Wang Xiaohui, Xu Lin, Zhang Na, Zhao Ying
Department of Anesthesiology, Cheeloo College of Medicine, Qilu Hospital (Qingdao), Shandong University, Qingdao, China.
Department of Anesthesiology, Qilu Hospital of Shandong University, Jinan, China.
Front Pediatr. 2022 Sep 12;10:965884. doi: 10.3389/fped.2022.965884. eCollection 2022.
Waardenburg syndrome (WS) is a rare genetic disorder that presents with clinical characteristics such as white forelock, congenital deafness, dystopia canthorum, and heterochromia iridis. It is mostly transmitted through an autosomal dominant mode, with a few genetic mutations. Children with WS often require surgical intervention at an early age and may have a difficult airway, which can be challenging for anesthesiologists.
We report the anesthetic management during cochlear implantation in a 14-month-old girl with WS who weighed 9 kg. In addition to hearing loss and delayed speech, she presented with motor developmental delay, chewing and swallowing impairments, and dietary bucking. Resistance was encountered during tracheal intubation after anesthesia induction, and the tracheal tube was successfully intubated after rotation.
We report the anesthetic management during cochlear implantation in a child with WS, briefly describe the research advances related to WS, and discuss the optimization of the perioperative management of these children, including airway management, anesthesia-related complications, and the use of anesthetics.
瓦登伯革氏综合征（WS）是一种罕见的遗传性疾病，具有白发斑、先天性耳聋、内眦异位和虹膜异色等临床特征。它大多通过常染色体显性模式遗传，有少数基因突变。患有WS的儿童通常在幼年时就需要手术干预，并且可能存在气道困难，这对麻醉医生来说是一项挑战。
我们报告了一名体重9公斤、患有WS的14个月大女孩在人工耳蜗植入手术期间的麻醉管理情况。除了听力丧失和语言发育迟缓外，她还存在运动发育迟缓、咀嚼和吞咽障碍以及饮食抗拒。麻醉诱导后气管插管时遇到阻力，旋转后气管导管成功插入。
我们报告了一名患有WS的儿童在人工耳蜗植入手术期间的麻醉管理情况，简要描述了与WS相关的研究进展，并讨论了这些儿童围手术期管理的优化，包括气道管理、麻醉相关并发症以及麻醉剂的使用。
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