Surgical Reconstruction for Severe Tracheal Obstruction in Morquio A Syndrome

Ann Thorac Surg. 2016 Oct;102(4):e329-31. doi: 10.1016/j.athoracsur.2016.02.113.

Abstract

Progressive tracheal obstruction is commonly seen in Morquio A syndrome and can lead to life-threatening complications. Although tracheostomy can address severe upper airway obstruction, lower airway obstruction, commonly associated with a narrow thoracic inlet and vascular compression, requires an alternative approach. We describe the case of a 16-year-old patient with Morquio A syndrome whose near-fatal tracheal obstruction was relieved by timely surgical tracheal vascular reconstruction with dramatic resolution of his respiratory symptoms.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Computed Tomography Angiography / methods
  • Echocardiography, Transesophageal / methods
  • Extracorporeal Membrane Oxygenation / methods
  • Follow-Up Studies
  • Humans
  • Imaging, Three-Dimensional*
  • Male
  • Mucopolysaccharidosis IV / complications*
  • Mucopolysaccharidosis IV / diagnostic imaging
  • Plastic Surgery Procedures / methods*
  • Rare Diseases
  • Risk Assessment
  • Severity of Illness Index
  • Sternotomy / methods
  • Surgical Flaps
  • Tracheal Stenosis / diagnostic imaging
  • Tracheal Stenosis / etiology*
  • Tracheal Stenosis / surgery*
  • Treatment Outcome