23rd International Conference on Oral and Maxillofacial Surgery, Kowloon, Hong Kong, 31 March - 03 April 2017, pp.375
Rett syndrome (RS) is a neurological disease that occurs only
in females and it manifests with mental retardation, seizures,
movement disorders, autistic behaviour and abnormal breathing.
Airway control is difficult due to limited mouth opening. Bruxism,
oculogyric crises, parkinsonism and dystonia are also common,
while myoclonus and choreoathetosis are seen infrequently. Respiratory and cardiac diseases, prolonged QT syndrome, diabetes
mellitus and anaesthetic agent sensitivity are significant considerations for anaesthesiologist. These features lead to the need for
special care. These children require a coordinated multi-specialist
oriented health care.
We present a 12-year-old female with Rett syndrome who
required extraction of impact right canine teeth. Teeth grinding
– bruxism, difficulties in chewing and swallowing, poor oral cavity hygiene, oral inflammation with hypertrophic gingiva were
observed in intraoral examination. She had loss of consciousness,
movement disorders, mental retardation, screaming fits, irritation,
anger, self-abusing behaviour and limited mouth opening. Therefore, sedation anaesthesia was planned for surgical procedures.
After standard monitorisation, patient sedated only with propofol
due to more sensitive to anaesthetics. There was no complication during sedation. After surgery, patient was awakened after
applying analgesic and transferred to recovery room.