A severe systemic presentation of pigmented villonodular synovitis in a child with underlying Chediak–Higashi syndrome

True, Victoria L; Monsell, Fergal; Smith, Tanya; Parke, Simon; Grier, D.; Schneider, Marion; Steward, Colin G.

doi:10.1097/bpb.0000000000000190

Cited by 3 publications

(3 citation statements)

References 12 publications

(14 reference statements)

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“…D-PVNS is a rare benign disease of the synovium and tendon sheath that rarely involves multiple joints. 11,12 Twenty-seven patients in our study had monoarticular involvement; only one patient had bilateral knees affected. Although genetic defects, trauma, and hemorrhage have been suggested as possible causes, 6 the etiology of PVNS remains unknown.…”

Section: Discussionmentioning

confidence: 83%

Short-Term Outcomes of Synovectomy and Total Knee Replacement in Patients with Diffuse-Type Pigmented Villonodular Synovitis

Zhou

et al. 2019

J Knee Surg

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Diffuse-type pigmented villonodular synovitis is a rare benign disease that causes disorders of the knee, including erosion of subchondral bone and cyst formation, with eventual osteoarthritis. The purpose of this study was to evaluate the short-term outcomes of synovectomy and total knee replacement in patients with the diffuse type of pigmented villonodular synovitis. From November 2011 to May 2015, we performed synovectomy and total knee replacement in 28 patients with diffuse pigmented villonodular synovitis diagnosed on the basis of histopathology of biopsy specimens. Clinical data were collected perioperatively and during follow-up for evaluation of surgical efficacy. No intraoperative complications were encountered. Mean operative duration was 73.4 minutes (range: 47–115 minutes); mean estimated blood loss was 223.9 mL (range: 50–600 mL). The mean duration of follow-up was 58.7 months (range: 36–84 months). Mean range of motion improved from 86.1 ± 11.3 degrees (range: 60–100 degrees) to 107 ± 11.4 degrees (range: 90–130 degrees). Average Knee Society clinical scores improved from 38.9 ± 9.5 (range: 17–54) to 84.4 ± 6.1 (range: 75–98); functional scores improved from 48.9 ± 13.1 (range: 25–80) to 84.6 ± 6.1 (range: 75–95; p < 0.05 for both). Postoperative radiographs showed no signs of prosthesis loosening, periprosthetic fractures, or dislocation. The short-term efficacy of synovectomy and total knee replacement in treating patients with diffuse pigmented villonodular synovitis was satisfactory.

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Section: Discussionmentioning

confidence: 83%

Short-Term Outcomes of Synovectomy and Total Knee Replacement in Patients with Diffuse-Type Pigmented Villonodular Synovitis

Zhou

et al. 2019

J Knee Surg

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“…Multifocal PVNS is rare, particularly in childhood. [1,2] We report a rare case of multifocal PVNS affecting over 20 joints in a 7-year-old female to highlight the importance of considering PVNS as a differential diagnosis in children presenting with long-term joint swelling and to reduce the occurrence of misdiagnosis.…”

Section: Introductionmentioning

confidence: 99%

Multifocal pigmented villonodular synovitis in a child

Zhao

Zhou²,

Hua³

et al. 2016

Medicine

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Introduction:Pigmented villonodular synovitis (PVNS) is a rare, benign proliferative disorder of the synovial membrane that typically presents in adults and affects a single joint. Multifocal PVNS is very rare, particularly in childhood. We reported a rare case of multifocal PVNS affecting over 20 joints in a child.Clinical procedure:A 7-year-old female patient had a 6-month history of multifocal joints swelling with mild pain. She was diagnosed as polyarticular juvenile idiopathic arthritis at a local hospital. Naproxen, methotrexate, infliximab, and pavlin were used to treat the patient for 2 months. However, the treatment had no effect, the joints swelling remained. The patient was then transferred to our hospital. Physical examination revealed multiple joints swelling, especially in the shoulders joints. Puncture fluid from a shoulder joint was bloody. Magnetic resonance imaging (MRI) revealed synovial thickening and hemosiderin deposition. Biopsy of joint synovium found villous nodules, the invasion of foam cells, and hemosiderin deposition. By collecting all of the evidence, the diagnosis of PVNS was confirmed.Conclusions:PVNS was easily misdiagnosed as rheumatoid arthritis and the formal treatment was usually delayed. This case described here is the first case of PVNS involving such a large numbers of joints that has been reported in the literature.

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“…It has a predilection for large joints, comonly affecting the knee and hip. Diagnosis is a challenge as patients present with various symptoms, nonspecific to the disease [3][4][5][6].…”

Section: Introductionmentioning

confidence: 99%

The Importance of Precise Imaging in Villonodular Synovitis of the Hip: A Radiology Case Report

Belecciu¹,

Muntean²,

Gyurka³

2017

UCMS

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Villonodular synovitis (VS) is a rare condition classified as a benign proliferative disorder of the synovium. An increasing number of cases describe nonspecific symptoms and diagnosis remains a challenge in corectly assessing the disease. We report the case of a 17-year-old patient that presents with persistent and worsening joint pain of the left hip, unresponsive to medication. A radiography, CT-scan (8-slice, 1G) and MRI (Philips 1 Tesla) are performed for imaging purposes. MRI findings include an intra-articular mass seen as a low to medium signal intensity heterogenous image. MRI combined with CT remains the most accurate imaging solution to diagnose such disease and particular attention is needed to correctly assess VS.

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A severe systemic presentation of pigmented villonodular synovitis in a child with underlying Chediak–Higashi syndrome

Cited by 3 publications

References 12 publications

Short-Term Outcomes of Synovectomy and Total Knee Replacement in Patients with Diffuse-Type Pigmented Villonodular Synovitis

Short-Term Outcomes of Synovectomy and Total Knee Replacement in Patients with Diffuse-Type Pigmented Villonodular Synovitis

Multifocal pigmented villonodular synovitis in a child

The Importance of Precise Imaging in Villonodular Synovitis of the Hip: A Radiology Case Report

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