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Anaesthesia and Rett syndrome: a case report.

D Maguire1, C Bachman

  • 1Department of Anaesthesia, University of Manitoba Children's Hospital, Winnepeg.

Canadian Journal of Anaesthesia = Journal Canadien D'Anesthesie
|July 1, 1989
PubMed
Summary

Rett syndrome patients can undergo spinal fusion surgery safely. Anesthesia and surgery did not worsen respiratory control issues in a 14-year-old girl with this neurological disorder.

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Area of Science:

  • Neurology
  • Pediatric Surgery
  • Anesthesiology

Background:

  • Rett syndrome is a rare neurodevelopmental disorder primarily affecting females.
  • Key features include cognitive impairment, autistic behaviors, motor deficits, and abnormal breathing patterns.
  • Spinal deformities often necessitate surgical intervention, such as spinal fusion.

Observation:

  • A 14-year-old female diagnosed with Rett syndrome underwent spinal fusion surgery.
  • General anesthesia was administered for the procedure.
  • The patient's respiratory control, a known vulnerability in Rett syndrome, was closely monitored.

Findings:

  • The surgery and general anesthesia did not lead to any observable worsening of the patient's respiratory control defect.
  • The patient experienced hypothermia and ongoing blood loss during the procedure.
  • A normal anion gap acidosis was noted but was not linked to the underlying Rett syndrome.

Implications:

  • This case suggests that spinal fusion surgery under general anesthesia can be managed safely in patients with Rett syndrome.
  • Careful perioperative monitoring is crucial to manage potential complications like hypothermia and blood loss.
  • Further research could explore anesthetic protocols and surgical outcomes in this specific patient population.

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