Something that I recently promised Dr. James S.T. Yao, I will be working to publish on my stent removal and extended remote endarterectomy cases and techniques. Meanwhile, here is a talk.
Nutcracker Syndrome: The Renal Vein Transposition
Case Report
Patient is a 43 year old woman who had been having bouts of severe left sided abdominal pain for several years with worsening episodes of nausea and vomiting resulting in several visits to the emergency room. She has also had microscopic hematuria. Gastrointestinal workup including gastric emptying study, esophagogastroduodenoscopy and colonoscopy were negative, as was a workup for kidney stones. Eventually she was referred to my clinic for management of nutcracker syndrome. She denied lower abdominal pain nor excessive menstrual bleeding.
On examination, she was tender over the left kidney and flank. Laboratory examination was positive for microscopic hematuria. CT venography (below) showed an obstruction of her left renal vein by the superior mesenteric artery. Drainage via gonadal vein was not demonstrated, and no pelvic varices or complex of retroperitoneal veins was apparent.
Duplex showed the narrowing in the left renal vein and spectral Doppler showed elevated velocities across the compression caused by the superior mesenteric artery (below). The collecting system was not obstructed.
Treatment options included endovascularization with a large stent in the left renal vein, left renal vein transposition to a lower position on the inferior vena cava, left renal autotransplantation, and left nephrectomy. Stent placement comes with a degree of risk for cardiopulmonary embolism which may require a sternotomy to fish out an errant stent. The risk for this in the US is because the largest nitinol stents available are 14mm in diameter which might result in undersizing in a vein that could easily dilate to well over 20mm. Larger nitinol stents for venous applications are available in Europe but currently are not approved in the US (yet). Wall stents, while certainly wide enough, have the problem of being long and stiff when not fully deployed. A 22×35 Wall stent may be 50mm long if deployed inadvertently into a tributary vein or contrained at the narrowing. Because it slides easily, passing balloons in or out can cause it to slip out of position. Because this stent elongates when compressed and packed, deployment is challenging and it is prone to “watermelon seeding,” a set up for embolism. It does have the virtue of easy reconstraining.
My friend and recent host for Midwest Vascular Surgery Society Travelling Fellowship, Dr. John V. White, in Chicago, seems to have solved this problem by a multistep process of predeploying a temporary suprarenal IVC filter, deploying a stent (whatever fits), leaving the filter as a protection against stent migration for 4 weeks until the stent permanently seats itself through scarring/intimal ingrowth, then removing the filter.
I chose to perform venography and renal vein transposition. The patient was placed in a supine position on a hybrid angiographic operating room table and her right femoral vein was accessed. She was placed in 15 degrees reverse Trendelenberg which is about the upper limit possible. Venography below.
After heparinizing and clamping, the renal tributary was taken with a 5mm cuff –this would ensure proper length without narrowing the IVC.
The vein was anastomosed and flow was excellent by pulse Doppler.
She recovered well but had a longer stay because of an ileus, being discharged on day 5. Because she lived at a distance, and came back for followup the following week prior to boarding a plane for home. She no longer had the left sided abdominal pain and there was no hematuria. CT showed excellent drainage through the transposed vein.
Followup will be periodic (6 monthly) renal venous duplex from home. Given that there was minimal tension on the repair, I expect this to do well.
Discussion:
Nutcracker syndrome is one of the many unfortunate consequences of our bipedal lifestyle. The small intestines hang like baggy sausages off the branched stems of the superior mesenteric artery (SMA), and in some individuals, the SMA compresses the left renal vein against the aorta. The left renal vein receives up to 12-15% of cardiac output via the left kidney, and with outflow obstruction, drains the blood through small collaterals. The renal venous hypertension results in swelling of the left kidney with subsequent left renal colic -with flank and abdominal pain, nausea, and vomiting. There is hematuria which can be gross or microscopic. With drainage via an incompetent gonadal vein, varicoceles can occur with discomfort in men and pelvic varices with pelvic congestion syndrome can occur in women.
Diagnosis is challenging because it is one of the relatively rare non-gastrointestinal causes of abdominal pain (table).
