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

Mastoiditis in children

Fifty-seven children were seen over a 10-year period, 1984-1994, at two large pediatric referral centers with a diagnosis of mastoiditis. Twelve had acute infection and 45 had chronic manifestations. Clinical presentations and recovered bacterial pathogens were identical to those reported in earlier literature although the incidence of both acute and chronic mastoiditis has decreased markedly since 1950. The availability of computed tomographic (CT) scans during this decade has improved the management of chronic disease by defining the location of cholesteatomas and the extent of disease as well as possible anatomic variations and potential complications encountered during surgery. CT scanning is indicated in acute disease when there is suspicion of chronic suppuration or destruction of the mastoid