Airway Management of a Child With Hunter's Syndrome

Abstract

Hunter's syndrome, a very rare genetic disorder caused by a missing or malfunctioning enzyme iduronate-2-sulfatase, resulting in the accumulation of glycosaminoglycans in lysosomes. We report the airway management of an 8-year-old male child diagnosed as Hunters syndrome came with complaints of shortness of breath, lower respiratory tract infection and acute exacerbation of asthma. Admitted in pediatric ICU with severe wheeze and respiratory distress. Started on HFNC. Despite HFNC symptoms got worsened. So, planned for elective intubation. Child has large, protruded tongue, short neck. Difficult airway cart made ready. One attempt of video laryngoscopy and one attempt of normal laryngoscopy done. Unable to intubate so proceeded with tracheostomy as child was desaturating