Anesth Pain Med.  2018 Apr;13(2):173-175. 10.17085/apm.2018.13.2.173.

Anesthetic management of a pediatric patient with Rett syndrome: A case report

Affiliations
  • 1Department of Anesthesiology and Pain Medicine, Ulsan University Hospital, Ulsan, Korea. nurunbab@hotmail.com

Abstract

Rett syndrome is a neurodevelopmental disease that almost always affects female patients. It is caused by mutations in MeCP2 in the majority of cases. Patients diagnosed with Rett syndrome may experience serious adverse events even with smaller amounts of medication for sedation and anesthesia. The major anesthetic concerns associated with Rett syndrome are lack of cooperation, abnormal continuous limb movements, abnormal respiratory control, difficult positioning secondary to scoliosis, and altered sensitivity to painful stimuli. Because of the risks caused by these problems, anesthesiologists should be aware of the specific anesthetic concerns of patients with Rett syndrome in order to safely administer anesthesia. Here, we describe the management of a pediatric patient diagnosed with Rett syndrome.

Keyword

MeCP2 mutations; Postnatal neurological disorder; Rett syndrome

MeSH Terms

Anesthesia
Dyskinesias
Extremities
Female
Humans
Rett Syndrome*
Scoliosis

Reference

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