Gholmieh Linda, El Assaad Rosanne
Anesthesiology, Lebanese American University Medical Center, Beirut, LBN.
Cureus. 2024 Sep 24;16(9):e70099. doi: 10.7759/cureus.70099. eCollection 2024 Sep.
Wolf-Hirschhorn syndrome (WHS) is a rare genetic disorder caused by 4p chromosome microdeletion, characterized by distinctive craniofacial features, growth delay, short stature and slow height gain, variable degrees of intellectual disabilities, epilepsy, and congenital heart disease. Anesthetic management in pediatric patients with WHS requires a tailored approach due to the complex multisystem involvement, and the optimal strategy remains undetermined despite several published case reports. We present the airway management of an eight-year-old patient with WHS undergoing strabismus surgery using a supraglottic device. Although our patient's distinctive craniofacial features of WHS suggested a potential for difficult intubation; after mitigating the risk of aspiration, a flexible laryngeal mask was successfully placed on the first attempt, offering a viable alternative to the challenges of tracheal intubation.
沃尔夫-赫希霍恩综合征（WHS）是一种由4号染色体微缺失引起的罕见遗传病，其特征为独特的颅面特征、生长发育迟缓、身材矮小和身高增长缓慢、不同程度的智力残疾、癫痫和先天性心脏病。由于存在复杂的多系统受累情况，WHS患儿的麻醉管理需要采取个性化方法，尽管已有多篇病例报告发表，但最佳策略仍未确定。我们介绍了一名8岁WHS患儿在使用声门上装置进行斜视手术时的气道管理情况。尽管我们的患者具有WHS独特的颅面特征，提示有插管困难的可能性；但在降低误吸风险后，首次尝试就成功置入了柔性喉罩，为气管插管的挑战提供了一种可行的替代方法。
