Pelvic congestion syndrome (PCS) is a condition often seen in multiparous women of premenopausal age, and is characterized by chronic pelvic pain and dyspareunia associated with pelvic varicosities.
Presentation
PCS may present with a variety of symptoms which may be nonspecific. Pelvic pain and postcoital ache, particularly on the left side, are the most classic symptoms of PCS. Patients may experience an acute and severe pelvic fullness or a chronic and dull pain. Pain may worsen with sitting, standing, or just before menstrual periods. Women with PCS may have other nonspecific symptoms like pelvic tenderness, lethargy, depression, vaginal discharge, dysmenorrhea, rectal discomfort or an increase in frequency of urination. Patients may present with tenderness over the ovaries or uterus during physical examination. Pelvic fullness, ovarian tenderness and chronic pain are the specific manifestations of PCS.
Workup
Definitive diagnosis of PCS is often a challenge and may take a long time for many patients. Interventional radiology helps in evaluation of the condition once other pelvic causes are ruled out. Many traditional imaging techniques may miss diagnosis of PCS as venous distention may not be clear or may be absent in the image. Ultrasound with Doppler imaging provides a good image of venous blood flow in the pelvic region [8]. Doppler diagnosis is used when the ovarian veins are greater than 4 mm in diameter, and there is a retrograde blood flow in left ovarian vein [9]. MRI is yet another imaging modality primarily used in the diagnosis of PCS. This imaging is also very sensitive in locating and assessing pelvic varices.
Laparoscopy is a method for direct visualization of the varices and helps to rule out other causes of chronic pelvic pain like endometriosis. But in many patients, laparoscopy may not give a positive image. Pelvic venogram gives information on dynamic blood flow along with measurements of both ovarian and pelvic veins [10]. Diagnostic venogram also has the benefit of performing embolotherapy as a treatment, when needed. A transfundal pelvic venogram, in which a catheter is placed into the myometrium, shows venous abnormalities in the uterus. But ovarian and pelvic varices may be missed in the venogram.
Treatment
Pharmacological treatment options often try to address the chronic pain associated with PCS. Pain relief may be obtained by non-steroidal anti-inflammatory drugs. Other medical treatment methods include progestins, danazol, phlebotonics, dihydroergotamine, and hormone replacement therapy. Medroxyprogesterone acetate is used to increase venous contraction.
Extraperitoneal resection of the left ovarian vein is the surgical method used to improve symptoms of PCS [11]. Hysterectomy is useful in relieving the symptoms to a certain extent, but 33% of the patients were reported to have residual pain after the treatment. Laparoscopic ligation of ovarian veins is a popular treatment option, but has the disadvantages of significant morbidity, and hospital stay for the procedure. Pelvic vein embolization therapy is effective in reducing morbidity associated with surgery. This procedure has become more popular as the accepted treatment modality for treating PCS [12].
Some of the possible complications associated with embolization include coil migration, renal vein thrombosis, and perforation of ovarian vein. Chances of recurrence can be reduced by using bilateral venography and embolization of both ovarian and iliac veins. Often treatment is done in a staged way starting with embolization of right and left ovarian varices followed by pelvic varices after about a month.
Prognosis
PCS may cause distress to many women. None of the treatment modalities currently used are fully successful in treating the condition. Chronic pain may cause anxiety, depression, and physical worries [5]. Endovascular treatment has a good prognosis when compared to many other treatment options. Laparoscopic ligation of ovarian veins results in remission of pain and reduced pelvic varicosities for about a year [6]. But surgical management elevates the risk of pelvic adhesion formation, and thus increases morbidity. Ovarian and pelvic venous embolization gives a good prognosis [7].
Etiology
Multiple factors are thought to be involved in the etiology of this syndrome. PCS may be classified on the basis of variations in etiology. This includes PCS caused by:
- Anatomic dysfunction
- Psychosomatic dysfunction
- Hormonal dysfunction
- Latrogenically induced dysfunction
- Neuropathic dysfunction
An incompetent venous valvular system in the pelvis causes stasis, congestion and pain that are characteristic of this syndrome. It is reported that pregnancy induces an increase in the capacity of ovarian veins up to 60 times the normal. This may continue for more than six months postpartum and makes pregnancy one of the major risk factors for developing this syndrome. Uterine malposition is also considered as a possible etiology of PCS by anatomic dysfunction.
Studies show that stress affects the functioning of smooth muscle and secretory cells leading to psychosomatic effects. These lead to chronic congestion of vessels and, ultimately, PCS. PCS of psychosomatic origin was supported by studies that showed patients with this syndrome tend to be more neurotic than normal women.
Women with PCS were found to have many other hormonally induced conditions like multicystic ovaries, larger uterus, and thicker endometrium [2]. This indicates the possibility of the condition being triggered by hormonal dysfunction. Hormonal changes lead to alterations in intraluminal pressure leading to weakening of ovarian vein walls. Valvular incompetence of this kind plays a key role in development of congestion and valvular stasis.
