Anaesthetic management in a neonate with a double-outlet right ventricle undergoing repair of leaking myelomeningocele.

Neonates comprise a special population of patients undergoing surgery, and the presence of congenital defects further complicates their anaesthetic management. This report describes a neonate born with Arnold-Chiari malformation and a ruptured myelomeningocele, who was later found to also have a double-outlet right ventricle. This report outlines the careful selection of anaesthetic agents and perioperative strategies tailored to the patient's compounded neurologic and cardiac congenital defects while catering to the surgical requirements. The patient underwent repair of the MMC under total intravenous anaesthesia induced with midazolam, ketamine and atracurium and maintained with a combination of dexmedetomidine and fentanyl. The combination of dexmedetomidine and fentanyl appears to be a viable option for neonates with rigid perioperative haemodynamic goals and a need for neuroprotection. We report the safe use of dexmedetomidine for total intravenous anaesthesia pending more robust evidence in this patient population.