Peripartum management of a patient with dopamine beta-hydroxylase deficiency, a rare congenital cause of dysautonomia.

We present the first reported case involving the peripartum anaesthetic management of dopamine beta-hydroxylase deficiency in a 22-year-old primigravida with high-grade placenta praevia. Elective caesarean section was performed at 36 weeks gestation with a combined spinal-epidural regional anaesthetic technique. Extensive preparation was undertaken to manage the consequences of obstetric haemorrhage and consideration given to potential pharmacological sensitivities suspected to exist in patients with this rare disorder affecting sympathetic nervous system function. An uncomplicated caesarean section was performed from which the patient recovered well to be discharged home with a healthy baby.