This article summarizes the clinical characteristics of vascular etiologies of pelvic pain: pelvic venous disorders, vasculitis, and ischemic pelvic disease, and the vague symptoms which can be confused with endometriosis, pelvic organ prolapse, and inflammatory bowel disease.
General Assessment
Patient history should include information about the onset, location, timing, and nature of pelvic pain as well as the presence of GI symptoms, systemic or autoimmune symptoms, varicose veins, and leg swelling. Thorough physical examination of the abdomen, pelvis, and skin should be performed with particular attention to the presence of varicose veins on the genitals, pelvic girdle, and lower extremities. History and physical examination should guide diagnostic imaging, which may include transvaginal ultrasound, ovarian vein duplex ultrasound, external abdominal ultrasound, biopsy, venography studies, or intravascular ultrasound. Additional tests including an ankle-brachial index or antibody titers may assist diagnosis in the setting of suspicion for arterial insufficiency or autoimmune vasculitis respectively.
Conditions
Pelvic Venous Disorders
Pelvic venous disorders refer to pelvic pain originating from dilation of the pelvic venous vasculature in the setting of pelvic venous hypertension. These disorders include pelvic congestion syndrome (PCS, pelvic venous insufficiency), nutcracker and May-Thurner syndromes (obstruction). Due to hormonal effects on the vasculature, pelvic venous disorders are most commonly seen in women of child-bearing age, however, they also can present in post-menopausal women on hormone replacement therapy. Treatment guidelines have yet to establish a consensus with respect to the use of antiplatelet and anticoagulation interventions for patients with pelvic venous disorders.
Pelvic Congestion Syndrome
Pelvic Congestion Syndrome (PCS) reportedly accounts for up to one-third of pelvic pain in women (Meissner and Gibson, 2015) – surprising given the lack of research we have on this entity. It involves pelvic venous dilation secondary to venous insufficiency. For pelvic congestion syndrome to be diagnosed, pelvic pain must be present for greater than 6 months with variable symptoms of dyspareunia, dysuria, and dysmenorrhea, typically present throughout the menstrual cycle, but often intensifying during menses. Characteristically, the pain is least severe in the morning and worsens throughout the day with upright position. GI symptoms may be present as well. Varicose veins are seen in the majority of women with PCS: vulvar-vaginal varicose veins in up to one-third, and lower extremity in up to 90%. The diagnostic test of choice is pelvic MR venogram, but the varicosities can also be visualized on ultrasound. Treatment is guided by symptoms, presentation, and patient preferences; options include medications (medroxyprogresterone acetate or the gonadotropin-releasing hormone analog, goserelin, not appropriate for long term use), surgery (hysterectomy and oophorectomy, not appropriate for childbearing age groups), and endovascular procedures (embolization, sclerotherapy). When endovascular therapy is elected, bilateral embolization or sclerotherapy of the internal iliac veins and the ovarian veins may be necessary to achieve success. Of note, between 66-100% of treated patients experience complete or partial symptom improvement (Meissner and Gibson, 2015).
Nutcracker Syndrome
Nutcracker syndrome typically presents with flank pain, hematuria, renal hilum varicosities, and pelvic pain secondary to compression of the left renal vein. This compression produces ovarian or gonadal vein dilation, reflux into the uterine venous plexus (or hydroceles in men), and painful pelvic venous varicosities. Treatment modalities include surgery and endovascular interventions. Open surgical left renal vein transposition is the treatment of choice; for patients undergoing surgical treatment, complete resolution of symptoms has been observed in 87% of patients during a mean follow up of 37 months (Velasquez et al, 2018; Erben et al, 2015). While long term success has not yet been established for endovenous stents in the left renal vein, early results are promising.
May-Thurner Syndrome
May-Thurner syndrome (and pelvic external venous compression) describes outflow obstruction of the iliac vein secondary to compression between the iliac artery and posterior bony structures and is more common on the left side. Symptoms are commonly vague and include symptoms of PCS as well as lower leg swelling, pain, and varicosities. Signs include venous hypertension and can even lead to deep venous thrombosis (Hernandez-Rodriguez et al 2009; Obara 2018). Intravascular ultrasound is used to confirm the diagnosis and to guide treatment. Treatment options include surgical angioplasty usually with stenting of the vessel. Of note, one year after iliac venous stenting, 80% of those with pelvic venous congestion symptoms from May-Thurner syndrome reported resolution of pain and dyspareunia (O'Brien and Gillespie, 2015). Embolization (internal iliac and gonadal veins) may be considered if angioplasty or stenting fails to resolve the obstruction or venous hypertension (O'Brien and Gillespie, 2015).
