Delayed-onset progressive pseudorheumatoid dysplasia with secondary synovial chondromatosis.

A 25-year-old man presented to us with progressive multiple joint pain, enlargement, and restricted movements. X-rays showed platyspondyly, multiple epiphyseal widening, synovial chondromatosis, and decreased bone stock and cortical thickness. Genetic testing showed biallelic pathogenic variants in CCN6 which confirmed the diagnosis of progressive pseudorheumatoid dysplasia. Supportive care, physical therapy, genetic and psychological counselling were provided to the patient.

Osteocondromatosis sinovial de rodilla: una causa infrecuente de gonalgia en edad pediátrica / Synovial chondromatosis of the knee. A rare cause of knee pain in pediatric age

La osteocondromatosis sinovial es una metaplasia benigna de la membrana sinovial que afecta a 1 de cada 100 000 personas, en su mayoría adultos, y es extremadamente infrecuente en edad pediátrica. Predomina en grandes articulaciones, sobre todo la rodilla, y la sintomatología es, por lo general, inespecífica. Dado que la radiografía simple no suele ser concluyente, se recurre a la resonancia magnética nuclear y a la tomografía axial computarizada para orientar el diagnóstico. Se expone el caso de una paciente de 10 años de edad con gonalgia y dismorfia en la patela izquierda de seis meses de evolución, con diagnóstico de osteocondromatosis sinovial. Se presenta el caso dado que se trata de una entidad muy rara en niños, pero que requiere un tratamiento quirúrgico precoz para evitar sus posibles complicaciones, como la destrucción articular progresiva o la malignización a condrosarcoma.

Synovial osteochondromatosis consists of a synovial metaplasia which affects 1 per 100 000 people. It is a very rare disease among children. It typically affects large joints of the body, especially the knee. Due to the lack of specificity of the signs and symptoms and X-Ray images, imaging tests such as nuclear magnetic resonance or computerized tomography are frequently needed for diagnosis.We report a case of a ten-year-old female patient with a six months history of pain and deformity of left patella which was diagnosed with synovial osteochondromatosis. This case highlights the importance of clinical suspicion, not only because it is an extremely rare disease in children, but also because it needs a surgical treatment as soon as possible in order to avoid consequences it might have in pediatric age, as joint destruction or malignization to chondrosarcoma.

Ultrasound-guided synovial Tru-cut biopsy: indications, technique, and outcome in 111 cases.

OBJECTIVE: To investigate the diagnostic performance of ultrasound-guided synovial biopsy. METHODS: Clinical notes, pathology and microbiology reports, ultrasound and other imaging studies of 100 patients who underwent 111 ultrasound-guided synovial biopsies were reviewed. Biopsies were compared with the final clinical diagnosis established after synovectomy (n = 43) or clinical/imaging follow-up (n = 57) (mean 30 months). RESULTS: Other than a single vasovagal episode, no complication of synovial biopsy was encountered. One hundred and seven (96 %) of the 111 biopsies yielded synovium histologically. Pathology ± microbiology findings for these 107 conclusive biopsies comprised synovial tumour (n = 30, 28 %), synovial infection (n = 18, 17 %), synovial inflammation (n = 45, 42 %), including gouty arthritis (n = 3), and no abnormality (n = 14, 13 %). The accuracy, sensitivity, and specificity of synovial biopsy was 99 %, 97 %, and 100 % for synovial tumour; 100 %, 100 %, and 100 % for native joint infection; and 78 %, 45 %, and 100 % for prosthetic joint infection. False-negative synovial biopsy did not seem to be related to antibiotic therapy. CONCLUSION: Ultrasound-guided Tru-cut synovial biopsy is a safe and reliable technique with a high diagnostic yield for diagnosing synovial tumour and also, most likely, for joint infection. Regarding joint infection, synovial biopsy of native joints seems to have a higher diagnostic yield than that for infected prosthetic joints. KEY POINTS: • Ultrasound-guided Tru-cut synovial biopsy has high accuracy (99 %) for diagnosing synovial tumour. • It has good accuracy, sensitivity, and high specificity for diagnosis of joint infection. • Synovial biopsy of native joints works better than biopsy of prosthetic joints. • A negative synovial biopsy culture from a native joint largely excludes septic arthritis. • Ultrasound-guided Tru-cut synovial biopsy is a safe and well-tolerated procedure.

