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

Victoria L True, Fergal P Monsell, Tanya A Smith, Simon C Parke, David J Grier, Marion E Schneider, Colin G Steward

Research output: Contribution to journalArticle (Academic Journal)peer-review

2 Citations (Scopus)

Abstract

Pigmented villonodular synovitis (PVNS), a condition of synovial hyperproliferation that mostly affects large joints, is rare in children and conventionally lacks systemic symptoms. This report describes a complex paediatric patient who underwent bone marrow transplantation to control the accelerated phase of the Chediak-Higashi syndrome. Diffuse PVNS developed in one knee 2.75 years later. Progression of PVNS was accompanied by the development of severe systemic symptoms, which resolved rapidly following subtotal surgical debridement. The patient remains well with minimal elevation of inflammatory marker levels 10.5 years later. As PVNS and Chediak-Higashi syndrome are both very rare diseases we propose a potential unifying hypothesis for this combination.

Original languageEnglish
Pages (from-to)526-9
Number of pages4
JournalJournal of Pediatric Orthopaedics Part B
Volume24
Issue number6
DOIs
Publication statusPublished - Nov 2015

Keywords

  • Biopsy
  • Bone Marrow Transplantation
  • Chediak-Higashi Syndrome
  • Debridement
  • Female
  • Humans
  • Infant
  • Knee Joint
  • Magnetic Resonance Imaging
  • Severity of Illness Index
  • Synovitis, Pigmented Villonodular
  • Case Reports
  • Journal Article

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