Complex regional pain syndrome in pediatric patients with severe factor VIII deficiency

Two boys with severe factor VIII deficiency that initially presented with acute onset of joint pain and swelling consistent with an uncomplicated hemarthrosis are reported. When appropriate management failed to provide resolution of symptoms, alternate diagnoses were considered. Both boys ultimately had complex regional pain syndrome (CRPS) diagnosed. The delay in diagnosis contributed to prolonged patient discomfort and lack of appropriate therapy. Complex regional pain syndrome encompasses a group of disorders that are characterized by pain severity or duration disproportionate to that expected. It is uncommon in the pediatric population. Because early diagnosis and appropriate treatment may improve outcome, it is important for practitioners to consider CRPS in the differential diagnosis of persistent pain in children with hemophilia.