Version May 2024
INTRODUCTION — The pelvic venous syndromes, which include pelvic congestion syndrome and vulvar varicosities, are poorly understood disorders of the pelvic venous circulations.
●Pelvic congestion syndrome (PCS) is characterized by chronic pelvic discomfort exacerbated by prolonged standing and coitus in women who have periovarian varicosities on imaging studies. The etiology of PCS is unclear and the optimum treatment is uncertain. Development of an evidence-based approach to managing these patients has been limited by the absence of definitive diagnostic criteria.
●Vulvar varicosities result from venous obstruction, increased venous pressure, and venous insufficiency, most commonly during pregnancy. They may be isolated or associated with varices of the lower extremity, and they may occur as part of PCS.
This topic will discuss the clinical presentation, evaluation, and management of pelvic venous disease. Related content on venous disease is presented separately.
●(See "Pathophysiology of chronic venous disease".)
●(See "Overview of lower extremity chronic venous disease".)
In this topic, when discussing study results, we will use the terms "woman/en" or "patient(s)" as they are used in the studies presented. However, we encourage the reader to consider the specific counseling and treatment needs of transgender and gender expansive individuals.
VULVOVAGINAL VARICOSITIES
Epidemiology — It is estimated that 4 percent of women have had vulvar varicosities [1]. They usually occur during pregnancy and typically regress spontaneously within six weeks postpartum. They are rare in nulliparous women. When they occur in nonpregnant women, they generally present in the second or third decade of life [2].
Pathogenesis/pathology — Vulvar varicosities are dilated venous channels that probably develop from a combination of proximal venous obstruction and valvular incompetence, which results in increased venous pressure. Their anatomy has been defined by direct injection and surgical dissection of the varices.
The vascular drainage of the female external genitalia includes the dorsal superficial clitoral, bulbovestibular, profundus clitoral, and posterior labial veins. Because these perineal veins do not have valves, they are susceptible to the development of varices. Up to one-half of vulvar varicosities arise from an incompetent great saphenous vein, which drains the superficial and deep external pudendal veins and posteromedial tributaries.
Insufficiency of the internal iliac and ovarian veins may also contribute to development of varicose veins over the territories of their main tributaries (ie, the internal pudendal and obturator veins). Such varices can be large, have frequent anastomoses, and involve the vulva and posteromedial aspect of the thigh [3-6]. Rarely, vulvar varices arise from the round ligament vein, mimicking varicocele in the male [1,7].
Vulvar varicosities are most common in pregnant women because anatomic and physiologic changes associated with pregnancy result in pelvic venous congestion. Proposed mechanisms include (1) increased pelvic blood flow, which impairs venous return via the femoral veins and thus contributes to venous congestion in the lower limbs and pelvis, and (2) mechanical compression of the inferior vena cava/iliac veins by the gravid uterus, which increases venous pressure distally [6,8-10]. In addition, hormonal changes likely contribute by causing vasodilation.
Vulvar varices in nulliparous women are also caused by local venous insufficiency and incompetence; genetic factors likely play a role in predisposition to these venous changes. Rarely, vulvar varicosities can result from pelvic tumors that limit venous drainage [11].
Clinical manifestations — Women with vulvar varices are often asymptomatic [1]. Symptomatic women may describe vulvar discomfort, swelling, and pressure that are exacerbated by prolonged standing, exercise, and coitus. In nonpregnant women, symptoms may be aggravated by menses and may represent a manifestation of PCS [1,3,12-15].
Diagnosis — The diagnosis of vulvar varicosities is based on physical examination (picture 1). Patients should be examined in both the supine and upright positions, since the latter enhances filling of the veins. The varicosities appear as purple-blue distensible folds in the vaginal mucosa and labia or as a grape-like cluster of veins ("bag of worms") in the enlarged vulva (figure 1) [8,9,16]. They may be isolated or associated with varices of the buttocks, thigh, or lower leg.
There is no consensus on indications for imaging. Imaging is not necessary to confirm the diagnosis, which is evident on physical examination, but may be useful in therapeutic planning. For example, if surgery is planned for treatment of the varicosities, most vascular surgeons would obtain duplex ultrasound studies of the lower extremity veins (even in the absence of lower leg varicosities) to determine whether connections to the saphenous system are present, as these connections would alter the treatment approach. (See "Diagnostic evaluation of lower extremity chronic venous disease", section on 'Venous duplex ultrasound'.)
Some clinicians perform magnetic resonance (MR) imaging or computed tomography (CT) to evaluate pelvic and gonadal veins in all patients with vulvar varicosities, and, if imaging is positive, they perform venography if an intervention such as coil embolization is planned.
