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

BACKGROUND: Acute 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. CONCLUSION: In 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.