Newly Published
Education  |   March 2020
Airway Anatomy of an Adult with Hurler’s Syndrome
Author Notes
  • From the Departments of Anesthesiology and Critical Care Medicine (N.M.D., O.A.I., C.M.T., T.P.T.) and Otolaryngology/Head and Neck Surgery (N.M.D.), Johns Hopkins University School of Medicine, Baltimore, Maryland.
  • Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are available in both the HTML and PDF versions of this article. Links to the digital files are provided in the HTML text of this article on the Journal’s Web site (www.anesthesiology.org).
    Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are available in both the HTML and PDF versions of this article. Links to the digital files are provided in the HTML text of this article on the Journal’s Web site (www.anesthesiology.org).×
  • Correspondence: Address correspondence to Dr. Dalesio: ndalesi1@jhmi.edu
Article Information
Images in Anesthesiology / Airway Management / Endocrine and Metabolic Systems / Respiratory System
Education   |   March 2020
Airway Anatomy of an Adult with Hurler’s Syndrome
Anesthesiology Newly Published on March 25, 2020. doi:https://doi.org/10.1097/ALN.0000000000003278
Anesthesiology Newly Published on March 25, 2020. doi:https://doi.org/10.1097/ALN.0000000000003278
Hurler’s Syndrome is a rare genetic disorder characterized by a deficiency in the enzyme α-L-iduronidase, leading to an accumulation of glycosaminoglycans in the soft tissues. Without treatment, these patients experience severe multisystem dysfunction leading to airway obstruction, cardiopulmonary failure, and eventually death at 5 to 10 yr of age.1  Allogeneic hematopoietic stem cell bone marrow transplantation significantly reduces disease progression, increasing survival into the third decade of life.2 
These videos are from a 19-year-old patient with Hurler’s Syndrome presenting for surgery whereby a “cannot intubate” scenario occurred requiring urgent supraglottic airway placement and intubation using a flexible bronchoscope through the supraglottic airway. While maintaining spontaneous ventilation and after passing the bronchoscope through the supraglottic airway, the aryepiglottic folds are engorged and appear edematous (Supplemental Digital Content, video 1, http://links.lww.com/ALN/C343). After advancing the scope past the engorged tissue, the vocal cords could be seen in addition to an out-pouching cephalad to the anterior commissure. Polysaccharide deposition between the tracheal rings made discerning tracheal structures difficult (Supplemental Digital Content, video 2, http://links.lww.com/ALN/C344).