Others perform pelvic venography in all patients prior to any invasive therapy since vulvar varicosities caused by incompetence of ovarian veins and/or internal iliac veins may recur after local therapy, thus this information may change the primary or secondary treatment approach [1,3,4,6,17-19]. As an example, in a series of 25 women with vulvar varices (with and without PCS) who underwent transjugular venography, 15 women (60 percent) had left ovarian vein reflux and thus underwent coil embolization rather than local therapy [4]. All had complete resolution of symptoms without any procedure related complications (access infection, coil migration or venous perforation).
If vulvar varicosities are associated with chronic pelvic pain, pelvic imaging (eg, MR, CT, venography) should be performed as part of the diagnostic evaluation for PCS [1,3-7]. (See 'Diagnostic evaluation' below.)
Differential diagnosis — Differential diagnosis of vulvar varices includes the following [15]:
●Venous malformations, which are rare, congenital, dysmorphic vessels in the vulva. They typically present as bluish, easily compressible, nonpulsatile masses that increase in size with maneuvers that increase venous pressure. Unlike vulvar varicosities, venous malformations are often symptomatic (particularly during menses or pregnancy) and slowly grow over the patient's lifetime; they do not regress [8,16,20,21]. In addition, they can be complicated by spontaneous intralesional thrombosis.
Doppler ultrasound, magnetic resonance imaging, and direct injection venography have been used to distinguish between varicosities and venous malformations, but an imaging study is only required to confirm the diagnosis if invasive treatment is planned.
●Hematoma, which should be suspected in the setting of recent trauma. (See "Evaluation and management of female lower genital tract trauma" and "Management of hematomas incurred as a result of obstetric delivery".)
●Hernia, which is characterized by absence of blue/purple coloration and by increased bulging when the patient bears down or coughs. (See "Classification, clinical features, and diagnosis of inguinal and femoral hernias in adults".)
●Soft-tissue neoplasm. (See "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers".)
●Hemangiomas [20,22-25] usually present within the first few months of life and can grow rapidly. They appear as superficial red papules or nodules, or as deep raised skin-colored nodules, which often have a bluish/black hue. Hemangiomas that persist into adulthood may cause symptoms due to periodic thrombosis. Visual inspection and history can differentiate between varicosities and hemangiomas. Imaging is indicated in selected cases. (See "Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and complications".)
●Arteriovenous malformation – These lesions increase in size over time due to arterial inflow and almost always require some type of ablative therapy.
●Bartholin gland cyst, which appears as a soft, painless mass in the area of the Bartholin gland (ie, medial labia majora or lower vestibular area). (See "Bartholin gland masses".)
Complications — Occasional complications include deep and superficial thrombophlebitis, ulceration, and hemorrhage [8,10].
Management — Treatment depends on whether vulvar varicosities are present in isolation or associated with leg varicosities and/or PCS.
Pregnant women — During pregnancy, noninvasive therapy is preferred because regression commonly occurs postpartum [10,26]. There are no data to support specific therapies or one therapy over another. Supportive therapies that have been suggested include vulvar support and compression (pelvic supporter), leg elevation, compression with support hose, sleeping on the left side, exercise, and avoidance of long periods of standing or sitting [15].
Vulvar varicosities are not a contraindication to vaginal delivery. Theoretically, a laceration of large vulvovaginal varicosities could result in significant hemorrhage, but no such hemorrhages have been reported in the literature. This may be because obstetricians advise patients with massive varicosities to undergo cesarean delivery or because the varicosities collapse after delivery and their low blood flow allows bleeding to be readily controlled.
Rarely, thrombosis of vulvar varicosities occurs during pregnancy. Conservative treatment with oral analgesics is usually effective but, local excision of the thrombus may be considered [10]. (See "Approach to treating symptomatic superficial venous insufficiency", section on 'Surgical options'.)
Sclerotherapy — The treatment of symptomatic varices that persist for more than three months postpartum or arise in the nonpregnant state is based on size and symptoms. As with lower extremity varicosities, vulvar varices can be treated with sclerotherapy, ligation and excision, or embolization [3,4,9,17,19,27,28]. These techniques are described elsewhere. (See "Injection sclerotherapy techniques for the treatment of telangiectasias, reticular veins, and small varicose veins" and "Approach to treating symptomatic superficial venous insufficiency", section on 'Intervention for axial vein reflux'.)
