Embolization Therapy: Principles and Clinical Applications, 1 Ed.

Pelvic Congestion Syndrome

Howard Richard

Pelvic pain can be divided into acute pain, recurrent pelvic pain, and chronic pelvic pain. Acute pain rarely lasts more than 1 month. It typically resolves of its own accord. Recurrent pain is pain that occurs on a regular basis such as the cyclic pain of dysmenorrhea or the episodic pain of dyspareunia. Chronic pelvic pain is defined as pain located primarily in the pelvis that lasts more than 3 to 6 months.13 There are many different causes of chronic pelvic pain, and textbooks have been written delineating the differential diagnosis. It is important to note that although chronic pelvic pain often originates with a biologic underpinning, patients will develop psychological and sociologic components with the passage of time.4 The psychological aspects such as depression and sociologic aspects such as interpersonal relationship dysfunction should be considered in the management of these problems.

Chronic pelvic pain can be attributed to gynecologic, uterine or extrauterine, urologic, gastrointestinal, musculoskeletal, or multiple causes. With the discussion of chronic pain based on pelvic venous congestion, we are chiefly concerned about extrauterine gynecologic pain. Richet5 first described pelvic venous varicosities in 1857. Grossly dilated or varicose veins with marked venous incompetence can be noted in women, particularly following pregnancy. Taylor6 reported a connection between the varicosities and pelvic pain in 1949. These varices can extend to the perineal tissues and even to the buttocks and the upper legs.7,8 Dilated ovarian intrauterine vascular plexus can be identified at laparoscopy (Fig. 59.1). This can be challenging secondary to the positive pressure used in laparoscopy. In fact, up to 90% of patients with negative laparoscopy potentially have pelvic congestion syndrome (PCS).9

The clinical features of PCS consist of a deep-seated pelvic pain, classically seen in multiparous women. The pain worsens with prolonged standing or walking and classically improves when in a recumbent position. The patients can have a congestive dysmenorrhea with symptoms worsening before menses. Patients can suffer deep dyspareunia with a postcoital ache, which may last for several days.10,11 This can be accompanied by emotional disturbance such as depression and sociologic turmoil in the patient’s interpersonal relationships.

The clinical signs of PCS include external varices involving the external genitalia and superficial vulvar and perivulvar tissue (Fig. 59.2).12,13 Patients may have pain to palpation over the ovarian point, which is two-thirds of the distance from the anterior superior iliac spine to the umbilicus. When evaluating the cervix in these patients, there may be excess clear mucoid discharge. The cervix may be blue secondary to venous engorgement. On bimanual examination, cervical motion tenderness may be found.10,14

Ovarian vein anatomy contributes to the pathogenesis of PCS. In normal patients, ovarian venous return is aided by spontaneous contractility. In some women, the ovarian vein smooth muscle undergoes age-related thinning.15When this is associated with an increase in venous diameter during pregnancy, the valves are no longer able to function properly. This can result in venous insufficiency. The vascular endothelium is known to contain estrogen receptors, which are related to the trophic response of vascular smooth muscle. In addition, nerves are identified within layers of the veins. The vascular filling and engorgement is also capable of generating pain signals in some patients by releasing pain mediators such as substance P.16 The internal iliac vein can also convey pressure to the periovarian and periuterine vascular plexus. These veins have valves in approximately 10% of patients, and this represents a potential pathway for reflux into the periuterine and periovarian vascular plexus in the event of valvular dysfunction.

Varicose vein development may also be related to genetic structural vein wall anomalies.17 This can be exacerbated by hormonal and hemodynamic factors related to pregnancy. The combination of genetic predisposition and pregnancy can combine to create the environment for the formation of pelvic varicosities. These venous structures can generate pain signals upon distention in some, but not all, women. Therefore, in some patients, the combination of incompetent distended veins with the ability of the veins to generate pain signals with distention results in the syndrome of pelvic venous congestion.

