Pelvic Congestion Syndrome and Its Relationship to Varices of the Lower Extremities

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1 250 JDMS 25: September/October 2009 Pelvic Congestion Syndrome and Its Relationship to Varices of the Lower Extremities A Literature Review KATHLEEN WHEELOCK, RVT Pelvic congestion syndrome occurs when the ovarian veins are incompetent; it is a common cause of chronic pelvic pain, which often goes undiagnosed and is also a cause of lower extremity varicose veins. Through the review of pelvic venous anatomy, the complexity of pelvic congestion syndrome and the relationships that create this chain reaction of symptoms can be diagnosed, and a holistic course of treatment can be applied. Key words: pelvic congestion syndrome, varicose veins, chronic pelvic pain, embolization therapy, ovarian vein From Cascade Vascular Diagnostics, Tacoma, Washington. Correspondence: wheelock.kathleen@gmail.com DOI: / Pain originating from the lower abdomen lasting more than six months or chronic pelvic pain is the cause of 10% to 40% of all gynecological referrals each year. 1 A large number of these cases are attributed to pelvic congestion syndrome: incompetence of the ovarian veins. These veins connect directly back into both the superficial and deep veins of the lower extremities. If a patient has undiagnosed pelvic congestion syndrome and measures are taken to eliminate varicose veins in the legs, the varicose veins may become recurrent due to the underlying condition. By reviewing the anatomy of pelvic veins and their communications to the veins of the lower extremities, as well as reaching a better understanding of the importance of diagnosing and treating pelvic congestion syndrome, more holistic treatment options can be developed for patients suffering from this often underdiagnosed condition. The symptoms of pelvic congestion syndrome are not very specific and thus cause a challenge for clinicians; they include pelvic pain, dyspareunia,

2 PELVIC CONGESTION SYNDROME / Wheelock 251 dysmenorrhea, and dysuria. All other pelvic pathologies need to be eliminated before pelvic congestion syndrome can be diagnosed; nutcracker syndrome is another known cause of ovarian vein reflux, and portal hypertension can also cause secondary ovarian vein incompetence. Pelvic congestion syndrome is recognized when the diameter of the ovarian vein measures greater than 5 mm by transvaginal sonography and is visually tortuous 2 ; not all women who meet the diameter criteria will have reflux in the ovarian vein or even have pelvic symptoms. Ovarian vein reflux is seen in approximately 10% of the female population; of this group, 60% or more can develop pelvic congestion syndrome. 1 Pelvic varicoceles are found in approximately half of women with chronic pelvic pain. 3 The cause of pelvic congestion syndrome is still unknown, but a hormonal linkage is assumed based on the fact that it is only seen in premenopausal women. 1 It is also theorized that uterine malposition can lead to kinking of the ovarian vein, causing pelvic congestion syndrome. Some believe that the chances of developing pelvic congestion syndrome increase with each pregnancy, as pregnancy causes the ovarian vein to dilate. It is possible, though rare, for nulliparous women to have pelvic congestion; the cause in this case is likely due to a congenital absence of valves in the upper portion of the ovarian vein. An autopsy series cited by Gültaşli et al. 2 reported that valves were absent in the ovarian veins 6% of the time on the right and 15% on the left in the sample population. Varicose veins in the lower extremity related to pelvic congestion syndrome can be in atypical locations such as on the posterior aspect of the upper thigh. There are numerous communications between the pelvic veins and the lower extremities; one such connection is between the inferior gluteal vein and the profunda femoris vein. The obturator vein also communicates directly with the profunda femoris vein, and the internal iliac vein communicates with the greater saphenous vein. 4 A study of 530 patients with pelvic congestion syndrome conducted in Spain found that 96% had reflux from the tributaries of the internal iliac vein to the lower extremities; in 64% of those patients, reflux was noted via the superficial and deep systems. 5 Primary venous incompetence involves only the superficial system, typically the greater or long saphenous vein. The risk of developing primary venous insufficiency increases with age, is more commonly seen in women, and is three times more common than secondary venous insufficiency. 6 Twelve percent of adults seek treatment for symptomatic primary venous incompetence each year. 6 Primary venous incompetence is caused by a weakening of the vein wall over time, and it is most commonly seen in the third decade of life and is seen in patients who spend a lot of time standing. This kind of venous incompetence can be treated in several different ways, including vein stripping, vein ligation, radiofrequency ablation, laser ablation, or a combination of these techniques. Secondary venous incompetence, deep system incompetence leading to perforator incompetence and eventually superficial incompetence, is typically caused by deep vein thrombosis damaging the valves; this then leads to dilatation of the vein by putting more stress on the more distal valves. Other ways of developing secondary venous insufficiency include a congenital absence of valves (typically autosomal dominant, developing early in life), trauma, and the infusion of irritant solutions that are hypotonic or hypertonic. Untreated venous incompetence can lead to hyperpigmentation of the skin in the gaiter region of the distal lower extremity and eventually ulcers caused by venous stasis. Patients with secondary venous incompetence are at an increased risk of developing deep vein thrombosis and superficial thrombophlebitis. Diagnosis Patients who are suspected of having pelvic congestion syndrome based on risk factors such as having more than one child and chronic pelvic pain must have both transabdominal and transvaginal sonography to rule out other pelvic pathologies. Common causes of chronic pelvic pain include but are not limited to endometriosis, ovarian cysts, uterine retroversion, interstitial cystitis, hernia formation, and pelvic congestion syndrome. 7 Some findings that help isolate pelvic congestion syndrome are ovarian point tenderness

