REVIEW ARTICLERichard P. Cambria, MD, Section Editor

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Diagnosis and treatment of the pelvic congestionsyndromeMarlene T. O’Brien, MD, PhD,a and David L. Gillespie, MD,b Rochester, NY; and Fall River, Mass

Background: Chronic pelvic pain accounts for up to 30% ofoutpatient gynecologic visits in the United States, potentiallyaffecting up to 40% of the female population during theirlifetime. Pelvic congestion syndrome (PCS) is defined aschronic pelvic pain resulting from reflux or obstruction of thegonadal, gluteal, or periuterine veins, sometimes associatedwith perineal or vulvar varices. It can also be caused bycompression of the left renal vein (LRV) between the superiormesenteric artery and the aorta, also known as the nutcrackersyndrome. Whereas PCS accounts for up to 30% of patientspresenting with chronic pelvic pain, it is frequently under-diagnosed. We reviewed the literature to investigate the cur-rent state of the diagnosis and treatment of this disorder.Methods: An online database search was performed withMEDLINE. MeSH headings included PCS, chronic pelvicpain, ovarian vein reflux, nutcracker syndrome, renal veinobstruction, pelvic varicosities, labial varicosities, emboliza-tion, treatment, and therapies.Results: Our MEDLINE search revealed more than 3756 ref-erences to chronic pelvic pain. Specific references to PCS,pelvic chronic pain, ovarian vein reflux, nutcracker syndrome,

the Division of Vascular Surgery, University of Rochester School ofedicine and Dentistry, Rochestera; and the Division of Vascular anddovascular Surgery, Heart and Vascular Center, Southcoast Healthcarestem, Charlton Hospital, Fall River.b

or conflict of interest: none.rint requests: David L. Gillespie, MD, RVT, FACS, Chief, Division ofascular and Endovascular Surgery, Heart and Vascular Center, South-ast Health System, Charlton Hospital, 363 Highland Ave, Fall River,A 02720 (e-mail: gillespied@southcoast.org).editors and reviewers of this article have no relevant financial relation-ips to disclose per the Journal policy that requires reviewers to declineview of any manuscript for which they may have a conflict of interest.-333Xyright � 2015 by the Society for Vascular Surgery.://dx.doi.org/10.1016/j.jvsv.2014.05.007

renal vein obstruction, pelvic varicosities, labial varicosities,embolization, treatment, and therapies, however, includedonly 260 references. Thirty-seven references were small seriesincluding fewer than 50 patients or individual case reportsdocumenting medical, surgical, or endovascular treatment ofPCS. The majority of these papers demonstrated successfultreatment of symptoms from PCS with embolization of one orboth ovarian veins in addition to treatment of refluxing in-ternal iliac vein branches. In addition, open surgery and, morerecently, endovascular stenting of LRV obstruction haveshown some promise in alleviating symptoms attributed tonutcracker syndrome.Conclusions: Diagnosis of PCS requires a careful history,physical examination, and noninvasive imaging. Several largecase series have demonstrated the efficacy of embolotherapy inthe reduction of pelvic pain; thus, it is the most favoredtreatment option for patients with PCS. For patients withoutflow obstruction due to nutcracker syndrome, a limitednumber of studies have demonstrated remission of symptomswith stenting of the LRV as an alternative to open surgery. (JVasc Surg: Venous and Lym Dis 2015;3:96-106.)

Chronic pelvic pain is described as the presence oflower abdominal or pelvic pain for longer than 6 months.Chronic pelvic pain accounts for up to 30% of outpatientgynecologic visits in the United States, potentially affectingup to 40% of the female population during their lifetime.1,2

Pelvic congestion syndrome (PCS) is defined as chronicpelvic pain often associated with perineal or vulvar varicesresulting from reflux or obstruction of the gonadal, gluteal,or periuterine veins. First described clinically by Richet in

1857, the existence of pelvic varicosities was documentedin 1949 by Taylor. PCS accounts for up to 30% of patientspresenting with chronic pelvic pain and is characterized bysymptoms of dysmenorrhea, dysuria, and dyspareunia.1 Itcan often be found in conjunction with vulvar and pelvicvarices in women and with varicoceles in men. In additionto causing a fair amount of physical pain and discomfort,PCS also carries a psychological burden and is often foundin conjunction with increased levels of anxiety, stress, anddepression. Patients with PCS are primarily premenopausaland range in age from 20 to 45 years, although most pre-sent in their second and third decades of life. Genetic orethnic predilections are unclear; however, a family historyand multiparity are both risk factors. Venous outflowobstruction resulting from left renal vein (LRV) compres-sion due to either the superior mesenteric artery (SMA)in nutcracker syndrome or uterine malposition is also animportant although less common factor in the develop-ment of PCS. Treatment of PCS consists of hormone ther-apy, embolotherapy, sclerotherapy, and endovascular andopen surgery. Although no randomized prospective trialshave studied the efficacy of such therapies, several studiesfrom single institutions have demonstrated efficacy for

mailto:gillespied@southcoast.orghttp://dx.doi.org/10.1016/j.jvsv.2014.05.007http://crossmark.crossref.org/dialog/?doi=10.1016/j.jvsv.2014.05.007&domain=pdf

Fig 1. Pelvic venous anatomy. CIV, Common iliac veins; IIV,internal iliac veins; IVC, inferior vena cava; LOP, left ovarianplexus; LOV, left ovarian vein; LRV, left renal vein; LUP, leftuterine plexus;ROP, right ovarian plexus;ROV, right ovarian vein;RUP, right uterine plexus. (Modified with permission. � 2014Intermountain Vein Center.)