- Mesenteric ischemia
- Median arcuate ligament syndrome
- Nutcracker syndrome
- Neuromuscular
- Urolithiasis
- Inflammatory aortitis/arteritis
- Hypersplenism
- Portal hypertension
- Arterial aneurysm
- Infections
- Pelvic Congestion Syndrome
- Endometriosis
- Hernias
A history of left sided abdominal pain, flank pain, nausea, vomiting, associated pelvic pain, and episodes of hematuria are diagnostic. Examination is typically positive for left renal tenderness and flank tenderness. Laboratory examination include urinalysis for hematuria. Duplex, while technically challenging, will show renal venous compression with velocity elevation or loss of respirophasicity, CTA will typically show obstruction of the left renal vein with filling of collaterals, as will MRV.
Venography should be done in a stepwise manner (White protocol) to fully demonstrate the maldistribution of blood. That is the key word, maldistribution. I learned from my fellowship with Dr. White that performing venography in as upright a position as possible recreated the pathophysiology, the physics, particularly for pelvic congestion and nutcracker. Remember, this is a disease of bipedalism, of upright posture. Many negative studies done supine become positive, as the contrast will fall to where it prefers to go. As I have stated in the past, on the venous side, demonstrating drainage has different imaging needs than demonstrating flow. Pathologic venous drainage has three characteristics:
- Varicose veins develop as an end stage process
- Reversal of flow or reflux is demonstrated, particularly into small tributary veins
- The midline is crossed in these usually small, now larger, collateral veins
While pressure gradients are nice if they are large, they are difficult to assess when they narrow to 1-2mmHg, particularly if they vary with cardiac cycle and respiration. Because we are assessing drainage, the distribution of contrast and the direction it goes is particularly important, and far more sensitive than pressure measurements.
Venography was done per a modification of Dr. White’s protocol for pelvic congestion:
- Steep reverse Trendelenberg
- Hand injection 10mL half diluted contrast, gently as to not create false reflux
- Runs with catheter in left EIV, right EIV, left renal vein, right renal vein
- With pelvic congestion workup, add selective bilateral gonadal and internal iliac veins.
I have started transposing gonadal veins when they have enlarged from chronic reflux (link, ref 2). Renal vein transposition was chosen because her ovarian vein was competent and too small to transpose (ref 1-3). While the patency rate of stents in veins seems to be acceptable, long term data is unavailable. Also, venographic appearances are deceiving -see the in-vivo measurement of the left renal vein after dissection:
References:
- White, J. et al, Left ovarian to left external iliac vein transposition for the treatment of nutcracker syndrome. J Vasc Surg Venous Lymphat Disord. 2016;4:114–118.
- Miler R, Shang E, Park W. Gonadal Vein Transposition for the Treatment of Nutcracker Syndrome. Annals of Vascular Surgery 2017, July 6. in press. http://dx.doi.org/10.1016/j.avsg.2017.06.153
- Markovic J, Shortell C. Right gonadal vein transposition for the treatment of anterior nutcracker syndrome in a patient with left-sided inferior vena cava. J Vasc Surg Venous Lymphat Disord.2016 Jul;4(3):340-2. doi: 10.1016/j.jvsv.2015.09.002.
- Erben Y, Gloviczki P, Kalra M, Bjarnason H, Reed NR, Duncan AA, Oderich GS, Bower TC. Treatment of nutcracker syndrome with open and endovascular interventions. J Vasc Surg Venous Lymphat Disord. 2015 Oct;3(4):389-96. doi: 10.1016/j.jvsv.2015.04.003.
Case report up on Annals of Vascular Surgery -Gonadal Vein Transposition for Nutcracker Syndrome
A case report co-authored by my recently graduated trainees, Drs. Roy Miler and Eric Shang. An update to be presented at Midwest Vascular Surgery Society Meeting in Chicago.
When Hybrid Seems Better: Carotid Trauma As a Model For All Trauma
History
The patient is an 80 year old woman with lung cancer who was getting a port placed at her home institution. It was to be a left subclavian venous port, but when access was not gained, a left internal jugular venous port was attempted, but after the intitial stick and sheath placement, pulsatile bleeding was recognized and the sheath removed. Hemostasis was achieved with clips and the wound closed and a right internal jugular venous port was placed. The postprocedural CXR shown above showed tracheal deviation and numerous clips from the initial port placement attempt, and a CT scan with contrast (unavailable) showed a carotid pseudoaneurysm of 3cm projecting posteriorly behind the pharynx/esophagus. She was kept intubated and sedated, and transferred for management.
