Mucopolysaccharidosis and anaesthesia: the impact of enzyme replacement therapy on modern airway management in Asian experience

Mei-Chi Lin; Chien-Chuan Chen

Volume : VIII, Issue : I, January - 2018

Mucopolysaccharidosis and anaesthesia: the impact of enzyme replacement therapy on modern airway management in Asian experience

Mei Chi Lin, Chien Chuan Chen

Abstract :

Background: Mucopolysaccharidoses (MPS) are hereditary storage diseases, and airway management is typically worse in these patients as disease progresses. The impact of enzyme replacement therapy (ERT) on the incidence of difficult airway management has not been adequately documented in Asian patients.

Methods: A retrospective chart review of 28 patients (13 patients receiving ERT) with MPS who had received 72 anaesthetics at Mackay Memorial Hospital between January 2004 and Feuary 2014. The first endpoint of this study is to evaluate the impact of ERT on onchoscopic findings and airway management. The second endpoint is to examine airway problems during anaesthesia and determine the role of the new intubation device in MPS patients.

Results: A problem in airway management at induction of anaesthesia occurred in 10 of 67 (15%) anaesthetics in 6 of 28 patients (21%). The success rates of use of the intubation device for MPS patients were 89%, 90%, 83%, 100% and 100% for the classic laryngoscopy, laryngeal mask airway, awake fie–optic onchoscopy, video laryngoscopy and rigid onchoscopy, respectively. Three patients were still documented as having difficult intubation even after long–term ERT. Interestingly, 54% of MPS patients could have a stationary airway condition, evident from preoperative onchoscopic evaluation, and have a smooth anaesthesia intubation scenario after long–term ERT even having undergone therapy since they were teenagers.

Conclusions: Long–term ERT in Asian MPS patients might help maintain the airway condition in a steady state even having undergone therapy since they were teenagers. Choice of the newer alternative intubation device might help greatly in this scenario.