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Safety of anesthesia in mucopolysaccharidoses - A comparative retrospective cohort study on more than 600 cases
Simon Moser1, Paul Harmatz2, William Rhoads2
1UCSF Benioff Children's Hospital Oakland, 747 52nd St., Oakland, CA 94609, USA; Institute for Inherited Metabolic Diseases and Department of Pediatrics, Paracelsus Medical University Salzburg, Strubergasse 21, 5020 Salzburg, Austria.
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.
Insights
Anesthesia for patients with Mucopolysaccharidosis (MPS) is safer than previously reported, with a low complication rate of 1.06%. Strategies like specialized anesthetist allocation and fiberoptic intubation may further improve safety for MPS patients undergoing general anesthesia.
Area of Science:
- Anesthesiology
- Pediatric Anesthesia
- Rare Diseases
Background:
- Mucopolysaccharidosis (MPS) patients face significant risks during general anesthesia.
- There is a lack of robust evidence and guidelines for managing anesthesia in MPS patients.
- Perioperative complications are common in this vulnerable population.
Purpose of the Study:
- To analyze anesthesia procedures and complication rates in Mucopolysaccharidosis patients.
- To compare complication rates with previously published data.
- To identify factors that may improve anesthesia safety in MPS patients.
Main Methods:
- A single-center retrospective chart review of 660 anesthesia procedures in 83 MPS patients (2010-2022).
- Comparison with a published cohort of 67 patients undergoing 269 anesthesia procedures.
- Quantitative analysis of demographics, MPS types, anesthesia techniques, surgical procedures, and complication rates.
Main Results:
- A low overall complication rate of 1.06% (7 cases) was observed.
- Supraglottic airway was the most common airway type (77%).
- Complication rates were significantly lower than in a previously reported cohort (1.06% vs. 25.6%).
Conclusions:
- Anesthesia for MPS patients can be achieved with a low complication rate.
- Specialized care, including dedicated anesthetists and advanced airway techniques like fiberoptic intubation, may enhance safety.
- Further research and standardized protocols are needed to optimize anesthetic management for MPS patients.
