Journal of Vascular Surgery
Volume 42, Issue 2 , Pages 275-280, August 2005

Endovascular stenting in the treatment of pelvic vein congestion caused by nutcracker syndrome: Lessons learned from the first five cases

Presented at the Seventeenth Annual Meeting of the American Venous Forum, Feb 9–13, 2005, San Diego, Calif.

Service de Chirurgie Vasculaire, Centre Hospitalier Universitaire Nord

Received 7 February 2005; accepted 30 March 2005.

Article Outline

Background

Compression of the left renal vein between the aorta and the superior mesenteric artery is a rare but possibly underestimated condition. Surgical correction (42 cases reported in the literature) can be performed by means of a variety of different techniques. Although endovascular stenting is well accepted for iliocaval occlusive disease, it has been poorly evaluated in this indication. We describe five patients who were treated for nutcracker syndrome by using stenting and analyze the nine cases previously reported.

Methods

From November 2002 to September 2004, five women (mean age, 34.7 years) were admitted for endovascular treatment of a nutcracker syndrome. They all had incapacitating pelvic congestion syndrome, including two with a history of left ovarian vein embolization; moreover, two had left lumbar pain, and three had hematuria. The mean preoperative venous disability score was 2.4. The patients underwent a gynecologic examination and laparoscopy to eliminate other causes of pelvic pain. The laparoscopy revealed large pelvic varicose veins and no signs of endometriosis. Duplex scan, computed tomographic scan, and iliocavography revealed left renal vein compression, with proximal distention and collateral pathways, with dilatation and permanent reflux in the left ovarian vein in the three patients who had not had prior embolization. The mean renocaval pullback gradient was 4.3 mm Hg. A percutaneous endovascular procedure, during in which a self-expanding metallic stent was implanted, was performed under general anaesthesia.

Results

Technical success was achieved in all cases. One case of stent migration occurred: the stent was pulled down in the inferior vena cava, with uneventful follow-up (mean, 14.3 months). One month later, patients were all improved and stents were patent at the duplex scan examination, without restenosis. The mean venous disability score was 1. No further left ovarian vein reflux was evident at duplex scan in patients who did not have prior embolization. Pelvic pain recurred in one patient who had initially improved, and endometriosis was diagnosed 15 months after the procedure. Two other patients, who received 40-mm-long stents, had a secondary recurrence of the symptoms caused by stent dislodgement. The two other patients were asymptomatic.

Conclusions

This study shows that stenting is feasible, but some guidelines should be followed, mainly the use of long stents protruding into the inferior vena cava. Stenting can eliminate the symptoms of the condition, and the technique is only very slightly invasive. Further experience and follow-up are needed before accepting such a procedure for treatment of the nutcracker syndrome.

 

The endovascular treatment of large-vein occlusive disease is well accepted for iliocaval and superior vena cava lesions. Many publications have shown that it is feasible, effective, and safe.1, 2 In addition, the midterm results are satisfactory.

The nutcracker phenomena is caused by arterial compression of the left renal vein between the superior mesenteric artery and the aorta.3 It is symptomatic on occasion and can even be incapacitating. As stenting has given good results in the treatment of May-Thurner syndrome, another form of compression of a large vein by an artery, endovascular therapy for the nutcracker syndrome was first proposed in 1996 by Neste.4 We report our experience of the endovascular treatment of the nutcracker syndrome and review all the cases published.

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Methods 

From November 2002 to September 2004, five women aged 30 to 39 years (mean, 34.7 years) were admitted to the vascular surgery department for endovascular treatment of a nutcracker syndrome. All but one had had a previous pregnancy, and none had a history of deep vein thrombosis. Two had prior left ovarian vein embolization 19 months and 13 months before that improved their symptoms for 2 and 3 months, respectively, before recurrence associated with the development of left lumbar pain. One had had endovascular stenting for May-Thurner syndrome. Pelvic surgery had previously been performed in three: a left ovariectomy in one, for a left ovarian cyst in one, and a right ovariectomy in one. One patient had a history of bilateral great saphenous vein stripping.

All of the patients had long-standing incapacitating pelvic congestion syndrome (mean duration, 3.4 years). These symptoms were associated with left lumbar pain in three and microscopic hematuria in two. Mean preoperative venous disability score5 was 2.4 (range, 2 to 3). In addition, one patient had left limb atypical varices with venous insufficiency symptoms.

