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Multicentric Disappearing Bone Disease treated with Arthroplasty.
C K Chan1, Razif-Ma Mohamed1, A A Azlina1
1NOCERAL, Department of Orthopaedic Surgery, Faculty of Medicine, University of Malaya, Petaling Jaya, Malaysia.
Malaysian Orthopaedic Journal
|May 30, 2017
Summary
Gorham disease, a rare condition causing bone loss, affected a woman with knee and hip lesions. Surgical joint replacements were successful, providing long-term pain relief and stability.
Area of Science:
- Orthopedics
- Rare Diseases
- Bone Metabolism
Background:
- Gorham disease, also known as disappearing bone disease, is an extremely rare condition characterized by progressive osteolysis.
- This case highlights the challenges in diagnosing and managing multicentric Gorham disease.
Observation:
- A middle-aged woman presented with a significant bone defect in her left tibia, leading to knee joint subluxation and antalgic gait.
- She subsequently developed rapid osteolysis of her right femoral head, mimicking Gorham disease progression.
- Bone biopsy confirmed Gorham disease in both affected sites.
Findings:
- The patient underwent successful total knee replacement for her left knee, achieving pain relief.
- Following a year of radiographic monitoring to confirm cessation of osteolysis, a total right hip replacement was performed.
- Five years post-surgery, both arthroplasties remain stable, and the patient is pain-free.
Implications:
- This case demonstrates the feasibility of surgical management for Gorham disease affecting major joints.
- Successful arthroplasty in Gorham disease can lead to long-term pain relief and functional restoration.
- Early diagnosis and staged surgical intervention are crucial for optimal outcomes in multicentric Gorham disease.
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