How to manage groin pain in a patient with Ehlers-Danlos syndrome (EDS)?

Management of Groin Pain in Ehlers-Danlos Syndrome

The management of groin pain in patients with Ehlers-Danlos syndrome (EDS) should focus on treating the most prominent symptoms while addressing the underlying joint hypermobility and tissue fragility, with occupational therapy and bracing being the most effective initial interventions. 1

Understanding EDS-Related Groin Pain

Groin pain in EDS patients typically stems from:

• Joint hypermobility leading to hip instability and subluxations
• Pelvic floor dysfunction
• Tissue fragility affecting ligaments and tendons
• Visceral hypersensitivity from gastrointestinal manifestations

Diagnostic Approach

1. Assess joint hypermobility using the Beighton score:

   • Score ≥5 indicates significant hypermobility
   • Consider applying 2017 diagnostic criteria for hEDS if positive 2

2. Rule out gastrointestinal causes:

   • Test for celiac disease (higher prevalence in EDS patients) 2
   • Consider anorectal manometry, balloon expulsion test, or defecography if lower GI symptoms are present 2
   • Evaluate for pelvic floor dysfunction, especially rectal hyposensitivity 2

3. Screen for comorbidities:

   • Postural orthostatic tachycardia syndrome (POTS)
   • Mast cell activation syndrome (MCAS)
   • These conditions frequently coexist with EDS and can exacerbate pain 2

Treatment Algorithm

First-Line Interventions

1. Occupational therapy and bracing (70% improvement rate) 1

   • Custom hip bracing to improve stability
   • Pelvic support devices
   • Activity modification techniques

2. Physical therapy

   • Focus on core and hip stabilization exercises
   • Avoid hyperextension and joint stress
   • Gentle strengthening of supporting muscles

3. Complementary/alternative treatments

   • Most commonly used approach in EDS patients 1
   • Heat therapy, gentle massage
   • Mindfulness and relaxation techniques

Second-Line Interventions

1. Pharmacological management:

   • NSAIDs for acute pain flares
   • Acetaminophen for baseline pain management
   • Muscle relaxants for associated muscle spasms
   • Avoid opioids as they should not be used specifically to treat pain in EDS 2

2. Neuromodulators (use with caution):

   • Consider tricyclic antidepressants, SNRIs, or gabapentinoids
   • Note: 47% of EDS patients report adverse effects with these medications 1

Advanced Interventions

For refractory groin pain:

1. Interventional procedures:

   • Trigger point injections
   • Ultrasound-guided hip joint injections
   • Nerve blocks (ilioinguinal, genitofemoral)

2. Surgical consultation (limited role):

   • Reserved for cases with clear anatomical abnormalities
   • Higher complication rates due to tissue fragility
   • Careful patient selection is crucial

Special Considerations

• Gastrointestinal management may be necessary if GI symptoms contribute to groin pain 2
• POTS management if present: increased fluid/salt intake, compression garments, and exercise training 2
• MCAS treatment if suspected: histamine receptor antagonists and mast cell stabilizers 2

Pitfalls to Avoid

1. Overreliance on opioids - can lead to dependence without addressing underlying issues 2
2. Aggressive physical therapy - may worsen symptoms due to tissue fragility
3. Ignoring comorbidities - POTS and MCAS can amplify pain perception
4. Delayed diagnosis - can lead to chronic pain and disability 3
5. Overlooking pelvic floor dysfunction - common in EDS patients 2

Monitoring and Follow-up

• Regular assessment of pain levels and functional status
• Adjustment of bracing and supportive devices as needed
• Periodic reevaluation for complications or new symptoms

By following this structured approach to managing groin pain in EDS patients, clinicians can help improve pain control, maintain function, and enhance quality of life while minimizing the risk of complications.