Anesthetic management of a pediatric patient with Rett syndrome: A case report.
10.17085/apm.2018.13.2.173
- Author:
A Ran LEE
1
;
Hyung Kwan LEE
;
Young Ung KIM
;
Jae Ho LEE
;
Ho Jun KANG
;
Se Hun PARK
Author Information
1. Department of Anesthesiology and Pain Medicine, Ulsan University Hospital, Ulsan, Korea. nurunbab@hotmail.com
- Publication Type:Case Report
- Keywords:
MeCP2 mutations;
Postnatal neurological disorder;
Rett syndrome
- MeSH:
Anesthesia;
Dyskinesias;
Extremities;
Female;
Humans;
Rett Syndrome*;
Scoliosis
- From:Anesthesia and Pain Medicine
2018;13(2):173-175
- CountryRepublic of Korea
- Language:English
-
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.