Gynecologists had referred all of the patients, and a complete gynecologic workup was performed, including a clinical examination, pelvic transparietal and endovaginal echography, and laparoscopy. In all cases, no other cause of pelvic pain was found, and the results of echography and laparoscopy showed the presence of large varices around the uterus (left side only in three).

After 3 days of a no-residue diet, a duplex scan was performed on an empty stomach in our department with a Toshiba Powervision SSA 380A and a 3–6 MHz probe. The celiac region was examined with horizontal, sagittal, and transversal sections. In cases of nutcracker syndrome, the left renal vein was stenosed between the superior mesenteric artery and the aorta, and this was always associated with proximal left renal vein dilatation (Fig 1). Flow was absent or at least there was no respiratory fluctuation. Left ovarian vein dilatation with permanent reversed flow was found in three patients (two patients had left ovarian vein occlusion caused by a prior embolization).

Angio computed tomographic (CT) scan showed left renal vein stenosis (Fig 2, A) with proximal dilatation (Fig 2, B). In the three patients with a patent left ovarian vein, it was dilated and quickly became opacified (Fig 2, C).

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  • Fig 2. 

    Angio computed tomography scan aspect of a patient with nutcracker syndrome. A, Stenosis of the left renal vein between the superior mesenteric artery and the aorta (black arrow). B, Proximal dilatation of the left renal vein (black arrow). C, Dilatation and early opacification of the left ovarian vein (black arrow).

The procedures were performed in the operating room with a portable C arm. Under local anesthesia, a percutaneous puncture of the left common femoral vein gave access to a 7F sheath. Selective catheterization of the left renal vein was performed with a 7F RDC guiding catheter (Cordis, Johnson & Johnson, Miami, Fla) and a 5F Cobra angiographic catheter (Cook, Bloomington, Ind). A ngiography was performed, and in all cases, it revealed dye stagnation in the left renal vein with opacification of the collateral pathways (Fig 3, A). In the three previously nonembolized patients, the left ovarian vein was dilated and opacified down to the pelvis. The renocaval pullback gradient was 4 to 5 mm Hg (mean, 4.3 mm Hg).

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  • Fig 3. 

    Angiographic view of the nutcracker syndrome. A, Stenosis of the left renal vein with dye stagnation and large collateral pathways (left gonadic vein, hemiazygos vein). B, Massive reflux in the left gonadic vein. C, After stenting, the collateral pathways have disappeared and the left renal vein is well opacified.

Once nutcracker syndrome was confirmed, general anesthesia and intravenous heparin were given. An 11F sheath was inserted into the left common femoral vein and the Terumo guidewire (Tokyo, Japan) was changed for an Amplatz Super Stiff guidewire (Boston Scientific, Natick, Mass). Angioplasty was first performed with a 15-mm-diameter balloon (Maxi LD, Cordis, Johnson & Johnson). A self-expanding metallic stent (Wallstent, Boston Scientific-Schneider, Minneapolis, Minn) was then deployed in the left renal vein and dilated with a balloon. Completion angiography was performed before the sheath was retrieved (Fig 3, B).

Follow-up was performed by clinical examinations and duplex scanning at 1, 3, 6, and 12 months, and then yearly or if symptoms recurred. In that case, or if changes were noted on the duplex scan, a CT scan and an angiography were performed.

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Results 

Technical success was achieved in all patients. Angioplasty alone, although there was no residual mark on the balloon when inflated, always failed to relieve the stenosis. The first patient received a 20-mm-diameter, 60-mm-long Wallstent that migrated into the retrohepatic inferior vena cava and was pulled down 5 cm with an Amplatz Goose Neck Snare Kit (ev3 Inc, Plymouth, Minn). However, it adopted a transversal position in the inferior vena cava just cephalad to the left renal vein and was left in place because further attempts failed. A 16-mm-diameter stent was then deployed in this patient and in the other patients as well.

No postoperative complications occurred. All patients received low-molecular-weight heparin after the operation and were discharged on nadroparin for 15 days and clopidogrel for at least 6 months. Two patients were also given fluindione for 6 months.

Follow-up was 4.2 to 26.5 months (mean, 14.3 months). At 1 month, all patients had improved. A duplex examination showed the absence of residual stenosis with a good flow in the left renal vein. No further left ovarian vein reflux was observed on the duplex scan of the patients who did not have prior embolization. Although we do not have a rational explanation—unless it would be unseen collateral pathways—surprisingly, the two patients with prior embolization had improvement not only of the left flank pain but also of the pelvic congestion syndrome.

