Education  |   November 2016
Images in Anesthesiology: Child with Junctional Epidermolysis Bullosa, Hoarseness, and Nasal Obstruction Demonstrating Severe Laryngeal Stenosis
Author Notes
  • From the Department of Anesthesia, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio.
  • Address correspondence to Dr. Aronson: lori.aronson@cchmc.org
Article Information
Education / Images in Anesthesiology / Airway Management / Central and Peripheral Nervous Systems / Pediatric Anesthesia / Respiratory System
Education   |   November 2016
Images in Anesthesiology: Child with Junctional Epidermolysis Bullosa, Hoarseness, and Nasal Obstruction Demonstrating Severe Laryngeal Stenosis
Anesthesiology 11 2016, Vol.125, 1044. doi:10.1097/ALN.0000000000001201
Anesthesiology 11 2016, Vol.125, 1044. doi:10.1097/ALN.0000000000001201
A 5-YR-OLD, 19.7-kg boy with junctional epidermolysis bullosa (JEB) presented for evaluation of nasal obstruction, hoarseness, stridor worsened by crying, and snoring. Microlaryngoscopy and bronchoscopy revealed a significantly deformed and swollen epiglottis with concealed laryngeal anatomy (fig. A) and severe laryngeal stenosis secondary to granulation tissue obscuring the vocal cords (fig. B) with normal subglottis and trachea (anatomic structures not shown). With these symptoms and degree of laryngeal stenosis, we anticipated slow inhalational induction, increased end-tidal carbon dioxide, airway obstruction, and challenging control of anesthetic depth. Maintenance with dexmedetomidine or propofol and restricting opiates may be preferred in order to preserve spontaneous ventilation and airway tone while minimizing carbon dioxide retention.
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