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.
Subject(s)
Bone Marrow Transplantation/adverse effects , Chediak-Higashi Syndrome/complications , Debridement/methods , Knee Joint/pathology , Synovitis, Pigmented Villonodular/diagnosis , Biopsy , Chediak-Higashi Syndrome/surgery , Female , Humans , Infant , Knee Joint/surgery , Magnetic Resonance Imaging , Severity of Illness Index , Synovitis, Pigmented Villonodular/etiology , Synovitis, Pigmented Villonodular/surgeryABSTRACT
The aim of this paper is to describe the first reported use of computed tomography (CT) guided lumbar sympathetic block as treatment of a case of complex regional pain syndrome (CRPS) in a child. The potential aetiology of CRPS is discussed in relation to the mechanism of action of local anaesthetics used in the block. Based on the successful treatment of this child and the documented success of its use in adults, we conclude that despite the minimal dose of radiation given, CT guided lumbar sympathetic block is an important treatment option in CRPS in children.