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interventions that have alleviated symptoms related to thedisease.

ANATOMY

The ovaries and uterus are drained by both the internaliliac and gonadal veins (Fig 1). The internal iliac vein passesslightly medial and posterior to the internal iliac artery,joining the external iliac to form the common iliac vein.Its tributaries are divided into parietal and visceral. Parietaltributaries are the superior and inferior gluteal, sciatic, sacral,ascending lumbar, and obturator veins. Visceral tributariesare the internal pudendal, middle hemorrhoidal, and vesico-prostatic plexuses in men and the uterine, gonadal, and ves-icovaginal plexuses in women. In 27% of cases, the internaliliac vein drains by means of two separated trunks. Rarely, itcan drain directly into the inferior vena cava (IVC). Valvesare found infrequently on the internal iliac veins (10% ofcases on the main trunk and 9% on its tributaries).3

Ovarian veins provide drainage of the parametrium,cervix, mesosalpinx, and pampiniform plexus, forming arich anastomotic venous plexus with the paraovarian, uter-ine, vesical, rectal, and vulvar plexuses (Fig 1). Two orthree trunks form a single ovarian vein at L4, with theleft ovarian vein draining into the LRV and the rightovarian vein draining directly into the IVC in the majorityof women. In up to 10% of women, the right ovarian veinmay also drain into the right renal vein instead of the IVC.Studies have shown that normal ovarian veins have anaverage diameter of less than 5 mm.3 Valves are presentin these veins, mainly in the distal third. Ahlberg et al3

found no ovarian vein valves on the left side in 15% andnone on the right side in 6%. In those in whom valvesare present, they are incompetent in 40% on the left andin 35% on the right. Ovarian vein reflux has been reportedin 10% of female renal transplant donors, up to 60% ofwhom develop PCS.4 Up to 47% of asymptomatic parouswomen have left ovarian reflux and enlarged mean ovariandiameters ranging from 7 to 12 mm on computed tomog-raphy (CT) scan.5 Thus, because of variation in anatomyand the variability of correlation between both anatomicand functional imaging and clinical symptoms, the diag-nosis of PCS is primarily a clinical one that is often deducedfrom a process of elimination in conjunction with imagingsuggestive of venous incompetence or obstruction. On thebasis of clinical presentation and hemodynamic pathophys-iologic findings, four main types of pelvic venous circula-tion disorders have been recognized: vulvar variceswithout accompanying symptoms of pelvic congestion(although vulvar varices may be seen with any type of pelviccongestion), isolated insufficiency of the hypogastric veinand its tributaries, gonadal vein reflux, and obstruction ofthe gonadal outflow by mesoaortic compression of theLRV (nutcracker syndrome).2,6 The most common ofthese is gonadal vein reflux due to incompetent valves.2,7

PATHOPHYSIOLOGY

Because of the paucity of functioning valves and theproximity of the pelvic veins to several structures, pelvic

varicosities can develop by two mechanisms, reflux causedby incompetent valves and obstruction. The cause ofvalvular incompetence is unknown, although hormonalfactors are thought to play a significant role. During preg-nancy, estradiol inhibits vasoconstriction and induces uter-ine enlargement with selective dilation of the ovarian anduterine veins, placing more stress on the valves. Multipa-rous women are more likely to develop pelvic venousincompetence. Conversely, vasoconstrictors have shownsome efficacy in alleviating the symptoms of PCS byimproving venous return through compression of thevein. In women diagnosed with PCS, the injection of dihy-droergotamine produces a 35% reduction in diameter ofthe pelvic veins and a decrease in pain.8

PCS may also result from obstruction of ovarian veinoutflow. The most common cause of obstruction is thecompression of the LRV between the SMA and the aorta,also known as the nutcracker syndrome (Fig 2).9 Distalobstruction can lead to increased venous pressure and sub-sequent venodilation, valvular incompetence, and tortuos-ity of the ovarian vein, resulting in the development of anelevated pressure gradient between the LRV and the venacava, a finding that is normally absent. The presence ofan elevated LRV-IVC pressure gradient may be suggestive

Fig 3. Patient with disfiguring vulval varices of intrapelvic origin.(Reprinted from Scultetus AH, Villavicencio JL, Gillespie DL, KaoTC, Rich NM. The pelvic venous syndromes: analysis of ourexperience with 57 patients. J Vasc Surg 2002;36:881-8, withpermission from Elsevier.)