Synovial Chondromatosis.

Synovial chondromatosis is a rare, benign condition of unknown etiology in which the synovium undergoes metaplasia leading to cartilaginous nodules that ultimately break free, mineralize, and even ossify. The most commonly involved joint is the knee. Patients may be asymptomatic or may present with pain, swelling, and limited range of motion. Plain radiographs can be diagnostic and mineralized nodules are pathognomonic. Recommended treatment involves arthroscopic or open removal of loose bodies with or without a synovectomy to prevent further articular and periarticular destruction and to relieve symptoms.

A systematic literature review of synovial chondromatosis and pigmented villonodular synovitis of the hip.

OBJECTIVES: Benign synovial diseases of the hip including Synovial Chondromatosis (SC) and Pigmented Villonodular Synovitis (PVNS) are devastating diseases. Initially, patients present with hip pain unrelieved by conservative measures. The diagnosis of PVNS and SC are often delayed, leading to progression of joint damage. The purpose of this review is to present the latest on the diagnosis, management, and prognosis of SC and PVNS of the hip. METHODS: An extensive systematic search of MEDLINE and PUBMED Databases was performed. Data parameters were set from 2005 to present day with set inclusion criteria. Systematic reviews were excluded. RESULTS: 427 abstracts were identified, with 12 articles meeting all inclusion criteria. Eight studies focused on SC, and 5 on PVNS. 233 patients with SC of the hip and 98 patients with PVNS of the hip were identified, a total of 331 patients. DISCUSSION: Benign Synovial disorders of the hip are rare. In patients with chronic hip pain secondary to benign synovial disorders, early diagnosis and surgical intervention demonstrate good outcomes, and patients may benefit due to prevention of morbidity from further joint destruction. There is no clear consensus between higher successes through open versus arthroscopic surgical debridement. In the final phase of benign synovial disorders of the hip, THA of different types based on the patient's age should be considered.

[Synovial chondromatosis of the right temporomandibular joint: report of one case].

Synovial chondromatosis of the temporomandibular joint(TMJ) is an extremely rare condition. A case of synovial chondromatosis with postoperative recurrence and involvement of base of skull was reported. The clinic pathological features, diagnosis and treatment were discussed with literature review.

[Synovial chondromatosis in the temporomandibular joint: report of 2 cases].

The clinical manifestations, radiographic findings, intraoperative view, histopathologic features of synovial chondromatosis（SC） in the temporomandibular joint (TMJ) were summarized in 2 cases. Preoperative symptoms included preauricular pain（2/2）, swelling（2/2） and limitation of mouth opening (0/2). X-ray findings showed widened joint space. The articular surface destructed and irregular stippled calcifications were seen in the infratemporal fossa by CT scan in one case. MRI showed multiple small nodular formations in the articular cavity. There was no malocclusion and limitation of mouth opening after surgery. As a rare disease of the temporomandibular joint， SC often exists in superior spaces. The clinical manifestations lack specificity. Detection of calcified loose bodies on radiography was helpful to diagnosis, while final diagnosis was dependent on histopathologic examination with characteristic cartilaginous nodules in the synovial membrane.

Metotrexato en condromatosis sinovial: una serendipia / Methotrexate in sinovial chondromatosis: a case of serendipity

La condromatosis sinovial es una metaplasia idiopática benigna de la membrana sinovial que afecta a 1/100.000 habitantes, en una relación hombre/mujer de 3 a 1 entre los 30 y 50 años. Predomina en grandes articulaciones como rodilla (70%), cadera (20%) y hombro (19%), y en menor proporción en codo y tobillo. Puede ser primaria o secundaria. La etiología es desconocida. La resolución es quirúrgica ya sea por artroscopia o por cirugía a cielo abierto, no existiendo otra alternativa terapéutica. Se presenta el caso clínico de un paciente con condromatosis sinovial en hombro derecho, que se comporta como una artropatía erosiva, indicándose metotrexato y resolviendo casi totalmente los nódulos condromatosos.