On examination, her vital signs were stable. She had 2cm of tracheal deviation and swelling was apparent at the base of the neck. While my trainees may be better versed at this than I at the particulars of this, my old general surgery trauma training kicked in, as she had a Zone I neck carotid injury, 
which in my experience is highly morbid despite how stable the patient was. Point again to trainees, this is no different from someone having stabbed this patient with a knife at the base of the neck. My options were:
- Open repair
- Endovascular repair from femoral access
- Hybrid repair
Open Repair
Open repair is the approach of choice for zone 2 injuries because aerodigestive tract injuries can also be addressed and the exposure is straightforward. For Zone 1 injury, the exposure is potentially possible from a neck exposure, but in my experience, jumping into these without prepping for a sternotomy puts you into a situation without a plan B. The exposure of the carotid artery at this level becomes challenging with hemorrage from the artery once the compression from the hematoma or pseudoaneurysm is released. A sternotomy in this elderly woman, while not optimal, may be necessary if open control is required, but the best plan is to avoid this.
Endovascular Options
This should be a straightforward repair from an endovascular approach, even with the larger sheath required for the covered stents. A purely endovascular approach is problematic for two reasons. One, cerebral protection devices are built for bare carotid stents and not peripheral stent grafts, but this is not prohibitive -it should be fine. Without a planned drainage, the hematoma would be left behind which could cause prolonged intubation and problems with swallowing -both an issue for an elderly patient battling lung cancer. Endovascular access could provide proximal control for an open attempt from above, but instrumenting from the arch in an 80 year old has a known 0.5-1% stroke rate.
Hybrid Repair
A hybrid open approach with exposure at the carotid bifurcation offers several advantages. With control of the internal carotid artery, cerebral protection is assured while the carotid artery is manipulated. At the end of the procedure, the internal carotid can be backbled through the access site with the common carotid artery clamped. The hematoma could be avoided until the stent graft is deployed. An unprotected maniplation in the arch can be avoided. Once the stent graft is deployed, drainage of the hematoma can be performed.
This required setting up a table off the patient’s left that allowed the wire to lie flat to be manipulated by my right hand. The carotid bifurcation was accessed through a small oblique skin line incision and the common, internal, and external carotid arteries, which were relatively atherosclerosis free, were controlled with vessel loops. The patient was heparinized. The internal carotid was occluded with the loop, and the common carotid below the bifurcation was accessed and an 8F sheath with a marker tip inserted over wire. Arteriography showed the injury and pseudoaneurysm.
The location of the injury based on CT and on this angio would have baited a younger me into directly exposing it, but experience has taught me that which occasionally you can get away with it, the downsides -massive hemorrhage, stroke, need for sternotomy, just aren’t worth it. The sheath was brought across the injury and a Viabahn stent graft was deployed across the injury.
The hemorrhage was controlled and the hematoma was then exposed and drained -the cavity was relatively small and accepted the tip of a Yankauer suction easily. A Jackson-Pratt drain was placed. The access site was repaired after flushing and retrograde venting as described.
She recovered rapidly after extubation postop. She was able to breath and swallow without difficulty and had suffered neither stroke nor cranial nerve injury. The drain was removed on postop day 2.
The patient recently returned for a 6 month followup. Duplex showed wide patency of her stent.
More gratifyingly, her port was removed as her cancer was controlled with an oral regimen.
Discussion
Let me start with my bias that all penetrating trauma should be approached in a hybrid endovascular OR. It is a natural setting for trauma and this case illustrates that. In a hybrid operating room, central aortic and venous injuries can be controlled endovascularly while open repair, including salvage packing, can be done. Excess morbidity of central vascular exposures can be avoided. Temporary IVC filters can be placed if indicated (becoming rarer and rarer). Cardiopulmonary bypass can be started.
In this patient, hybrid therapy brought the best of both techniques and avoided many of the pitfalls of the purely open or endovascular approach. For stable zone I penetrating injuries of the neck, it is clear that this is a reasonable approach.