Fig 2. Ovarian vein obstruction (nutcracker syndrome). Duplex(A), computed tomography (CT) scan (B), and angiographic (C)views of the nutcracker syndrome. (Reprinted from Hartung O,Grisoli D, Boufi M, Marani I, Hakam Z, Barthelemy P, et al.Endovascular stenting in the treatment of pelvic vein congestioncaused by nutcracker syndrome: lessons learned from the first fivecases. J Vasc Surg 2005;42:275-80, with permission fromElsevier.)

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of nutcracker syndrome, but not in isolation without symp-toms or evidence of varicosities, as will be further discussed.Uterine malposition with ovarian kinking is anotheralthough less common cause of outflow obstruction alongwith May-Thurner syndrome, a condition in which the leftcommon iliac vein is compressed by the right common iliac

artery. This compression can sometimes lead to deepvenous thrombosis. Regardless of the etiology, the endresult of ovarian vein outflow obstruction is the develop-ment of numerous refluxing varicosities, cross-pelvicvenous collaterals, and painful venous congestion of theperineal vasculature (Fig 3).

PRESENTATION

Patients with PCS have usually seen primary care andgynecologic specialists before being referred to a vascularspecialist. Many patients will present with chronic, dull,lower abdominal pain often accompanied by dyspareuniaand bladder irritability and urgency.1 The pain is typicallyrelieved by lying down and exacerbated by standing upor increased intra-abdominal pressure, such as during preg-nancy and the premenstrual period. Pain during inter-course or during the postcoital period is not uncommon.Other symptoms of pelvic congestion are nonspecific andvariable in intensity. Affected women may have fullness inthe legs, generalized lethargy, depression, abdominal orpelvic tenderness, vaginal discharge, dysmenorrhea,swollen vulva, lumbosacral neuropathy, rectal discomfort,and nonspecific gastrointestinal symptoms.2 Differentialdiagnosis in these patients is lengthy and includes pelvic in-flammatory disease, endometriosis, pelvic tumors, intersti-tial cystitis, and inflammatory bowel disease.

Clinical examination often reveals vulvar varicosities(Fig 3) together with an engorged cervix and pain on

Fig 4. Algorithm for workup of pelvic congestion syndrome(PCS). Patients present to primary care physicians with complaintsof chronic pelvic pain and possibly dysmenorrhea, dysuria, anddyspareunia. A thorough physical and pelvic examination shouldbe conducted, looking for perineal, vulvar, and lower extremityvarices and ovarian point tenderness. The patient should then bereferred to a vascular laboratory for pelvic and lower extremity(LE) duplex ultrasound (US) examination, performed preferablywhile standing. If US demonstrates an ovarian vein of more than6 mm, dilated tortuous arcuate veins in the myometrium thatcommunicate with bilateral pelvic varicose veins, slow blood flow(6 mm,16 although 7 mm has also been sug-gested as a cutoff.5,14 Park et al16 found the positive pre-dictive value of a 6-mm-diameter ovarian vein for thediagnosis of PCS caused by the ovarian vein to be 83.3%,and this number has been widely accepted since. In truth,whereas an ovarian vein size criterion is one component ofPCS, its diagnosis is favored on the basis of a combination

of ovarian vein dilation, clinical symptoms, and these veno-graphic findings: dilated tortuous arcuate veins in the myo-metrium that communicate with bilateral pelvic varicoseveins, slow blood flow (

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Both the internal iliac and the genital veins should beimaged to look for dilation and reflux, including imagingwith the Valsalva maneuver. The obturator, sciatic, and in-ternal pudendal veins should also be imaged. Collateralpathways can be found in patients with PCS. In addition,duplex scanning should evaluate the common iliac veins,IVC, and renal veins to search for venous obstruction.The authors think that all patients suspected of havingPCS should also undergo lower extremity duplex scanlooking for common femoral vein reflux into the perineumthrough tributary veins even in the absence of lower ex-tremity symptoms, given that the absence of lower extrem-ity varicosities does not necessarily preclude the diagnosisof PCS.6,11,12

If pelvic US with color duplex imaging is highly sug-gestive of PCS, proceeding to venography and potentialintervention is not unreasonable. However, in patientswith normal or nondiagnostic findings on pelvic US andpersistent symptoms, MDCT venography and MRVshould be considered as they have a higher sensitivity forlower pelvic varices and also yield more discriminating in-formation about the surrounding anatomy. First describedas tools for the diagnosis of pelvic varicosities in 1999,traditional CT venography and MRV provide usefulanatomic data in the diagnosis of PCS, particularly if thecause is compressive, as with May-Thurner or nutcrackersyndrome.17 The abrupt narrowing of the LRV with anacute angle (beak sign) has been shown to have 91.7%sensitivity and 88.9% specificity in diagnosis of nutcrackersyndrome.17 The limitations to traditional CT and mag-netic resonance imaging (MRI) are that static images pro-vide few data about the direction of flow within pelvicveins. These studies are normally conducted while the pa-tient is lying down, so they may underestimate the extentof collateral networks or ovarian vein enlargement. Theemergence of MDCT and time-resolved MRV (TR-MRV), however, has bridged the gap between staticanatomic and dynamic functional imaging.

