关键词: Waardenburg syndrome airway anesthesia management cochlear implantation rare disease Waardenburg syndrome airway anesthesia management cochlear implantation rare disease

来  源:   DOI:10.3389/fped.2022.965884   PDF(Pubmed)

Abstract:
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要:
未经证实:Waardenburg综合征(WS)是一种罕见的遗传性疾病,具有临床特征,例如白色的前额,先天性耳聋,反乌托邦角胸,和异色症虹膜。它主要通过常染色体显性模式传播,有一些基因突变.患有WS的儿童通常在很小的时候就需要手术干预,并且可能有困难的气道。这对麻醉师来说可能是个挑战。
UNASSIGNED:我们报告了一名体重为9公斤的患有WS的14个月大女孩在人工耳蜗植入过程中的麻醉管理。除了听力损失和说话延迟,她出现了运动发育迟缓,咀嚼和吞咽障碍,和饮食呕吐。麻醉诱导后气管插管时遇到阻力,旋转后气管插管成功。
UNASSIGNED:我们报告了一名患有WS的儿童在人工耳蜗植入过程中的麻醉管理,简述与WS相关的研究进展,并讨论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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