Paroxysmal nocturnal haemoglobinuria. Peri-operative management of a patient with Budd-Chiari syndrome.

The peri-operative management of a patient with paroxysmal nocturnal haemoglobinuria and associated hypoplastic anaemia, who underwent successful elective surgery for the complication of Budd-Chiari syndrome, is described. Anaesthesia in patients with paroxysmal nocturnal haemoglobinuria should be based on drugs and materials least likely to cause complement activation. The reasons for use of a technique based on benzodiazepines, opioids, isoflurane and the avoidance of nitrous oxide are discussed.