The synovial chondromatosis is a benign idiopathic metaplasia ofthe synovial membrane which affects one in 100,000 inhabitants. Itis 3 times more common in males, aged between 30 and 50 yearsold. It is commonly found in large joints such as knee (70%), hip(20%) and shoulder (19%) and less frequently in elbow and heel. Itcan be primary or secondary. The etiology is still unknown.The resolution is surgical by means of arthroscopy or open surgery,existing no other therapeutic alternatives.We present a male patient with primary synovial chondromatosis inthe right shoulder, leading to an erosive arthropathy. Treatment withmethotrexate resolved almost entirely the cartilaginous nodules.

Secondary synovial chondromatosis of the shoulder.

Synovial chondromatosis is classified as either primary or secondary. Primary synovial chondromatosis results from a proliferation of chondrocytes in the synovial membrane leading to the formation of cartilaginous loose bodies. Secondary synovial chondromatosis is a rare condition characterized by the growth of separated particles from the articular cartilage or osteophytes in joint diseases. The present article aims to report the secondary chondromatosis of the shoulder and to discuss the clinical manifestations, pathogenesis, diagnosis, histological findings and management of this condition.

Up-regulation of interleukin-6 and vascular endothelial growth factor-A in the synovial fluid of temporomandibular joints affected by synovial chondromatosis.

Our aim was to explore important inflammatory mediators for synovial chondromatosis in the temporomandibular joints (TMJs) by analysing synovial fluid. Samples were collected from 10 patients with unilateral synovial chondromatosis of the TMJ. Control samples were obtained from 11 subjects with no symptoms in the TMJ. Concentrations of aggrecan, interleukin (IL)-2, IL-4, IL-5, IL-6, IL-8 (CXCL8), IL-10, interferon (IFN)-γ, tumour necrosis factor (TNF)-α and vascular endothelial growth factor (VEGF)-A were measured in the samples of synovial fluid, and the results in the two groups compared. The tissues from the affected TMJ were examined histologically and immunohistochemically. Of the proteins evaluated, the concentrations of aggrecan, IL-6, and VEGF-A were significantly higher in the group with synovial chondromatosis. The immunohistochemical analysis showed that the synovial cells around the osteocartilaginous nodules were vigorously expressing VEGF-A. IL-6 and VEGF-A are thought to have important roles in the pathology of synovial chondromatosis of the TMJ.

Two rare involvement sites: synovial chondromatosis.

Synovial chondromatosis (SC) is a rarely encountered benign disease of unknown etiology characterized by multiple joint mouse, as well as metaplastic cartilage points in the joint, bursa and tendon sheaths. Although single joint involvement is frequently encountered, multiple joint involvements may also occur. In this study, two patients with SC in the right shoulder joint and in both ankles were presented, reviewed according to literature.

[Synovial chondromatosis]. / Sinovijalna hondromatoza.

Synovial chondromatosis is an uncommon benign disorder of the synovial membrane ofjoints, tendon sheaths, or bursae characterized by the formation of multiple cartilaginous nodules or osseus loose bodies. It is usually a monoarticular disease, and 33 different localizations have been described until now. The aim of this review article is to present the newest knowledge on the etiology, diagnosis, differential diagnosis, and management of synovial chondromatosis. Malignant transformation to chondrosarcoma is an unusual but possible complication. It is closely connected with recurrence rate and usually occurs many years after surgical treatment. More specific details related to surgical treatment of most often affected joints, i.e. shoulder, elbow, hip, knee, and ankle are reported as well at the end of this review article.

Clinical and radiologic findings of synovial chondromatosis affecting the temporomandibular joint.

