Anaesthesia and the Mucopolysaccharidoses: A Survey of Techniques and Problems

Abstract

Mucopolysaccharide inJiltration oJ tissues in Hurler, Hunter, San Filippo, Maroteaux-Lamy, Morquio and Scheie syndromes causes problems to anaesthetists in the management oJ the airway and in myocardial Junction. Recent experience at the Royal Children's Hospital, Melbourne, is presented with illustrations highlighting these problems.

Otorhinolaryngological manifestations of the mucopolysaccharidoses

“…As these conditions may affect any organ system, the systemic effects of the disease process must be borne in mind before considering even seemingly minor surgical intervention [7]. Indeed, such is the risk of anaesthesia in severely affected patients that anaesthesia would be unwise for non-life threatening procedures.…”

Otolaryngologic Manifestations of the Mucopolysaccharidoses

“…Characteristic findings include a large head, flat nose, upturned nostrils, coarse facial features, hirsutism, corneal clouding, macroglossia, prominent forehead, joint stiffness, skeletal deformities (dysostosis multiplex), recurrent respiratory infections, otitis media, upper airway obstruction, persistent rhinorrhea, and obstructive hydrocephalus. The diagnosis is usually established by 6 to 24 months, and death ensues by age 10 Hunter's syndrome (MPS II) is clinically distinguished from MPS I by the lack of corneal clouding, a longer life expectancy, less severe mental retardation, and an X-linked pattern of inheritance. Type II A is more severe in its manifestations and is clinically detectable by age 2 to 4 years, with death occurring in the second decade.…”

Anaesthesia and the mucopolysaccharidoses: a review of patients treated by bone marrow transplantation

“…The withdrawal of cyclopropane makes these observations of historical interest only. Kempthome and Brown (1983) consider upper airway obstruction to be a greater problem than seen in this series. They report difficulty even with an oropharyngeal airway, and suggest that the insertion of a nasopharyngeal airway together with pulling the tongue forward may be required to relieve the obstruction.…”