Two patients who received a 40-mm-long, 16-mm-diameter Wallstent had recurrence of the symptoms at 3 and 4 months postoperatively. In both, duplex and CT scanning showed that the stent had moved to the right side of the inferior vena cava, allowing the superior mesenteric artery to compress the left renal vein (Fig 4). Both patients are currently under close surveillance.

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  • Fig 4. 

    Stent displacement after 3 months. A, Computed tomography scan shows the stent lying on the right side of the inferior vena cava and the superior mesenteric artery compressing the left renal vein on the left side of the stent. B, Angiography shows the stent largely protruding in the inferior vena cava.

Different pelvic pain developed in one patient at 15 months. Imaging techniques found endometriosis, even though no signs of endometriosis had been found 17 months before at the preoperative workup (pelvic echography, duplex scan, CT scan, and laparoscopy). A hysterectomy was performed after medical treatment failed.

The two other patients are asymptomatic at 4.2 and 26.5 months after the procedure.

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Discussion 

The nutcracker syndrome is a relatively rare—but may be underestimated—entity that can need correction. Pastershank6 was the first to propose surgical treatment of this condition. To date, 42 cases treated by a variety of techniques other than endovascular stenting have been reported (Table I).6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23 All of these procedures gave good results, but they are invasive and even sometimes radical, such as nephrectomy.23

Table I. Published cases of treatment of nutcracker syndrome without endovascular stenting
Technique (references)NComplicationsResults
LRV transposition7, 8, 9, 10, 11, 12, 13182 RPHAsymptomatic, 17
2 ileusHematuria, 1
1 DVT
Autotransplantation14, 157 Asymptomatic, 7
LRV bypass165 Asymptomatic, 4
Improved, 1
External stenting17, 183 Asymptomatic, 3
Gonadocaval bypass183Residual gradient 2Improved, 3
SMA transposition191 Asymptomatic, 1
Surgical angioplasty201 Asymptomatic, 1
Renal fixation211 Asymptomatic, 1
LRV phlebolysis61
BA221 Asymptomatic, 1
Nephrectomy231
Total42 Asymptomatic, 35
Improved, 5
Unknown, 2

LRV, Left renal vein; SMA, superior mesenteric artery; BA, balloon angioplasty; RPH, retroperitoneal hematoma; DVT, deep vein thrombosis.

Although most of the cases reported occurred in men,18 only women were in our cohort, because they were all initially referred for pelvic congestion syndrome.24 Their venous disability score was ≥2, and all of these young women had been limited in their activities for >5 years. The first two patients had prior left ovarian vein embolization, because we initially failed to diagnose the nutcracker syndrome on the duplex scan. The diagnosis was made at follow-up when the patients came back with left lumbar pain and recurrent pelvic pain.

Our experience leads us to believe that the diagnostic criteria on imaging techniques (duplex scan, CT, magnetic resonance angiography, angiography) should associate left renal vein stenosis with proximal distention and the presence of collateral pathways. It must be emphasized that stenosis is not always well highlighted by angiography. An intravascular ultrasound scan could visualize it more accurately, as it does in May-Thurner syndrome.25 The main collateral pathway is the left gonadic vein, which was dilated and incompetent in all of our patients; this sign can be seen on CT scan, as it has early contrast enhancement during the portal phase.

Treatment was decided when the patients had clinical signs of incapacitating nutcracker syndrome (left flank pain, hematuria), or pelvic congestion syndrome (after elimination of others causes by a complete workup including laparoscopy), or both, associated with a significant stenosis of the left renal vein based on the association of duplex scan, CT scan, and angiographic and renocaval pullback gradient (>3 mm Hg) criteria.

