What is the best management approach for a patient with Ehlers-Danlos (EDS) syndrome and avascular necrosis (AVN) of the hip?

Management of Avascular Necrosis of the Hip in Ehlers-Danlos Syndrome

For patients with EDS and AVN of the hip, surgical intervention (hip replacement) is ultimately required for symptomatic disease, but must be approached with extreme caution due to tissue fragility, vascular vulnerability, and high complication rates specific to EDS patients. 1, 2

Initial Diagnostic Approach

When a patient with EDS presents with persistent hip pain, the diagnostic pathway differs from standard AVN evaluation:

• Obtain MRI of both hips immediately if standard radiographs are normal, as MRI is the preferred diagnostic method for AVN and bilateral involvement is common 3
• Avoid invasive diagnostic procedures including arteriography, which is contraindicated in EDS due to risk of fatal vascular complications 2, 1
• Determine EDS subtype urgently through genetic testing if not already confirmed, as vascular EDS (Type IV) carries dramatically different surgical risks than hypermobile or classical types 3, 1

Medical Management Strategy

Before considering surgical intervention, optimize medical management:

• Initiate celiprolol (beta-blocker with vasodilatory properties) if not already prescribed, particularly for vascular EDS, to reduce vascular morbidity 3, 4
• Maintain blood pressure in normal range with aggressive hypertension treatment to minimize vascular complications 3, 4
• Ensure adequate vitamin D and calcium supplementation to address bone health, as vitamin D deficiency is common and can worsen bone density 3
• Screen for and treat secondary causes of decreased bone density including hypogonadism, which may contribute to AVN progression 3

Preoperative Vascular Surveillance

All EDS patients require comprehensive vascular imaging before any surgical intervention:

• Perform baseline imaging from head to pelvis using Doppler ultrasound, low-radiation CT angiography, or cardiovascular MRI to evaluate the entire aorta and branch vessels 1, 4
• Document any arterial tortuosity, aneurysms, or dissections that would increase surgical risk 3, 4
• Obtain cardiology and vascular surgery clearance before proceeding with orthopedic surgery 1, 4

Surgical Considerations and Timing

The decision to proceed with hip replacement requires careful risk-benefit analysis:

For Vascular EDS (Type IV):

• Surgery carries extremely high mortality risk due to arterial fragility and bleeding complications 2, 5
• Delay surgery as long as possible with conservative management including pain control, activity modification, and assistive devices 2
• When surgery is unavoidable, it must be performed at a center with expertise in EDS by a multidisciplinary team including vascular surgery on standby 1, 4

For Hypermobile and Classical EDS:

• Surgery is less immediately life-threatening but still carries increased risks of wound dehiscence, bleeding, and joint instability 6, 5
• Proceed with surgery when conservative management fails and quality of life is significantly impaired 3

Intraoperative Management

Meticulous surgical technique is mandatory to minimize complications:

• Use pledgeted sutures for all anastomoses and tissue repairs to prevent tearing through fragile tissues 1
• Minimize tissue trauma through gentle handling and avoid excessive retraction 1, 5
• Have blood products immediately available due to increased bleeding risk from vascular fragility 5
• Consider regional anesthesia carefully, as epidural hematoma risk may be increased, though data is limited 5

Postoperative Surveillance

Extended monitoring is essential due to delayed complications:

• Prolonged hospital observation with close wound monitoring for dehiscence 2
• Serial imaging surveillance to detect vascular complications including pseudoaneurysm formation 2
• Annual vascular surveillance imaging should continue lifelong for all EDS patients 1, 4

Critical Pitfalls to Avoid

• Never perform closed reduction under general anesthesia for any hip pathology in EDS, as avascular necrosis of the femoral head developed in 4 of 5 hips treated this way 7
• Do not use opioids for chronic pain management due to high risk of dependence and worsening of gastrointestinal symptoms common in EDS 8
• Avoid invasive vascular imaging including diagnostic catheter angiography, which has caused fatal complications in EDS patients 1, 2
• Do not proceed with surgery without multidisciplinary team involvement including genetics, cardiology, vascular surgery, and anesthesia with EDS expertise 1, 4

Multidisciplinary Team Structure

Management requires coordination across multiple specialties:

• Medical genetics for definitive EDS subtype classification and genetic counseling 1, 8
• Cardiology for cardiovascular risk assessment and blood pressure optimization 1, 4
• Vascular surgery for preoperative vascular assessment and intraoperative standby 1, 4
• Orthopedic surgery with specific experience in EDS complications 6, 7
• Anesthesiology familiar with EDS-specific perioperative risks 5

Conservative Management Options

While most symptomatic AVN ultimately requires surgery, maximize conservative approaches first:

• Physical therapy focusing on joint protection and strengthening surrounding musculature 6
• Assistive devices including canes or walkers to offload the affected hip 3
• Pain management through non-opioid modalities including NSAIDs (if no contraindications), acetaminophen, and topical agents 8
• Activity modification to reduce mechanical stress on the hip joint 6