The anaesthetic management of a patient with tuberous sclerosis underg
oing two-stage scoliosis surgery is described. The patient suffered fr
om severe mental retardation, seizures and facial angiofibromas. Gener
al anaesthesia using isoflurane and nitrous oxide in oxygen, supplemen
ted with opioid analgesia and hydralazine, and labetalol to induce hyp
otension, appeared to be satisfactory. Postoperative recovery was dela
yed and complicated by pleural effusion, sputum retention and mild sei
zures. Tuberous sclerosis is an autosomal dominant disease well known
for its neurocutaneous manifestations. Other organs such as the heart,
lungs and kidneys may be involved. The potential problems in the anae
sthetic management of a patient with tuberous sclerosis are discussed.