In evaluating patients for the diagnosis of pelvic venous congestion, various imaging modalities have demonstrated dilated veins in asymptomatic women. Ultrasound, computed tomography (CT), and magnetic resonance imaging scans are able to clearly define dilated ovarian veins. However studies have failed to demonstrate any correlation between the presence of dilated veins and the presence of symptomatic pelvic venous congestion.1820 On imaging examination, patients with pelvic venous congestion can have polycystic-appearing ovaries. When evaluating patients with PCS and polycystic-appearing ovaries, patients with PCS demonstrate an increased number of a atrophic follicles consistent with follicular atresia.21 This is a distinct difference from patients with polycystic ovary disease and normal patients. Although both patients with pelvic venous congestion and polycystic ovary disease can demonstrate elevated androstenedione content, patients with pelvic congestion demonstrated a decreased response of estradiol to follicle-stimulating hormone (FSH) as contrasted with the normal response of estradiol to FSH in patients with polycystic ovary disease and normal patients.22,23

Functional imaging can be performed with catheter venography. Selective catheterization and retrograde venography of the left renal vein only allows visualization of ovarian vein in patients with incompetent valves. However, in some patients, the proximal valve may be competent. Therefore, selective ovarian venography is required to allow passive reflux to extend all the way down the ovarian vein into the pelvic veins. In some patients, the reflux can extend to the perivulvar or upper lower extremity veins. Sometimes, this reflux can be seen extending into the periuterine venous plexus and then ascending in the internal iliac veins (Fig. 59.3). It is possible to perform direct transuterine venography with injection of contrast directly into the uterus (Fig. 59.4). Beard et al.14 first described this technique in the mid-1980s. A grading schema was developed to define the abnormalities on transuterine venography, taking the following factors into account: an ovarian vein diameter greater than 8 mm, a tortuous venous plexus with flow from side to side, and a delay in washout of contrast from the pelvis. The scoring system was devised in which a score of 5 or more gives a diagnostic sensitivity of 91% and specificity of 89% on the diagnosis of pelvic venous congestion syndrome.

Treatment options for pelvic venous congestion syndrome include hysterectomy and bilateral oophorectomy. This surgery is often used in patients with chronic pelvic pain because some of these patients will have a working diagnosis of adenomyosis, endometriosis, or uterine fibroids. Beard et al.10 reported a study of 36 patients who were diagnosed with PCS and treated with hysterectomy and bilateral oophorectomy. At 1-year follow-up, 12 of the 36 patients had some residual pain, and 1 patient had no significant change in her pain. Therefore, two-thirds of the patients were cured with removal of uterus and ovaries, which can likely be attributed to ligation of the periuterine periovarian venous plexus. Unfortunately, this also shows us that one-third of the patients had persistent pain despite the surgery. Patients with persistent pelvic venous congestion after hysterectomy may benefit from transvenous embolization.

DEVICE/MATERIAL DESCRIPTION

Venous embolization for PCS can be performed via the internal jugular or femoral venous approach. When the procedure is performed via the internal jugular venous approach, a 6-Fr 55 cm straight vascular sheath is used. This allows for stable access to the inferior vena cava. The left renal vein can be catheterized with a vertebral curve or Cobra catheter (Cook Bloomington, Indiana). Both of these catheters will usually allow access to the left ovarian vein. In some cases, the sheath can be advanced into the left renal vein and a reverse curve catheter such as a Roush inferior mesentric (RIM) (Cook Bloomington, Indiana) can be used to gain access to the left ovarian vein. Catheterization of the right ovarian vein is often accomplished with either an multi purpose B (MPB) (Cook Bloomington, Indiana) curve or a Cobra catheter. Evaluation of the pelvic veins is often accomplished with a Berenstein (Cordis Bridgewater, New Jersey) or vertebral curve catheter.

When the procedure is performed via the femoral venous access, a reverse curve sheath such as the Hopkins curve sheath (Cook Bloomington, Indiana) can be used in the left renal vein. Reverse curve catheters such as Simmons-type catheters (Cook Bloomington, Indiana) can be used to gain access to the right ovarian vein.

