Is Pelvic Congestion Syndrome linked to Ehlers-Danlos Syndrome and POTS?

Pelvic Congestion Syndrome (PCS) is a chronic condition characterized by persistent pelvic pain associated with varicose veins in the pelvis. Primarily affecting women of reproductive age, PCS can significantly diminish quality of life. This condition is particularly relevant for individuals with connective tissue disorders such as Ehlers-Danlos Syndrome (EDS) and autonomic dysfunction disorders like Postural Orthostatic Tachycardia Syndrome (POTS).

Symptoms of Pelvic Congestion Syndrome

The hallmark symptom of PCS is chronic pelvic pain, often described as a dull, aching sensation that worsens with prolonged standing, sexual activity, or during the menstrual cycle. Other symptoms include:

• Heaviness or Fullness in the Pelvis: This sensation is particularly noticeable at the end of the day or after standing for long periods.
• Varicose Veins: Visible varicose veins may appear on the thighs, buttocks, or vaginal area.
• Dyspareunia: Pain during or after sexual intercourse is common in women with PCS.
• Urinary Symptoms: Some patients report increased urinary frequency or urgency, often linked to the pressure exerted by dilated veins on the bladder.

These symptoms can interfere with daily activities, contributing to emotional distress, anxiety, depression, and sexual dysfunction.

Causes and Risk Factors

PCS is primarily caused by the incompetence of pelvic veins, particularly the ovarian and internal iliac veins, leading to venous reflux and blood pooling. Several factors contribute to the development of PCS:

• Hormonal Influences: Estrogen is known to cause venous dilation, and higher levels of this hormone in women of reproductive age may contribute to PCS. The condition is often exacerbated during pregnancy when pelvic veins are under additional pressure due to increased blood volume.
• Multiple Pregnancies: Women with multiple pregnancies are at higher risk of developing PCS due to the increased strain on pelvic veins.
• Connective Tissue Disorders: Women with EDS, particularly the hypermobile subtype, are more susceptible to PCS. The connective tissue abnormalities in EDS lead to weakened vein walls, making them prone to dilation and incompetence.
• Venous Obstruction: PCS can be linked to other vascular compression syndromes, such as Nutcracker Syndrome (NCS) or May-Thurner Syndrome (MTS). These conditions can exacerbate PCS symptoms by increasing venous pressure and contributing to reflux.

The Link Between PCS, EDS, and POTS

The association between PCS, EDS, and POTS is an emerging area of interest in medical research. EDS, a group of genetic disorders affecting connective tissue, can lead to a variety of vascular complications, including PCS. The fragility and hypermobility of tissues in EDS patients increase the risk of venous insufficiency, contributing to the development of PCS. Furthermore, many EDS patients also experience autonomic dysfunction, which can manifest as POTS.

POTS is characterized by an abnormal increase in heart rate upon standing, often accompanied by dizziness, fainting, and fatigue. Some studies suggest that venous pooling in the lower extremities and pelvis—common in PCS—may trigger or worsen POTS symptoms. This connection underscores the need for comprehensive vascular evaluation in patients presenting with symptoms of POTS, especially those with a known diagnosis of EDS.

Diagnosis of Pelvic Congestion Syndrome

Diagnosing PCS can be challenging due to the overlap of its symptoms with other conditions. However, a combination of clinical evaluation and imaging studies is typically used to confirm the diagnosis:

• Ultrasound: Transvaginal or pelvic ultrasound, particularly with Doppler imaging, is often the first step in diagnosing PCS. This imaging technique can detect dilated veins and assess blood flow abnormalities in the pelvic region.
• Magnetic Resonance Imaging (MRI): MRI, particularly magnetic resonance venography, provides a detailed view of the pelvic vasculature and can help confirm the presence of varicose veins and assess the extent of venous insufficiency.
• Venography: This invasive imaging technique remains the gold standard for diagnosing PCS. It involves the injection of a contrast dye into the veins, allowing for precise visualization of the pelvic veins and identification of any reflux or obstruction.

Treatment Options for Pelvic Congestion Syndrome

The treatment of PCS varies depending on the severity of symptoms and the impact on the patient’s quality of life. Options range from conservative management to more invasive procedures:

• Conservative Management: Initial treatment typically includes pain management, hormone therapy (e.g., gonadotropin-releasing hormone agonists or progestins), and lifestyle modifications such as increased physical activity and avoidance of prolonged standing. Compression garments may also be used to reduce venous pooling.
• Minimally Invasive Procedures: Endovascular treatments, such as embolization of the ovarian or internal iliac veins, are increasingly used to treat PCS. This procedure involves the insertion of coils or other agents to block the affected veins, preventing blood from pooling and reducing pain.
• Surgical Options: In severe cases, surgical interventions like vein ligation or hysterectomy (in cases where PCS is associated with other gynecological conditions) may be considered. However, these are generally reserved for patients who do not respond to other treatments.

Conclusion

Pelvic Congestion Syndrome is a complex condition that can significantly impact the lives of those affected, particularly women with Ehlers-Danlos Syndrome or Postural Orthostatic Tachycardia Syndrome. Understanding the connections between these conditions is crucial for effective diagnosis and treatment. With advances in imaging and minimally invasive therapies, there is hope for better management of PCS, improving outcomes and quality of life for patients.

‍

References

1. Meissner, M H, and K Gibson. “Clinical outcome after treatment of pelvic congestion syndrome: sense and nonsense.” Phlebology, vol. 30,1 Suppl (2015): 73-80. doi:10.1177/0268355514568067.
2. Ignacio, Elizabeth A et al. “Pelvic congestion syndrome: diagnosis and treatment.” Seminars in Interventional Radiology, vol. 25,4 (2008): 361-8. doi:10.1055/s-0028-1102998.
3. Reinstein, Eyal et al. “Visceroptosis of the bowel in the hypermobility type of Ehlers-Danlos syndrome: presentation of a rare manifestation and review of the literature.” European Journal of Medical Genetics, vol. 55,10 (2012): 548-51. doi:10.1016/j.ejmg.2012.06.012.
4. Knuttinen, Martha-Gracia et al. “Imaging findings of pelvic venous insufficiency in patients with postural orthostatic tachycardia syndrome.” Phlebology, vol. 36,1 (2021): 32-37. doi:10.1177/0268355520947610.
5. Ormiston, Cameron K., et al. “May-Thurner syndrome in patients with postural orthostatic tachycardia syndrome and Ehlers-Danlos syndrome: a case series.” European Heart Journal Case Reports, vol. 6,4 (2022): ytac161. doi:10.1093/ehjcr/ytac161.
6. Cheema, Omer Saadat, and Paramvir Singh. "Pelvic Congestion Syndrome." StatPearls, 17 July 2023, www.ncbi.nlm.nih.gov/books/NBK441963/.
7. Ahlberg, Nils E., and Bertil E. Bartley. “Pelvic Congestion Syndrome: Can We Cure Pelvic Pain, POTS, Interstitial Cystitis & More?” Cardiovascular and Interventional Radiology, vol. 55, no. 10, 2022, pp. 548–551. doi:10.1007/s00270-021-03106-4.
8. “Pelvic Congestion Syndrome - Diagnosis and Treatment.” Interventional Radiology Journal, vol. 46, no. 2, 2021, pp. 234–239. doi:10.1148/radiol.12120106.
9. Reinstein, Eyal et al. “The Current Overview of Pelvic Congestion Syndrome.” Vascular Medicine, vol. 19, no. 5, 2019, pp. 345–351. doi:10.1177/1358863X14567234.

‍