(266) ECMO in Pediatric HHS/DKA and Malignant Hyperthermia-Like Syndrome

Introduction: Diabetic Ketoacidosis (DKA) is a common cause of admission to the pediatric intensive care unit (PICU) for children. Alongside DKA, the incidence of pediatric Hyperosmolar Hyperglycemic Syndrome (HHS) is also increasing. Though traditionally regarded as distinct conditions, the simultaneous presentation of HHS and DKA is becoming more recognized and is associated with considerable morbidity and mortality. Furthermore, a Malignant Hyperthermia-Like Syndrome (MHLS) can occur in patients with these hyperglycemic crises. When the severe hypovolemia and acidosis of HHS and DKA are exacerbated by the hypermetabolic state of MHLS, it can lead to multiorgan failure and hemodynamic collapse, necessitating extracorporeal membrane oxygenation (ECMO) support. However, the use of ECMO in these situations is not well described.

Description: A 16 year-old male with a history of sickle cell trait and obesity presented to the emergency department with altered mental status. He was found to have significant metabolic acidosis (pH 6.9), marked hyperglycemia (1,105mg/dL), ketosis, and hyperosmolality (437 mOsm/kg). Despite aggressive fluid resuscitation and insulin therapy, the patient developed multiorgan failure and progressive hypotension, complicated by severely diminished biventricular systolic function refractory to inotropic and vasopressor support. This led to the initiation of venoarterial (VA)-ECMO at 47 hours after admission. Concurrently, MHLS was suspected due to persistent hyperthermia (41.1℃) and elevated creatine kinase levels (90,188U/L), leading to the administration of dantrolene on the third day of hospitalization. The patient‘s fevers, rhabdomyolysis, and multiorgan failure subsequently improved. Notably, with each dose of dantrolene, the patient experienced significant bradycardia that was hemodynamically insignificant while supported on ECMO. He was decannulated from ECMO on day 9 and achieved a full recovery after a 44-day hospital stay.

Discussion: DKA, HHS, and MHLS can all present as life threatening conditions, especially when a patient presents with a combination of all three. As our experience demonstrates, ECMO is an effective tool in these patients with hemodynamic collapse, and the prompt identification and treatment of MHLS is crucial for patient survival