Rett syndrome is a progressive neurological disorder that occurs only in females and it manifests with mental retardation, seizures, movement disorders, autistic behavior, and abnormal breathing. A 4.5-year-old female child with Rett syndome underwent dental restoration under sedoanalgesia. Her physical examination was normal except for sterotyped movements. We recommend that anesthesiologists should have knowledge about this disorder’s characteristics, complications, potential associated problems, and important aspects of anesthetic drug selection
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