3 252 JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY September/October 2009 VOL. 25, NO. 5 FIGure 1. Computerized tomography (CT) image (above) and transvaginal sonography image of pelvic varices. Published with permission from LearningRadiology.com. on physical examination (94% sensitive and 77% specific) or dilated ovarian vein on transvaginal sonography with reversed flow (caudal). 1 Also seen on a sonogram are dilated arcuate veins in the uterine myometrium and polycystic changes in the ovary. 3 It is important to note that the polycystic changes of the ovary are not characteristic of those seen with polycystic ovarian syndrome. The reason for the polycystic change in the ovaries is still unknown but was stated to be three times more prevalent in women with pelvic congestion syndrome than the control group in the Park et al. 3 study on the use of transabdominal and transvaginal sonography in diagnosing pelvic congestion syndrome (Figure 1). 8 Transabdominal and transvaginal sonography can be used as a screening tool to decide which patients should go on to further testing studies, but they cannot conclusively support the existence of pelvic congestion syndrome in a particular patient. Selective ovarian venography remains the gold standard for diagnosing pelvic congestion syndrome. As Ganeshan et al. 1 pointed out, contrastenhanced magnetic resonance venography (MRV) may soon become the initial noninvasive modality for diagnosing pelvic congestion syndrome. MRV allows for the measurement of time from injection to the appearance on venography and, in the case of nutcracker syndrome, can demonstrate the source of the varices. In addition, MRV can show clinicians other pelvic pathologies that may coexist, 1 thus eliminating the need for transabdominal and transvaginal sonography to rule out pelvic pathologies and reducing the time and cost for the patient. Traditional ovarian venography can diagnose pelvic congestion syndrome when one or more of the following are present: ovarian vein diameter greater than 1 cm, uterine venous engorgement, congestion of the ovarian plexus, and filling of pelvic veins across the midline or into the lower extremity. 1 For asymptomatic patients, the finding of pelvic congestion syndrome as a cause of lower extremity varicose veins can be a clinical challenge. There is a significant relationship between pelvic varices and venous insufficiency in the lower extremities. 2 Patients presenting with atypical varicose veins or recurrent varicose veins after stripping or ablation could have secondary varicosities due to pelvic congestion syndrome. In the study by Gültaşli et al., women with chronic pelvic pain were studied to determine an association between the ovarian vein incompetence and that of lower extremity incompetence. None of these patients had been previously diagnosed with lower extremity insufficiency. Although no statistical relationship was found between the number of births for a patient and the prevalence of pelvic varices, a correlation was found between the prevalence of pelvic venous insufficiency and the presence of lower extremity venous insufficiency. 2 Patients were treated with embolization or ligation of the ovarian vein; this was found to be successful in 58% of cases. 2 A combination of extraperitoneal

