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Pelvic Congestion Syndrome
Introduction
Pelvic congestion syndrome is a pelvic venous syndrome that is frequently misdiagnosed and difficult to manage. This syndrome is a relatively common cause of chronic pelvic pain in women of reproductive age, accounting for nearly 10% to 20% of gynecologic consultations. However, only 40% of these cases are referred to subspecialists or specific care teams for further evaluation. Although the etiology of pelvic congestion syndrome still remains unclear, it is thought to result from a combination of factors, including genetic predisposition, anatomical abnormalities, hormonal factors, damage to the vein wall, valve dysfunction, reverse blood flow, hypertension, and dilatation[1][2]. Although transcatheter venography represents the gold standard for pelvic congestion syndrome diagnosis, it is performed after inconclusive noninvasive imaging, such as Doppler ultrasound, computed tomography (CT) scan, and magnetic resonance imaging (MRI). Once the diagnosis is confirmed, the treatment options for pelvic congestion syndrome include medical, surgical, and endovascular therapies. Medical and surgical treatments are less effective compared to transcatheter pelvic vein embolization. This latter has been proven to be a safe, effective, and durable therapy for treating pelvic congestion syndrome.[3]
Etiology
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Etiology
Pelvic congestion syndrome, also known as pelvic venous insufficiency, is due to the incompetency of the internal iliac vein, the ovarian vein, or a combination of the venous structures of the pelvis. This syndrome is often the underlying cause of otherwise unexplained chronic pelvic pain. Nearly 10% of women have isolated ovarian varices, and of this 10%, about 60% have pelvic congestion syndrome.[4] Chronic pelvic pain is characterized as more than 6 months of persistent or intermittent pain localized in the pelvis.[5] The overall prevalence of pelvic congestion syndrome ranges from 6% to 27% worldwide and remains a significant challenge for women's healthcare providers. Chronic pelvic pain may be caused by pelvic congestion syndrome in up to 30% of women.[6] More recently, pelvic congestion syndrome is estimated to be present in up to 75.5% of patients with pelvic varicose veins, as reported in a 10-year retrospective analysis of a 600-female patient cohort conducted by Gavrilov et al.[7]
The exact etiology of pelvic congestion syndrome is unclear and is most likely dependent on multiple factors. The congestion of the pelvic veins can be due to hormones, venous insufficiency of the valves, venous obstruction, and secondary to concurrent medical conditions, such as peripheral artery disease. The release of pain-inducing substances due to increased dilatation of the veins along with stasis is a likely cause of the pain in pelvic congestion syndrome.[8]
Epidemiology
Pelvic congestion syndrome mainly affects premenopausal, multiparous women.[9] There has been no reported occurrence of the syndrome in menopausal women.[10] In patients with chronic pelvic pain, the prevalence of the disease is nearly 30%.[11]
Pathophysiology
In pelvic congestion syndrome, the abnormal dilation of the interlinked venous channels of the internal iliac and ovarian veins is often implicated. The ovarian plexus drains into the ovarian veins on both sides, whereas the hemorrhoidal, utero-ovarian, sacral, and vesicular venous plexuses drain into the internal iliac veins. The broad ligament has the internal iliac and ovarian veins running through it.[2]
There can be incompetence of the internal iliac veins and the inferior vena cava. However, most of the cases of the pelvic varices are identified in the ovarian veins. Moreover, about 60% of these develop pelvic congestion syndrome.[12] In the majority of pelvic congestion syndrome cases, incompetency of the internal pudendal and broad ligament parametrial branches are involved. The pelvic venous reflux into the lower limb or vulvar varicosities is often associated with the incompetency of the branches of the circumflex femoral and obturator veins.[13]
The primary vein insufficiency is due to either the absence of the venous valves or the incompetency of the valves. In such patients, the congenital absence of the ovarian valves has been reported in 6% of patients on the right side and 13% to 15% on the left side. There are incompetent valves in 35% to 46% of women on the right and 41% to 43% on the left. The inclination of multiparous women to develop pelvic congestion syndrome can be due to the 50% increased pelvic vein capacity due to physiological changes during pregnancy. These changes can lead to retrograde blood flow and incompetency of the valves. Even 6 months after pregnancy, these vascular changes can persist.[14]
Secondary pelvic vein incompetence is often due to external compression of the vein, leading to venous outflow obstruction. Different causes of external compression include the nutcracker phenomenon, also known as left renal vein entrapment syndrome. This phenomenon is due to the compression of the left renal vein between the superior mesenteric artery and the abdominal aorta. Similarly, the compression of the left common iliac vein by the right internal iliac artery in May-Thurner syndrome can also lead to such results.[15][16] Regional overload in the venous channels can lead to pelvic venous congestion. This regional overload might be due to left renal vein thrombosis (with renal cell carcinoma), tumor thrombosis in the inferior vena cava, cirrhosis, congenital malformations of arteriovenous and venous channels, and retro aortic left renal vein.[17]
Both varicoceles and pelvic congestion syndrome are considered diseases of the pelvic veins, which include the gonadal veins, tributaries of the internal iliac veins, and parametrial and uterine veins in females. The course of the spermatic and ovarian veins may explain the seemingly left-sided prevalence of both conditions. The left gonadal vein drains into the higher-pressure left renal vein, as opposed to the right renal vein, which drains directly into the inferior vena cava.[18]
Improvement of symptoms after menopause indicates the influence of hormones on pelvic congestion syndrome. Estrogen is a venous dilator and can thus produce the venous dilation implicated in the pathophysiology of pelvic congestion syndrome.[10] However, despite the lack of clinically confirmed cases of postmenopausal pelvic congestion syndrome systematically reported to date, isolated references of postmenopausal patients who fulfill diagnostic criteria and experience a lasting alleviation of symptoms after respective standard treatment strongly suggest its incidence[19].
