(740) Uncommon Occurrence: Brain Herniation From Invasive Group A Strep

Introduction: Group A Streptococcus (GAS), commonly a noninvasive bacterial infection in children resulting in strep throat, is usually treatable with antibiotics. Post-COVID-19 pandemic, the CDC has observed an increase in invasive GAS infections, with 20,000 to 27,000 cases annually. Invasive manifestations include cellulitis with bacteremia, necrotizing fasciitis, pneumonia with bacteremia, and rare neurological infections. Mortality ranges from 1,800 to 2,400 cases annually. This report details a rare case of invasive GAS leading to brain herniation.

Description: A healthy 13-year-old presented with left eye swelling and encephalopathy preceded by an upper respiratory tract infection. He rapidly decompensated while in the ED requiring intubation. He had significant lactic acidosis, high vasoactive requirements and developed DIC. CT head showed an intraorbital abscess and pneumocephalus adjacent to the orbit. MRI showed evidence of cerebritis and meningitis with DWI restriction changes across the bifrontal cortices but without focal epidural or subdural collection. Imaging showed no evidence of intracranial hypertension. No surgical intervention was performed due to lack of a drainable fluid collection and DIC.
After 12 hours he neurologically decompensated and lost all brain stem reflexes and bilateral pupils became fixed and dilated. He required significant escalation of all vasoactive and inotropic support. CT head confirmed diffuse bilateral cerebral edema with bilateral uncal and tonsillar herniation. He was taken off life support per families wishes. Initial blood cultures were positive for Streptococcus pyogenes.

Discussion: Invasive GAS infections have high morbidity and mortality rates. CNS involvement is rare, constituting 0.2%–1% of childhood bacterial meningitis cases. All cases were associated with preceding viral illness. Reports of GAS-induced brain herniation in children are scarce. A retrospective study reviewed five cases of invasive GAS with neurologic sequelae, with one associated mortality. Treating invasive intracranial GAS necessitates neurosurgical intervention and prolonged antibiotic therapy. Education on invasive GAS is essential for effective prevention and treatment.