Navigating Aortic Fragility and Seizure Risk in a Child with Marfan Syndrome Undergoing Hypospadias Repair: The Potential of Dexmedetomidine-Based Anesthesia–A Case Report

Abstract

Marfan syndrome poses significant anesthetic risks, including cardiovascular instability and tissue fragility, which are further complicated in pediatric patients with epilepsy. We present the anesthetic management of a 13-year-old boy with Marfan syndrome and controlled epilepsy undergoing elective urethroplasty. To prioritize hemodynamic stability for aortic protection and prevent perioperative seizures, general anesthesia was induced and maintained with sevoflurane and a continuous dexmedetomidine infusion (loading dose 0.5 µg/kg; maintenance 0.2 µg/kg/h). This multimodal approach provided effective sympatholysis and analgesia without utilizing neuraxial blockade. Intraoperative hemodynamics remained stable, and the patient was extubated smoothly without coughing or agitation, followed by an uncomplicated discharge on the second postoperative day. Dexmedetomidine demonstrated efficacy as a pivotal adjunct in this complex case, enabling a non-neuraxial strategy and ensuring a smooth emergence to minimize perioperative morbidity in high-risk pediatric patients with connective tissue and neurological comorbidities