Advanced Techniques for Securing Airway in Mucopolysaccharidoses and the Impact of New Therapeutic Approaches

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.