Posterior spinal fusion in Sotos' syndrome.

Abstract

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.