Anaesthesia and airway management in mucopolysaccharidosis

Abstract This paper provides a detailed overview and dis-cussion of anaesthesia in patients with mucopolysacchari-dosis (MPS), the evaluation of risk factors in these patients and their anaesthetic management, including emergency airway issues. MPS represents a group of rare lysosomal storage disorders associated with an array of clinical mani-festations. The high prevalence of airway obstruction and restrictive pulmonary disease in combination with cardio-vascular manifestations poses a high anaesthetic risk to these patients. Typical anaesthetic problems include airway obstruction after induction or extubation, intubation diffi-culties or failure [can’t intubate, can’t ventilate (CICV)], possible emergency tracheostomy and cardiovascular and cervical spine issues. Because of the high anaesthetic risk, the benefits of a procedure in patients with MPS shoul

Hearing loss as a complication of stapes surgery

During a 10-year period (1984-1994) 1229 stapes operations for otosclerosis were performed at our respective institutions by experienced surgeons well trained in the various techniques. Procedures included 691 stapedectomies, 234 small-fenestrae stapedotomies, and 304 revision operations. These primary and revision cases resulted in 20 ears with severe sensorineural hearing loss or anacusis noted during the immediate postoperative period. This article will critically evaluate those procedures that resulted in profound hearing loss and attempt to determine possible reasons for this occurrence. It is hoped that these data will allow surgeons to identify before and/or during surgery patients at risk for development of this complication and therefore decrease the overall morbidity rate of this exacting procedure