Intrauterine devices used in contraception and tubal sterilization procedures are both presumed to be associated with the development of PCS. In one of the studies, about 60% of the patients with PCS were found to have undergone tubal ligation process earlier [3]. Malformed vessels of PCS produce neurotransmitters which are considered to be responsible for the development of this syndrome.
Epidemiology
PCS is more commonly seen in women of reproductive age, particularly those below the age of 45 years. Pregnancy increases the risk of ovarian congestion and, thus, PCS. The syndrome is rarely found in women who have not been pregnant. About 30% of the women with chronic pelvic pain is diagnosed with PCS, and the pain is caused exclusively by the condition. About 15% of patients with chronic pelvic pain have PCS along with other pelvic pathology. Ovarian varices are also an important risk factor in the development of PCS. About 60% of the patients with ovarian varices develop this syndrome.
Pathophysiology
Pathogenesis of PCS is multifactorial. Deficiency in the valves of the ovarian veins is one of the key factor that lead to pathogenesis of this syndrome. Many hormonal and mechanical factors contribute to the development of pelvic varices, a major etiological factor of PCS. Both ovarian and pelvic varices result in the chronic, dull aching pain characteristic of PCS.
Multiple previous pregnancies often lead to an increase in intravascular volume and vein capacity to over 60%. Distention of veins results in incompetent valves. Pregnancy is also associated with weight gain and changes in the pelvic structure, both of which result in venous obstruction. This leads to accumulation of blood in the veins resulting in engorgement and clotting. Nerves in the surrounding tissues are affected and pain ensues [4].
Estrogen, the main female hormone, is known to weaken the walls of veins. Thus, levels of estrogen in the body, particularly in premenopausal women, is presumed to be associated with the syndrome. Retroaortic left renal vein causes obstruction in left ovarian vein and pelvic varices. Pelvic varices may also result from compression of the left common iliac vein against the spine.
Vein congestion may be caused secondarily by portal hypertension, increased flow of blood in the pelvic veins, and vascular malformations. Pelvic varices are formed by portosystemic shunt in portal hypertension. Pelvic tumors may occasionally cause ovarian vein distention by venous outflow obstruction or by an increase in the flow of blood.
Prevention
Controlling the risk factors associated with the condition is the only known preventive measure for PCS.
Summary
Pelvic congestion syndrome (PCS) is a common condition often seen in multiparous women of premenopausal age. It is characterized by chronic pelvic pain and dyspareunia associated with the presence of ovarian and pelvic varicosities. Both hormonal and mechanical factors play and important etiological role in the development of varicosities. Blood flow through the congested veins of pelvis region results in chronic pain, pressure and heaviness. It may also manifest with dysmenorrhea, and post coital pain.
About 10% of the women in the general population have ovarian varices, of which 60% may develop this syndrome [1]. Imaging techniques are used to locate and evaluate the dilated and tortuous uterine and ovarian vessels, which extend to the side walls of the pelvis. Some of the major risk factors of the syndrome include two or more pregnancies, and hormonal dysfunction. Embolization is considered to be a safe and effective way to treat PCS.
Patient Information
Pelvic congestion syndrome (PCS) refers to the condition caused by the presence of varicose veins in ovary or pelvis. It is characterized by a chronic, dull pain in the pelvis which may increase with standing for a long time. PCS is a common gynecologic problem seen in women in reproductive age. Both mechanical and hormonal factors are known to cause this syndrome. Some of the factors that increase the chances of developing PCS include hormonal changes, previous multiple pregnancies, polycystic ovaries, and fullness of leg veins.
Chronic, dull pain in the pelvic region is the classic, characteristic symptom of this condition. The pain may be felt in the lower abdomen and lower back. It may increase during menstrual periods, after intercourse, after standing for a long time, and during pregnancy. Other symptoms of PCS are not so specific and includes increased frequency of urination, abnormal bleeding during menstrual periods, vaginal discharge, and presence of varicose veins in vulva and buttocks. A thorough pelvic examination will help in ruling out the chances of other diseases that may lead to chronic pain. Imaging techniques are used to confirm the diagnosis of PCS. The techniques include pelvic venography, in which a dye is injected into the veins to make them visible during X-ray. MRI is also used in diagnosing the affected veins in PCS. Pelvic and transvaginal ultrasound are other techniques commonly used in locating and assessing the condition.
Embolization is a minimally invasive procedure in which a catheter is inserted into the vein and directed to affected vein using imaging methods. Tiny coils are inserted into the affected vein to relieve the pressure inside the vessel. Other treatment methods depend on the symptoms. Analgesics are recommended to reduce the pain. Hormonal therapy is suggested in some cases to control the symptoms. Other surgical options include hysterectomy with removal or tying of affected veins. Of the different methods, embolization is considered to be a safe and effective method to treat PCS.
References
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