow-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 legpain. Cavogram demonstrated high-grade stenosis at the junction of theinferior and middle thirds of the stent tract (arrow), at the level of the venousanastomosis / recanalization. Angioplasty with 12, 14 and 16mm balloons wasperformed with good technical results, symptomatic relief and unremarkablefollow-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 recurrenthigh-grade stenosis at the same segment (arrow) and angioplasty wasperformed with a 14mm balloon. Technical success and symptom relief wereachieved. 1-month follow-up CTV and 2-month follow-up cavogram showedwide stent patency.

A B

D EC

Post baangiop

Post balloonangioplasty



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 veinIVC to treat extensive iliofemoral and popliteal thrombosis. There were no early complications of the procedure. Three secondary angioplasties were requirediffuse neo-intimal hyperplasia as well as at two and three years post-procedure for focal stenoses at the point of venous anastomosis /recanalization. Otherrecurrence of DVT, severe lower extremity pain or skin changes associated with chronic venous stasis. There was incidental minimal separation of the overlathe common femoral and profunda veins at one-month follow-up without pseudoaneurysm as well as chronic vague non-specific lower extremity, pelvic andpresence 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-expandingobstruction 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 Lmetastatic 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(11).



SUMMARY & TEACHING POINTS

Regarding indications for DVT thrombolysis, the exact population is not defined. According to the American College of Chest Physicians, the ind

>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 riskis 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 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 8

Villalta Scale – clinical scoring system of signs and symptoms (either binary, yes/no; categorical, none, mild, moderate, severe; or continuousvalidated 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 lipodermatosclhypertension, 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 syndro



QUESTION

Which of the following collateral pathways of chronic SVC/IVC obstructileast 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 leacommon?

A. Internal and external mammary pathway This is a common collateral pathway athe internal mammary, superior epigastric, and inferior epigastric veins and superof the thorax.

B. Azygos-hemiazygos pathway This pathway predominates unless it is poorly deveobstructed at its confluence with SVC

C. Lateral thoracic pathway This is a common collateral pathway and includes the l

thoracic, thoracoepigastric, superficial circumflex, long saphenous, and femoral vcollaterize to the IVC

D. Vertebral pathway This s a common pathway and includes the innominate, verteintercostal, lumbar, and sacral veins to collaterize to the azygos and internal mampathways

E. Cavoportal collateral pathway – although an important collateral pathway, it is lethan the others mentioned above. Flow is directed from the vena cava to the por

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 R



SORRY, THAT’S INCORRECT.

Which of the following collateral pathways of chronic SVC/IVC obstruction is the leacommon?

A. Internal and external mammary pathway This is a common collateral pathway athe internal mammary, superior epigastric, and inferior epigastric veins and superof the thorax.

B. Azygos-hemiazygos pathway This pathway predominates unless it is poorly deveobstructed at its confluence with SVC

C. Lateral thoracic pathway This is a common collateral pathway and includes the la

thoracic, thoracoepigastric, superficial circumflex, long saphenous, and femoral vcollaterize to the IVC

D. Vertebral pathway This s a common pathway and includes the innominate, verteintercostal, lumbar, and sacral veins to collaterize to the azygos and internal mampathways

E. Cavoportal collateral pathway – although an important collateral pathway, it is lethan the others mentioned above. Flow is directed from the vena cava to the por

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 R



REFERENCES

1. Takehara N, Hasebe N, Enomoto S, Takeuchi T, Takahashi F, Ota T, Kawamura Y, Kikuchi K. Multiple and recurrent systemic thrombotic events

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 Ma

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 thromband 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 symptomdeep 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 recanalizGastroenterol 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 cava: midterm results. J En dovasc 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 20

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 ca2004 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 casesRadiol. 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 proxthrombosis. Arterioscler Thromb Vasc Biol. 2010 Apr;30(4):669-74.

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

SIR RFS Case Series: A Painful Swollen Leg

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