What is the success rate of bilateral total Temporomandibular Joint (TMJ) joint replacement in a 20-year-old female with a history of Ehlers-Danlos syndrome?

Success Rates of Bilateral TMJ Joint Replacement in Ehlers-Danlos Syndrome

Bilateral total temporomandibular joint (TMJ) replacement in patients with Ehlers-Danlos syndrome (EDS) is associated with significantly higher complication rates and should be considered with extreme caution, as these patients are at increased risk for poor surgical outcomes.

Understanding Ehlers-Danlos Syndrome and TMJ Involvement

Ehlers-Danlos syndrome is a rare connective tissue disorder with several subtypes, with vascular EDS (vEDS) being the most severe form:

• Prevalence of vEDS is approximately 1/50,000 to 1/200,000 1
• Caused by pathogenic variants in the COL3A1 gene affecting type III collagen assembly 1
• Results in significant loss of mechanical strength in arteries and hollow organs
• Life expectancy is reduced to an average of 51 years 1

TMJ disorders are extremely common in patients with EDS:

• Nearly 98% of patients with hypermobile EDS (hEDS) report TMJ symptoms 2
• Most common symptoms include TMJ pain (95%), clicking (90%), and jaw fatigue (80%) 2
• TMJ luxation/dislocation occurs in 44% of hEDS patients 2

Success Rates and Complications

The literature on bilateral total TMJ replacement specifically in EDS patients is limited, but available evidence suggests concerning outcomes:

• Case reports document "extensive surgical journeys" with multiple failed TMJ replacements in EDS patients 3
• Patients with EDS are at higher risk for:
  • Increased bleeding during and after surgery
  • Delayed wound healing
  • TMJ pain refractory to treatment 4
  • Arterial complications including dissections, aneurysms, and ruptures 1

Treatment Approach for TMJ Disorders in EDS

Conservative Management First

Less invasive approaches should be exhausted before considering total joint replacement:

• TMJ arthroscopy has shown promising results in EDS patients:
  • A case series of 18 female patients with EDS Type III showed improvement in mouth opening from 23.4 mm to 27.8 mm after arthroscopy 5
  • All patients were asymptomatic at long-term follow-up (average 62 months) 5

Surgical Considerations

If surgery is deemed necessary:

1. Patient Selection and Risk Assessment:

   • Comprehensive evaluation of vascular status through non-invasive imaging 1
   • Thorough assessment of connective tissue involvement throughout the body
   • Genetic testing to confirm specific EDS subtype, as vascular subtype carries highest risk 1

2. Surgical Planning:

   • Custom alloplastic TMJR is generally preferred over stock devices for complex cases 1
   • Current prostheses have up to 20 years of follow-up data with favorable outcomes in general populations 1
   • However, these outcomes may not translate to EDS patients

3. Perioperative Management:

   • Meticulous hemostasis during surgery
   • Careful soft tissue handling
   • Consideration of celiprolol (a beta-blocker with vasodilatory properties) for patients with vEDS 1

Potential Complications

Patients with EDS undergoing TMJ replacement face higher risks of:

• Facial nerve injury (most common complication at 7.8% incidence) 6
• Surgical site infection
• Postoperative pain management challenges
• Potential airway concerns due to swelling 6
• Implant instability and loosening of screws 1
• Relapse of TMJ ankylosis 1
• Vascular complications, particularly in vEDS patients 1

Alternative Approaches

For some EDS patients with TMJ disorders, less invasive procedures may be more appropriate:

• Condylectomy has been reported successful in a 21-year-old woman with EDS who had recurrent TMJ dislocation 7
• Orthognathic surgery has been successfully performed in selected EDS patients, suggesting that with careful patient selection, some jaw surgeries can be safe and effective 4

Conclusion

The decision to perform bilateral total TMJ replacement in a young patient with EDS must be approached with extreme caution. While alloplastic TMJR is increasingly considered the gold standard for end-stage TMJ disorders in the general population 1, the unique challenges posed by EDS significantly increase the risk of complications and treatment failure. Less invasive approaches like arthroscopy should be thoroughly explored before considering total joint replacement.