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Pigmented villonodular synovitis in children. A case report
J Bruns1, T Schubert, G Eggers-Stroeder
1Department of Orthopedic Surgery, University of Hamburg, Germany.
Insights
Pigmented villonodular synovitis (PVS) is a rare joint disease. Early diagnosis and surgical treatment, like total synovectomy, can prevent further joint damage.
Area of Science:
- Orthopedics
- Pediatric Rheumatology
- Surgical Pathology
Background:
- Pigmented villonodular synovitis (PVS) is a rare neoplastic proliferation of the synovium.
- It primarily affects young adults and children, potentially leading to secondary osteoarthritis.
- Early diagnosis and intervention are crucial to preserve joint function.
Observation:
- This article details a rare case of diffuse PVS in an 11-year-old girl.
- Clinical presentation, diagnostic challenges, and treatment strategies are discussed.
- The importance of high clinical suspicion for PVS in pediatric patients is highlighted.
Findings:
- Diagnostic procedures for PVS have varying efficacy.
- Preoperative histological confirmation is often not possible.
- Early invasive investigations like arthroscopy are recommended for definitive diagnosis.
Implications:
- Arthroscopy or arthrotomy followed by total synovectomy is the recommended surgical approach.
- Prompt surgical management can prevent the development of osteoarthritic lesions.
- This case underscores the importance of considering PVS in pediatric joint pathology and the efficacy of timely surgical intervention.
Abstract:
Pigmented villonodular synovitis (PVS) is a rare disease of the synovial layer in joints, seen particularly in children. Early diagnosis allows treatment with resection of the affected synovial tissue, avoiding secondary osteoarthritic lesions of the normally unaffected joint structures. In this article, symptoms, clinical findings, and the diagnosis procedure are reported in a rare case of diffuse PVS in an 11-year-old girl. Diagnostic procedures are of differing value, and it seems that the most important factor is suspicion of this disease even in children. Since preoperative verification of the histological diagnosis is impossible, early invasive investigation such as arthroscopy are recommended to confirm the histological character of the lesion. Arthrotomy or arthroscopy followed by total synovectomy is recommended as the surgical treatment of first choice. Our patient was treated by total synovectomy via an arthrotomy of the affected knee joint after histological diagnosis has been confirmed by arthroscopic excision and histological analysis. Follow-up examinations 4 and 6 months postoperatively revealed no signs of recurrence.