Salvage: A different approach to graft infections in the groin
The principles of salvage are in rescuing valuable undamaged goods in the setting of catastrophe. This guided me when a patient was flown in from an outside institution to our ICU with a saline soaked OR towel in his right groin -he had had an aorto-bifemoral bypass for aorto-iliac occlusive disease a year prior, but had never properly healed his right groin wound which continued to drain despite VAC therapy and wound care. On revealing his groin, this is what I saw:
A CT scan was sent with the patient but has been lost to time, and it showed a patent aorto-bifemoral bypass send flow around an occluded distal aorta and iliac arteries. The graft did not have a telltale haze around it nor a dark halo of fluid which signaled to me that it was likely well incorporated and only sick in the exposed part. The patient was not septic, but had grown MRSA from the wound which was granulating from the extensive wound care that had been delivered.
I felt that it would be possible to move his anastomosis point more proximally on the external iliac in a sterile field (figure above), and then close, then endarterectomize the occluded external iliac artery after removing the distal graft, then after vein patching, cover the repair with a sartorius muscle flap. It would salvage the remaining graft and avoid a much larger, more intense operation which was plan B. To prepare for that, I had his deep femoral veins mapped.
The patient was prepped and draped, the groin was excluded by placing a lap pad soaked in peroxide/betadine/saline solution (recipe for “brown bubbly” liter saline, a bottle of peroxide, a bottle of betadine), and covering with an adesive drape. The rest of the abdomen was then draped with a second large adhesive drape. A retroperitoneal (transplant-type) right lower quadrant incision was made (below) and the external iliac artery and graft were exposed. As predicted on CT, the graft was well incorporated.
The external iliac artery was opened and focally endarterectomized of occlusive plaque (image below). The adventitia had good quality despite the longstanding occlusion.
The graft was mobilized and transected and anastomosed end to side to this segment of artery (below). Dissecting was made difficult by how well incorporated it was.
The wound was irrigated (with brown bubbly) and closed, dressed, and sealed over with the adhesive drape. The groin wound was then revealed and the graft pulled out (below).
Remote endarterectomy using a Vollmer ring was used -in this case I didn’t use fluoroscopy given the short distance to the terminus of the plaque which i had mobilized in the pelvis.
The plaque came out easily and was not infected appearing. It is shown below ex vivo.
A segment of saphenous vein was harvested from the patient medially and the arteriotomy was patched. The sartorius muscle was mobilized and applied as a flap over this. The wound was irrigated with brown bubbly and packed open with the intention of VAC application.
The patient healed very rapidly and remains infection free. I had used this approach on several occasions in the past and twice more recently. It truly is salvage as it preserves the uninfected graft while never exposing it to the infection in the process of operating. It avoids having to remove the whole graft which then damages the left side -I have seen other surgeons take this approach elsewhere taking a all-or-nothing approach to graft infection to considerable morbidity to the patient. It avoids having to harvest deep femoral vein -another large operation to which the body responds truculently. The patient recently came by for his 4 year followup, still smoking, but legs preserved.
Followup At 4 Years
The patient came back in followup -it has been 4 years since his infection was repaired. He was complaining of short distance claudication. His wound healed well and remains closed. CTA shows along with his short segment SFA occlusion which we will treat, a widely patent R. EIA (below).
The remote endarterectomy of the external iliac artery remains patent. Compare this to the preop CTA which I found and wasn’t available when I posted this case originally:
The chronically occluded EIA can be readily seen. The artery shown in the current CTA is that recanalized artery.
The Closest Thing to an Off Pump CABG -a Carotid Subclavian Bypass to Treat Unstable Angina
Patient is a 77 year old man with history of HTN, hyperlipidemia, former smoking, and CAD with CABGx5 and bilateral lower extremity bypasses who developed unstable angina consisting of neck and throat pain. He underwent catheterization at an outside hospital and found to have 100% LAD occlusion, a diseased, small patent left main and left circumflex (the profunda femoral artery of the heart!), 100% RCA occlusion, a patent but diseased SVG to distal RCA, and a patent LIMA graft to distal LAD but with severe plaque and near occlusion of his proximal left subclavian artery.
