Perioperative considerations for adult patients with Dravet syndrome in regional centres

Abstract

Dravet syndrome (DS) is a rare and intractable severe form of epilepsy presenting in infancy with frequent prolonged myoclonic seizures and neurodevelopmental impairment, associated with aSCN1Agene mutation. Seizures are often triggered by temperature fluctuations and hyperthermia. This report presents a woman in her late adolescence with DS complicated with intractable catamenial epilepsy, a sex-specific form of epilepsy with seizure activity prominent during phases of the menstrual cycle. The patient underwent general anaesthesia for a hysteroscopy, cervical dilatation and endometrial curettage with Mirena insertion to improve seizure control. Her perioperative care was optimised for seizure prevention with continuation of antiepileptic medications, strict temperature monitoring and control, optimised anaesthetic agents encompassing induction with propofol and fentanyl with maintenance sevoflurane, followed by attentive postoperative care and monitoring. This case demonstrates that general anaesthesia can safely be delivered to adult patients with DS in rural and regional areas with thorough perioperative planning.