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Coordinated approach to spinal and tracheal reconstruction in a patient with morquio syndrome.

Coordinated approach to spinal and tracheal reconstruction in a patient with morquio syndrome. International journal of pediatric otorhinolaryngology Kiessling, P., Stans, A. A., Dearani, J. A., Boesch, R. P., Fogelson, J. L., Matsumoto, J. M., Haile, D. T., Balakrishnan, K. 2020; 128: 109721

Abstract

Morquio syndrome (Mucopolysaccharidosis IVA) is an autosomal recessive lysosomal storage disease with manifestations ranging from mild to severe phenotype. Mechanical spinal cord injury and airway insufficiency are major causes of mortality. A 17-year-old male patient with severe Morquio syndrome presented with cervical and upper thoracic spinal stenosis with spinal cord myelopathy, and progressive severe tracheal stenosis. Coordinated care among otolaryngology, orthopedic surgery, neurosurgery, anesthesiology, cardiovascular surgery, radiology, and pulmonology teams facilitated the successful planning and execution of two major surgical interventions in rapid succession. This is the first description of a successful coordinated spine and airway repair in the literature.

View details for DOI 10.1016/j.ijporl.2019.109721

View details for PubMedID 31639621