Coordinated approach to spinal and tracheal reconstruction in a patient with morquio syndrome. (January 2020)

• Record Type: Journal Article
• Authors: Kiessling, Patrick; Stans, Anthony A.; Dearani, Joseph A.; Boesch, R. Paul; Fogelson, Jeremy L.; Matsumoto, Jane M.; Haile, Dawit T.; Balakrishnan, Karthik
• Is Part Of: International journal of pediatric otorhinolaryngology. Volume 128(2020:Jan.)
• Publication Date: 2020-01
• Subjects: Pediatric airway -- Airway modeling -- Airway stenosis/reconstruction -- Multidisciplinary -- Mucopolysaccharidosis -- Tracheal stenosis; Otolaryngology -- Periodicals; Pediatrics -- Periodicals; Otolaryngology -- Periodicals; Pediatrics -- Periodicals; Oto-rhino-laryngologie -- Périodiques; Pédiatrie -- Périodiques; 618.9209751
• DOI: 10.1016/j.ijporl.2019.109721
• Languages: English
• ISSNs: 0165-5876

Abstract

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.