NECROTIZING FASCIITIS SECONDARY TO CLOSTRIDIUM SEPTICUM IN A PATIENT WITH MYD88 DEFICIENCY

Abstract

Introduction MyD88 deficiency impairs toll-like receptor and interleukin-1 receptor mediated immunity. This results in bacterial infections of the upper respiratory tract and skin and predisposes patients to invasive infection with pyogenic bacteria. Clostridium septicum is an anaerobic bacteria that rarely affects children. This case discusses a patient with MyD88 deficiency presenting with necrotizing fasciitis secondary to Clostridium septicum. Case Description Our patient is a 9-year old female with known MyD88 deficiency and history of multiple invasive bacterial infections. She presented with knee and abdominal pain and had extensive subcutaneous air on x-ray. She was immediately taken to the operating room for debridement and wound VAC placement and was found to have necrotizing fasciitis, necrotizing myositis, and necrotizing cellulitis secondary to Clostridium septicum. She was started on broad spectrum antibiotics that were later narrowed based on culture sensitivities. She then developed further extension of her disease and antibiotics were adjusted. Throughout her hospitalization she underwent numerous debridements, hyperbaric oxygen therapy, and multiple IVIG infusions. Antibiotic therapy was adjusted throughout hospitalization and narrowed by time of discharge. Discussion This patient's presentation of necrotizing fasciitis, necrotizing myositis, and necrotizing cellulitis secondary to Clostridium septicum is unique. This organism is typically found to infect adults with malignant disease, such as colorectal cancer. In other reported pediatric cases of Clostridium septicum, it has been associated with neutropenia. This case is important to report, as it may lead to discovery of additional pathways in which MyD88 deficiency may alter immune response. MyD88 deficiency impairs toll-like receptor and interleukin-1 receptor mediated immunity. This results in bacterial infections of the upper respiratory tract and skin and predisposes patients to invasive infection with pyogenic bacteria. Clostridium septicum is an anaerobic bacteria that rarely affects children. This case discusses a patient with MyD88 deficiency presenting with necrotizing fasciitis secondary to Clostridium septicum. Our patient is a 9-year old female with known MyD88 deficiency and history of multiple invasive bacterial infections. She presented with knee and abdominal pain and had extensive subcutaneous air on x-ray. She was immediately taken to the operating room for debridement and wound VAC placement and was found to have necrotizing fasciitis, necrotizing myositis, and necrotizing cellulitis secondary to Clostridium septicum. She was started on broad spectrum antibiotics that were later narrowed based on culture sensitivities. She then developed further extension of her disease and antibiotics were adjusted. Throughout her hospitalization she underwent numerous debridements, hyperbaric oxygen therapy, and multiple IVIG infusions. Antibiotic therapy was adjusted throughout hospitalization and narrowed by time of discharge. This patient's presentation of necrotizing fasciitis, necrotizing myositis, and necrotizing cellulitis secondary to Clostridium septicum is unique. This organism is typically found to infect adults with malignant disease, such as colorectal cancer. In other reported pediatric cases of Clostridium septicum, it has been associated with neutropenia. This case is important to report, as it may lead to discovery of additional pathways in which MyD88 deficiency may alter immune response.