As we have some experience in venous stenting for iliocaval occlusive disease,2 we have been very interested in the first cases of stenting to treat nutcracker syndrome. So far, nine cases of endovascular stenting have been published worldwide (Table II).4, 13, 18, 26, 27, 28, 29

Table II. Published cases of endovascular stenting for nutcracker syndrome
NSymptomsHematuriaStentFollow-up (months)Results
Neste41 WS
Segawa261 FNoneYes70×20-mm covered stent6Hematuria
Scultetus181 FPCSYes60-mm Palmaz24Improved
Park271 MVaricoceleYes18-mm Niki stent4Asymptomatic
Chiesa281 MFlank painYes16×30-mm WS + 20×40-mm Mem
Lin133 MFlank painYes12×40-mm WSNSAymptomatic migration 1
van der Laan291 FFlank painYes 15Improved

WS, Wallstent; F, female; PCS, pelvic congestion syndrome; M, male; Mem, Memotherm; NS, not stated.

Some technical points should be emphasized. General anesthesia was used because our experience in May-Thurner syndrome under local anesthesia revealed that stent deployment and angioplasty are very painful in that specific indication. Moreover, the postoperative course showed that lumbar pain was present for a few days.

The left common femoral vein should be preferred for percutaneous access to make left renal vein catheterization easier. A super stiff guidewire is needed and should be placed downwards in the left gonadic vein to allow the stent to cross the stenosis. Then the guidewire should be retrieved and pushed away cautiously into the renal vein before stent deployment.

One of the main technical end points is the type of stent to use. According to Raju1 and our own experience2, 30 in venous stenting, self-expanding retrievable metallic stents should be used, and the Wallstent is the only commercially available stent that has all these criteria. In line with Segawa,26 we used a 20-mm-diameter stent for our first patient. The stent was correctly positioned, but it moved into the inferior vena cava during balloon angioplasty. An attempt to retrieve it with a Goose Neck failed when the stent took a transversal orientation after 5 cm, and further attempts failed. Despite the risk of thrombosis, we decided not to perform surgery. The patient received fluindione for 6 months and clopidogrel for 1 year, and no complications had occurred after 26 months.

This complication was caused by the anatomy of the left renal vein. The length on the left side of the superior mesenteric artery is too short to put the stent in while staying in its trunk, so during angioplasty, the stent shortened, then was not stabilized on both side of the stenosis, and moved. We then used a 16-mm-diameter Wallstent with an excellent result.

For patients 1 and 2, we used 60-mm-long stents, and angiographic control showed they were slightly protruding into the inferior vena cava. For the two next patients, 40-mm-long stents were used, with an excellent completion angiography. Symptoms disappeared until the third and the fourth month, when both patients had secondary stent dislodgement. These complications proved the efficiency of the treatment but also showed that 40-mm-long stents are too short. Lin13 had the same complication using a 12-mm-diameter, 40-mm-long, Wallstent.

We now believe that the 16-mm-diameter, 60-mm-long Wallstent should be considered as the base of sizing for left renal vein stenting but should be fit to patient morphology and anatomic measurements taken during the procedure. As with all venous stenoses, stents need to be stabilized, so long stents that lean on both sides of the stenosis should be used.

Protrusion of the stent in the inferior vena cava does not seem to cause any complication. It can be assimilated to what Raju1 recommended to do for stenting in the May-Thurner syndrome: stent largely protruding into the inferior vena cava. In our own experience of venous stenting, two stents were left in a transversal position in the inferior vena cava after migration during stenting without adverse effect after 26 and 48 months. Moreover, inferior vena cava filters, although mainly inserted in case of thrombotic event and in most cases without heparin or warfarin therapy, have long-term patency rates of >90% at 10 years.31

Postoperative drug treatment is an important issue. Three patients were given low-molecular-weight heparin for 15 days and clopidogrel for 6 months, like we commonly do for May-Thurner syndrome. These durations were chosen empirically, as no guidelines for drug therapy exist. Two patients were also given fluindione, one because the stent migrated into her inferior vena cava and the other at the surgeon’s choice.

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Conclusion 

The nutcracker syndrome is an emerging indication for venous endovascular stenting. This only slightly invasive treatment can eliminate the symptoms of the condition. Stenting can be used, provided that the same techniques as those in the May-Thurner syndrome are used, that is, the stent should protrude into the inferior vena cava. Further studies should report if this approach has adverse effects and if such a treatment can be accepted.

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Acknowledgment 

We thank Dr Michel Perrin, who agreed to sponsor this work at the 2005 meeting of the American Venous Forum.

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References 

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 Competition of interest: none.

PII: S0741-5214(05)00539-2

doi:10.1016/j.jvs.2005.03.052

Journal of Vascular Surgery
Volume 42, Issue 2 , Pages 275-280, August 2005

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