The approach to treatment of vulvar varicosities is derived from case reports and small series; no randomized trials have been performed. Based on expert opinion, sclerotherapy is the preferred treatment for isolated vulvar varicosities with no evidence of great or small saphenous vein obstruction or reflux on lower extremity duplex ultrasonography. More commonly, there is evidence of saphenous venous insufficiency with duplex ultrasound and evidence of direct communication from the saphenofemoral junction to the vulvar varicosities. In these cases, the leg veins are generally treated first and the treatment response of the vulvar varices is assessed. Local sclerotherapy can be utilized as adjunctive therapy for vulvar varices, if necessary. (See "Overview of lower extremity chronic venous disease" and "Diagnostic evaluation of lower extremity chronic venous disease", section on 'Venous duplex ultrasound'.)
●Procedure – A solution of sodium tetradecyl sulfate (or other sclerosant [eg, polidocanol]) is injected directly into the varices; the concentration of the solution depends on the size of the veins. The patient wears a compression girdle for 48 hours after each treatment session and usually at least two to three treatment sessions are required. Patients are typically uncomfortable for three to four days, but pain can be managed adequately with oral analgesics.
The injected varicosities become firm and fibrotic after sclerotherapy, with improvement in pain, pressure, and swelling within seven days of treatment. Case series have described marked improvement (100 percent response) at 6- to 12-month follow-up [3,9].
Thromboses within the varices can occur if they are not compressed adequately after injection. The treatment is microthrombectomy. Other potential complications include allergy to the medication and ulceration or hyperpigmentation of the skin over the varicosities, but these complications have not been reported.
Ligation — Surgical treatment for vulvar varices by ligation of incompetent veins and perforators requires multiple incisions, but can provide symptomatic relief. However, dyspareunia and cosmetic disfiguration secondary to perineal scarring are potential future complications [27,28].
Isolated vulvar varicosities can be treated with so-called "stab" or "ambulatory phlebectomy," which utilizes very small 1 to 2 mm skin incisions through which sequential venous segments are avulsed. Surgical ligation is not performed because the incisions are too small; instead, hemostasis is achieved by compression with a bandage or stocking.
Ablation — Laser or radiofrequency ablation is rarely possible, as varicosities of the vulva generally do not meet criteria for these treatment modalities because of the size and location of the veins. (See "Laser and light therapy of lower extremity telangiectasias, reticular veins, and small varicose veins" and "Techniques for radiofrequency ablation for the treatment of lower extremity chronic venous disease".)
PELVIC CONGESTION SYNDROME
Relevant pelvic venous anatomy — Pelvic congestion syndrome (PCS) is a medical condition that can cause chronic pelvic pain but has not been fully characterized. PCS has been associated with venous reflux, cranial (proximal) iliocaval stenosis, and/or obstruction of the gonadal, gluteal, periuterine, or perineal veins. It can also be caused by meso-aortic compression of the left renal vein (LRV) between the superior mesenteric artery and the aorta (meso-aortic angle <15 degrees, commonly referred to as Nutcracker syndrome) [29], secondary to retro- or circumaortic LRV [30], or agenesis of the common or external iliac veins [31]. Although a genetic disorder may be a risk factor, familial cause and multiparity are commonly associated in the development of PCS.
There is venous drainage of both ovaries and uterus through both internal iliac veins (IIV) and gonadal veins. Tributaries of IIV are divided into parietal and visceral veins.
●Parietal tributaries include the superior and inferior gluteal, sciatic, sacral, ascending lumbar, and obturator veins.
●Visceral tributaries include the internal pudendal, middle hemorrhoidal, uterine, gonadal, and vesicovaginal plexuses.
Understanding the uniqueness of that anatomy is critical in understanding the possible causes of PCS, such as compression on the left IIV by overlying right common iliac artery as in May-Thurner syndrome (MTS), which may propagate reflux and initiate PCS. Reflux in the left ovarian vein (LOV) is more common than in the right as the latter drains directly into IVC. Also, compression of LRV between superior mesenteric artery and aorta can generate severe reflux of the LOV.
Epidemiology — It is difficult to establish the true incidence of PCS given the lack of definitive diagnostic criteria. It primarily affects multiparous women in the reproductive age group [1,13,14,32]. No cases have been reported in menopausal women [1,13,33]. The prevalence of PCS is up to 30 to 40 percent in patients presenting with chronic pelvic pain in whom no other obvious pathology can be found [12,32,34-36].
Pathophysiology — The etiology of PCS is unclear. Multiple investigators have observed gross dilatation, incompetence, and reflux of the ovarian veins in women with PCS and have thus attributed PCS to underlying venous pathology [1,12,32,37,38]. In this model, anatomic and/or hormonal factors lead to venous insufficiency of the ovarian veins and/or internal iliac veins, resulting in periovarian pelvic varicosities [1,17,36,39,40]. Tuboovarian varicoceles have been termed the female equivalent of testicular varicocele [12,38,41].
