Posterior spinal fusion in Sotos' syndrome.
Sotos' syndrome (synonym: cerebral gigantism) is the association of mental retardation, macrocephaly and prenatal onset of accelerated growth. The rapid skeletal growth may account for a 4% incidence of scoliosis. General anaesthesia using halothane or enflurane in nitrous oxide and oxygen, with opioid supplementation and labetalol to induce moderate hypotension, appeared to be a satisfactory technique for corrective spinal surgery. The potential problems are discussed, with mental retardation and sometimes aggressive behaviour contraindicating a "wake-up" test. Extradural somatosensory evoked potential monitoring is a satisfactory alternative. Hook failures seem more likely than in patients undergoing surgery for adolescent idiopathic scoliosis.
Suresh
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Anaesthetics Unit, Royal London Hospital, Whitechapel.
D000293:Adolescent; D000768:Anesthesia, General / Q000379:methods*; D001927:Brain Diseases / Q000150:complications; D005877:Gigantism / Q000150:complications*; D006801:Humans; D008297:Male; D012600:Scoliosis / Q000150:complications / Q000601:surgery*; D013123:Spinal Fusion / Q000379:methods*; D013577:Syndrome