Mucopolysaccharidosis (MPS) is a genetic disease that alters tissue morphology, generating functional and anatomical alterations. This explains why they represent a population group with a high risk of airway difficulties given soft tissue deformities as well as a frequent requirement of surgical interventions to solve the mechanical problems caused by their disease. A descriptive observational study was carried out by analyzing the medical records of patients diagnosed with MPS undergoing procedures that required general anesthesia or sedation at the Fundación Cardioinfantil during the period of time between 01/01/2018 and 01/01/2020, supported by the fact that the Fundación Cardioinfantil is a world reference center for this type of disease, considered an orphan given its low worldwide incidence. The objective of this study was to describe the anesthetic experience during these procedures, specifically the demographic characteristics of the population studied, the anesthetic procedure and the approaches used in airway management, as well as the complications associated with the anesthetic procedure in relation to the disease. In our general results we observe concordance with the findings of international studies in patients with MPS where airway complications occur with a much higher incidence compared to the general population, therefore we recommend that anesthetic interventions in these patients be performed in centers of high complexity and experience in airway management regardless of the risk of the procedure.