He had an NSTEMI. His vitals signs stabilized in the coronary care unit and he was sent to a telemetry floor. Whenever he walked, he would get the jaw pain, and this would also occur sporadically while recumbent.
On examination, he had no left brachial pulse, only a monophonic signal there, and bounding femoral pulses where there were the origins of bilateral femoral-tibial bypasses. His radial artery pulse was diminished on the right and absent on the left. Both saphenous veins had been harvested as were arm veins for the left leg bypass.
CTA shows the left subclavian artery to be occluded at its origin.
Cardiac surgery, interventional cardiology, and vascular surgery were called in for consultation. Cardiology consultation (Drs. Kapadia and Shisheboor) felt, and I agreed, that the left subclavian lesion was a poor candidate for recanalization and stenting. CT Surgery (Dr. Faisal Bakaeen) and I had a long discussion regarding alternate conduits, as he had unknown radial but likely radial artery disease, and had all usable veins previously harvested. I brought up a free RIMA graft -I had worked with Dr. Daniel Swistel, in NYC as a resident, who was Dr. George Green’s protege, and as a medical student at P&S I scrubbed Dr. Green’s final cardiac case. He routinely performed bilateral ITA bypasses decades before all-arterial revascularizations were routine. I get enthusiastic talking about cardiac disease! Walking through all the options -does anyone use deep femoral vein as coronary bypass conduit -we agreed ultimately that the best option would be a carotid-subclavian bypass with plenty of backup.
At its heart, it would be this vascular surgeon’s attempt at an off-pump single vessel CABG (above). Preparations were made with cardiac anesthesia and cardiac surgery to place an IABP (intra-aortic balloon pump) if he became unstable. For my part, the operation was straightforward, but I was going to have to go about it efficiently. I also figured that with a clamp beyond the LIMA takeoff, no significant change would occur to the coronary flow from the LIMA graft. So I hoped as I worked very deliberately. We kept him on the hypertensive side during the case.
The operation went well. The patient’s angina resolved and a followup CT showed the patent bypass feeding the LIMA and LAD.
His resting angina resolved. He followed up a month later and was very pleased. Moreover, he had a brachial and radial artery pulse and a general weakness of the left arm that he never complained about before lifted.
Discussion
The carotid subclavian bypass is something that really needs to be in the armamentarium of a modern vascular surgeon. Though out of print, Wylie’s Atlas (the unabridged, multivolume version) is available used through online sellers, and is useful for elucidating the anatomy which boils down to avoiding cutting the important structures -the phrenic nerve, the vagus nerve, the brachial plexus, branches of the subclavian including the vertebral artery, while cutting away muscles -lateral head of sternocleidomastoid, any part of the omohyoid, the anterior scalene muscle. And dividing the lymphatic duct if encountered. And tunneling under the jugular vein. And minding the buttery fragility of the SCA. The best technical paper out there is by Dr. Mark Morasch and it mostly deals with carotid-subclavian transposition (reference 1) but has excellent figures on bypass as well. I do both transposition and bypass, but for brevity, I prefer bypass.
This is not a unique problem, having been reported in the literature. An unusual variant of this is coronary sbuclavian steal syndrome (reference 2), which refers to reversal of flow in the LIMA bypass in the setting of subclavian artery occlusion and left arm exertion -which was not the case here, but interesting enough to mention. Here, it was a straightforward case of managing the hemodynamics. The key point of operating on such a patient was having the surety of quick response in the case of ischemic heart failure -we operated in the cardiovascular operating rooms with rows of perfusion pumps and balloon pumps and VADs and ECMOs at the ready. Indeed, this result could not have been so straightforward and routine seeming without the combined effort and experience of the whole Heart and Vascular Institute from nursing to consultant staff.
Reference
- Morasch MD. Technique for subclavian to carotid transposition, tips, and tricks. J Vasc Surg 2009;49:251-4.