●Anatomic contributors – The main underlying anatomic causes of PCS are thought to be venous reflux, obstruction, or a combination.
•Reflux – Venous reflux likely results from the relative infrequently of venous valves combined with hormonal factors that promote venodilation (eg, increased levels of estrogen during pregnancy), both of which may contribute to PCS and resultant pelvic pain. As evidence, one study of 35 patients reported the injection of dihydroergotamine led to a significant reduction in diameter of the pelvic veins and reduced associated pain [42].
•Obstruction – Venous outflow obstruction may be secondary to venous compression. Examples include May-Thurner syndrome (MTS), which results from compression of iliac veins/IVC by overlying iliac arteries, and Nutcracker syndrome, which can be caused by compression of the LRV between the superior mesenteric artery and aorta or secondary to retro-aortic left renal vein. Venous compression may significantly increase the intravascular pressure which, in turn, may cause more dilated and tortuous veins that then exacerbate valvular incompetence, resulting in more distended and tortuous veins.
An additional discussion of MTS syndrome is presented separately. (See "May-Thurner syndrome".)
●Role of pregnancy – The higher prevalence of PCS in multiparous women may be related to the 50 percent increase in pelvic vein capacity during pregnancy, which may lead to venous incompetence and reflux in the nonpregnant state [1]. The increased frequency of PCS symptoms on the left side may be due to extrinsic compression of the left renal vein between the aorta and superior mesenteric artery ("nutcracker phenomenon"; ie, pelvic congestion, left flank pain, and hematuria) [43-45], or because valvular incompetence of the ovarian vein due to absent ovarian vein valves is more common on the left [46]. In asymptomatic women, ovarian vein valves are missing in 15 percent of women on the left side and 6 percent of women on the right, and nearly one-half of these women have valvular incompetence on at least one side.
●Estrogen – The absence of PCS in menopause has been attributed to the decline in estrogen, which acts as a venous dilator. This hypothesis is supported by observations that pharmacologic or surgical induction of a hypoestrogenic state may result in improvement or resolution of symptoms [1,4,47,48].
•Etiology of pain – Ovarian vein dilatation, stasis, and/or reflux on pelvic venography are common findings in multiparous premenopausal women, and most of these women are asymptomatic [12,41]. Why these findings are associated with chronic pelvic pain in some women, but not in others, is unclear. A causal relationship has not been proven, but is supported by limited data showing pain relief upon administration of venoconstrictors or ovarian vein ligation/embolization. For example, a trial that randomly assigned 12 women with PCS to treatment with dihydroergotamine (a selective venoconstrictor) or saline placebo during an acute attack of pain noted that narrowing of dilated pelvic veins and reduction of venous congestion on venography was associated with diminution in pain [39]. Other small studies of women with chronic pelvic pain, venous congestion, and reflux by either Doppler ultrasound and/or venography and no evidence of pelvic pathology at laparoscopy have reported improvement in pain after sclerotherapy, embolization, or venous ligation [18,40,49-52]. (See 'PCS with vulvar varices' below.)
Clinical presentation — PCS is characterized by pelvic pain of at least six months duration. PCS can cause chronic dull aching pelvic pain characterized by the "three Ds" (dysmenorrhea, dysuria, and dyspareunia). PCS usually presents in the second or third decade of life and carries a significant psychological and anxiety burden that is often associated with vulval and gluteal varicosities. Pain usually worsens through the day, and is exacerbated by standing, straining, coitus, or pregnancy. The patients may present with abdominal wall tenderness and gross varicosities in the lower abdomen, vulva, and gluteal regions. The patient may also complain of sharp exacerbations of pain, dysmenorrhea, deep dyspareunia, and urinary urgency. Gluteal, vulvar, and/or thigh varices may also be present [1,3]. Patients with proximal compression syndromes, such as MTS or Nutcracker, may present with limb swelling or hematuria, respectively.
Diagnostic evaluation — There are no definitive diagnostic criteria for PCS. In general, the diagnosis is based on a combination of characteristic symptoms, tenderness on physical examination, and documentation of pelvic vein dilatation or incompetence, after exclusion of other causes for these nonspecific findings. The general approach to evaluation of women with chronic pelvic pain is discussed in detail separately. (See "Chronic pelvic pain in adult females: Evaluation".)
Physical examination — In a patient with characteristic symptoms, the diagnosis is supported by bimanual examination showing marked ovarian tenderness, cervical motion tenderness, and uterine tenderness with direct palpation. In an observational study including 57 women with pelvic pain, the combination of tenderness on abdominal examination over the adnexa and history of postcoital ache was 94 percent sensitive and 77 percent specific for discriminating PCS from other pelvic pathologies [13,32].
