Cao Jiang, Lv Xin, Chen Lin, Luo Yu, Wu Jianmei, Yu Song
Department of Pediatric Orthopedics, Affiliated Hospital of Zunyi Medical University, Zunyi, 563000, China.
Zunyi Medical And Pharmaceutical College, No. 8 North Section of Ping An Avenue, Xinpu New District, Zunyi, Guizhou Province, 563000, China.
BMC Pediatr. 2025 Jul 11;25(1):549. doi: 10.1186/s12887-025-05905-0.
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.
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.
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.
急性肾上腺皮质功能不全（肾上腺危象）作为术后低血压的一个原因，在儿科患者中并不常见且报道不足。夏科 - 马里 - 图斯（CMT）病是一种遗传性运动和感觉神经病变，常与脊柱侧弯并存。严重脊柱侧弯的手术矫正由于手术创伤大及手术时间长而涉及显著应激。
一名13岁女性，诊断为CMT病和严重脊柱侧弯（胸弯Cobb角92°），接受了后路截骨矫正手术。术后第1天，患者出现持续性低血压（收缩压<90 mmHg），对液体复苏或多巴胺血管升压药支持无反应。术后第4天，促肾上腺皮质激素（ACTH）刺激试验显示皮质醇水平为323.1 nmol/L，在生理应激情况下较低，同时ACTH浓度显著降低至4.72 pg/ml，确诊为急性肾上腺皮质功能不全。氢化可的松治疗使血压逐渐恢复正常，术后第8天恢复到术前水平。肾上腺激素水平恢复正常后停用类固醇治疗。
在接受严重脊柱侧弯手术的儿科患者中，不明原因的术后低血压需要对肾上腺危象进行全面评估，尤其是在存在潜在慢性疾病或营养不良的情况下。
