Macrophage activation syndrome triggered by Candida parapsilosis-related cochlear implant infection.

BACKGROUND

Cochlear implant-related infections may rarely lead to life-threatening systemic complications. Macrophage Activation Syndrome (MAS) is a severe and potentially fatal hyperinflammatory condition. MAS associated with CI infection has not been previously reported in pediatric patients.

CASE PRESENTATION: We present a 13-year-old female patient who developed MAS secondary to Candida parapsilosis-related sepsis associated with cochlear implant (CI). Despite broad-spectrum antibacterial and antifungal therapy, the patient's clinical course deteriorated with pancytopenia, hyperferritinemia, and acute liver failure. She fulfilled HLH-2004 criteria and was diagnosed with MAS. Multidisciplinary treatment approach included plasmapheresis, high-dose pulse corticosteroids and CI explantation, resulted in clinical recovery. Implant site cultures revealed that Candida parapsilosis, a fungal pathogen known for biofilm formation and implant-related infections. Following explantation, the patient showed gradual improvement with immunosuppressive therapy and supportive care. Successful cochlear implant reimplantation was performed two months later.

CONCLUSION

CI-related infections and related systemic complications are rare but severe. This is the first reported pediatric case of MAS triggered by cochlear implant-related sepsis. Early recognition of MAS and multidisciplinary management are essential to improve outcome and survival.