Complex Regional Pain Syndrome (Crps) in a Hemophilia Child with Inhibitor: A Rare Interactable Cause of Joint Pains

ABSTRACT

Introduction: Joint bleeding is the main cause of joint pain in hemophilia patients and can lead to chronic joint disease which also happens to be one of the significant causes of disability and joint pain in these patients. Furthermore, ComplexRegional Pain Syndrome (CRPS), despite being a very rare complication, should be considered in cases of persistent intractable pain, especially in the pediatric group. Clinical symptoms in CRPS include severe chronic pain, edema, and decreased range of motion. CRPS management is critical to allowing the function and ability of the joint to restore. Method(s) This study aims to report a hemophilia case with intractable pain and his underlying diagnosis. A 14-year-old severe hemophilia A patient with high titer/responder inhibitor was on on-demand treatment by Bypassing Agents (BPAs). Result(s) During the disease course, his right knee became a target joint due to recurrent bleedings. Consequently, he underwent Radiosynoviorthesis (RSO) and treatment with BPAs. After three days of improving, he got an increasing fever and severe right knee pain. The COVID-19 test result was negative, but Staph. Aureus was found in the synovial fluid, and treatment began with Vancomycin and Rifampin. After several days, his condition and laboratory markers were improved, However, intractable disabling pain remained constant regardless of augmented combination therapy with FEIBA and rFVIIa. Parallelly, morphine was prescribed due to the Pain Management counseling. However, the pain began to rise as the morphine dosage declined. As a result, CRPS proposed to be the leading cause of pain, and after several prolonged special physiotherapy sessions, pain reduced significantly, only one BPA was continued and he was ambulated again. Discussion/Conclusion: The current case indicates that CRPS is a rare complication in patients with bleeding disorders which has been reported rarely till now. Nonetheless, it should be considered a diagnosis in complicated patients with recurrent hemarthrosis due to its debilitating and destructive nature.