Imaging — Imaging is performed to document the characteristic pelvic venous changes, which support the diagnosis. Although the absence of pelvic venous changes helps to exclude PCS, the presence of these abnormalities is not diagnostic, as incompetent and dilated ovarian veins are common, nonspecific findings. In one study, they were seen on computed tomography in 47 percent of asymptomatic multiparous women [41]. In another study, 10 percent of 273 healthy female kidney donors who underwent preoperative abdominal aortography before left nephrectomy had retrograde flow in an incompetent left ovarian vein [53]. Approximately 40 percent of the women with ovarian varices did not have PCS symptoms.
In addition, although dilatation of the ovarian vein is necessary but not sufficient for diagnosis, there is no consensus on the optimum cut-off for ovarian vein diameter in PCS, and no validated measure for venous congestion or tortuosity [54].
Relevant imaging tests include:
Pelvic ultrasound — Pelvic ultrasonography is typically the first imaging study performed as it can exclude pelvic neoplasms and uterine pathology as potential causes of pain. For patients with suspected PCS, this dynamic tool can provide direction of flow, detect varicosities, anatomic characterization, and determine the localized area of concern. Limitations of ultrasound include the technology itself, skill and experience of the operator, and patient characteristics such as obesity or significant overlying bowel gas.
●Technique – Pelvic ultrasound is performed while the patient is fasting and after one day of a no-residue diet with both transabdominal 5 MHz and transvaginal probes. To uncover underlying reflux, the pelvic ultrasound study should be performed in standing or steep reverse Trendelenburg position. A Valsalva maneuver may help to examine reflux of ovarian veins. In addition, the pelvic ultrasound study should evaluate reflux in common femoral veins and reflux in saphenofemoral junction as well. However, absence of deep lower extremity reflux does not preclude the possibility of having PCS.
Ultrasound may not detect venous changes in affected women because imaging is generally performed with the patient in the supine position when the veins are flaccid. Sensitivity is higher if the study is performed with the patient in the upright position or by asking her to Valsalva. Despite these maneuvers, there is still a poor correlation between ultrasound findings and venography for the presence or absence of pelvic varices [1].
●Criteria for varices – We confirm PCS using the ultrasound findings of ovarian vein diameter of ≥6 mm, slow blood flow (<3 cm/second), and retrograde venous blood flow in the left ovarian vein combined with the clinical vulval varicosities with the three Ds (dysmenorrhea, dysuria, and dyspareunia). The criteria for pelvic ultrasound diagnosis of varices include the visualization of dilated ovarian veins >6 mm (image 1), although 7 mm has also been suggested as a cutoff. One study reported a positive predictive value of 83.3 percent for a 6 mm diameter ovarian vein for the diagnosis of PCS [55]. A meta-analysis of six studies comparing transvaginal ultrasound with venography as the standard for the diagnosis of PCS reported sensitivity and specificities ranging from 83 to 100 percent for the findings of dilated uterine veins >5 mm and pelvic varicoceles [56].
Additional ultrasound findings include dilatation of the left ovarian vein with reversed caudal flow, presence of tortuous and dilated pelvic venous plexuses, dilated arcuate veins crossing the uterine myometrium, and variable duplex waveform in varicoceles during the Valsalva's maneuver [1,12,13,32,40,55,57].
●Other findings – Dilated pelvic veins in the adnexal regions or in the outer one-third of the myometrium (ie, arcuate veins) are common findings on pelvic sonograms. Dilated arcuate veins have no clinical significance, unless associated with other dilated pelvic veins in women with chronic pelvic pain. Focal dilation of these veins could be due to prior instrumentation, trauma, or complications of pregnancy.
Computed tomography — Computed tomography (CT) venography can offer useful anatomic information in the diagnosis of PCS, especially to examine the possibility of venous compression disorders such as May-Thurner and Nutcracker syndromes. Although CT is not dynamic and does not reflect volumetric variations during the cardiac cycle. CT venography with three-dimensional reconstruction may help with mapping out all anatomic consideration before endovascular intervention. An ovarian vein is considered incompetent if it is completely opacified during the arterial phase of CT angiography.
Magnetic resonance angiography — Time-resolved MRA (TR-MRA) may be beneficial in determining anterograde versus retrograde flow in the ovarian veins.
Venography — Although venography is the "gold standard" for diagnosis of PCS, it is typically performed only at the time of endovascular intervention.
