Anaesthesia and Rett syndrome: a case report

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.