Background and Goal of Study: 

Mucopolysaccharidosis (MPS) represents a group of rare lysosomal storage disorders associated with a progressive organ dysfunction (1). Typical anaesthetic problems include difficult airway management, cardiovascular and cervical spine issues (1). Objective: To review one hundred twenty-six (N126) anaesthetic charts following twenty children over a 10-year period with emphasis on airway problems.

Materials and Methods:

A descriptive retrospective audit chart review of 20 patients with MPS who had received anaesthetics at the Niño Jesús Children’s Hospital during the time frame January 2010–March 2017. We evaluated the type of anaesthesia, the airway devices used and the and frequency perioperative complications. Data are presented as mean and standard deviation, percentage.

Results: 100% of the procedures were performed with general anaesthesia. Primary airway management for the 126 procedures included 76 laryngeal masks (60.31%). Of 38 endotracheal tube (30,15%), 22 were managed by fiberoptic bronchoscopy (17.46%) and 12 with facial masks (9,52%). The most frequent complication was laryngospasm (4,69%) and the worst complication was a can’t oxygenate can’t intubate scenario which was resolved with an urgent tracheostomy. Discussion: The most important aspect MPS patients' anaesthetic considerations is airway management. The anticipation of difficulties and preparation are of paramount importance. Even if not chosen as the first option, we highly recommend having a fiberoptic bronchoscope readily available in case other methods fail. Further, the use of LM to rescue the airway is recommended due to consistently good results. Nowadays, newly developed airway devices have greatly increased the safety of MPS.

Conclusion(s): In our experience, a laryngeal mask is a very good option for minor surgery with children with MPS. Adverse events are less frequent using fiberoptic bronchoscopy intubation as the first option.

03 / Mucopolysaccharidosis cases: ten years of experience in airway management.