When performed, many investigators recommend selective ovarian and internal iliac venography in patients with symptoms consistent with PCS, with or without vulvar varicosities [1,3,4,6,17-19]. The procedure involves catheterization of the right and left ovarian veins via a percutaneous femoral or jugular approach. Ovarian venograms in patients with PCS usually demonstrate abnormally dilated ovarian veins (>10 mm in diameter), sluggish blood flow, reflux causing retrograde fill and congestion of the ovarian venous plexus, tortuosity of venous plexuses, uterine venous engorgement, and filling of pelvic veins across the midline (image 2 and image 3) [1,4,12,17,36,38,40,57,58].
Venous reflux often occurs into the thigh with upright positioning and Valsalva. There also may be evidence of internal iliac vein dilatation and reflux. Depending on the population studied, as many as 80 percent of premenopausal women undergoing venography have pelvic varicosities and venous stasis [12,37,39,40,53]. Some of these women are asymptomatic, while others have chronic pelvic pain with no other identifiable etiology for their symptoms.
Venography is probably more sensitive than ultrasonography since it can be performed in the upright or semi-upright position with a tilting table. The Society for Vascular Surgery (SVS) and the American Venous Forum (AVF) clinical practice guidelines recommend retrograde ovarian and internal iliac venography as the test of choice for the diagnosis of pelvic venous disorders. In addition to making the diagnosis, it provides an opportunity for concurrent therapeutic transcatheter embolization or sclerotherapy (image 4) [58]. (See 'Management of PCS' below.)
Pelvic vein pressures measured at venography have not been correlated with symptoms.
Differential diagnosis — The differential diagnosis of chronic pelvic pain is long. The causes and evaluation of chronic pelvic pain are discussed in detail separately. (See "Chronic pelvic pain in nonpregnant adult females: Causes" and "Chronic pelvic pain in adult females: Evaluation".)
Treatment options — There is no single approach to treatment of PCS and the optimum treatment is not known. Therapy is individualized based on symptoms.
Endovascular therapy — Transcatheter embolization of ovarian and/or pelvic veins has become the core therapy for patients with PCS. Embolic agents include coils, vascular plugs, glue, and sclerosant foam. Success rates of up to 90 percent have been reported after three years of follow-up. A series including 56 patients with chronic pelvic pain reported a mean pain decrease of 65 percent over 12 months of follow-up after embolization [52]. In a different study of 67 patients with PCS who underwent ovarian vein coil embolization, 82 percent reported pain reduction, satisfaction with the procedure, and did not pursue further treatment [59]. Regression of vulvar varicosities has been reported after successful ovarian vein embolization [60].
●Vascular access – While the patient is in the supine position, we use ultrasound-guided micropuncture to access the common femoral or internal jugular veins. Next, we upgrade to a 5-French sheath and selectively cannulate the left renal vein with a vascular catheter followed by selective cannulation of ovarian vein. Then, the glide wire and glide catheter are advanced into the ovarian vein. We confirm intraluminal cannulation with 5 mL of contrast material with the patient in the reverse Trendelenburg position, if possible. Incompetent ovarian veins present with venous dilation and reflux of contrast material into the pelvis. Once correct placement is confirmed, we advance the guide catheter to the pelvic brim. Another 5 mL contrast is injected to reconfirm the location and to outline any cross-pelvic collaterals.
●Vascular obstruction – In our practice, we use microcatheter and microcoils. Framing microcoils are placed first and hydrogel coils are placed second. This technique helps to accelerate venous thrombosis and minimize the risk of coil malposition or displacement. For cases with large veins (>12 mm), using coils at least 30 or 50 percent larger than the diameter of target varicose vein with slow deployment is advised because of the possibility of coil migration [51,61].
●Adjunctive sclerotherapy – Sclerotherapy of the IIV can be used adjunctively with balloon occlusion technique and sclerosants, such as sodium tetradecyl sulfate, are injected slowly.
●Unilateral versus bilateral treatment – The decision to treat one or both ovarian veins should be on a case-to-case basis with attention to the severity of symptoms, degree of reflux in ovarian veins, and the distribution of the pelvic varicosities. Patients with significant bilateral ovarian vein reflux along with a large number of pelvic varicosities would likely benefit from bilateral embolization of both ovarian veins, while patients with localized left-sided ovarian vein reflux with moderate varicosities may benefit from unilateral embolization. Clinical judgment guided by experience, clinical presentation, and the magnitude of disease should be used to guide timing of embolization therapy in conjunction with other therapeutic modalities to relieve any compression on renal or iliac veins.
Surgery — The main goal of surgical intervention is to relieve compression and/or control reflux [62]. Initial successful resection was retroperitoneal through an open incision [62]; complications included retroperitoneal hematoma, ileus, prolonged hospital stay, and risk of anesthesia. Successful laparoscopic transperitoneal ovarian vein ligation has also been reported [49]. In the author's opinion, open surgery may only be considered in selected patients with failed endovascular therapy with the presence of life-limiting symptoms.
