Purpose <p>Systemic sclerosis (SSc) is a rare autoimmune disorder marked by fibrosis, vascular abnormalities, and immune dysfunction. In advanced cases, it can lead to severe craniofacial complications, including mandibular condylar and ramus resorption, which can significantly impair jaw function and quality of life. We present a rare case of bilateral complete mandibular condylar and ramus resorption in a patient with advanced SSc treated with extended custom total TMJ reconstruction.</p> Patient concerns &amp; clinical findings <p>A 46-year-old female with long-standing SSc who presented with severe temporomandibular joint (TMJ) dysfunction, malocclusion, and airway compromise. Imaging revealed bilateral complete resorption of the mandibular condyles and rami, resulting in functional limitations and aesthetic concerns.</p> Diagnosis, interventions, and outcomes <p>The primary diagnosis was advanced SSc with bilateral TMJ destruction. Surgical reconstruction was performed using extended patient-fitted bilateral total TMJ prostheses (eTMJR). Initial postoperative healing was complicated by soft tissue tension and poor vascularity, common in SSc patients. Adjunctive hyperbaric oxygen therapy (HBOT) was utilized to enhance wound healing.Over several years of follow-up, the patient maintained stable prosthetic function without complications or infection, improved occlusion, and satisfactory aesthetic outcomes.</p> Conclusion <p>This report illustrates the surgical challenges, multidisciplinary decision-making and long-term success of extended custom TMJ reconstruction in advanced SSc patients. Presurgical assessment with a multidisciplinary approach and consideration of adjunctive therapies such as HBOT were helpful in achieving desirable functional and aesthetic outcomes when considering potential surgical intervention.</p>

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Extended temporomandibular joint prosthetic reconstruction of total condylysis in the setting of severe scleroderma: a case report

  • Mohammed Amir Rais,
  • Mervat E. Mahmoud,
  • W. Jonathan Fillmore

摘要

Purpose

Systemic sclerosis (SSc) is a rare autoimmune disorder marked by fibrosis, vascular abnormalities, and immune dysfunction. In advanced cases, it can lead to severe craniofacial complications, including mandibular condylar and ramus resorption, which can significantly impair jaw function and quality of life. We present a rare case of bilateral complete mandibular condylar and ramus resorption in a patient with advanced SSc treated with extended custom total TMJ reconstruction.

Patient concerns & clinical findings

A 46-year-old female with long-standing SSc who presented with severe temporomandibular joint (TMJ) dysfunction, malocclusion, and airway compromise. Imaging revealed bilateral complete resorption of the mandibular condyles and rami, resulting in functional limitations and aesthetic concerns.

Diagnosis, interventions, and outcomes

The primary diagnosis was advanced SSc with bilateral TMJ destruction. Surgical reconstruction was performed using extended patient-fitted bilateral total TMJ prostheses (eTMJR). Initial postoperative healing was complicated by soft tissue tension and poor vascularity, common in SSc patients. Adjunctive hyperbaric oxygen therapy (HBOT) was utilized to enhance wound healing.Over several years of follow-up, the patient maintained stable prosthetic function without complications or infection, improved occlusion, and satisfactory aesthetic outcomes.

Conclusion

This report illustrates the surgical challenges, multidisciplinary decision-making and long-term success of extended custom TMJ reconstruction in advanced SSc patients. Presurgical assessment with a multidisciplinary approach and consideration of adjunctive therapies such as HBOT were helpful in achieving desirable functional and aesthetic outcomes when considering potential surgical intervention.