Frequency of Adnexal Torsion in HEDS Compared to the General Population

Adnexal torsion is a medical emergency that occurs when an ovary or fallopian tube twists, cutting off its blood supply. While this condition is uncommon in the general population, it raises an important question for those with hypermobile Ehlers-Danlos syndrome (HEDS): how often does adnexal torsion occur in people with HEDS?

What Is Adnexal Torsion?

Adnexal torsion involves the twisting of the ovary and sometimes the fallopian tube, which can cause severe pelvic pain, nausea, and vomiting. If untreated, it can lead to loss of ovarian function due to ischemia. This condition requires prompt diagnosis and surgical intervention to save the affected tissue.

In the general population, adnexal torsion is relatively rare. It accounts for about 2.7% of all gynecologic emergencies and occurs in approximately 5 per 100,000 women annually. The risk is higher in women of reproductive age, especially those with ovarian cysts or masses.

What Is HEDS and Why Could It Affect Adnexal Torsion Risk?

Hypermobile Ehlers-Danlos syndrome (HEDS) is a connective tissue disorder characterized by joint hypermobility, skin elasticity, and tissue fragility. The syndrome affects collagen, a key structural protein in the body, which can lead to increased tissue laxity.

This increased laxity may affect the ligaments that support the ovaries and fallopian tubes, potentially making adnexal torsion more likely. Ligaments that are more flexible or weaker could allow the adnexa to twist more easily.

How Often Does Adnexal Torsion Occur in People with HEDS?

Research specifically quantifying the frequency of adnexal torsion in HEDS is limited. However, clinical observations and case reports suggest that adnexal torsion may be more common in this group than in the general population.

Evidence from Case Reports and Clinical Observations

• Several case studies describe women with HEDS experiencing adnexal torsion, sometimes recurrently.

• These cases often involve younger women with no other typical risk factors like ovarian cysts.

• The ligamentous laxity in HEDS is thought to contribute to the increased risk.

  
  

Estimated Frequency Compared to General Population

While exact numbers are not well established, some specialists estimate the risk of adnexal torsion in HEDS patients could be several times higher than in women without connective tissue disorders. For example, if the general population risk is about 5 per 100,000 annually, the risk in HEDS might be closer to 15–20 per 100,000 or more, based on clinical experience.

More research is needed to confirm these estimates, but the trend suggests a meaningful increase.

Why Does This Matter for Women with HEDS?

Understanding the increased risk of adnexal torsion in HEDS has practical implications:

• Early Recognition: Women with HEDS and sudden pelvic pain should be evaluated promptly for adnexal torsion.

• Preventive Care: Gynecologists may consider closer monitoring of ovarian health in HEDS patients.

• Surgical Planning: Surgeons may need to be aware of tissue fragility and ligament laxity when treating adnexal torsion in HEDS.

• Patient Education: Women with HEDS should be informed about symptoms of adnexal torsion to seek care quickly.

  
  

Women with HEDS should be alert to symptoms that could indicate adnexal torsion:

• Sudden, severe pelvic or lower abdominal pain, often on one side

• Nausea and vomiting accompanying the pain

• Abdominal tenderness or swelling

• Pain that may come and go if the torsion is intermittent

  
  

Prompt medical attention is critical because delayed treatment can lead to loss of ovarian function.

Diagnosis typically involves:

• Pelvic ultrasound with Doppler to assess blood flow to the ovary

• Clinical examination and patient history

• Sometimes MRI or CT scans if ultrasound is inconclusive

  
  

Treatment requires surgery to untwist the ovary and restore blood flow. In some cases, the ovary may need to be removed if it is severely damaged.

For women with HEDS, surgeons may take extra care due to fragile tissues and increased bleeding risk.

While adnexal torsion cannot always be prevented, women with HEDS can take steps to manage their health:

• Regular gynecologic check-ups to monitor ovarian cysts or masses

• Immediate evaluation for pelvic pain, especially if sudden and severe

• Discussing their HEDS diagnosis with healthcare providers to ensure tailored care

• Considering lifestyle adjustments to reduce strain on pelvic ligaments, such as avoiding heavy lifting or high-impact activities during flare-ups

  
  

The Need for More Research

The link between HEDS and adnexal torsion highlights a gap in medical research. More studies are needed to:

• Quantify the exact risk of adnexal torsion in HEDS

• Understand the underlying mechanisms of increased torsion risk

• Develop guidelines for screening and management specific to HEDS patients

  
  

This knowledge will help improve outcomes and quality of life for women with HEDS. Knowing that Adnexal torsion is a risk factor women who develop similar symptoms can let their medical team know they have HEDS and to rule the condition out.

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