Role of laparoscopy — Characteristic pelvic venous changes can sometimes be visualized at laparoscopy and laparotomy performed for other indications. However, these findings are common and nonspecific in asymptomatic patients [1,12,13]. Laparoscopy is less sensitive than venography for diagnosis of venous congestion. Venographic studies in women following normal laparoscopy for chronic pelvic pain have demonstrated dilatation of the major pelvic veins and congestion in the ovarian plexuses and broad ligaments in more than 80 percent. As with pelvic ultrasonography, diagnostic laparoscopy is generally utilized to exclude other causes of chronic pelvic pain. (See "Chronic pelvic pain in nonpregnant adult females: Causes".)
Management of PCS
PCS without vulvar varices — Although the data supporting medical management of PCS are limited, we suggest a trial of medical therapy as first-line treatment. The risks of medical therapy are low, particularly when compared with the risks of invasive procedures. Supporting data come from three small randomized trials that reported improved pain scores [35,47,63] and improved venography scores [35,63] in women treated with goserelin (3.6 mg per month), medroxyprogesterone acetate (30 to 50 mg per day), or etonogestrel implant. Additionally, women treated with medroxyprogesterone acetate reported rapid return of pain after cessation of treatment, which further suggests that hormonal therapy suppressed pain [47]. Limitations of these studies include small size [35,47,63] and lack of a placebo group [35].
Women who do not respond to medical therapy can pursue invasive treatment, but the optimal procedure is not known, as randomized trials have not been performed. Reported procedures include embolization or sclerotherapy of the ovarian veins with or without the internal iliac veins [40,43,48,52,57-59,64-66], laparoscopic or open ligation of the ovarian veins [18,28,49,53], and hysterectomy with bilateral salpingo-oophorectomy (BSO) for women who have completed childbearing [48,67]. The existing studies are difficult to compare because they use different diagnostic criteria for PCS; lack control groups; involve different venography, embolization, and surgical techniques; fail to provide standardized evaluation of symptoms before and after treatment; and have variable durations of follow-up.
Despite the above limitations, the reported technical success rates of ovarian vein embolization range from 89 to 100 percent with clinical success rates of 58 to 100 percent (follow-up period of up to five years) [17,48,54,68-73]. Complication rates of 4 to 8 percent have been reported for ovarian vein embolization [70,71]. Surgical ligation of the ovarian vein has been associated with improvement in pain in approximately 75 percent of patients [54]. Hysterectomy with BSO may be helpful for women with PCS, but the data are inconsistent. In one study of 36 women who underwent hysterectomy with BSO, 67 percent of patients reported no pain at one year [67], while a different study of 27 women who underwent the same procedure reported a nonsignificant trend toward improvement at one year [48]. Hysterectomy with BSO is an option for women who have failed other treatment modalities, but women are counseled that the surgery may not resolve their pain.
PCS with vulvar varices — If vulvar varices are present, evidence from case reports and small series shows that treatment of ovarian vein reflux leads to reduction in the size of vulvar varicosities [18,28]. Surgical approaches and embolization likely result in similar outcomes, although some data are inconsistent [3]. We prefer embolization to vein ligation because it is safe, well-tolerated, and allows for a minimally invasive approach. Direct vulvar sclerotherapy or local excision of varices can be performed for persistent disease [3,19]. One case report utilized ultrasound-guided foam sclerotherapy of vulvar and lower left varicosities to avoid ovarian vein embolization in a patient with ovarian reflux and mild PCS which resulted in improvement in pelvic symptoms and resolution of varicosities [74].
PCS with simultaneous left renal vein compression syndrome ("nutcracker syndrome") — The nutcracker syndrome results from compression of the left renal vein by the superior mesenteric artery. This entrapment can cause symptoms of pelvic venous congestion with retrograde venous flow and a dilated gonadal vein. Treatment can be targeted at the renal vein, gonadal vein, or both. Options include embolization of the gonadal veins, laparoscopic gonadal vein ligation, and/or treatment of the renal vein compression. The optimal treatment is unclear as the data are limited and mainly consist of case reports [32].
●Gonadal vein occlusion – Two different case reports describe successful treatment of two women with PCS secondary to nutcracker syndrome using laparoscopic gonadal vein ligation [75,76]. In a case series of 48 women with nutcracker phenomenon, gonadal vein embolization was successfully performed in 96 percent and resulted in long-term benefits in 75 percent [43].