CT venography with three-dimensional reconstructionhas been shown to be an effective road map before varicosevein surgery and in the visualization of venous webs, suchas those found in May-Thurner syndrome.17 The proce-dure should be timed for evaluation of the portal, genital,and renal veins, and separate imaging should be performedlater for evaluation of the pelvic and iliocaval veins. Pelvicvarices are imaged as dilated, tortuous, enhanced tubularstructures around the uterus and ovary, with possibleextension into the broad ligament and pelvic side wall.They can also involve the paravaginal venous plexus. Anovarian vein is considered incompetent if it is completelyopacified during the arterial phase of CT angiography.5

Time-resolved MRA (TR-MRA) is particularly helpfulfor the detection of PCS because of its ability to accuratelydetermine whether anterograde or retrograde flow in theovarian vein is present. Patients are placed in the supine po-sition with their arms at the side. Kim et al describe a pro-tocol for single-pulse TR-MRA whereby imaging of thepelvis is performed in the coronal plane at rapid 2- to 5-

second intervals for 1 to 3 minutes after peripheral intrave-nous injection of 0.1 mmol/kg body weight of nondilutedgadopentetate dimeglumine or gadobenate dimeglumineat a rate of 2 mL/s. This is followed by a saline bolus of20 mL at 2 mL/s. Maximum intensity projections ofeach three-dimensional data set are generated in both thecoronal and sagittal planes. Compared with T2/T2-weighted MRI, TR-MRV has a better image conspicuityto discriminate ovarian vein reflux. Most recently, the sensi-tivity, specificity, and accuracy of TR-MRA were comparedwith those of conventional venography, with resultsdemonstrating excellent agreement and the implicationthat TR-MRA is the best noninvasive means for diagnosisof pelvic venous reflux and PCS in symptomatic patients.14

One caveat to MRI, however, is that follow-up imagingwith magnetic resonance can be limited because of the arti-factual effects of embolization coils placed endovascularly.

Venography is the “gold standard” for diagnosis ofPCS; however, it should be reserved for concomitant inter-vention or if noninvasive imaging is equivocal (Fig 5). It isperformed under local anesthesia through the commonfemoral or basilic vein approach.11 Patients should havea urinary catheter inserted to prevent the bladder fromfilling with contrast medium and obscuring visualizationof the pelvic venous drainage. It should image the fourveins responsible for venous return from the pelvis: both in-ternal iliac veins and both gonadal veins. The study shouldbe performed with and without a Valsalva maneuver andpreferably with a tilt table in reverse Trendelenburg.1Venography should not be used as the primary imagingmode for diagnosis of PCS but rather reserved for patientswho have suspected PCS necessitating intervention basedon prior noninvasive imaging or in patients whose noninva-sive imaging is equivocal (Fig 4).

NONSURGICAL TREATMENT

Reports have varied in the literature over the decadeson nonsurgical treatment of PCS, including progestins,danazol, phlebotonics, gonadotropin-releasing hormone(GnRH) receptor agonists with hormone replacement ther-apy, dihydroergotamine, nonsteroidal anti-inflammatorydrugs, and psychotherapy. Whereas psychotherapy hasbeen used in conjunction with ovarian suppression to treatthe symptoms of PCS,18 there are no studies to show thatpatients who have been diagnosed with PCS will benefitfrom psychotherapy in isolation. For women with chronicpelvic pain of unknown etiology, the benefits of a multidis-ciplinary approach have been described, yet the utility of psy-chological intervention to alleviate pain is unclear.19

With medical therapy, the primary goal is either to sup-press ovarian function or to cause vasoconstriction ofdilated veins. Medroxyprogesterone acetate (MPA) andthe GnRH analogue goserelin have been used with limitedeffects (Table I). MPA may be given orally 30 mg/day for6 months. Goserelin acetate is dosed as an injection of3.6 mg monthly during a 6-month period.20 As chemicalovarian ligation has numerous side effects, estrogenreplacement therapy is frequently required as well.

Fig 5. Ovarian vein reflux. A, Selective left ovarian vein injectionshows free reflux into broad ligament. Maximum left ovarian veindiameter is 12 mm. B, Left ovarian vein is successfully embolized

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Although these therapies provide some relief, their effectsare transient, particularly those of MPA. Results fromstudies using daily oral administration of MPA demonstratevisual analog scale for pain score improvements whilereceiving therapy, but stable results 9 months after treat-ment were obtained only when psychotherapy was coadmi-nistered with MPA.18 This result reinforces earlyobservations on the close relationship between the psycho-logical and somatic symptoms of PCS. When GnRH ago-nists were compared with daily MPA, both showedefficacy, with GnRH agonists showing more improvementsin pain symptoms, depressive symptoms, and sexual func-tion 12 months after completion of treatment. Side effectsfrom progestins principally included bloating and anaverage 5-pound weight gain during 4 to 6 months;GnRH agonists were associated with hot flashes, nightsweats, vaginal dryness, and mood changes.8,18,20 Becauseof these side effects, combined with limited efficacy, med-ical therapy is not favored for long-term treatment ofPCS. Medical treatment also often diminishes fertility.On the other hand, for patients with mild to moderatesymptoms who would like to delay endovascular or surgicaltreatment, medical therapy is not an unreasonable choice.A discussion with the patient about the positives and neg-atives of medical therapy vs intervention with potentialadjuvant psychotherapy should be a part of treatment,addressing the side effects and benefits of these options.

