Abstract
Rett syndrome is a genetic neurodevelopmental disorder which occurs in females and includes autism, spasticity, hypotonia, microcephaly, scoliosis, stereotyping, abnormal respiratory control and seizures. They are at an increased predisposition for QT interval prolongation and cardiac arrhythmias. An 8-year-old severely intellectually impaired girl with Rett syndrome was referred to us for anaesthesia for multiple dental abscess drainage and rehabilitation. Her frequency of convulsions had increased and she convulsed every time an attempt was made to feed her. The pain of chewing exacerbated the convulsions. The cornerstone of our management was to provide adequate pain relief, anaesthesia without muscle relaxant and opioids, and judicious use of local anaesthetics. We discuss the anaesthetic management and its advantages and limitations in this case report.