●Renal vein occlusion – In a case series of 5 women with combined PCS and nutcracker syndrome who were treated with endovascular stents placed in the left renal vein, all five had successful stent placement and reported improvement at one month [77]. However, pelvic pain recurred in one woman (who was ultimately diagnosed with endometriosis) and two women had secondary recurrence of symptoms caused by stent dislodgement. The remaining two women were asymptomatic at 4 and 26 months postprocedure.
●Combined treatment – In one case report of a woman with nutcracker syndrome and ovarian vein insufficiency who also had severe genital varicosities, the woman was successfully treated with balloon dilation of the renal vein, embolization of the insufficient left ovarian vein, and subsequent embolization of genital varicose veins by left hypogastric vein coil [78].
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Female pelvic pain".)
SUMMARY AND RECOMMENDATIONS
Vulvar varicosities
●Description and contributing factors – Vulvar varicosities are dilated veins that appear as grape-like clusters of veins on the vulva ("bag of worms"). They are caused by venous obstruction, increased pressure, and venous insufficiency and generally appear during pregnancy and regress postpartum. Hormonal and anatomic factors play a role in pathogenesis. They can arise in isolation, in association with leg/buttock varices, or as part of pelvic congestion syndrome. (See 'Vulvovaginal varicosities' above.)
●Diagnosis – The diagnosis of vulvar varicosities is based on physical examination. Patients should be examined in the supine as well as upright position, which enhances filling of the veins. There is no consensus on indications for imaging. Some clinicians perform magnetic resonance imaging or computed tomography to evaluate pelvic and gonadal veins in all patients with vulvar varicosities and, if imaging is positive, they perform venography if an intervention such as coil embolization is planned. Others omit noninvasive testing and perform diagnostic venography in all patients. (See 'Diagnosis' above.)
●Treatment options
•Pregnant patients – We suggest conservative therapy of vulvar varices during pregnancy (Grade 2C). (See 'Pregnant women' above.)
•Nonpregnant patients – For nonpregnant women with persistent bothersome vulvar varices originating from the saphenous vein, we suggest sclerotherapy as first line treatment (Grade 2C). Duplex ultrasonography should be performed prior to the procedure to exclude lower extremity venous insufficiency. If lower extremity venous reflux or obstruction is present, the leg varices should be treated first and the response of vulvar varices assessed. Direct vulvar sclerotherapy or local excision of varices can be performed subsequently, if needed. (See 'Sclerotherapy' above.)
•Ovarian vein reflux – For patients with vulvar varices and incompetence of pelvic and gonadal veins, we suggest treatment of ovarian vein reflux first (Grade 2C). Embolization can be performed at the time of diagnostic venography and generally leads to reduction in the size of vulvar varicosities. Local sclerotherapy can be performed subsequently, if needed. (See 'PCS with vulvar varices' above.)
Pelvic congestion syndrome
●Clinical presentation – PCS is characterized by a dull ache or heaviness that increases premenstrually; with prolonged standing, postural changes, walking, or activities that increase intraabdominal pressure; and after intercourse (postcoital ache). Dysmenorrhea, deep dyspareunia, and urinary urgency may also occur. (See 'Clinical presentation' above.)
●Imaging findings – Imaging studies in affected women reveal periovarian varicosities. Although the absence of pelvic venous changes helps to exclude PCS, the presence of these abnormalities is not diagnostic, as incompetent and dilated ovarian veins are common, nonspecific findings.
●Diagnosis – The diagnosis of PCS is based on a combination of characteristic symptoms, tenderness on physical examination, and documentation of pelvic vein dilatation or incompetence, after exclusion of other causes for these nonspecific findings. (See 'Diagnostic evaluation' above.)
●Initial treatment – We suggest a trial of medical therapy with medroxyprogesterone acetate, etonogestrel insert, or gonadotropin-releasing hormone (GnRH) agonist as first line therapy for treatment of PCS (Grade 2C). Patients who are refractory to medical therapy can then consider embolization or sclerotherapy. For patients with both PCS and left ovarian vein compression syndrome ("nutcracker syndrome"), options include embolization of the gonadal veins or laparoscopic gonadal vein ligation, treatment of the renal vein compression, or both. (See 'PCS without vulvar varices' above.)
●Treatment of PCS and vulvar varices – In patients with PCS and vulvar varices, we suggest treatment of ovarian vein reflux first (Grade 2C). This generally leads to reduction in the size of vulvar varicosities. Local sclerotherapy can be performed subsequently, if needed. (See 'PCS with vulvar varices' above.)
●Role of laparoscopy – Diagnostic laparoscopy is generally utilized to exclude other causes of chronic pelvic pain. (See 'Role of laparoscopy' above.)
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