SURGICAL TREATMENT

Surgical treatment of PCS is still an accepted therapyfor a select group of patients who have debilitating symp-toms, are acceptable surgical candidates, and are refractoryto medical or endovascular therapy. Surgery for ovarianreflux can provide symptomatic relief for patients, withthe drawbacks of scarring, morbidity, prolonged hospitalstay, and extended healing time (Table I). Extraperitonealresection of the left ovarian vein to treat PCS was first re-ported by Rundqvist et al21 in 1984. In properly selectedpatients, this operative method was shown to providesymptomatic improvement in two thirds of patients withPCS.10 In 2003, Gargiulo et al22 published the largest se-ries of laparoscopic transperitoneal ovarian vein ligation in23 women with a 1-year follow up. They reported com-plete resolution of the patient’s symptoms with this tech-nique. This technique involves accessing the right ovarianvein by incising the posterior peritoneum below the mesen-tericoparietal fossa and accessing the left ovarian vein byreflecting medially the left colon and incising the posteriorperitoneum covering the aorta below the inferior duodenal

to proximal aspect. Note circumaortic left renal vein (LRV). C,Right ovarian vein dilation and reflux shown on selective injection.Note small distal parallel ovarian vein channels (frequent finding).(Reprinted from Cordts PR, Eclavea A, Buckley PJ, DeMaioribusCA, Cockerill ML, Yeager TD. Pelvic congestion syndrome: earlyclinical results after transcatheter ovarian vein embolization. J VascSurg 1998;28:862-8, with permission from Elsevier.)

Table I. Summary of treatment modalities for pelvic congestion syndrome (PCS)

Study (year) Patients Treatment Time, months Complications% Clinicalimprovement

MedicalFarquhar et al (1989) 22 MPA vs MPA þ psychotherapy 9 Weight gain, bloating 73Reginald et al (1989) 84 MPA vs placebo 9 Weight gain, bloating 75Soysal et al (2001) 47 GnRH agonist 12 Hot flashes, mood swings,

night sweats65

EmbolotherapyEdwards et al (1993) 1 Coils 12 None 100Sichlau et al (1994) 3 Coils 22.8 1 recurrence 67Cordts et al (1998) 9 Coils, coils and gelatin 13.4 2 recurrences 88.9Maleux et al (2000) 41 Sclerosing agents 19.9 Glue migration 4% 58.5Venbrux et al (2002) 56 Sclerosing agents 22.1 3.6% coil migration;

5.4% recurred65

Scultetus et al (2002) 57 Coils, sclerosing agents, excision 25-288 None 75.4Pieri et al (2003) 33 Sclerosing agent 6.5 None 61Kim et al (2006) 127 Sclerosing agent and coils 45 5% recurrence 83Kwon et al (2007) 67 Coils 48 None 82Creton et al (2007) 24 Coils and phlebectomy 36 4.2% recurrence, 4.2%

coil migration76

Asciutto et al (2009) 35 Coils 45 None 47Castenmiller et al (2013) 43 Coils None 88Hocquelet et al (2013) 33 Coils 26 1 failure to catheterize 61Laborda et al (2013) 202 Coil 60 3% groin hematoma, 2%

coil migration, 0.5%reaction to contrast material

93.8

SurgeryRundqvist et al (1984) 15 Extraperitoneal resection of

left ovarian vein67.2 6.7% wound infection, 6.7%

bleeding73.3

Beard et al (1991) 36 Bilateral oophorectomy andhysterectomy

12 Infertility, morbidity, longhospital stay

67

Mathis et al (1995) 1 Transperitoneal laparoscopicligation of ovarian vein

None 100

Gargiulo et al (2003) 23 Transperitoneal laparoscopicligation of ovarian vein

12 Ileus, hematoma 74

GnRH, Gonadotropin-releasing hormone; MPA, medroxyprogesterone acetate.

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fold. The drawbacks to this procedure include higher sur-gical morbidity and several complications, such as deepvenous thrombosis, retroperitoneal hematoma, paralyticileus, and mechanical ileus caused by intestinal adhesion.Hospital stay and recovery time are also limiting factorswith surgical ovarian vein ligation.22 Surgery should beconsidered in patients with lifestyle-limiting symptomsthat have recurred despite embolotherapy.