Embolization can be performed with a sclerosant slurry. Various sclerosants have been used. Hypertonic saline mix with Gelfoam (Ethicon, Sommerville, New Jersey) slurry, Sotradecol (Angiodynamics Inc., Quensbury, New York), Sotradecol foam, and sodium morrhuate have all been used with success. Typically, this is performed in conjunction with embolization using pushable coils, detachable coils, and vascular plugs. The use of glue as an embolic agent for this indication has been described as well.24

TECHNIQUE

Venous embolization of the ovarian and internal iliac veins is typically performed with intravenous conscious sedation. The internal jugular and or femoral venous access routes can be employed based on operator preference. Left ovarian veins classically arise from the left renal vein (Fig. 59.5). Left renal venography can demonstrate reflux into an incompetent left ovarian vein. However, some patients will have a competent cephalad left ovarian vein valve, and in these patients, selective left ovarian venography is required to confirm the diagnosis of ovarian reflux. Following catheterization of the left ovarian vein, the catheter can be advanced into the pelvis, whereupon venography can demonstrate the periuterine and periovarian venous plexus. Marked stasis of contrast and accumulation and pooling of contrast in the pelvis is consistent with the diagnosis of pelvic venous congestion. Embolization is then performed using a combination of sclerosant slurry and coils or vascular plugs. The embolization is performed at several levels throughout the course of the ovarian vein. This is important because reflux into patent tributaries of the ovarian vein can lead to treatment failure via collateral pathways that circumvent coils placed distally in the target vein. In addition, it is not uncommon to find parallel channels and these should be selectively catheterized and embolized independently.

Catheterization of the right ovarian vein is often challenging. Classically, a right renal venogram will demonstrate a landmark for the right ovarian vein. The right ovarian vein is classically located immediately caudal to the confluence of the right renal vein and the inferior vena cava. Variant anatomy exists, and on occasion, the right ovarian vein can be identified as a branch of the right renal vein (Fig. 59.6). Alternatively, the right ovarian vein may enter the inferior vena cava caudal to the confluence of the right renal vein and the inferior vena cava (Table 59.1). Following catheterization of the right ovarian vein, catheterization and embolization is performed in a similar fashion to that of the left ovarian vein.

For most patients, performance of ovarian venography and embolization without an assessment of the internal iliac veins represents an incomplete evaluation of PCS. Selective internal iliac venography is performed to identify persistently abnormal periovarian and periuterine vascular plexus. Balloon occlusion venography can be helpful in challenging anatomy. Once these venous plexus are identified, they can be embolized with sclerosants alone or with sclerosants and coils. If using coils, care must be taken to ensure the coils do not migrate and/or embolize to the lungs; coils can be placed via an over-the-wire Fogarty occlusion balloon catheter (Cook Bloomington, Indiana) to minimize this risk. As outlined in an earlier chapter, introducing the leading aspect of a coil into side branches can help anchor the coil nest and minimize the risk of migration. Following embolization of the right internal iliac venous plexus, the left internal iliac venous plexus can be catheterized and embolized in a similar fashion.

CLINICAL APPLICATIONS

In patients with pelvic venous congestion, ovarian and internal iliac venous embolization can provide significant clinical relief. The first report of transcatheter embolization of the ovarian veins was described by Edwards et al. in 1993.25 Since that time, several studies have been published demonstrating the success of embolization at addressing the pain associated with PCS. Most of the studies published to date involve performance of a unilateral or bilateral ovarian vein coil embolization without embolization of one or both internal iliac veins. These data, however, demonstrate the shortcomings of this approach. For example, Capasso et al.26 reported on ovarian vein embolization in 19 patients (13 underwent unilateral ovarian vein embolization and 6 underwent bilateral ovarian vein embolization). After a mean of 15.4 months follow-up, significant relief was experienced in 57.9%, partial relief in 15.8%, and no relief in 26.3% of patients. Similarly, Kwon et al.27 described their experience with ovarian vein embolization in 67 patients (unilateral embolization in 65 patients, unilateral embolization in 2 patients). They found significant relief in 82%, no relief in 15%, and worsening symptoms in the remaining 3% of patients. Other studies evaluating ovarian vein embolization for PCS have had similar findings.28,29 Although the use of isolated ovarian vein embolization can still provide most patients with significant symptomatic improvement, the failure rate seen in these studies supports the addition of internal iliac venography and embolization to a treatment protocol. Interestingly, Gandini et al.30reported their experience with bilateral ovarian vein embolization using a sclerosant in 38 patients and found significant relief in 100% of patients after a mean follow-up period of 12 months.30

