What is the best management approach for a patient with hypermobile Ehlers-Danlos Syndrome (hEDS) and childhood-onset hip joint asymmetry?

Management of Childhood-Onset Hip Joint Asymmetry in Hypermobile Ehlers-Danlos Syndrome

For a patient with hEDS and childhood-onset hip joint asymmetry, prioritize conservative management with physical therapy and bracing while delaying orthopedic surgery, as surgical outcomes are significantly worse in hEDS patients compared to those without connective tissue disorders. 1

Primary Management Strategy

Orthopedic surgery should be delayed in favor of physical therapy and bracing because patients with hEDS hypermobile type experience decreased stabilization and pain reduction outcomes from surgical interventions compared to those without EDS. 1 This recommendation is particularly critical given that the hip asymmetry developed during childhood, suggesting an underlying structural abnormality that may represent developmental dysplasia or growth-related deformity superimposed on the connective tissue disorder.

Conservative Treatment Approach

• Initiate low-resistance exercise programs to improve joint stability by increasing muscle tone around the asymmetric hip joints. 1

• Physical therapy with myofascial release techniques is essential and often necessary before patients can participate effectively in exercise programs. 1 Research demonstrates that physiotherapy benefits proprioception and pain in hEDS patients, with improvements seen regardless of intervention type over 4-8 week programs. 2

• Bracing should be considered as part of the conservative management strategy to provide external joint stabilization. 1

Pain Management Protocol

Given the chronic nature of musculoskeletal pain in hEDS with structural hip abnormalities:

• Engage a pain management specialist early in the treatment course, as chronic pain is a hallmark feature requiring specialized expertise. 1

• Start with gabapentin as first-line pharmacologic therapy, titrating to 2400 mg daily in divided doses for neuropathic pain components. 1

• Consider neuromodulators including tricyclic antidepressants (particularly amitriptyline 75-100 mg), SSRIs, SNRIs, or pregabalin (75-300 mg every 12 hours) as alternatives or adjuncts. 1

• Paracetamol (acetaminophen) is safe for pain management in this population. 1

• Never prescribe NSAIDs as they can worsen gastrointestinal symptoms and are generally contraindicated in hEDS. 1

• Avoid opioids specifically for chronic pain management. 1

Monitoring and Multidisciplinary Coordination

• Coordinate care among multiple specialists including orthopedics, physical medicine and rehabilitation, pain management, and potentially cardiology (for aortic root monitoring with echocardiogram every 2-3 years). 1

• Consider vitamin C supplementation as it serves as a cofactor for cross-linking of collagen fibrils and may improve hypermobility. 1

• Ensure calcium and vitamin D supplementation for bone health, combined with low-impact weight-bearing exercise. 1

• Consider DXA scan if height loss greater than one inch occurs, as bone health may be compromised. 1

Non-Pharmacologic Interventions

• Cognitive Behavioral Therapy (CBT) is strongly recommended for chronic pain management, promoting patient acceptance and development of adaptive behaviors. 1

• Yoga is recommended for chronic musculoskeletal pain, including back pain and headaches. 1

Critical Pitfalls to Avoid

• Do not rush to surgical correction of the hip asymmetry, as the evidence clearly shows inferior outcomes in hEDS patients. 1 The structural abnormality that developed during childhood cannot be adequately stabilized through surgery given the underlying connective tissue disorder.

• Avoid aggressive physical therapy that causes pain or iatrogenic injuries, as many hEDS patients report harm from inappropriate interventions. 3 Programs should emphasize gradual progression and joint protection.

• Do not overlook systemic manifestations including autonomic dysfunction (POTS), gastrointestinal symptoms, and psychological comorbidities that commonly accompany hEDS and may impact rehabilitation tolerance. 1, 4

Surgical Considerations (If Conservative Management Fails)

If the hip asymmetry progresses to severe degenerative joint disease despite conservative management, recognize that untreated developmental hip abnormalities inevitably lead to early arthritis. 5 However, even in this scenario, delay surgery as long as possible given the poor outcomes in hEDS, and ensure the patient understands that mechanical failure rates are higher compared to arthroplasty for other indications. 5