4 PELVIC CONGESTION SYNDROME / Wheelock 253 vein ligation in addition to ligation of the branches of the internal iliac vein was found to be curative in 77% of cases. 2 The question of how to treat pelvic congestion is varied; embolization and ligation are both common. Prior to these two treatments, the main source of relief for the patient was the removal of the uterus and ovaries, putting the patient into premature menopause and eliminating the hormonal contribution to pelvic congestion syndrome. Treatments The safety of ovarian vein embolization was questioned by Maleux et al. 9 in a study published by the Journal of Vascular and Interventional Radiology in Ovarian vein embolization was performed in 41 patients, the majority of whom underwent unilateral embolization. In this procedure, glue was injected via catheter into the ovarian vein to occlude the vein, and in some cases, microcoils were also placed in the vein and then glued in place. There is a risk of migration of the glue fragments, which can cause either a pulmonary embolism or deep vein thrombosis. Microcoils can cause artifacts in subsequent magnetic resonance imaging studies; to avoid this complication in the future, stainless steel coils have been replaced by platinum coils. 10 In the Maleux et al. 9 study, relief of symptoms typically occurred two to three weeks after embolization; 68% of the sample population noted marked improvement in their preembolization symptoms. It was noted that a better clinical response to treatment was observed in multiparous women compared to uniparous women. 9 No nulliparous women were involved in this study. When embolization treatment fails, the next step is a hysterectomy. 9 The long-term results of the Maleux et al. 9 study found that embolization treatment is a viable alternative to hysterectomy in some patients. Treatment of pelvic venous congestion by embolization of the ovarian and internal iliac vein tributaries has been shown to alleviate not only chronic pelvic pain but also varices of the lower extremities. 4 Greiner and Gilling-Smith 4 conducted a study of 41 women with recurrent varicose veins of the lower extremity after multiple treatments. Evaluation determined that all of the women had pelvic congestion syndrome that was believed to be the cause of their lower extremity varicose veins. Of the patients, 77% showed significant improvements in the clinical study conducted by Greiner and Gilling-Smith 4 for embolization treatment. Venbrux et al. 10 sought to observe the effects that embolization treatment for pelvic congestion syndrome has on the menstrual cycle and chronic pelvic pain. The sample population in this study included 56 women ranging in age from 16 to 66 years, all of whom received bilateral ovarian vein embolization. To further reduce the risk of recurrence of varices via the internal iliac vein tributaries, the internal iliac veins were bilaterally thrombosed. The study found that 96% of the patients noted improvement in their chronic pelvic pain. 10 These findings support the findings of Maleux et al. 9 and Gültaşli et al. 2 ; both indicated that embolization is a long-term treatment for pelvic congestion syndrome. There were no significant changes with regard to menstrual cycle pain or duration. 10 It is important to note that one of the patients in this study went on to have a normal, healthy, full-term infant after receiving embolization treatment, although no studies to date have examined the effects of embolization-treated ovarian and internal iliac veins on pregnancy. 10 Conclusion Pelvic congestion syndrome treated by embolization therapy is an effective treatment for alleviating chronic pelvic pain and varicose veins of the lower extremity. This condition, if untreated, can cause the patient lifelong pain and suffering. If clinicians are able to recognize the signs of this often underdiagnosed condition, unneeded and ultimately ineffective surgical procedures to relieve lower extremity varicose veins can be avoided, saving the patient time and money. References 1. Ganeshan A, Upponi S, Hon L, Uthappa MC, Warakaulle DR, Uberoi R: Chronic pelvic pain due to pelvic congestion syndrome: the role of diagnosis and interventional radiology. Cardiovasc Intervent Radiol 2007;30:

5 254 JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY September/October 2009 VOL. 25, NO Gültaşli NZ, Kurt A, Ipek A, et al: The relation between pelvic varicose veins, chronic pelvic pain and lower extremity venous insufficiency in women. J Diagn Intervent Radiol 2006;12: Park SE, Lim JW, Ko YT, et al: Diagnosis of pelvic congestion syndrome using transabdominal and transvaginal sonography. Am J Roentgenol 2004;182: Greiner M, Gilling-Smith GL: Leg varices originating from the pelvis diagnosis and treatment. Vascular 2007;15(2): Villavicencio JL: Recurrent leg varices and intrapelvic and extrapelvic venous connections: it s anatomy my dear Watson. Vascular 2007;15(2): Zwiebel WJ, Pellerito JS: Introduction to Vascular Ultrasonography. 5th ed. Philadelphia, Elsevier Saunders, Gambone JC: The enigma of chronic pelvic pain: systematically tracing the cause. J Fam Pract 2003;15(12) Herring W: Learning Radiology. Philadelphia, Albert Einstein Medical Center, Maleux G, Stockx L, Wilms G, Marchal G: Ovarian vein embolization for the treatment of pelvic congestion syndrome: long-term technical and clinical results. J Vasc Intervent Radiol 2000;11: Venbrux AC, Chang AH, Kim HS, et al: Pelvic congestion syndrome (pelvic venous incompetence): impact of ovarian and internal iliac vein embolotherapy on menstrual cycle and chronic pelvic pain. J Vasc Intervent Radiol 2002;18:

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