Vasculitis
Vasculitis is a rare cause of pelvic pain secondary to vessel inflammation and immune cell infiltration into the vascular wall (Roane, 1999). Pelvic pain secondary to vasculitis can occur as an isolated gynecologic vasculitis or as part of one of the established vasculitides: Takayasu’s arteritis, giant (temporal) cell arteritis, granulomatosis with polyangiitis (formerly Wegener’s), eosinophilic granulomatosis with polyangiitis (formerly Churg Strauss), microscopic polyangiitis, or polyarteritis nodosa (Traisak et al, 2016; Hernandez-Rodriguez et al, 2009; National Institute of Neurological Disorders and Stroke, 2020). Of the aformentioned vasculitides, giant (temporal) cell arteritis is the most common. Patients with vasculitis may present with fever, fatigue, weight loss, joint pain, ocular inflammation, upper and lower airway involvement, absent or diminished pulses, or purpura (Merkel, 2019; National Institute of Neurological Disorders and Stroke, 2020). Diagnostic testing includes identifying antibodies common in autoimmune vasculitis, such as ANA and ANCA. Chest x-ray, vascular imaging, or biopsy may also aid in diagnosis (Merkel, 2019). Cornerstones of treatment include glucocorticoids and/or immunomodulatory therapies (Roane, 1999). It is important to be aware of vasculitis in the differential diagnoses of these patients to prevent delay in diagnosis and treatment.
Ischemic Pelvic Disease
Ischemic pelvic disease refers to an acute or chronic reduction of blood flow in the vessels supplying the pelvis. A common cause is atherosclerosis in the aorta and in the common and internal iliac arteries, resulting in luminal narrowing (Obara, 2018; Antunes-Lopez et al, 2019). Risk factors include smoking, hypertension, diabetes mellitus, hyperlipidemia, and physical inactivity (National Heart, Lung and Blood Institute, 2019; Davies, 2019). Patients may present with numbness, tingling, cramps, and aches in the pelvis, buttocks, thighs, legs, or feet, and sexual dysfunction. Muscle atrophy, poor hair growth, incomplete wound healing, and formation of skin ulcers may also be observed. Intermittent claudication, limb and/or pelvic pain exacerbated by activity and alleviated by rest, is characteristic and can be managed with cilostazol, which has vasodilatory action (National Heart, Lung and Blood Institute, 2019). Diagnosis is made typically by ankle-brachial index (or rarely, penile-brachial index in men) to assess arterial insufficiency; ultrasound may also be used to assess blood flow. In rare instances, including equivocal results in first-line diagnostic testing, CTA, MRA, or invasive vascular imaging may assist with the diagnosis (National Heart, Lung and Blood Institute, 2019; Davies, 2019). Treatment focuses on smoking cessation as well as medical management of hyperlipidemia, elevated blood sugar, and hypertension. Cardioprotective drugs including aspirin and clopidogrel are indicated in the patient with ischemic pelvic disease or peripheral artery disease. If medical intervention fails, treatment is indicated with endovascular or surgical revascularization of discrete vascular lesions.
Conclusion
For the patient who presents with pelvic pain, vascular etiologies are important to consider. The history and physical exam can help guide diagnostic imaging and laboratory evaluation. Treatment strategies are varied and depend on clinical presentation, severity, comorbidities, and patient preferences.
This article is a summary of vascular conditions associated with pelvic pain found in Chapter 10 of Facing Pelvic Pain: A Guide for Patients and Their Family Members (De E, Stern TA (eds), due for publication September 2020; Massachusetts General Hospital Psychiatry Academy, via Amazon). https://www.facingpelvicpain.org/ The publication, written for the lay public but detailed enough for providers, provides a comprehensive discussion of causes and treatment for pelvic pain of all etiologies.
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