Perioperative Management of a Hospitalized Patient with Williams Syndrome Undergoing Teeth Extraction under General Anesthesia

Abstract

Williams syndrome is a congenital disease accompanied by “elfin-like” facial features, cardiovascular disease with supravalvular aortic stenosis, and delayed development. Because of intellectual disability, it may be difficult for patients with Williams syndrome to adapt to changes in environment such as hospitalization. However, some reports have suggested that ambulatory anesthesia should not be performed for patients with this syndrome because of the possibility of perioperative death caused by their cardiovascular disease. We report herein the perioperative management of a patient with Williams syndrome undergoing extraction of third molar teeth under general anesthesia.

The patient was an 18-year-old woman. She was nervous even during medical interview in the hospital ward. According to her mother, she had an extreme fear of hospitalization and surgery. Therefore, we tried not to frighten her and to make her feel relaxed during hospitalization. We performed only easy medical acts that she had previously been able to accept, such as blood pressure measurement and auscultation. One hour before surgery, we secured vascular access and started intravenous sedation with midazolam in the hospital ward. During intravenous sedation, she was calm and exhibited neither respiratory depression nor glossoptosis, etc. The induction of anesthesia was smooth. As a number of papers have reported, mask ventilation and tracheal intubation were not difficult despite micrognathia. She exhibited no problems during surgery. In order not to make her feel anxiety after awakening, we injected midazolam after extubation. After surgery, she was calm and relaxed with her parents together in the hospital ward. She was in good condition and was discharged from the hospital on the next day. To manage hospitalized patients with Williams syndrome, it is necessary to avoid events and medical actions that may cause the patients to feel stress or anxiety.