History and Physical
The concurrent presence of venous varices with pelvic pain in premenopausal women does not always mean that they are causally related. Even in asymptomatic females, the dilation and incompetency of pelvic veins are common findings, making it challenging to identify which patients have chronic pelvic pain due to pelvic congestion syndrome.
The pain associated with pelvic congestion syndrome presents as a dull ache or a sensation of heaviness in the pelvis, lasting for 3 to 6 months, and can be unilateral or bilateral. However, the pain can switch from one side to the other. Factors such as walking, postural changes, lifting, and long-standing positions increasing the abdominal pressure can exacerbate the pain. The paint is often exacerbated before or during the menstrual period. The intensity of pain worsens with each subsequent pregnancy and during or after sexual intercourse. The time of the day also affects the intensity, with the pain worsening at the end of the day.[20]
If the findings of characteristic pelvic pain are present, a physical examination can help in formulating the final diagnosis. The uterine tenderness, ovarian tenderness, and cervical motion tenderness on direct palpation during bimanual examination in a patient presenting with a complaint of chronic pelvic pain support the diagnosis of pelvic congestion syndrome.
In a study involving 57 females with pelvic pain, the combination of a history of postcoital pain with tenderness over the adnexa during physical examination demonstrated 77% specificity and nearly 94% sensitivity in differentiating pelvic congestion syndrome from other pathologies of pelvic origin.[11]
Evaluation
The presence of characteristic pelvic venous changes on imaging supports the diagnosis of pelvic congestion syndrome, but it is not necessary to form the final diagnosis. Dilated ovarian veins with incompetency of the valves are commonly found in asymptomatic women.[21]
Patients with pelvic congestion syndrome, in whom an intervention is planned, require evaluation for pelvic venous reflux with ultrasound, retrograde internal iliac or ovarian venography, CT, or MRI.[22]
Ultrasound
Pelvic ultrasound is the first-line imaging modality for pelvic congestion syndrome. Ultrasound helps rule out the presence of pelvic masses or uterine problems as the underlying cause of pelvic pain. The pelvic anatomy, ovarian changes, uterine enlargement, and dilated uterine and ovarian veins can be evaluated using color-Doppler and conventional B-mode ultrasounds.[23]
Retrograde flow of blood with an increase in the size of the left ovarian vein and a decrease in velocity of the blood flow can be observed using ultrasonography. Enlarged, tortuous pelvic venous channels can be noted. The incompetency of valves in the pelvic varicose veins can be noted using Valsalva's maneuver. These varicoceles show variable duplex waveforms on such maneuvers. Polycystic changes of the ovary are also observed in patients with pelvic congestion syndrome.[8][11]
Criteria for varices: The diagnosis of pelvic congestion syndrome can be confirmed using ultrasound findings. Criteria include an ovarian vein diameter of ≥6 mm, a slow blood flow of <3 cm/s, and retrograde venous blood flow in the left ovarian vein combined with the clinical vulval varicosities with the 3 Ds (dysmenorrhea, dysuria, and dyspareunia). The criteria for pelvic ultrasound diagnosis of varices include the visualization of dilated ovarian veins >6 mm, although 7 mm has also been suggested as a cutoff. A study reported a positive predictive value of 83.3% for an ovarian vein diameter of 6 mm in diagnosing pelvic congestion syndrome.[24]
Computed Tomography and Magnetic Resonance Imaging
The anatomical details of the pelvic vasculature and tissue of the pelvic cavity can be easily visualized using CT and MRI. As CT uses radiation, it is not recommended in premenopausal women.[25]
Magnetic resonance venography is a promising noninvasive imaging technique for diagnosing pelvic vasculature varices. However, its specificity for venous pathologies is low, as the patient is in a supine position for this examination.[26]
The direction and velocity of flow in different vascular channels can be assessed with phase-contrast velocity mapping, which is an MRI-based technique. This technique can be used to evaluate pelvic veins.[27]
Venography
Ovarian and iliac catheter venography is considered the gold standard for diagnosing pelvic vascular congestion. The ovarian veins are catheterized using percutaneous jugular and femoral pathways. The distension of the venous channels is better assessed when a venogram is performed during Valsalva. Venographic diagnostic findings of pelvic congestion syndrome include incompetent pelvic veins (with a diameter of more than 5-10 mm) and congestion of flow in venous channels of ovarian, pelvic, vulvovaginal, and thigh veins. Venous reflux in ovarian veins can also be noted.[25]