OBJECTIVES: Our aim was to study the clinical and radiologic findings of synovial chondromatosis (SC) affecting the temporomandibular joint (TMJ) and provide references for diagnosis and treatment. STUDY DESIGN: Twenty patients confirmed as SC histopathologically were included in the investigation. Nineteen cases were treated with arthrotomy and 1 case with arthroscopy. The clinical manifestations, radiographic findings, histopathologic features, surgical treatment, and differential diagnosis were combined to study this disease. The SC features of TMJ on magnetic resonance (MR) images were summarized on proton density-weighted (PD) and T2-weighted images. RESULTS: Synovial chondromatosis of TMJ occurred more often in women and on the right. Symptoms included preauricular pain, swelling, limitation of mouth opening, crepitations, and deviation on opening. The detection rate of calcified loose bodies was 30% on conventional radiographs and 53.3% on computerized tomography. Multiple small ring-like or tubular signals could be seen on PD and T2-weighted MR images. SC mainly affected the superior joint space; it could involve the inferior space when a perforated or deformed disc was present. SC could extend into intracranial fossa, infratemporal fossa, and lateral pytergoid muscle. CONCLUSIONS: The characteristic ring-like signals could be found on MR images of the patients with SC of TMJ. This lesion should be differentially diagnosed with TMJ disorders and preauricular masses. Considering its recurrence and the different behavior of SC in different patients, various treatment strategies should be considered.

Interventional radiology of synovial chondromatosis in the temporomandibular joint using a thin arthroscope.

This case report describes, for the first time, the use of interventional radiology following arthroscopy with a thin arthroscope for the diagnosis and treatment of synovial chondromatosis (SC) of the temporomandibular joint (TMJ). Therapeutic joint irrigation concomitant with arthroscopic observation of the superior joint compartment (SJC) was performed in a patient with functional TMJ pain caused by SC. The thin arthroscope was inserted with the aid of an X-ray fluoroscope. Arthroscopy confirmed that the SC was at disease stage II and that synovitis existed throughout the SJC. The initial maximal interincisor opening (MIO) was 30 mm, while after the joint-irrigation procedure the MIO was 40 mm. In addition, the TMJ pain was greatly reduced after joint irrigation. Our findings demonstrate that interventional radiology with a thin arthroscope is clinically useful for the diagnosis and primary treatment of patients with painful SC in the TMJ.

Arthroscopy in primary synovial chondromatosis of the hip: description and outcome of treatment.

Between 1985 and 2000, 120 patients underwent arthroscopic management for primary synovial chondromatosis of the hip. We report the outcome of 111 patients with a mean follow-up of 78.6 months (12 to 196). More than one arthroscopy was required in 23 patients (20.7%), and 42 patients (37.8%) went on to require open surgery. Outcomes were evaluated in greater detail in 69 patients (62.2%) treated with arthroscopy alone, of whom 51 (45.9%) required no further treatment and 18 (16.2%) required further arthroscopies. Of the 111 patients, 63 (56.7%) had excellent or good outcomes. At the most recent follow-up, 22 patients (19.8%) had undergone total hip replacement. Hip arthroscopy proved beneficial for patients diagnosed with primary synovial chondromatosis of the hip, providing good or excellent outcomes in more than half the cases.

Synovial chondromatosis in an elite cyclist: a case report.

Primary synovial chondromatosis is a rare disorder that can present as chronic hip and groin pain. It is characterized by formation of osteocartilaginous nodules arising from the synovium. We report the first case, to our knowledge, of an Olympic-caliber cyclist, in her mid thirties, with primary synovial chondromatosis of the left hip. Clinical examination showed decreased internal rotation, external rotation, forward flexion, and abduction of the left hip. A radiograph of the left hip showed slight hip-joint narrowing centrally. A magnetic resonance imaging arthrogram showed a small anterior labral tear and innumerable small intermediate-intensity filling defects situated diffusely within the joint fluid. Fluoroscopically guided injection of the left hip with local anesthetic and cortisone produced temporary pain relief. Conservative treatment was marginally helpful. Results of a rheumatology workup were unremarkable. Arthroscopic removal of loose bodies and synovectomy were performed. The diagnosis of primary synovial chondromatosis was confirmed by histologic examination. At the 17-month follow-up, our patient was essentially pain free and had returned to her previous athletic activities.

Synovial osteochondromatosis in a 9-year-old girl: clinical and histopathological appearance.

This article reports the clinical and histopathological appearance of synovial osteochondromatosis in the right knee joint of a 9-year-old girl. Initial symptoms such as monoarticular joint swelling led to further diagnostic procedures with the aid of different imaging techniques including X-ray examination and MRI. The definitive diagnosis was based on histopathological findings after performing arthroscopy of the affected joint and the intraoperative appearance of the synovial lesions. Besides antiphlogistical drug-therapy, the treatment of choice is synovectomy.