Mohammed Yousef M, Khan Safeera
Anesthesia and Pain Management, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.
Medicine, Damascus University, Damascus, SYR.
Cureus. 2020 Sep 22;12(9):e10582. doi: 10.7759/cureus.10582.
Airway management in patients suffering from mucopolysaccharidoses (MPS) is one of the most difficult anesthesiologic challenges. MPS is a group of rare, inherited diseases caused by the absence or malfunctioning of lysosomal enzymes needed to break down macromolecules called glycosaminoglycans (GAGs). MPS is associated with clinical symptoms and physical features, which all together contribute to the high incidence of difficulty in providing airway during surgical procedures. We used PubMed as our main database (PubMed Advanced Search Builder) to search for relevant literature. At first, we looked for the prevalence of MPS worldwide. Then, we searched for airway management complications in the MPS population using the keywords: "Mucopolysaccharidoses," "Anesthesia complications," and "airway management." Another search was carried out to look for new therapeutic agents and explore their impacts on body organs. We reviewed the finalized articles to explore how anesthesiologists used different airway techniques. We discovered that video laryngoscope and I-gel aided fiber-optic intubation have been available in recent years and have been used uneventfully in several patients. We presented recommendations regarding preoperative and intraoperative preparation to avoid airway-related complications in the future. We realized that many therapy approaches had been suggested, especially after further understanding of the pathophysiology of MPS. However, more investigation needs to be conducted to determine their efficacy and explore if there is any impact on airway management.
黏多糖贮积症(MPS)患者的气道管理是麻醉领域最具挑战性的难题之一。MPS是一组罕见的遗传性疾病,由分解称为糖胺聚糖(GAGs)的大分子所需的溶酶体酶缺乏或功能异常引起。MPS与临床症状和身体特征相关,这些因素共同导致手术过程中气道管理困难的发生率很高。我们以PubMed作为主要数据库(PubMed高级搜索生成器)来搜索相关文献。首先,我们查找了全球范围内MPS的患病率。然后,我们使用关键词“黏多糖贮积症”“麻醉并发症”和“气道管理”在MPS人群中搜索气道管理并发症。还进行了另一项搜索,以寻找新的治疗药物并探讨它们对身体器官的影响。我们回顾了最终确定的文章,以探讨麻醉医生如何使用不同的气道技术。我们发现近年来视频喉镜和I-gel辅助纤维支气管镜插管已可用,并在一些患者中顺利使用。我们提出了关于术前和术中准备的建议,以避免未来出现气道相关并发症。我们意识到已经提出了许多治疗方法,特别是在对MPS的病理生理学有了进一步了解之后。然而,需要进行更多的研究来确定它们的疗效,并探讨是否对气道管理有任何影响。