Heliox in the management of respiratory failure in a Morquio A syndrome patient with trachea narrowing

Abstract

Morquio A Syndrome is a mucopolysaccharide deposition disease where patients can develop respiratory failure due to airway narrowing by polysaccharide deposition, anatomical distortion and compression of the airway. Definitive management with surgery is high risk, only performed in the highly specialized centers, and by the time they develop respiratory failure they may not be a candidate for surgery due to difficulty intubation. Consequently, they often end up with long term BiPAP dependent and suffer from side effects of abdominal, facial pain and depression. Heliox is low density gas mixture which can facilitate oxygen delivery in narrowed airway. Here we report a case of Morquio A syndrome patient with 24-h BiPAP dependence and successfully treated with Heliox and weaned off BiPAP for 4 hours a day with improved quality of life.