EMBOLOTHERAPY

Since its introduction in 1993, transcatheter ovarian veinembolization (Fig 5) has become the mainstay for treatmentof PCS secondary to ovarian and pelvic venous incompe-tence.23 Several embolic agents, including sclerosantfoam,24 glue,7 Amplatzer plugs,25 and coils,2,11,26 havebeen described.Whereas a fairly large body of data regardingtranscatheter ovarian vein embolization exists, these studiesare limited to relatively small clinical series and retrospectivereviews. The success rates for the reduction of chronic pelvicpain in these studies range from 47% to 94% with averagefollow-ups of 12 to 36 months (Table I).

First described by Edwards et al23 in 1993 with a singlecase report of bilateral ovarian vein embolization, the tech-nique has grown and been used widely with relative

success. Sichlau et al27 reported on ovarian vein coil embo-lization in 1994, citing success in three patients with onerecurrence after 1 year. Cordts et al28 described ninewomen undergoing ovarian vein embolization in 1998,89% of whom demonstrated relief of symptoms with tworecurrences at 2 years of follow-up. Interestingly, no pub-lished data demonstrate a significant difference in out-comes between unilateral and bilateral ovarian veinembolization. A study of 41 patients using enbucrilateand lipiodized oil as embolic agents demonstrated a nearly60% total symptomatic relief with no difference in outcomebetween bilateral and unilateral ovarian vein embolization.7

Investigators have most commonly employed the visualanalog pain scale to measure severity of symptoms beforeand after embolotherapy. A larger series of 56 patients in2002 demonstrated a mean decrease in pain by 65% afterembolization.29 More recent studies have reported successby pain reduction at a rate nearing 85%.26 Some studiessuggest that ovarian vein embolization, followed by inter-nal iliac vein embolization at a later date, improves out-comes and prevents recurrence.2,29 Kim et al30 found an83% improvement in symptoms in 131 patients with amean follow-up of 45 months, of whom 85% received in-ternal iliac vein embolization. Venbrux et al29 reported

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similar results with ovarian vein embolization succeeded byinternal iliac vein treatment and a follow-up of 38 months.Of note, patients with isolated ovarian vein reflux had out-comes superior to those of patients with isolated internaliliac vein reflux or combined disease.1,2 Ovarian vein embo-lization did result in a significant improvement of symp-toms for those with isolated ovarian reflux, but theresults of embolization with combined reflux did not reachstatistical significance.2

Patients with vulvar and lower extremity varicositieshave been studied with regard to regression of varicoseveins after embolotherapy for PCS. There has been moresuccess in the regression of vulvar than of lower extremityvaricosities after ovarian vein embolization.12,31 To date,there has been no large study that demonstrates a signifi-cant change in clinical, etiologic, anatomic, and pathologic(CEAP) classification after embolotherapy for PCS,although several studies report >80% reduction in vulvarvaricosities and symptoms after embolization for ovarianvein insufficiency.

Right femoral vein access is the most commonapproach for venography and embolization, althoughtransjugular, basilic, and transbrachial approaches havebeen reported with technical success. The average numberof coils per vein has been reported at six, with spring coilsbeing the most common form of embolization.11

The technique of transcatheter embolotherapy forovarian and pelvic varices is straightforward, although thereis some variation in the literature. With a femoral or rightinternal jugular approach, a 6F sheath is guided into thevena cava. For assessments of the left or right ovarianvein, access is facilitated with a Cobra or Sim 1 catheter.Once access is obtained, the sheath is guided into the renalvein for coaxial support. Next, with use of a glide wire andglide catheter, the ovarian vein is catheterized. An injectionof 10 mL of contrast material with the patient in the reverseTrendelenburg position is performed. Incompetent ovarianveins will show venous dilation and reflux of contrast mate-rial into the pelvis. The guidewire and catheter should thenbe moved down the ovarian vein to just above the pelvicbrim. Another injection confirms reflux of contrast materialinto the pelvic veins, cross-pelvic collaterals, and any thighor vulvar varicosities.

Modern embolotherapy of the main trunk of the ovarianvein or the iliac veins is facilitated by a microcatheter systemand microcoils. In general, framing coils are placed first, fol-lowed by gel coils to promote venous thrombosis. Sclero-therapy of the hypogastric veins can be used adjunctively.With a balloon occlusion technique, sclerosants such as so-dium tetradecyl sulfate are injected (Fig 2).

One major drawback to coil embolization is undoubt-edly coil migration into the pulmonary system, which hasbeen reported in 2% of patients after coiling of the internaliliac vein.32 Larger caliber veins (>12 mm) increase the riskof this complication. To prevent coil migration to the pul-monary artery, the diameter of the coils should be at least30% or 50% larger than the diameter of the left internal iliacveins.32 Other complications include perforation of the

ovarian vein, flank pain, postprocedural fevers, and veni-puncture site hematomas.29,30

