Ring 18 syndrome is a rare chromosomal abnormality, with about 70 cases reported worldwide. The clinical manifestations of this syndrome include congenital heart disease, a short height, craniofacial abnormalities such as microcephaly and micrognathia, hypothyroidism, and mental retardation. We report the general anesthetic management of a patient with ring 18 syndrome undergoing dental treatment.
A 20-year-old male (weight, 28.8 kg ; height, 127 cm) with ring 18 syndrome was scheduled to undergo dental treatment under general anesthesia. The clinical manifestations of this patient included mental retardation, a short height, hypertelorism, a flat nasal bridge, micrognathia, a carp-shaped mouth, aortic valve regurgitation, and pulmonary regurgitation. Because of the anticipated difficulty caused by the patient's mental retardation, he was scheduled to undergo dental treatment under general anesthesia. Prior to entering the operation room, he was sedated with oral midazolam. Upon the induction of general anesthesia, an oral airway, laryngeal mask, video laryngoscope, and broncofiberscope were prepared. General anesthesia was induced with propofol and remifentanil. Since mask ventilation was easy to perform, we administered rocuronium. Fortunately, intubation by visual identification of the larynx and the glottis using a Macintosh laryngoscope was possible. However, nasotracheal intubation was impossible because of the rhinostenosis. General anesthesia was maintained using sevoflurane and remifentanil. To avoid regurgitation, we maintained his heart rate at more than 60 bpm and avoided any increase in systemic vascular resistance. Extubation was performed without difficulty after the patient was fully awake. No complications were observed during or after the general anesthesia.
When providing anesthesia for patients with ring 18 syndrome, anesthesiologists should evaluate the congenital heart disease preoperatively and prepare for unanticipated airway difficulties.