- Cua B et al. Review of coronary subclavian steal syndrome. J Cardiol. 2017 Apr 14. pii: S0914-5087(17)30090-4. doi: 10.1016/j.jjcc.2017.02.012. [Epub ahead of print]
Chronic IVC occlusion causing venous claudication and ulcers requires treatment
The patient is a gentleman from out of state who had called about problems he was having with walking and with leg ulcers that wouldn’t heal. He is in his seventies and has a pacemaker for an arrhythmia for which he was on Xarelto. He also had type II diabetes. He had bilateral lower extremity deep venous thromboses 6 years prior requiring IVC filter placement. The filter occluded, and it resulted in sudden sharp and debilitating pain in both legs with walking short distances -some days only 50 paces.He described it as an unbearable pain in calves and thighs that felt like his legs were going to burst. He also had ulcers on his legs that would heal with ministration but soon recur. This was all despite being quite active, with regular workouts, and being fit. He was compliant with compression. He sent a CT scan done last year (below).
It showed an Optease retrievable vena cava filter that was occluded and the iliac systems bilaterally (right above and left below) were chronically occluded with patent vena cava above and femoral confluences bilaterally below.
He had no hypercoagulability nor ongoing recent DVT’s. I thought there was a good chance that we would be able to recanalize the occluded iliocaval segment and he flew in for a consultation, and he was pencilled into the schedule ahead of his visit.
Examination revealed a fit and trim man in his 70’s in no apparent distress. He had bilateral leg edema that was moderate with small superficial and tender ulcers of the right posterior distal calf. Pulses were normal. He was taken to our hybrid suite and venography from femoral vein access in the proximal thigh in the supine position revealed his right and left iliac venous systems to be occluded (below figures).
Wire access into the iliac systems was performed with Glidewire and Glidecatheter periodic venography to confirm that I had not exited the vein. Unlike the arterial system, extravasation from being extravenous does not have the consequence of bleeding, hematoma development, and pseudoaneurysm formation because of the low pressure, but it can be a long procedure and uncomfortable as well so these are done under general anesthesia.
Once position confirmed to be in the iliac vein, the vein was dilated to allow for greater ease of movement. In the case of the uncrossable filter, I switched to access from above via a right internal jugular vein access.
Once the wire crossed into the iliac vein from above, it was captured and brought out. While ballooning by itself is inadequate for revascularization, it greatly eases wire capture and on the right, it was done simply by driving the wire from above into the sheath. Wire capture wins access across the iliocaval and IVC filter occlusion from below.
Once wire access is done, ballooning across the filter is done from both sides. A large sheath is them delivered across the IVC filter. Finally, a Palmaz stent mounted on a large balloon is delivered and deployed. I chose to do this from the right access, and retracted the wire on the left -something done with some trepidation because of the great difficulty gaining this access, but with with prior balloon dilatation, reaccess is made easier. Also, plan B would be reaccess from above.
When this is accomplished, the left sided wire is reaccessed across this stent. This is the venous side analogue to gate access in EVAR (below).
Once this is done, the iliac veins are dilated to 14mm from the IVC to the common femoral arteries. large 18mm Wall stents are deployed in a kissing fashion from the caval stent into both iliac systems and dilated to 18mm.
After deployment, the Wall Stents are ballooned to 18mm. These stents were extended into the common femoral artery with 14mm nitinol stents.
Completion venography suggested successful iliocaval recanalization and revascularization but these procedures are not done without a final intravascular ultrasound (IVUS).
Intravascular ultrasound revealed incomplete expansion of the right common femoral stent. This was treated with another stent and ballooning with the result on the right.
Venography alone is insufficient in determining patency. As illustrated, IVUS ensures a durable outcome.
The leg ulcer was treated with an Unna’s boot. A word about the venerable Unna’s boot –it works. The dressing dries and compresses while the Zinc Oxide prevents bacterial growth. It is interesting that the dressing is so infrequently used nowadays but not so when you consider that it isn’t reimbursed. And patients generally hate it.
This revascularization has an excellent chance at working as the patient has no hypercoagulability and had a patent common femoral confluence bilaterally. As I had mentioned in a prior post, the idea in venous revascularization is connecting confluences that serve as inflow and outflow.
This will require followup, consisting of duplex, and it is advantageous that he is anticoagulated for his arrhythmia. It is becoming more apparent that those languishing with chronic venous insufficiency and its complications need the IVC and iliac veins interrogated with a duplex. When an obstruction is found, they should be treated with these techniques as a first line therapy.