For male patients with varicocele or female patientswith mild vulvar varices, sclerotherapy has proved to bean effective treatment. Several groups in Europe use sclero-sants exclusively for embolization of the spermatic vein inmen with varicocele.33 The most frequently used sclero-sants are 3% to 5% sodium tetradecyl sulfate (liquid orfoam) and polidocanol. A catheter is placed into the reflux-ing segment of the spermatic vein and injected with 3 or4 mL of sclerosant during a Valsalva maneuver. Depositionof sclerosant in the pelvic segment of the vein is more effec-tive than at a lumbar level. The catheter is then left at theorifice of the spermatic vein for 2 or 3 minutes to minimizeegress of the sclerosant.1 Scultetus et al6 reported successwith local excision and sclerotherapy for women withvulvar varices, whereas a paper from Australia reports suc-cessful US-guided foam sclerotherapy of vulvar varicositiessecondary to ovarian vein reflux.24

Ultimately, there remains an uncertainty as to theoptimal technique for ovarian vein embolization, althougha combination of coils and sclerosants has demonstratedclinical efficacy in a number of studies described beforeand is the most common published technique for ovarianvein embolization (Table I). There is currently no evidenceto suggest a difference in symptomatic relief with regard tounilateral vs bilateral ovarian vein embolization.7,30 Kim et alreported 83% clinical improvement with bilateral ovarianembolization followed by embolization of varicosities fromthe internal iliac vein 4 to 6 weeks later. On the basis ofthe current literature, the decision to treat one or bothovarian veins should depend on the severity of symptoms,the anatomy of the pelvic varicosities, and the degree ofreflux in each ovarian vein. A patient with a high degreeof reflux bilaterally and a number of bilateral varicositiesnetworking with the internal iliac veins would likely benefitfrom bilateral embolization, whereas a patient with left-sideddilation and reflux with moderate varicosities may benefitfrom unilateral embolization. Clinical judgment and experi-ence should be used to guide embolotherapy in conjunctionwith symptoms, anatomy, and functional studies.

NUTCRACKER SYNDROME

For patients with PCS secondary outflow obstructionfrom nutcracker syndrome, treatment should focus onrelieving anatomic compression rather than embolizationof outflow tracts. Diagnosis of nutcracker syndrome, asdescribed before, is based on a constellation of factors,including symptoms of pelvic congestion as well as flankpain, microhematuria, and suggestive imaging. An acute nar-rowingof theLRV, termed the beak sign, has a high sensitivityfor diagnosis of nutcracker syndrome.17 In addition, the ratioof the LRV at its narrowed vs its dilated portion is often usedas a diagnostic criterion, with the high end of normal beingaround 4:1; one study demonstrated that a value>4.9 corre-lates strongly with the presence of LRV compression.17 Inaddition, there is conflicting evidence regarding the utilityof LRV pressure gradients in the diagnosis of nutcracker

Table II. Summary of treatment modalities for nutcracker syndrome

Study (year) Patients Treatment Time, months Complications% Clinical

Improvement

EndovascularWei et al (2003) 1 Stent 3 None 100D’Archambeau et al (2004) 40 Embolization N/A 4% failure to catheterize 75Hartung et al (2005) 5 Stent 14.3 40% stent migration 40Kim et al (2005) 1 Stent 24 None 100Basile et al (2007) 3 Stent 16 None 100Zhang et al (2007) 20 Stent 15 6.7% migration 100Cohen et al (2009) 1 Stent 12 In-stent restenosis Required bypassChen et al (2011) 61 Stent 66 3.2% stent migration,

1.6% maldeployment,1.6% IVC protrusion

96.7

Wang et al (2012) 30 Stent 36 6.7% migration 100Surgery

Thompson et al (1992) 1 SMA transposition 12 None 100Shokeir et al (1994) 2 Autotransplantation 12 None 100Hohenfellner et al (2002) 8 LRV transposition 66.4 8.3% hematoma, 8.3% DVT 87.5Shen et al (2004) 2 LRV transposition 3 None 100Wang et al (2009) 7 LRV transposition 42.6 14% ileus, 29% hematoma 85.7Viriyaroj et al (2009) 1 Transperitoneal laparoscopic

gonadal vein ligation12 None 100

Marone et al (2011) 1 LRV anterior transposition 6 None 100Gong et al (2012) 3 Left spermatic vein ligation,

iliac vein anastomosis3 None 100

Li et al (2012) 1 Abdominal aortic transposition 38 None 100Xu et al (2013) 2 Laparoscopic inferior

mesentericegonadal vein bypass3 None 100

DVT, Deep venous thrombosis; IVC, inferior vena cava; LRV, left renal vein; N/A, not applicable; SMA, superior mesenteric artery.

JOURNAL OF VASCULAR SURGERY: VENOUS AND LYMPHATIC DISORDERS104 O’Brien and Gillespie January 2015

syndrome as studies have classified it as compensated ornoncompensated, with the compensated syndrome havingpressure gradients within normal ranges and the noncompen-sated syndrome being characterized by LRV hypertension.The normal pressure gradient between the LRV and IVC is1 mm Hg or lower, and various studies have identified LRVhypertension as a gradient >3 mm Hg.4 Therefore, whereasLRV hypertension is not diagnostic of nutcracker syndrome,measuring the pressure gradient during venography andstenting does provide a way to monitor the technical successof the procedure and has utility in some cases.