Laparoscopy
Chronic pelvic pain is a significant cause of gynecologic diagnostic laparoscopies. According to certain reports, more than 40% of such laparoscopic procedures are due to chronic pelvic pain.[28] The rate for the occurrence of pathological findings identified on laparoscopies in women with chronic pelvic pain ranges between 35% and 83%. In 20% of these cases, pelvic congestion is also identified.[29]
Treatment / Management
Pelvic Congestion Syndrome Without Vulvar Varices
Medical management is typically the first-line treatment for pelvic congestion syndrome due to its non-invasive nature and lower associated risks. This approach is often combined with pelvic floor physical and cognitive behavioral therapies.[30] Up to 70% of pelvic congestion syndrome-affected females may be adequately managed through these conservative treatment approaches, although few studies assess long-term efficacy.
Medical treatments: Pharmacological options for managing pelvic congestion syndrome include gonadotropin-releasing hormone (GnRH) agonists, danazol, combined oral contraceptives, progestins, phlebotonics, and nonsteroidal anti-inflammatory drugs.[31] Etonogestrel implant, goserelin, and medroxyprogesterone acetate have also been successful in alleviating the pain associated with pelvic congestion syndrome.[8] Improved pain relief is observed when medroxyprogesterone is combined with psychotherapy.[32] Goserelin, a GnRH agonist, has better results in controlling the pain compared to medroxyprogesterone acetate. However, it cannot be continued beyond 1 year because it is a GnRH agonist.[8] (A1)
Invasive treatments: Women who do not respond to medical therapy may consider more invasive treatments; however, the optimal procedure remains unclear due to a lack of randomized trials. These treatments include both surgical and non-surgical approaches. Surgical treatment has traditionally been considered the preferred approach for both conditions, although clinical practice is increasingly leaning towards percutaneous embolization as the definitive management for pelvic congestion syndrome.[30] Broadly, non-surgical procedures include embolization or sclerotherapy of the ovarian veins with or without the internal iliac veins.[28][33][34][35][36][37][38][39][34][28]
Surgical procedures for pelvic congestion syndrome include laparoscopic or open ligation of the ovarian veins [40][23][41][42] and hysterectomy with bilateral salpingo-oophorectomy for women who have completed childbearing.[34][43] However, the results of these treatments were not favorable.[22](A1)
Overall, interventional coil embolization of ovarian veins may be considered a safe and effective gold standard to alleviate symptoms of venous congestion and thus applies to pelvic congestion syndrome in certain cases.[44] A systematic review involving 473 patients who underwent interventional coil embolization reported clinical alleviation of symptoms in 82.1% to 100% of cases.[45] Complications were reported to be rare and comparably mild, such as local hematoma after cannulation. Recurrence rates were reported to be minimal. Laborda et al [46] reported a remission of pain in 93.9% of patients with a follow-up of 5 years, with approximately one-third of patients achieving complete symptom relief.(A1)
Pelvic Congestion Syndrome with Vulvar Varices
If vulvar varices are present, evidence from case reports and small series shows that the treatment of ovarian vein reflux reduces the size of vulvar varicosities.[40][23] Surgical approaches and embolization likely result in similar outcomes, although some data are inconsistent.[47] The ablation of incompetent veins can also be achieved by endovascular procedures using a minimally invasive approach. These procedures can be performed in an outpatient setting, leading to comparatively quick recovery and fewer complications.[48] Various agents, such as platinum embolization coils, glue, foam, or liquid sclerosants, can be used to induce endothelial damage in the incompetent vessels.[49](B2)
Differential Diagnosis