In general, the addition of internal iliac venography and embolization as well as the addition of sclerosants to the embolization technique has improved the results of this procedure. Venbrux et al.31 described their experience in 56 patients, with all patients undergoing bilateral ovarian vein embolization and 43 patients undergoing internal iliac vein embolization. Procedures in this study were performed with coils and a sclerosant. After a mean follow-up period of 22.1 months, significant or partial relief was seen in 96% of patients, whereas 4% of patients experienced no improvement in their symptoms. Similarly, Kim et al.32 reported their experience with 127 patients (106 undergoing bilateral ovarian vein embolization, 20 undergoing unilateral ovarian vein embolization, and 108 undergoing internal iliac vein embolization) treated with coils and sclerosants.32 They found significant relief in 85%, no relief in 12%, and worsening symptoms in 3% of their patients after a mean follow-up of 45 months. In 2013, Laborda et al.33 published the largest study evaluating ovarian and internal iliac vein embolization for PCS. There were 202 patients with chronic pelvic pain who were treated with coil embolization, leading to clinical success in 93.9% of patients after 5 years (179 patients completed the 5-year follow-up period); 33.5% of patients reported complete disappearance of their symptoms after embolization.33

Chung and Huh34 published a randomized study supporting the use of embolization in the treatment of PCS in 2003. They evaluated 106 patients with PCS and pelvic pain who were initially treated unsuccessfully with medroxyprogesterone. These patients were randomly assigned to three treatment groups: ovarian vein embolization with coils (n = 52), hysterectomy with bilateral salpingo-oophorectomy and hormone replacement therapy (n = 27), and hysterectomy with a unilateral salpingo-oophorectomy on the affected side (n = 27). All patients underwent venography before treatment to confirm the diagnosis of PCS. Based on the use of a visual analog scale to assess pain, embolization was significantly more effective at reducing pelvic pain compared to the other methods of treatment.

POTENTIAL COMPLICATIONS

Complications include access site injury such as a hematoma. This can manifest as pain at the catheter insertion site. As this is a venous procedure, these are typically self-limited. Nontarget embolization is always a concern, particularly with coil embolization. Migration of coils into the heart and pulmonary circulation has been reported.31,35 This can be minimized by either (1) not using coils in the internal iliac veins or (2) only placing coils into the internal iliac veins following ipsilateral ovarian vein embolization and behind an over-the-wire Fogarty balloon occlusion catheter to allow for safe controlled formation of a stable coil mass. Cardiac arrhythmias have also been reported after this embolization for PCS.36 Future fertility remains a question after embolization for PCS. However, Kim et al.32 reported that there were no significant changes in FSH, luteinizing hormone, or estradiol levels. In addition, they reported a 50% pregnancy rate in premenopausal women undergoing this procedure.

TIPS AND TRICKS

• Counsel patients that they should not expect improvement until 2–6 wk after the procedure.

• Counsel patients that they may set off airport metal detectors because of the embolization coils. Tell them to simply state that they have an implanted medical device.

• Start with a left ovarian vein. When performing the left ovarian venogram, image the reflux through the right ovarian vein to identify the right ovarian vein origin from the inferior vena cava.

• Balloon occlusion venography can demonstrate reflux into the periuterine venous plexus and facilitate catheterization.

• If you only embolize the ovarian veins, you may have patients experience an early relapse; these patients should be brought back to have the internal iliac veins embolized.

• In patients with PCS and hematuria, consider the nutcracker syndrome and measure a pressure gradient across the left renal vein.

SUMMARY

Ovarian and internal iliac vein embolization has been shown to be an effective treatment for the symptoms associated with PCS. Although prospective randomized trials comparing embolization with medical and/or surgical therapy need to be performed to gain a more comprehensive understanding of this procedure, available data suggest that this is a safe and effective minimally invasive treatment option for these patients.

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