title: Tracheomalacia in an adult with respiratory failure and Morquio syndrome.
abstract: Patients with Morquio syndrome can develop respiratory failure secondary to reduced chest wall compliance and airway collapse from irregularly shaped vocal cords and trachea. We report the case of a patient with Morquio syndrome whose clinical course was complicated by tracheomalacia. An obese 29-year-old female with Morquio syndrome presented with severe wheezing and tachycardia. One month prior to admission, she underwent elective spinal stabilization surgery, which resulted in fixed head flexion. The surgery was complicated by paraplegia and the need for mechanical ventilation via tracheostomy. Initial bronchoscopy revealed severe tracheomalacia, and the tracheostomy tube was changed to one with an adjustable flange. On 3 occasions over the next 20 days she had labored breathing with dramatically decreased V(T). Each time, bronchoscopy revealed almost complete occlusion of the distal end of the tracheostomy tube. Ventilation became much easier when the tracheostomy tube was advanced past the obstruction. After one month, she became febrile, severely hypoxemic, and her family decided to withdraw care. In patients with Morquio syndrome, close attention must be given to the patient's abnormal airways and malformed chest cage. Mechanical ventilation may be difficult because of upper-airway obstruction or low compliance imposed by the restrictive chest wall. Complete tracheal collapse can occur in these patients, especially with fixed head flexion.
authors.affiliation: Respiratory Care Department, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.
authors.forename: Carolyn J
authors.initials: CJ
authors.lastname: Pelley
authors.forename: Jean
authors.initials: J
authors.lastname: Kwo
authors.forename: Dean R
authors.initials: DR
authors.lastname: Hess
mesh_terms: D000328:Adult; D004632:Emergency Medical Services; D005260:Female; D006801:Humans; D009085:Mucopolysaccharidosis IV; D012131:Respiratory Insufficiency / Q000628:therapy*; D014133:Tracheal Diseases / Q000503:physiopathology*; D014481:United States