Forschung & Innovation
Publikationen
Safety of anesthesia in mucopolysaccharidoses - A comparative retrospective cohort study on more than 600 cases
PMU Autor*in
Florian B. Lagler
Alle Autor*innen
Simon Moser, Paul Harmatz, William Rhoads, Richard Rowe, Florian B. Lagler
Fachzeitschrift
MOLECULAR GENETICS AND METABOLISM
Kurzfassung
Background: Mucopolysaccharidosis (MPS) patients frequently require general anesthesia and have a high risk of perioperative complications. Strong recommendations and controlled studies are missing so far. Methods: We conducted a mono-center chart review of 660 anesthesia procedures, with comparison to a published cohort. Reported are quantitative analysis of demographics, MPS types, anesthesia methods, kinds of surgical procedures, rates and types of complications (e.g. respiratory, neurologic or cardiologic complications). Included were all MPS cases that had general anesthesia at the UCSF Benioff Children's Hospital Oakland between 2010 and 2022. We compared our results to another well-known report of 67 patients that underwent 269 anesthesia procedures (Dohrmann et al.) comprising 67 patients that underwent 269 anesthesia procedures. Results: In this study, we analyzed 660 anesthetic procedures on 83 MPS patients. The most frequently used airway type was the supraglottic airway (77 %), followed by intubation (15 %) and mask airway (8 %). Complications occurred in only 7 cases (1.06 %). These cases required 3 or more intubation attempts. Other publications generally report much higher rates. Dohrmann et al. 2020 reported a 25.6 % complication rate, with 55 % airway management problems and 35 % respiratory adverse events as the most common as well as a case of hypoxic cardiac arrest resulting in death as the most serious complications (Dohrmann et al., 2020). Discussion and conclusion: This is a large retrospective study on anesthesia for MPS patients. This report provides important hypotheses on how the safety of anesthesia in MPS could be improved. Allocation of all MPS cases to one anesthetist, proficient use of fiberoptic intubation, ensure patients maintain spontaneous breathing during airway device placement, additional safety standards and backup plans may reduce the complication rate and thus have an important impact on the outcome.
Keywords
ANESTHESIA, Mucopolysaccharidosis, Fiberoptic bronchoscope, Intubation, Laryngeal mask airway, Lysosomal storage disease