Ma Yangwei, Gao Jia, Huo Lianghong, Wang Fang
Department of Anesthesiology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Front Pediatr. 2024 Sep 13;12:1450017. doi: 10.3389/fped.2024.1450017. eCollection 2024.
Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) is an exceptionally rare condition. This case report highlights a child diagnosed with ROHHAD syndrome, presenting with a mediastinal tumor. ROHHAD syndrome is characterized by early onset obesity, hypothalamic dysfunction, autonomic dysfunction, inadequate ventilation, suspected seizures, and abnormal behavior. The presence of a mediastinal tumor necessitated surgical intervention. Key considerations during surgery included hypernatremia due to hypothalamic dysfunction, potential airway challenges, preoperative anemia, and hemodynamic fluctuations during the removal of the sizable mediastinal tumor. Comprehensive preparations ensured a safe operation. Notably, some children with this syndrome may exhibit symptoms such as decreased gastrointestinal function, polyuria, and thermoregulatory disturbances. Vigilance is essential during anesthesia assessment in these patients. Anesthesiologists should enhance their knowledge of this condition and tailor their management strategies based on individual clinical presentations and the specific planned surgical procedures.
快速进展性肥胖伴通气不足、下丘脑功能障碍和自主神经调节异常（ROHHAD）是一种极为罕见的病症。本病例报告重点介绍了一名被诊断为ROHHAD综合征且患有纵隔肿瘤的儿童。ROHHAD综合征的特征为早发性肥胖、下丘脑功能障碍、自主神经功能障碍、通气不足、疑似癫痫发作以及行为异常。纵隔肿瘤的存在需要进行手术干预。手术期间的关键考量因素包括下丘脑功能障碍导致的高钠血症、潜在的气道问题、术前贫血以及切除巨大纵隔肿瘤过程中的血流动力学波动。全面的准备工作确保了手术的安全。值得注意的是，一些患有此综合征的儿童可能会出现诸如胃肠功能减退、多尿和体温调节紊乱等症状。在对这些患者进行麻醉评估时，警惕至关重要。麻醉医生应加强对这种病症的了解，并根据个体临床表现和具体的手术计划制定相应的管理策略。
