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Multifocal pigmented villonodular synovitis in a child: A case report
Liang Zhao1, Kaiyu Zhou, Yimin Hua
1Department of Pediatrics Key Laboratory of Ministry of Education for Obstetric & Gynecologic and Pediatric Diseases and Birth Defects, West China Second University Hospital, Sichuan University, Chengdu, China.
Medicine
|August 19, 2016
Summary
This case report details a rare instance of multifocal pigmented villonodular synovitis (PVNS) in a child, affecting over 20 joints. Early misdiagnosis as juvenile idiopathic arthritis delayed treatment for this extensive PVNS case.
Area of Science:
- Rheumatology
- Pediatric Orthopedics
- Pathology
Background:
- Pigmented villonodular synovitis (PVNS) is a rare, benign synovial proliferation typically affecting a single joint in adults.
- Multifocal PVNS, especially in childhood, is exceptionally rare.
Observation:
- A 7-year-old female presented with a 6-month history of multifocal joint swelling and mild pain, initially diagnosed as polyarticular juvenile idiopathic arthritis.
- Treatment with naproxen, methotrexate, infliximab, and pavlin was ineffective, with persistent joint swelling.
- Physical examination revealed widespread joint swelling, particularly in the shoulders, with bloody joint fluid. MRI showed synovial thickening and hemosiderin deposition.
Findings:
- Biopsy confirmed PVNS with villous nodules, foam cell infiltration, and hemosiderin deposition.
- The diagnosis of PVNS was established based on clinical, imaging, and histopathological evidence.
- This represents the most extensive pediatric case of multifocal PVNS reported, involving over 20 joints.
Implications:
- PVNS can be misdiagnosed as other inflammatory arthropathies like rheumatoid arthritis, leading to treatment delays.
- Accurate and timely diagnosis of PVNS is crucial for appropriate management, especially in pediatric cases.
- This case highlights the importance of considering rare diagnoses in pediatric patients with persistent, multifocal joint swelling unresponsive to standard treatments.
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