The differential diagnoses for pelvic congestion syndrome align closely with those for chronic pelvic pain and should be evaluated similarly. The differential diagnoses include diseases of the urinary tract and gastrointestinal tract, musculoskeletal disorders, disorders of neurological origin, gynecological problems, and mental health disorders. Painful bladder syndrome, pelvic inflammatory disease, interstitial cystitis, endometriosis, pelvic neuralgia, irritable bowel syndrome, myofascial pain, and pelvic floor myalgia are the common causes of chronic pelvic pain. Accurately diagnosing the underlying cause of chronic pelvic pain remains challenging, even with advanced laparoscopic and diagnostic imaging techniques.[2][4]
Staging
Various grading systems have been developed to assess pelvic venous reflux and pelvic congestion syndrome.[30]
Yang et al [50] proposed a grading system for ovarian venous reflux to aid in pelvic congestion syndrome diagnosis based on the vasculature involved in time-resolved MR angiography. In this system, grade 1 reflux denotes isolated reflux of the left ovarian and parauterine veins, and grade 2 reflux is more severe and indicates a combination of grade 1 features and reflux in the right ovarian and internal iliac veins. Vulvar and thigh varices may also be present in grade 2 pathology.
Pelvic congestion syndrome may also be graded based on the pattern and duration of pelvic venous reflux, with increased grades associated with worsening symptoms. According to Gavrilov et al,[51] patients with pelvic varicosities may be subdivided into type I (reflux duration 1–2 s), type II (3–5 s), and type III (>5 s or spontaneous reflux in the absence of a loading test).
Prognosis
Response to Medical Management
Dihydroergotamine, an alpha-blocker, provides substantial relief in up to 95% of patients with pelvic congestion syndrome with chronic pelvic pain and can lead to a mean reduction of 35% in pelvic vein diameter. However, its adverse effects include dyspepsia, arrhythmias, and angina, which may limit its use.[52] A combination of medroxyprogesterone acetate and psychotherapy can be effective in up to 73% of patients, with similar results observed using progesterone implants, such as ImplanonTM or NexplanonTM[53].
Response to Invasive Treatments
Although studies are limited, a study conducted by Gavrilov et al [54] involving 277 female patients found that gonadal vein resection was superior to embolization with coils in terms of pelvic pain relief at 30 days (100% versus 73%), postoperative complications (11% versus 56%), and 3-year recurrence rate (5% versus 11%). The most common complication in both methods was pelvic vein thrombosis.
Overall, although cases of pelvic congestion syndrome are heterogeneous, available treatments provide pain relief for up to 70% of patients, with pain expected to improve following menopause.
Complications
Surgical treatments for pelvic congestion syndrome are associated with an increased rate of recurrent pelvic pain (20%) or residual pain (33%). Moreover, these procedures often result in aesthetic damage and prolonged hospitalizations.[55] The loss of gonadal function leading to the need for hormonal replacement is also an important complication of ovarian vein ligation and oophorectomy.[43]
Consultations
As treatment for pelvic congestion syndrome often requires interventional procedures when medical management fails, timely coordination and consultation with endovascular specialists is essential.
Deterrence and Patient Education
Pelvic congestion syndrome is prevalent in approximately 2.1% to 24% of women aged 18 to 50, highlighting the importance of educating patients with pelvic congestion syndrome. Patients should be educated regarding treatment adherence and informed of any relationship between their symptoms and menstrual cycle. When using bilateral oophorectomy, there is often a need for hormonal replacement. The patients should also be educated about these complications.[56]
Enhancing Healthcare Team Outcomes
Chronic pelvic pain accounts for approximately 10% to 20% of gynecologic consultations. Nearly 40% of these cases are referred to specialists for further evaluation. The diagnosis of pelvic congestion syndrome requires high clinical suspicion on the clinician's part. After diagnosis, medical or surgical management may be necessary. The radiological approach for embolization is also considered.
Given the complexity of PCS, effective management requires collaboration among an interprofessional team. This team typically includes primary care clinicians, gynecologists, and interventional radiologists. Coordination among these specialists is essential to achieving optimal patient outcomes and ensuring that all aspects of care are addressed in a timely and integrated manner.[57] This interprofessional collaboration is critical for improving patient outcomes and minimizing complications associated with the condition.
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