Dell'Olio Fabio, Lorusso Pantaleo, Siciliani Rosaria Arianna, Massaro Maria, Barile Giuseppe, Tempesta Angela, Grasso Salvatore, Favia Gianfranco, Limongelli Luisa
Department of Interdisciplinary Medicine, Complex Operating Unit of Odontostomatology Aldo Moro University Bari Italy.
Department of Emergency and Organ Transplantation, Operating Unit of Anesthesiology Aldo Moro University Bari Italy.
Clin Case Rep. 2023 Aug 10;11(8):e7657. doi: 10.1002/ccr3.7657. eCollection 2023 Aug.
The perioperative control of ammonia, reduction of stress, and administration of drugs tolerated in type 1 citrullinemia and Brugada pattern allowed the successful and uneventful management of general anesthesia in the study patient.
The aim of this study was to report the targeted perioperative management of general anesthesia (GA) adopted for dental extractions in a rare patient with type 1 citrullinemia and Brugada pattern. A male, Caucasian, adult type 1 citrullinemia patient needed dental extractions under GA. The medical history showed neurodevelopmental impairment, growth retardation, epilepsy, and a Type 2 Brugada electrocardiographic pattern in the second precordial lead. The authors focused the anesthesiologic protocol on the prevention of hyperammonemia and fatal arrhythmias. Changes in diet and 10% glucose solution administration prevented protein catabolism due to the fasting period (ammonia was 44 μmol/L preoperatively and 46 μmol/L postoperatively; glycemia was 120 g/dL preoperatively and 153 g/dL postoperatively). The patient received a continuous electrocardiogram, noninvasive blood pressure, pulse oximeter, entropy monitoring, train-of-four monitoring, and external biphasic defibrillator pads. Midazolam, remifentanil, and dexamethasone were administered for pre-anesthesia; thiopental and rocuronium for induction; remifentanil and desflurane for maintenance; sugammadex for decurarization. After the intraligamentary injection of lidocaine 2% with epinephrine 1:100,000 for local anesthesia, the patient developed a transient Type 1 Brugada pattern that lasted a few minutes. The whole procedure lasted 30 min. The patient's discharge to ward occurred 3 h after the end of GA. The perioperative management of ammonia, reduction of stress, and administration of drugs tolerated in Type 1 citrullinemia and Brugada pattern allowed the successful and uneventful administration of GA in the study patient.
围手术期对氨的控制、应激的减轻以及给予1型瓜氨酸血症和布加综合征可耐受的药物，使得本研究患者的全身麻醉得以成功且顺利进行。
本研究的目的是报告为一名患有1型瓜氨酸血症和布加综合征的罕见患者进行拔牙手术时采用的有针对性的围手术期全身麻醉管理。一名成年白种男性1型瓜氨酸血症患者需要在全身麻醉下进行拔牙。病史显示其存在神经发育障碍、生长发育迟缓、癫痫，且在第二胸前导联呈现2型布加综合征心电图模式。作者将麻醉方案重点放在预防高氨血症和致命性心律失常上。饮食调整和给予10%葡萄糖溶液可预防禁食期的蛋白质分解代谢（术前氨水平为44μmol/L，术后为46μmol/L；术前血糖为120g/dL，术后为153g/dL）。患者接受持续心电图、无创血压、脉搏血氧饱和度监测、熵监测、四个成串刺激监测以及外置双相除颤器电极片。麻醉前给予咪达唑仑、瑞芬太尼和地塞米松；诱导时给予硫喷妥钠和罗库溴铵；维持时给予瑞芬太尼和地氟醚；使用舒更葡糖钠进行肌松拮抗。在局部麻醉注射含1:100,000肾上腺素的2%利多卡因至韧带内后，患者出现持续数分钟的短暂1型布加综合征模式。整个手术持续30分钟。全身麻醉结束后3小时患者返回病房。围手术期对氨的管理、应激的减轻以及给予1型瓜氨酸血症和布加综合征可耐受的药物，使得本研究患者的全身麻醉得以成功且顺利进行。
