Overview on Anesthetic Management of Children with Mucopolysaccharidoses (MPSs)

Abstract

Objective: Mucopolysaccharidoses (MPSs) are hereditary disorders characterised by the accumulation of glycosaminoglycans (GAGs) due to the deficiency of lysosomal enzymes. These substrates accumulate in the skin, brain, heart, bone, liver, spleen, blood vessels, cornea and tracheobronchial airways. Patients with MPS disorders require several surgical interventions related to the deposition of GAGs most frequently otolaryngologic and ophthalmologic procedures. A wellknown problem with MPS disease is high incidence of difficult airway. Airway obstruction, difficulty in ventilation and oxygenation elevate the mortality rates of these patients under general anesthesia. Material and Methods: Charts of nine children with MPS who received general anesthesia performed by the authors at the otolaryngology and ophthalmology departments in the past five years were retrospectively reviewed. Results: Nine children received general anesthesia for 12 surgical procedures. One child died at preinduction, one case could not be intubated at his second surgery and it was cancelled, another case could not be intubated and an otolaryngologist performed an emergency tracheostomy in the emergency department for status epilepticus and respiratory distress. the others were uncomplicated under general anesthesia. Conclusion: An experienced anesthetic team should undertake anesthesia of all MPS cases. Awareness of MPS among anesthesiologists should be increased regarding to the problems of both syndrome and treatment in order to reduce morbidity and mortality.