Çekmen Nedim, Bihorac Edvin, Nur Mert
Department of Anesthesiology and Intensive Care Unit, Baskent University, Faculty of Medicine, Ankara, Turkey.
J Dent Anesth Pain Med. 2025 Apr;25(2):133-137. doi: 10.17245/jdapm.2025.25.2.133. Epub 2025 Mar 27.
1p36 deletion syndrome is characterized by a genetic deletion that frequently causes central nervous system, craniofacial, cardiac, and musculoskeletal anomalies. Perioperative management of patients with 1p36 deletion syndrome presents unique challenges due to multiple anomalies and potential complications. We present the successful anesthetic management of a 16-year-old patient diagnosed with 1p36 deletion syndrome who underwent general anesthesia for multiple dental procedures, including dental fillings, extractions, and tartar removal. The patient had micrognathia, hypotonia, flat eyebrows, a short neck, inability to sit without support, absence of speech and self-care skills, limited ability to follow simple commands, and poor cooperation. These factors increase the risk of difficult mask ventilation and intubation. To minimize aspiration risk, we successfully intubated the patient using rapid-sequence induction and intubation (RSII) method with cricoid pressure. A perioperative multidisciplinary team approach and comprehensive preoperative evaluation are crucial due to the existing anomalies, developmental and motor delays, and potential airway complications associated with this syndrome.
1p36缺失综合征的特征是一种基因缺失,常导致中枢神经系统、颅面部、心脏和肌肉骨骼异常。由于存在多种异常和潜在并发症,1p36缺失综合征患者的围手术期管理面临独特挑战。我们介绍了一名16岁被诊断为1p36缺失综合征患者的成功麻醉管理案例,该患者接受了包括补牙、拔牙和洗牙在内的多项牙科手术的全身麻醉。患者有小颌畸形、肌张力减退、眉毛扁平、颈部短、无支撑时无法坐立、无言语和自理能力、遵循简单指令的能力有限以及合作性差。这些因素增加了面罩通气困难和插管困难的风险。为了将误吸风险降至最低,我们采用快速顺序诱导插管(RSII)方法并施加环状软骨压迫,成功为患者进行了插管。鉴于该综合征存在的异常、发育和运动迟缓以及潜在气道并发症,围手术期多学科团队方法和全面的术前评估至关重要。