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Updated: Jun 19, 2026

Guidelines for Elective Pediatric Fiberoptic Intubation
Published on: January 17, 2011
[Difficult tracheal intubation using airway scope in a pediatric patient with Hunter syndrome]
Shima Taguchi1, Shinji Kusunoki, Hideki Fukuda
1Department of Anesthesiology and Critical Care, Hiroshima University Hospital, Hiroshima 734-8551.
Abstract:
Hunter syndrome, manifested by mucopolysaccharidosis II (MPS II), is a hereditary disorder caused by accumulation of glycosaminoglycans. An important issue in regard to anesthesia in affected individuals is airway management, because of gargoylism. An 8-year-old boy with MPS II was scheduled for adenotonsillectomy. We performed slow induction with sevoflurane, then utilized an Airway Scope (AWS; HOYA Corporation Tokyo, Japan), which enables operators to observe tube passage through the vocal cords with an LCD monitor, for tracheal intubation, because intubation guided by a fiberoptic bronchoscopy (FOB) was considered to be risky for glottic damage. Attempted tracheal intubations with the AWS alone and in combination with an FOB inserted through the tracheal tube failed, even though a clear image of the glottis was obtained. Finally, we inserted a stylet into the tracheal tube attached to the blade of the AWS and successful tracheal intubation was accomplished. The operation was completed uneventfully and the patient entered the ICU with his trachea intubated, because of pharyngeal and laryngeal edema. Although useful for difficult airway management, tracheal intubation with the AWS may be difficult when used in patients with a narrow oral cavity or small tracheal tube.
Insights
Airway Scope (AWS) intubation proved challenging in a Hunter syndrome (MPS II) patient due to gargoylism. A stylet-guided approach with AWS facilitated successful tracheal intubation, highlighting its utility in complex pediatric airway management.
Area of Science:
- Anesthesiology
- Genetics
- Pediatric Surgery
Background:
- Hunter syndrome (Mucopolysaccharidosis II) presents airway challenges due to gargoylism, complicating anesthesia.
- Effective airway management is critical for patients with MPS II undergoing surgical procedures.
Observation:
- An 8-year-old boy with MPS II required adenotonsillectomy, necessitating careful tracheal intubation.
- Airway Scope (AWS) was chosen for intubation to minimize risk of glottic damage compared to fiberoptic bronchoscopy (FOB).
Findings:
- Initial attempts with AWS alone and AWS-FOB combination failed to achieve tracheal intubation.
- Successful intubation was ultimately achieved by using a stylet within the tracheal tube attached to the AWS blade.
- The patient required continued tracheal intubation post-operatively due to pharyngeal and laryngeal edema.
Implications:
- This case demonstrates a modified AWS technique for successful tracheal intubation in a challenging pediatric airway case.
- AWS may present difficulties in patients with narrow oral cavities or small tracheal tubes.
- Further research into advanced airway management techniques for rare genetic disorders like MPS II is warranted.
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