Surgery for nutcracker syndrome has been reportedwidely (Table II). For young patients in good health with se-vere pain and persistent symptoms, open surgery has advan-tages of success and durability. LRV transposition,34 SMAtransposition,35 abdominal aortic transposition,36 and infe-rior mesenteric veinegonadal vein bypass37 have all beendescribed with relative success, although in small numbers(Table II). Surgical morbidity and renal ischemia time areboth drawbacks to open surgery. More recently, severalstudies have reported success in relieving the symptoms ofnutcracker syndrome by stenting the LRV.

LRV stenting for treatment of nutcracker syndromeand PCS was first described in 2005,38 and since then,studies primarily from China have demonstrated the safetyand efficacy of the procedure, particularly with self-expanding nitinol stents (Table II).9,39,40 Follow-up timeshave not exceeded 1 year. Although deployment of a renalstent is less invasive than open surgery, the postoperative

complications can include stent migration into the rightatrium, stent protrusion into the IVC, and stent migrationinto the hilar LRV.9,40 Wang et al recommended stentoversizing by 20% based on the diameter of the LRV atthe hilum as measured on US and MRI. Smaller stents,particularly 10- and 12-mm stents, are more likely tomigrate, according to Hartung et al.38 Chen et al9 recom-mended a stent length of 60 mm for stability within thefirst large branch of the LRV, postulating that if migrationdoes occur, the proximal end of the longer stent will prob-ably stay at the opposite wall of the IVC with the distal endremaining at the stenotic segment of the LRV to preventmigration into the heart.

The decision of open surgery vs endovascular treatmentfor nutcracker syndrome is also undoubtedly based on anat-omy. With anterior nutcracker syndrome, in which the LRVis compressed between the SMA and aorta, stenting mayprove to be sufficient to relieve symptoms. However, inthe case of posterior nutcracker syndrome, in which theLRV is compressed between the aorta and a vertebralbody, transposition may be necessary to relieve the obstruc-tion.4 Ultimately, surgery can provide a long-term solution.The outcomes for endovascular treatment are encouraging;however, stents are not permanent solutions, and for thisreason, in very young patients, surgery may be preferred.

CONCLUSIONS

The diagnosis and management of PCS continue toevolve. PCS remains an underdiagnosed cause of chronic

JOURNAL OF VASCULAR SURGERY: VENOUS AND LYMPHATIC DISORDERSVolume 3, Number 1 O’Brien and Gillespie 105

pelvic pain because of the difficulty in identifying varicositiesand ovarian vein reflux in patients who are supine. Thesymptoms of PCS are often lifestyle limiting, consisting ofchronic lower abdominal pain exacerbated by sitting orstanding, dyspareunia, dysuria, vulvar and lower extremityvaricosities, and pelvic tenderness. Nutcracker syndromecan also be manifested with hematuria and flank pain.Vascular surgeons in academic and community settingscan improve the diagnosis of PCS by educating primarycare physicians through in-service or continuing educationseminars on the signs and symptoms of PCS. Because PCSis frequently a diagnosis of exclusion, an awareness of itsprevalence within the primary care patient population canultimately increase the referral of appropriate patients tovascular specialists for further workup. Diagnosis of PCS re-quires a careful history, physical examination, and noninva-sive imaging with either transvaginal or transabdominal USwith color duplex imaging to visualize dilated and tortuousovarian veins. MDCT with three-dimensional reconstruc-tion and time-resolved MR angiographies are also usefulnoninvasive tests with the advantage of providing informa-tion about the surrounding anatomy as well as functionalinformation about retrograde flow. Once diagnosed, pa-tients with PCS should be offered embolotherapy as a pri-mary treatment option. Although conservative medicaltherapy with MPA or GnRH agonists has been reported,its effects are limited. Whereas the data in favor of embo-lotherapy are limited to a number of clinical series, success-ful reduction in pelvic pain can be achieved in 70% to 85%of patients who undergo embolization. Coil embolizationof one or both ovarian veins with sclerosing therapy ofbranching varicosities is the most widely reported andminimally invasive technique for alleviation of the symp-toms of PCS due to gonadal vein reflux. For patientswith outflow obstruction due to nutcracker syndrome, alimited number of studies have demonstrated remissionof symptoms with stenting of the LRV. Open surgeryinvolving renal vein transposition carries high success ratesbut should be reserved for patients who are young andsuitable surgical candidates.

AUTHOR CONTRIBUTIONS

Conception and design: DGAnalysis and interpretation: DG, MOData collection: DG, MOWriting the article: DG, MOCritical revision of the article: DG, MOFinal approval of the article: DGStatistical analysis: Not applicableObtained funding: Not applicableOverall responsibility: DG

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Submitted Nov 4, 2013; accepted May 25, 2014.

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Diagnosis and treatment of the pelvic congestion syndromeAnatomyPathophysiologyPresentationImagingNonsurgical treatmentSurgical treatmentEmbolotherapyNutcracker syndromeConclusionsAuthor contributionsReferences