Management of Anesthesia for Rubinstein-Taybi syndrome

Corresponding author: Cheon-Hee Park, M.D., Department of Anesthesia and Pain Medicine, Gwangju Christian Hospital, 37, Yangnim-ro, Nam-gu, Gwangju 503-715, Korea. Tel: 82-62-650-5154, Fax: 82-62-650-5116, E-mail: [email protected] This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. CC Rubinstein-Taybi syndrome (RTS) is a chromosomal mutation disease, which was first described by Rubinstein and Taybi in 1963. Respiratory infection such as aspiration pneumonia and congenital heart disease are leading causes of death during the one-year post-natal period. The patients with the diseases have difficulties in receiving intubations due to the craniofacial abnormality and anesthesia due to respiratory infection and arrhythmia [1]. A 10-year-old female patient weighing 40 kg (75-90 percentile) with a height of 135 cm (25-50 percentile) had RTS with a microdeletion of chromosome 16, and had no particular medications. She was classified with a grade 1 mental retardation. She usually suffered from sleep apnea syndrome, and visited our hospital for tonsillectomy (grade III) and adenoidectomy. She received surgery to correct the strabismus under general anesthesia at another hospital two years ago, but she was reportedly discharged without any side effects. Microcephaly, down slanting palpebral fissures and beaked nose were observed as RTS traits. Moreover, microstomia, macroglossia, narrow palate, micrognathia, and retrognathia were presented. Any abnormal findings were not present on the chest X-ray, electrocardiogram, and echocardiogram, but difficulties in managing her airways were expected since she was classified as Mallampati class III. She had been deprived of food for eight hours and was intra muscularly injected with 10 mg of famotidine for the risk of aspiration, and then was transferred to the operating room. However, she was extremely anxious; thus, she was administered ketamine (15 mg) and midazolam (1.5 mg) via IV injection under oxygen saturation monitoring, and was transferred to the operating room. She was attached to monitoring equipment such as an electrocardiogram, an oxygen saturation monitor, and a noninvasive blood pressure monitoring machine upon arrival at the operating room. Before induction, her vital signs were 120/74 mmHg (BP), 106 beats/min (HR), 20 times/min (RR), and 94% (SpO2). Oxygen (5 L/min) was provided to the patient via a mask, and the ProSeal LMA was prepared. 70 mg of propofol was administered via IV injection, and 25 mg of rocuronium was intravenously injected after confirming mask ventilation. One hundred seconds later, we performed an intubation with a cuffed tube (reinforced 5.5 mm). The laryngoscopic view was defined as Cormark & Lehane grade II. However, the tube did not advance further around the subglottic area; therefore, intubation was performed with the cuffed tube (reinforced 5.0 mm). At that time, vital signs were stable. We began operating by maintaining oxygen (1 L/min), air (2 L/min), and sevoflurane (1.5-2.5 vol%). Premature ventricular contraction was detected on the EKG three times in a row immediately after the surgery. Premature ventricular contraction continued for five minutes based on the 10 to 15 out of 110 beats/min. Therefore, we discontinued the surgery and waited, but it recovered to a normal rhythm without any other medication. After that, we resumed the operation. The vital signs during the surgery were 100-120/60-80 mmHg (BP), 90-120 beats/min (HR), 14-16 times/min (RR), and 99% (SpO2). We confirmed that self-respiration fully recovered by the time the surgery was completed, and then, glycopyrrolate (0.2 mg) and pyridostigmine (5 mg) were administered. As a result, we transferred the patient to the recovery room. The patient was extubated as she began opening her eyes and attempted extubation by herself. 2 L/min of oxygen was