MORQUIO SYNDROME AND ANESTHESIA

On the basis of a case of Morquio syndrome the following recommendations for anesthetic management of these patients are given: careful preoperative evaluation of pulmonary, cardiac, and neurologic function; avoidance of heavy sedation when pulmonary disease is severe; avoidance of stress to the cervical spine, especially extension during laryngoscopy; arterial cannulation for frequent blood-gas estimation and regulation of assisted or controlled ventilation; avoidance of hypocapnia with change in CSF pH and bicarbonate, reducing postoperative respiratory drive; avoidance of inappropriately large tidal volumes, although minute volumes need to be larger than predicted on a weight basis because of the relative preponderance of vessel-rich tissues in the body. Some of the difficulty in weaning this patient was felt to be due to the fact that he had become accustomed to large tidal volumes and was subjectively dyspneic breathing spontaneously; aggressive treatment of per- and postoperative sputum retention and infection, with chest physiotherapy and frequent suctioning; recognition of the patient with end-stage respiratory problems and serious consideration of the wisdom of subjecting him to anesthesia and major surgical intervention.