2023 Volume 51 Issue 1 Pages 10-12
Smith-Magenis syndrome (SMS) is caused by a heterozygous deletion at chromosome 17p11.2 or a RAI1 pathogenic variant. SMS is characterized by distinctive physical features, developmental delay, behavioral abnormalities, and sleep disturbance. We report our experience providing general anesthetic management to a patient with SMS requiring dental treatment.
A 6-year-old girl (weight, 21.1 kg ; height, 109 cm) with SMS was scheduled to undergo dental treatment under general anesthesia for 4.5 hours. She had hearing loss, severe sleep disturbance, and behavioral abnormalities. The behavioral abnormalities included self-injury, which occasionally lasted for periods of up to 2 hours. Her pediatrician had prescribed melatonin and aripiprazole. The melatonin was only effective for several hours ; upon awakening, she would attempt to consume whatever food was around her and would become aggressive and self-injurious if food was not available. Since it was deemed difficult for her to adhere to a preoperative fast, we decided to adjust the medication within her existing prescription. We also decided to use propofol for anesthesia maintenance to prevent self-injury arising from emergence excitement.
Although she awoke twice during the night before receiving anesthesia, she was able to maintain her fast without self-injury. General anesthesia was induced using oxygen and sevoflurane without restraint. We used propofol instead of sevoflurane to maintain the anesthesia. After the procedure, she awoke calmly, and extubation was performed without agitation. No complications were observed during or after the general anesthesia until hospital discharge.
When providing anesthesia to patients with SMS, it is important to take countermeasures to prevent sleep disturbances and behavioral abnormalities, especially the ability of the patient to adhere to the preoperative fast.
