Background: Primary Synovial Chondromatosis (PSC) is a rare benign tumor of the synovial membrane in which cartilage metaplasia produces calcific loose bodies within the articular space. Only a few cases are reported in the pediatric population and its etiology remains unknown. This condition typically affects large weight-bearing joints with pain, swelling and decrease range of motion. Due to its slow progressions, delayed diagnosis is frequent and differential diagnosis should consider other chronic arthritis and malignancies. While arthroscopic removal of loose bodies is the current treatment up to now, the association of partial or complete synovectomy is debated.
Case Presentation: We report about a 14-year-old girl with a long-lasting right shoulder pain, especially during movements or exercise, localized tenderness and hypotonia of the glenohumeral joint. No previous trauma was mentioned. Blood exams, Mantoux test and plain radiography of the right shoulder were unremarkable. Ultrasound imaging revealed echogenic and calcified bodies stretching the glenohumeral joint and dislocating the long head of biceps tendon. Magnetic resonance showed a "rice-grain" pattern of the right shoulder. From an arthroscopic surgery, multiple loose white bodies were removed within the synovial membrane, and synovial chondromatosis was confirmed by histological analysis. At one month follow up visit, the patient completely recovered without pain.
Conclusion: Synovial chondromatosis is a very uncommon cause of mono articular pain in children, especially when it affects shoulder. Pediatricians should keep in mind this condition to avoid delayed diagnosis and treatment, even in consideration of the low risk of malignant transformation. Through this case, we would highlight common diagnostic pitfalls and treatment of synovial chondromatosis.
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http://dx.doi.org/10.1186/s13052-022-01332-4 | DOI Listing |
J Hand Surg Asian Pac Vol
January 2025
Auckland Regional Centre for Plastic, Reconstructive and Hand Surgery, Middlemore Hospital, Auckland, New Zealand.
Synovial chondromatosis is a condition that rarely occurs in the hand and wrist. Recurrence and malignant transformation are two potential complications of the disease. We set out to retrospectively review 35 years of practice within our sarcoma and tertiary hand unit and identify recurrence or malignant transformation.
View Article and Find Full Text PDFRev Bras Ortop (Sao Paulo)
November 2024
Departamento de Reumatologia, Kassab Orthopedics Institute, Ksar Saïd, Tunísia.
Reichel syndrome or primary synovial chondromatosis (PSC) is an uncommon benign metaplastic condition that usually affects large joints. Though shoulder involvement was scarce, there are only a few cases in the pediatric population. A 14- year-old boy was admitted to the Pediatric Orthopedics department with right shoulder pain for 14 months.
View Article and Find Full Text PDFRev Bras Ortop (Sao Paulo)
November 2024
Disciplina de Ortopedia, Pontifícia Universidade Católica do Paraná (PUC/PR), Londrina, PR, Brasil.
Synovial chondromatosis is an uncommon, progressive, benign condition favoring synovial metaplasia resulting from the production of cartilaginous tissue as loose bodies within the joints. In rare cases, it can affect the interior of the shoulder joint and present with pain, edema, and impaired mobility. The diagnosis is challenging, requiring imaging techniques.
View Article and Find Full Text PDFJ Orthop Case Rep
December 2024
Department of Orthopedic Surgery, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India.
Introduction: Progressive pseudorheumatoid dysplasia is an autosomal recessively inherited rare disorder with features of growth retardation, multiple joint deformities, and intra-articular loose bodies.
Case Report: This case study is about a 34-year-old man who presented to us following trauma to his right leg. On general assessment, he was short statured with multiple deformities of both upper and lower limbs with local signs suggestive of proximal tibial fracture over his right tibia.
Virchows Arch
December 2024
Department of Pathology, The Johns Hopkins University School of Medicine, 401 N Broadway, Weinberg Building 2245, Baltimore, MD, 21231, USA.
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