What is Pelvic Congestion Syndrome

This often underdiagnosed condition can cause significant distress to sufferers. This article will detail symptoms, diagnosis and treatment options to help inform patients about their condition or possible diagnosis. 

Introduction

Pelvic congestion syndrome (PCS) was first described in 1831.¹ However, given the number of possible diagnoses for the main symptom of PCS, pelvic pain, diagnosis can be difficult.¹,^2,3 It is defined as chronic pelvic pain due to abnormally functioning and dilated pelvic veins.² Although dilated pelvic veins may be the cause of pelvic pain in a number of women (15-40% of women presenting with pelvic pain), dilated pelvic veins can also be present in those without symptoms.² 

PCS is most commonly diagnosed in women aged 20-40 years and rarely seen in post-menopausal women.²,³ 

Pathophysiology

Physical, mechanical and hormonal factors are thought to interplay.^2,3 The resultant obstruction or reflux of veins results in dilatation (or varicosities) of pelvic veins causing the aching pain that is the main symptom, akin to the pain felt from varicose veins in the legs.³ Drainage of the pelvic veins is complex involving plexuses (crossovers) and anatomy can vary between individuals.² Valves are present to prevent reflux or passage of blood in the wrong direction. Should they be absent (15% of the left ovarian vein, and 6% of the right ovarian vein valves are absent), blood can pool, causing dilatation.² Multiparity - multiple pregnancies - also seems to be involved. There is an increase in venous volume up to 60-fold in the pregnant state, and over time, this can cause incompetencies of the veins and dilatation.²,³

The pathophysiology of PCS pain is not fully understood. Suggestions include the distortion of the vessel wall release substances (neurokinins) which are known to contribute to inflammatory processes and pain. Physical irritation on adjacent nerves from dilated veins is also a possibility.²

Symptoms

The main symptom is of an aching pelvic pain that is non-cyclical (not related to menstrual cycle)  often worse after standing/walking and easing on lying down.²,³ Intercourse can also worsen pain and the pain can worsen before the menstrual cycle.²,³ Other symptoms can be present including urinary symptoms of needing to pass urine frequently and with urgency, and with perineal and lower limb varicose veins.²,³ However, there is no pathognomonic symptom of PCS with symptoms heavily overlapping with other causes. This makes PCS a challenging diagnosis and may result in underdiagnosis of PCS.¹,²,³

Pelvic pain can significantly and negatively affect the quality of life in women.^3,4 Those with chronic pelvic pain report higher rates of anxiety and depression.^3,4 This can also affect the patient's partner, who can often suffer associated emotional stress^.4

Diagnosis of pelvic congestion syndrome

The presenting patient will be evaluated for chronic pelvic pain, with full history and clinical examination being essential.⁵ Although not required for PCS, tests to rule out infections such as pelvic inflammatory disease as the cause for pains would include swabs for sexually transmitted infections.⁵

Ultrasound is a first-line test for pelvic pain and US with Doppler allows assessment of blood flow in the vessels including the veins to assess for incompetency. There are specific criteria for sonographic (Ultrasound) diagnosis of varicose veins and ultrasound allows dynamic visualisation which CT / MRI does not.²,³ 

CT and MRI allow more details and cross-sectional evaluation and CT does have a higher sensitivity for showing varicosities of the veins.²,³

Venography is a minimally invasive method of visualizing the veins requiring access to the veins from the groin usually and does involve some radiation.²,³ This is done by an interventional radiologist and allows dynamic flow assessments. Another added benefit is that treatment can be done at the same time.²,³  

Laparoscopy is performed as part of a workup for pelvic pain in many cases but may miss PCS in  80-90% of cases.²,³ Dilated veins may be compressed and not apparent because of the position of the patient for the procedure in addition to insufflation of air into the cavity.^2,3

Differential diagnoses

Other causes of Pelvic pain include:

Endometriosis

Pelvic inflammatory disease

Adenomyosis

Adhesion

Treatment Options

Therapeutic options are tailored to the patient's symptoms and needs.

 Non-surgical management

Medicine management includes using analgesia, including non-steroidal anti-inflammatory medications, given the postulated pathways involved in the symptoms of pain. Hormonal treatments (GnRH agonists)  to reduce the activity of the ovaries have some support in literature including one randomised control trial.^2,4

Therapy for chronic pain can be considered alongside other techniques as psychological interventions can reduce symptoms of pain anxiety and depression^.4

Invasive techniques

Surgical treatment 

Ligation (tying) of the veins during laparoscopy is an option for some cases.² Embolisation is another way of blocking a vessel that has been utilitsed and in trials has had positive results in terms of pain improvement for women - 70 80% reported improvement in pain. Embolisation can use sclerosing agents which block the vein or coils and can be done by interventional radiologists at the time of diagnostic procedures.¹,³

Hysterectomy remains an option for those where other treatment options have failed but appropriate discussions must be had before this level of surgery.² This does not always treat symptoms.¹

Unfortunately limited clinical trials have been undertaken in this area and evidence is limited to case series and cohort studies rather than gold-standard randomized controlled trials.¹,²

Summary

Pelvic Congestion Syndrome is a challenging diagnosis presenting with chronic pelvic pain. It has many symptoms that cross over with other causes of pelvic pain and require several investigations to both diagnose and exclude other causes. It can have a significant impact on the quality of life of those who suffer. There are many possible treatments which have had positive results but further research is needed.