Acute adrenal insufficiency-induced hypotension following spinal surgery in a pediatric patient with Charcot-Marie-Tooth disease and severe scoliosis: a case report.

backgroundAcute adrenocortical insufficiency (adrenal crisis) as a cause of postoperative hypotension is an uncommon and underreported condition in pediatric patients. Charcot-Marie-Tooth (CMT) disease, a hereditary motor and sensory neuropathy, frequently coexists with scoliosis. Surgical correction of severe scoliosis involves significant stress due to considerable surgical trauma and prolonged operative time. CASE DESCRIPTION: A 13-year-old female diagnosed with CMT and severe scoliosis (thoracic curvature Cobb angle 92°) underwent posterior osteotomy and corrective surgery. On postoperative Day 1, the patient experienced persistent hypotension (systolic blood pressure < 90 mmHg) that did not respond to fluid resuscitation or vasopressor support with dopamine. On postoperative Day 4, Adrenocorticotropic Hormone (ACTH) stimulation testing revealed a cortisol level of 323.1 nmol/L which was low in the context of physiological stress, along with a markedly reduced ACTH concentration of 4.72 pg/ml, confirming a diagnosis of acute adrenocortical insufficiency. Hydrocortisone treatment resulted in a gradual normalization of blood pressure, which returned to preoperative levels by postoperative Day 8. Steroid therapy was discontinued following normalization of adrenal hormone levels.

conclusionIn pediatric patients undergoing surgery for severe scoliosis, unexplained postoperative hypotension warrants thorough evaluation for adrenal crisis, especially in the context of underlying chronic illness or malnutrition.