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Life sciences · Journal article
BMC Anesthesiology · October 15, 2026
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Alström syndrome (AS) is a rare autosomal recessive disorder characterized by multi-system dysfunction including cardiomyopathy, hepatic impairment, and pathological obesity. We present the perioperative management of a pediatric AS patient with severe obesity (BMI 39.2 kg/m²) undergoing emergency laparoscopic ovarian detorsion. The anesthetic strategy utilized remimazolam-based balanced anesthesia with dosage adjustment based on obesity and hepatic dysfunction to address hemodynamic instability risks, resulting in stable intraoperative parameters and rapid postoperative recovery (extubation at 98 minutes after induction and discharge on postoperative day 3 without complications). This case suggests that remimazolam-based balanced anesthesia may be feasible and well tolerated in this high-risk scenario. This case highlights critical perioperative considerations for managing pediatric patients with rare metabolic syndromes complicated by severe obesity in emergent laparoscopic surgical settings.