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Published on: March 26, 2018
Morquio Syndrome for Aortic Valve Replacement: Anesthesia Concerns - A Case Report
Jupi Talukdar1, Surekha Vondutla1, Mamatha Munaf1
1Division of Cardiothoracic and Vascular Anaesthesiology, Department of Anesthesiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Abstract:
Morquio syndrome is a lysosomal storage disease that can lead to glycosaminoglycan deposition in the tissues. Skeletal deformities, cardiovascular complications, and airway abnormalities are well-recognized in this condition, specifically owing to thickened mucosa and epiglottis, making fiberoptic-guided intubation less successful. Our patient had severe aortic regurgitation and was posted for aortic valve replacement. Anesthetic challenges encountered during the management of the case, and a different approach to airway management are highlighted in this report. With increased survival of patients affected with Morquio syndrome, cardiac anesthesiologists might see an increasing number of similar patients requiring corrective cardiac surgery.
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